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Guidelines and Recommendations for

Targeted Neonatal Echocardiography and


Cardiac Point-of-Care Ultrasound in the
Neonatal Intensive Care Unit: An Update
from the American Society of
Echocardiography
Patrick J. McNamara, MD, MSc, FASE (Chair), Amish Jain, MD, PhD, FASE, Afif El-Khuffash, MB BCh,
Regan Giesinger, MD, FASE, Dany Weisz, MD, MSc, Lindsey Freud, MD, FASE, Philip T. Levy, MD,
Shazia Bhombal, MD, Willem de Boode, MB BS, Tina Leone, MD, Bernadette Richards, RDCS,
Yogen Singh, MB BCh, Jennifer M. Acevedo, ACS, John Simpson, MB BCh, FASE, Shahab Noori, MD,
and Wyman W. Lai, MD, FASE, Iowa City, Iowa; Toronto, Ontario, Canada; Dublin, Ireland; Boston, Massachusetts;
Atlanta, Georgia; Nijmegen, the Netherlands; New York, New York; Columbus, Ohio; Loma Linda, Los Angeles, and
Orange, California; Chicago, Illinois; and London, United Kingdom

Targeted neonatal echocardiography (TNE) involves the use of comprehensive echocardiography to appraise
cardiovascular physiology and neonatal hemodynamics to enhance diagnostic and therapeutic precision in
the neonatal intensive care unit. Since the last publication of guidelines for TNE in 2011, the field has matured
through the development of formalized neonatal hemodynamics fellowships, clinical programs, and the expan-
sion of scientific knowledge to further enhance clinical care. The most common indications for TNE include adju-
dication of hemodynamic significance of a patent ductus arteriosus, evaluation of acute and chronic pulmonary
hypertension, evaluation of right and left ventricular systolic and/or diastolic function, and screening for pericar-
dial effusions and/or malpositioned central catheters. Neonatal cardiac point-of-care ultrasound (cPOCUS) is a
limited cardiovascular evaluation which may include line tip evaluation, identification of pericardial effusion and
differentiation of hypovolemia from severe impairment in myocardial contractility in the hemodynamically unsta-
ble neonate. This document is the product of an American Society of Echocardiography task force composed of
representatives from neonatology-hemodynamics, pediatric cardiology, pediatric cardiac sonography, and
neonatology-cPOCUS. This document provides (1) guidance on the purpose and rationale for both TNE and
cPOCUS, (2) an overview of the components of a standard TNE and cPOCUS evaluation, (3) disease and/or clin-
ical scenario–based indications for TNE, (4) training and competency-based evaluative requirements for both
TNE and cPOCUS, and (5) components of quality assurance. (J Am Soc Echocardiogr 2024;37:171-215.)

The writing group would like to acknowledge the contributions of Dr. Regan Giesinger who sadly passed during the final
revisions phase of these guidelines. Her contributions to the field of neonatal hemodynamics were immense.

Keywords: Targeted neonatal echocardiography, Neonatal hemodynamics, Point-of-care ultrasound

From the Department of Pediatrics, University of Iowa, Iowa City, Iowa (P.J.M., the Department of Pediatrics, Evelina London Children’s Hospital, London,
R.G.); the Department of Pediatrics, University of Toronto, Toronto, Ontario, United Kingdom (J.S.); the Fetal and Neonatal Institute, Division of Neonatology,
Canada (A.J., D.W., L.F.); the Department of Paediatrics, Royal College of Children’s Hospital Los Angeles, Department of Pediatrics, Keck School of
Surgeons in Ireland, Dublin, Ireland (A.E.-K.); the Division of Newborn Medicine, Medicine, University of Southern California, Los Angeles, California (S.N.);
Boston Children’s Hospital, and the Department of Pediatrics, Harvard Medical CHOC Children’s Hospital, Orange, California (W.W.L.); and the University of
School, Boston, Massachusetts (P.T.L.); the Department of Pediatrics, Division California, Irvine, Orange, California (W.W.L.).
of Neonatology, Emory University and Children’s Healthcare of Atlanta, Atlanta, Reprint requests: American Society of Echocardiography, Meridian Corporate
Georgia (S.B.); the Department of Neonatology, Radboud University Medical Center, 2530 Meridian Parkway, Suite 450, Durham, NC 27713 (E-mail: ase@
Center, Radboud Institute for Health Sciences, Amalia Children’s Hospital, asecho.org).
Nijmegen, the Netherlands (W.d.B.); Vagelos College of Physicians and
0894-7317/$36.00
Surgeons, Columbia University, New York, New York (T.L.); the Heart Center,
Copyright 2023 Published by Elsevier Inc. on behalf of the American Society of
Nationwide Children’s Hospital, Columbus, Ohio (B.R.); Loma Linda University
Echocardiography.
School of Medicine, Loma Linda, California (Y.S.); the Department of
Pediatrics-Cardiology, Ann and Robert H. Lurie Children’s Hospital of Chicago, https://doi.org/10.1016/j.echo.2023.11.016
Northwestern University Feinberg School of Medicine, Chicago, Illinois (J.M.A.);
171
172 McNamara et al Journal of the American Society of Echocardiography
February 2024

TABLE OF CONTENTS gist and which involves comprehensive multimodal assessment of car-
diac function and hemodynamics. Since the initial guideline published
in 2011, the pool of neonatologists with expertise in TNE has
Background and Rationale for Targeted Neonatal Echocardiography 172 increased substantially, aided by highly successful formal subspeciality
Background 172 neonatal hemodynamic fellowship programs, facilitating skill dissem-
Rationale for TNE in the NICU 172 ination. In addition, scientific literature on the implementation of the
Neonatal Cardiovascular Physiology for the Hemodynamics Consul- TNE program has emerged.9,10 Normative data for standard echocar-
tant 173 diography indices for preterm and term infants have expanded, and
Indications for TNE in NICUs 173
newer methods for cardiac function assessment have been intro-
Indications for cPOCUS in the NICU 174
duced. Finally, the development of neonatal echocardiography simu-
TNE: Practical Aspects 174
Elements of Standard TNE 174 lation models in the past decade has prompted calls for its use to
Advanced Imaging and Measurements 177 support training in basic echocardiographic views and to identify
Research Tools and Emerging Measurements 183 structural abnormalities.6,11,12 These developments, catalyzed by the
The First Complete Echocardiogram 183 first edition of the TNE guideline 2011, have motivated the need to
The cPOCUS Evaluation 185 reconvene an expert panel to update and revise the guidelines incor-
TNE: Application of Imaging 185 porating scientific progress, and make them more relevant to contem-
Disease-Based Screening 185 porary neonatal practice. This article replaces the 2011 TNE guideline
Clinical Scenario–Based Screening 196 and has been expanded to provide recommendations for cardiac
TNE: Training and Accreditation 201
point-of-care ultrasound (cPOCUS). cPOCUS in neonatology is not
Previously Published Guidelines for Training in TNE 201
yet well defined, and there is significant overlap with TNE in scope13;
Knowledge Elements and Competencies for Clinical Practice in TNE 204
Proposed Training and Evaluation in TNE 204 in general, cPOCUS is a brief, qualitative, and less comprehensive
Training and Evaluation in Neonatal Hemodynamics 205 assessment of cardiac function, indwelling arterial or venous catheters
Practice Eligibility Route for Neonatologists With TNE Expertise 206 and life-threatening pericardial effusions.14 The recently published
Requirements for Training Programs in TNE and Neonatal Hemody- ASE recommendations for cPOCUS provide a framework for imag-
namics 206 ing and practice, although neonates were not included.15
cPOCUS: Training Recommendations 207
Quality Assurance for TNE and cPOCUS 207
Program Establishment 208
Rationale for TNE in the NICU
Conclusion 208 Longitudinal appraisal of cardiovascular status to maintain effective de-
Notice and Disclaimer 209 livery of oxygen and nutrients and removal of carbon dioxide and
waste products is pivotal for optimal organ function. The reliability of
clinical indices of systemic perfusion, such as heart rate, blood pressure
(BP), capillary refill time, urine output, lactate, and capillary refill time, is
BACKGROUND AND RATIONALE FOR TARGETED questionable.16-18 Echocardiography estimation of cardiac output (left
NEONATAL ECHOCARDIOGRAPHY ventricular [LV] and right ventricular [RV] output) is feasible. Older
pediatric echocardiography studies reported variable precision with
Background discordance rates of 630%. More recent neonatal comparative
Limitations in the clinical evaluation of the adequacy of circulatory evaluations with magnetic resonance imaging–derived estimates of car-
function in neonates have long been recognized, especially for preterm diac output showed higher correlation because of increased assessment
neonates. These limitations have led to the increased use of bedside and measurement rigor.19,20
echocardiography by neonatal clinicians to assess the hemodynamic The use of TNE in the setting of a hemodynamic consultation results
phenotype and cardiac function, especially in the past decade.1 in modification of management strategies in almost 40% of cases; of
Globally, more neonatal intensive care units (NICUs) have established note, the reported number is >80% for critical illnesses such as acute
formal programs to incorporate echocardiography in the diagnosis and pulmonary hypertension (PH) and systemic hypotension or
management of critically ill neonates.2,3 Recognition of the importance shock.9,21,22 In preterm infants with patent ductus arteriosus (PDA),
of comprehensive training, including high-quality echocardiography TNE is commonly used to better adjudicate those shunts which are he-
skills, has culminated in the development of formal, 1 year subspecialty modynamically significant and therefore predictive of adverse clinical
fellowship training programs in neonatal hemodynamics at centers in outcomes.23,24 In addition, it is used to adjust the duration of pharma-
the United States and Canada over the past decade. cologic treatment,25-27 and to select patients for and prevent clinical
Functional echocardiography was the first term used to describe instability after interventional closure (transcatheter device closure or
neonatologist-performed imaging assessments4 to distinguish it surgical ligation).28,29 Moreover, timely availability of TNE in NICUs
from primarily anatomy-focused echocardiography performed by a may allow emergent diagnosis and treatment of life-threatening compli-
cardiology service. However, over the past decade, different terms, cations such as cardiac tamponade, reveal incidental but highly relevant
such as neonatologist-performed echocardiography (NPE)5,6 and clini- findings such as malpositioned central venous catheters, and identify
cian-performed ultrasound,7 have been used interchangeably. In this structural heart defects.30 Comprehensive hemodynamic evaluation
article, we use the term targeted neonatal echocardiography (TNE), as including TNE may result in faster clinical recovery in preterm infants
proposed by the writing group of the American Society of with compromised systemic perfusion.31,32 In addition, TNE is used
Echocardiography (ASE) in 2011.8 TNE involves the use of echocar- to assess the severity of pulmonary vascular disease, transductal and in-
diography for neonates with hemodynamic perturbation, where clin- teratrial shunting, and myocardial performance during episodic acute
ical suspicion for major structural defects is low, performed by, or PH33 and may aid in risk stratification by identifying patients at highest
under direct supervision of, and interpreted by a trained neonatolo- risk for mortality or need for extracorporeal membrane oxygenation
Journal of the American Society of Echocardiography McNamara et al 173
Volume 37 Number 2

Abbreviations
(ECMO).34-37 Moreover, TNE men ovale and PDA) are key
PICC = Peripherally inserted
may provide information to physiologic determinants of
central catheter
ASE = American Society of guide more precise use of extrauterine transition.43 As pul-
Echocardiography pulmonary vasodilator therapies POCUS = Point-of-care monary vascular resistance
during acute severe hypoxemia ultrasound (PVR) falls secondary to lung
BP = Blood pressure
and enhanced evaluation of PVR = Pulmonary vascular expansion, flow across the PDA
BPD = Bronchopulmonary treatment response.38-40 There is reverses, thereby increasing pul-
resistance
dysplasia
increased evidence that TNE- monary blood flow which in
CDH = Congenital guided care enables earlier detec- RV = Right ventricular turn exerts shear force on the
diaphragmatic hernia tion and delineation of cardiovas- RVET = Right ventricular pulmonary vascular endothe-
CHD = Congenital heart cular compromise and supports ejection time lium. These changes prompt a
disease patient-tailored, physiology-based switch from production of vaso-
RVO = Right ventricular
hemodynamic management and output constrictor mediators to vasodila-
cPOCUS = Cardiac point-of- monitoring. In summary, TNE is tors, leading to a further drop in
care ultrasound a useful clinical adjunct to aid RVSP = Right ventricular PVR. The direction of flow across
neonatal cardiovascular assess- systolic pressure the PDA becomes increasingly
DS = Down syndrome
ment on the basis of a growing SBF = Systemic blood flow left to right. The postnatal rise
ECMO = Extracorporeal
recognition that it can provide he- in lung perfusion and elevation
membrane oxygenation SLPCV = Selective laser
modynamic information that of oxygen saturation, in combi-
photocoagulation of the
EF = Ejection fraction either complements what is clini- nation with an increase in brady-
communicating vessels
EI = Eccentricity index cally suspected or provides novel kinins and decrease in
physiologic insight.41 The integra- STE = Speckle-tracking prostaglandin levels promotes
FAC = Fractional area change tion of echocardiography derived echocardiography constriction of vascular smooth
HIE = Hypoxic-ischemic hemodynamic information, rele- TAPSE = Tricuspid annular muscles in the ductus arterio-
encephalopathy vant to an individual situation plane systolic excursion sus.45 Functional closure occurs
and directed by a specific clinical within the first 48 hours for
IDM = Infant of diabetic question, offers a blueprint from TNE = Targeted neonatal term-born infants, while
mother echocardiography
which to formulate a physiology- anatomic closure is typically
IVCT = Isovolumetric based diagnostic impression, TR = Tricuspid regurgitation completed within 14 to 21 days.
contraction time upon which cardiovascular sup- The higher pulmonary blood
TTTS = Twin-to-twin
IVRT = Isovolumetric port is based, and evaluate the transfusion syndrome flow from increased RV output
relaxation time response to therapeutic interven- (RVO) and the left-to-right PDA
tion.42 UVC = Umbilical venous shunt leads to an increase in pul-
LA = Left atrial catheter monary venous return and
LV = Left ventricular Neonatal Cardiovascular elevated left atrial (LA) pressure,
Physiology for the causing displacement of the flap of the foramen ovale over the rims of
LVEF = Left ventricular the fossa, limiting flow. Knowledge of these physiologic changes and
ejection fraction Hemodynamics
Consultant their timing is crucial to enable proper interpretation and integration
LVO = Left ventricular output of the hemodynamic information obtained using TNE, especially dur-
Neonates navigate complex car-
ing the perinatal period.
MV = Mitral valve diopulmonary sequences to tran-
The transition from fetal to postnatal life is more complex in pre-
sition from fetal to postnatal
NHTNE = Neonatal term infants with increased risk for hemodynamic compromise.42
hemodynamics and targeted circulation, including sudden
The potential determinants include immaturity of the myocardium,
neonatal echocardiography changes in lung volume and
persistence of fetal shunts, inherently smaller pulmonary vascular ca-
compliance, cardiac loading con-
NICU = Neonatal intensive pacity or adverse cardiovascular effects of lung disease and related
ditions and shunt physiology.43
care unit ventilation strategies, and differential cytokine and/or pharmacologic
Pulmonary vasodilation is further
responsiveness.43 The preterm myocardium is composed of underde-
NPE = Neonatologist- regulated by alveolar recruit-
veloped contractile mechanisms with disorganized myofibrils, imma-
performed echocardiography ment, lower carbon dioxide ten-
ture calcium handling system, and inadequately compliant collagen,
PA = Pulmonary artery sion, increase in oxygen tension,
all contributing to a myocardium with relatively reduced diastolic per-
surge of vasodilator prostaglan-
PAAT = Pulmonary artery formance and compliance, even more intolerant to an abrupt increase
dins, and release of endogenous
acceleration time in afterload (e.g., following removal of the placenta), and lack of
nitric oxide from endothelium.44
reserve to cope with reduced preload.46 The delay in physiologic
PAP = Pulmonary artery The rise in systemic vascular
drop in PVR secondary to lung disease coupled with the failure to in-
pressure resistance is aggravated by cold
crease cardiac outputs and persistence of fetal shunts can contribute
stress encountered after birth
PDA = Patent ductus to a maladaptive transition.47
and a surge in endogenous vaso-
arteriosus
constrictor substances during la-
PH = Pulmonary hypertension bor. Alterations in flow across Indications for TNE in NICUs
PI = Pulmonary insufficiency intra- and extracardiac fetal Several reports published over the past 10 years have highlighted the
shunts (e.g., ductus venosus, fora- typical indications where TNE has been used by clinicians in tertiary
174 McNamara et al Journal of the American Society of Echocardiography
February 2024

NICUs. Although these indications are often described in terms of should be limited to critical emergencies given the limited ability to
suspected pathologies, the decision to perform TNE is usually based detect mild or moderate disease and change over time. The following
on the interpretation of presenting symptoms. The typical pathologies section describes the images and measurements that should be per-
and their suggestive symptoms which may prompt a hemodynamic formed in all standard TNE examinations (Table 3).
evaluation with the use of TNE in neonates, as well as specific goals
of such an evaluation are listed in Table 1. It is important to note LV Systolic Function. There are three potential methods of
that if the presenting symptoms are suspicious of critical congenital measuring LV ejection fraction (LVEF) which are influenced by geo-
heart disease (CHD), or in conditions with known association with metric assumptions regarding the shape of the left ventricle. LVEF us-
CHD (e.g., congenital diaphragmatic hernia [CDH], trisomy 21), pa- ing M-mode imaging, which assumes that the left ventricle is circular in
tients must also receive a comprehensive echocardiogram that is re- cross-section, is the least recommended because of the inaccuracy of
viewed by a pediatric cardiologist in a timely fashion. In addition, if its geometric assumptions, among other things. The area-length, or
neonatologist-performed TNE is the first patient evaluation, the study hemicylindrical hemiellipsoid model, assumes that the ventricular
protocol should include the acquisition of images and views to base is a cylinder, and the apex is ellipsoid (Table 3, Figure 1). In
confirm normal cardiac structure and connections. If deviations contrast, the Simpson biplane method assumes the left ventricle to
from normal are recognized, then timely review by a pediatric cardi- be conical; therefore, circular in cross-section (Table 3, Figure 2). For
ologist is warranted. both area-length and Simpson measurements, the tracing should
occur at the endocardial–blood pool interface which typically has a
smooth, regular contour, and should include adjacent structures
Indications for cPOCUS in the NICU (e.g., papillary muscles) as within the cavity. In the transitional period,
cPOCUS is a brief bedside ultrasound examination of the heart when right-heart pressures are elevated (sometimes suprasystemic),
limited in its scope to specific clinical question. Point-of-care ultra- these assumptions may be less applicable. For optimal image acquisi-
sound (POCUS) is used for evaluations of other organ systems or tion the LV apex should move minimally between end-diastole and
as an aid during invasive procedures.48 Although some clinicians end-systole. Significant basal displacement of the apex during systole
who perform cPOCUS may also be trained in TNE, others may suggests image foreshortening. Normal ejection fraction (EF) is consid-
have less comprehensive training focused solely on the scope within ered 55% to 70%. Fractional shortening, like LVEF by M-mode imag-
POCUS. However, it is critical for all clinicians using ultrasound to ing, is commonly used but has several limitations. First, it assumes that
know and practice within the scope and limitations of their own the region sampled at the tips of the mitral valve (MV) leaflets is repre-
training and to seek support from more experienced operators sentative of global LV systolic function. Second, in the setting of septal
when needed. In older children and adults, the use of focused cardiac flattening the assumption that the left ventricle is circular may lead to
ultrasound in the emergency departments or intensive care units has significant inaccuracy in fractional shortening. The normal range is
become common and is used for a quick adjunctive evaluation in clin- considered 30% to 45% (Table 3, Figure 3).
ical scenarios such as hypovolemia, hypotension, low–cardiac output Recommendation
states, effusions, sepsis, and coronary conditions.49,50 In neonates,
however, the potential for encountering undiagnosed critical CHD LVEF should be measured using either the area-length or
and/or complex cardiopulmonary hemodynamic physiologic states Simpson biplane method.
inherently complicates the practice and scope of limited imaging mo-
dalities, needing detailed TNE assessments and experienced opera- LV Diastolic Function and LA Loading. Pulsed-wave Doppler
tors to interpret the physiologic impact of findings. Table 2 measurements may provide insights regarding the relative pressure
describes the potential clinical uses of a cPOCUS examination that differences between the left atrium and left ventricle, and left heart
may aid clinical decisions in a time-sensitive manner and the associ- filling. The peak velocity in the pulmonary vein may be low in the
ated pitfalls for operators to consider. Extreme caution is recommen- setting of PH and low pulmonary blood flow or high with a prominent
ded in practice of qualitative appraisal of heart function in diastolic wave in the setting of high-volume PDA shunt (Table 3,
symptomatic infants, particularly during the early transition because Figure 4). A-wave reversal in the pulmonary vein may be a normal
of the need to confirm normal cardiac anatomy. In addition, variant; however, in combination with other markers of LV diastolic
cPOCUS should not be used as a screening tool to detect CHD; how- dysfunction the duration and magnitude of the pulmonary vein A
ever, deviations from normal anatomy detected or suspected during a wave (in relation to the transmitral valve A wave) may be a marker
cPOCUS assessment should prompt referral to a pediatric cardiolo- of poor LV compliance resulting in backflow into the pulmonary veins
gist. It is thus strongly recommended to obtain early definitive imaging during the atrial phase. Most normal term and preterm infants have a
(pediatric echocardiography or standard TNE), wherever feasible, MV E/A ratio of <1 because of developmentally appropriate altered
particularly when patient symptoms remain unresolved despite ventricular compliance (Table 3, Figure 5). Because the velocity of
cPOCUS-guided management. It is therefore necessary for clinicians early (mitral E) flow is determined by the pressure gradient between
performing ultrasound to use their skills with caution and for institu- chambers, high MV E velocity (or MV E > A), may indicate a pressure-
tions to define the scope of performance and degree of oversight. or volume-loaded left atrium. Similarly, in mature neonates a low E
wave velocity may suggest impaired ventricular elastance.
Isovolumetric relaxation time (IVRT) is determined by the pressure
TNE: PRACTICAL ASPECTS gradient between chambers and is mostly used to estimate the relative
time it takes for pressure to build up in the left atrium sufficient to
Elements of Standard TNE overcome LV pressure, thus opening the MV and initiating ventricular
All TNE evaluations must be comprehensive because unexpected filling (Table 3, Figure 6). Shorter IVRT suggests either high LA pres-
physiologic findings which may modify clinical decisions are not un- sure (e.g., hemodynamically significant PDA) or high LV elastance
common. In addition, qualitative evaluation of cardiac function and vice versa. LA dilation may be seen in the setting of LA volume
Journal of the American Society of Echocardiography McNamara et al 175
Volume 37 Number 2

Table 1 Typical pathologies and associated symptoms that may prompt hemodynamic evaluation of patients using targeted
neonatal echocardiography in NICUs

Pathologies Suggestive clinical concerns Specific goals

PDA in premature neonates  Murmur i. Confirm diagnosis


 Bounding pulses, active precordium ii. Evaluate PDA size, shunt direction,
 Wide pulse pressure and flow pattern
 Hypotension and/or metabolic acidosis during iii. Evaluate signs of shunt magnitude
transition
 Worsening ventilation and efficacy of
oxygenation
 Pulmonary hemorrhage
 Screening to detect clinically silent large PDA in
high-risk patients (<27 wk gestational age)
Low SBF states during  Metabolic acidosis Evaluate ventricular volumes, systolic
transition in premature neonates  Elevated lactate performance, and outputs
 Poor urinary output
 Hypotension
 Low cerebral oxygen saturation (near-infrared
spectroscopy)
Acute PH  Acute hypoxic respiratory failure despite i. Confirm diagnosis and establish
adequate ventilation disease severity
 Preductal oxygen saturation (SpO2) $ 7%-10% ii. Ventricular function and outputs
vs postductal iii. Shunt presence and flow
characteristics
iv. Sequential assessments to monitor
progression
Shock  Hypotension Evaluate ventricular volumes, systolic
 Metabolic acidosis performance, and outputs
 Lactic acidosis
 Oliguria
 Hypotension (warm shock) or hypertension (cold
shock)
 Prolonged capillary refill time
Infants with perinatal asphyxia  Early routine evaluation in infants with evidence i. Evaluate biventricular systolic
of significant perinatal insult performance and outputs
 High cardiac troponin ii. As for shock and acute PH, when
 Signs of shock and/or acute PH relevant
 Low cerebral oxygen saturation
Chronic PH  Routine evaluation in at-risk preterm neonates at i. Confirm diagnosis and establish
36 wk postmenstrual age: moderate to severe severity
chronic lung disease, small for gestational age, ii. Evaluate RV size and systolic function
previous history of acute PH, oligohydramnios iii. Evaluate for presence of left-to-right
 Preterm neonates with moderate to severe shunts
chronic lung disease demonstrating signs iv. Rule out pulmonary vein disease/
suggestive of significant pulmonary vascular stenosis
disease: frequent hypoxemic episodes,
unexpected worsening, or lack of expected
improvement in respiratory course
CDH  Acute hypoxic respiratory failure despite i. Evaluate biventricular systolic
adequate ventilation performance and outputs
 Routine early evaluation recommended to define ii. Assess PA vs venous hypertension
normal anatomy and differentiate PA iii. Assess severity of PA hypertension
hypertension vs LV phenotype
Pericardial/pleural effusion  Sudden unexpected cardiorespiratory Confirm or rule out diagnosis.
deterioration in neonates with a central venous
catheter in situ
176 McNamara et al Journal of the American Society of Echocardiography
February 2024

loading (e.g., hemodynamically significant PDA) or pressure loading are established in some populations of term neonates (e.g., PH, hypoxic-
(e.g., high LV end-diastolic pressure due to diastolic dysfunction). It ischemic encephalopathy [HIE]).35,60
is conventional to use a ratio of LA to aortic root dimensions to quan- RVO is calculated using either the short- or long-axis plane; howev-
tify this, although LA volume has been used in some studies; however, er, both the velocity time integral and annulus should be measured in
neither has been validated with gold standard measures of cardiac vol- the same plane due to possible variations in annulus shape. Like LVO,
umes in neonates (Table 3, Figure 7). the angle of insonation should be minimized and the sample volume
should bisect the hinge point of the pulmonary valve (Table 3,
Recommendation Figure 13). Measurement of the pulmonary artery (PA) annulus should
All standard TNE studies should consider including be at precisely the same anatomic location where the sample volume is
transmitral flow (E/A ratio, IVRT) and a measure of LA placed to ensure consistency with longitudinal assessments. Branch PA
size. Measurement of pulmonary vein velocities may flow should be measured to screen for branch PA stenosis which may
be considered. interfere with the reliability of the estimate of RVO. RV diastolic func-
tion may be evaluated using tricuspid valve E/A ratio or Doppler tissue
imaging, but normative data are limited.
Systemic Blood Flow. In the absence of shunts, systemic blood
flow (SBF) can be calculated using the elements of the formula for
Recommendation
LV output (LVO) (Table 3, Figure 8). To optimize the accuracy of Objective measurement of RV systolic function including
the measurement it is essential that the angle of insonation with the TAPSE, RV FAC, and RVO should be performed.
aortic flow be as close to zero as possible and definitely <20 . In addi-
tion, sample volume position should bisect the hinge point of the
Pulmonary Hemodynamics. Assessment of mean PA pressure
aortic valve and measurement of the LVoutflow tract diameter should
(PAP) is based on the use of the modified Bernoulli equation to esti-
be at precisely the same anatomic location to ensure consistency with
mate either the mean PAP obtained from the peak velocity of a com-
longitudinal assessments. In the setting of a hemodynamically signifi-
plete jet of pulmonary insufficiency (PI) according to the calculation
cant PDA, where LVO is no longer a reliable estimate of SBF, surro-
mean PAP = 1/3 RV systolic pressure (RVSP) + 2/3 PA diastolic pres-
gate measurements may be useful. Diastolic flow reversal in the
sure or RVSP (obtained from the peak velocity of a complete tricuspid
descending aorta was the best predictor of cardiac magnetic reso-
regurgitant jet according to the equation RVSP = 4  tricuspid regur-
nance imaging derived estimates of PDA shunt volume.
gitation (TR) Vmax2 + estimated right atrial pressure) (Table 3,
Conventional markers such as PDA diameter or left atrium/aortic
Figures 14 and 15). It is essential that the line of insonation be parallel
root ratio performed poorly56 (Table 3, Figure 9). Reversal of diastolic
to the direction of the jet. It is also important to note that absence of
flow in the celiac artery, superior mesenteric artery and, less
TR or PI does not imply normal PAP. Also, in the setting of RV systolic
commonly, middle cerebral artery, which should have forward flow
dysfunction TR-derived estimates of RVSP may be lower than would
throughout diastole, are also associated with hemodynamic signifi-
be expected in the setting of normal RV function for the degree of
cance (Table 3, Figure 10). Absence of reversal should be interpreted
altered pulmonary hemodynamics.
with caution, particularly when end-organ pathology is present as dia-
PDA shunt direction, if present, provides a reliable indicator of the
stolic flow may also be influenced by organ resistance.
relative pressure between the systemic and pulmonary vascular beds
at the level of the great vessels. Of note, eccentricity index (EI) may be
Recommendation used to quantify RV pressure loading. Systolic EI is a ratio of the mid-
LVO should be routinely measured. Imaging to determine septum to the posterior wall diameter to the perpendicular diameter,
the diastolic flow direction centrally (descending aorta) parallel to the septal wall at the midpoint of the cavity (Table 3,
and peripherally (celiac artery, superior mesenteric artery, Figure 16). As the left ventricle is expected to be round, a normal sys-
middle cerebral artery) should be performed where ductal tolic EI is equal to 1, or a circular LV cross-section.61 Subjective eval-
shunt significance is in question. uation of septal flattening is unreliable, especially for mild to moderate
disease,55 so objective evaluation is recommended.
Assessment of PVR may be a useful adjunct in determining whether
RV Systolic Function. Subjective assessment, although common, is elevation in PAP relates to pulmonary vascular disease.62 Because
not recommended because of limited sensitivity, particularly for mild blood accelerates and reaches a maximum velocity more quickly in
and moderate disease.55 Because of the geometric limitations imposed a rigid circuit the relationship of PA acceleration time (PAAT) to total
by the conformation of the right ventricle, volume estimation is not RV ejection time (RVET) may be used as an indexed surrogate mea-
possible with two-dimensional echocardiography. As a result, surrogate sure of PVR (the so-called PVR index) (Table 3, Figure 17). A ratio
markers of EF are used. Fractional area change (FAC) (Table 3, of PAAT to RVET < 0.25 (some centers may use the inverse RVET/
Figure 11) may be measured in either the RV-focused four-chamber or PAAT > 4.0 which is more intuitive to parallel the directionality of
RV three-chamber views, although there is evidence of superior repro- changes in the index with changes in PVR) is suggestive of elevated
ducibility for the RV three-chamber view.57 In adults, the RV-focused PVR.57 The presence of notching of PA flow, similarly, reflects poor
four-chamber correlates well with cardiac magnetic resonance compliance in the pulmonary vascular bed and may be useful.
imaging–derived EF. For both views, identification of the endocardial–
blood pool interface may be challenging because of trabeculations or Recommendation
papillary muscles, however, the interface may be identified by its smooth
All standard TNE studies should include continuous-
contour. Tricuspid annular plane systolic excursion (TAPSE) is a measure
wave Doppler of any TR and/or PI jet, systolic EI, and
of longitudinal function that reflects RV EF58 and for which normative
evaluation of PA Doppler waveform for PVR index and
data have been established across most gestational age categories57,59
the presence of notching.
(Table 3, Figure 12). In addition, thresholds associated with poor prognosis
Journal of the American Society of Echocardiography McNamara et al 177
Volume 37 Number 2

Table 2 Typical indications for which a limited focused evaluation using cPOCUS may aid clinical decisions in NICUs

Indication Potential impact on decision-making Pitfalls

Central catheter tip location  Ensure tip is central and not too deep in RA or  Actual catheter tip may be difficult to
beyond and assist repositioning in real time identify in neonates
 Ensure UVC not intrahepatic, avoiding delay in  Artifacts can be mistaken for catheter
diagnosis and need for repeated radiography
Identification of effusions  Immediate identification or exclusion of  Must have knowledge about how to
 Sudden onset of shock (central pericardial/pleural effusion as a cause of shock, best drain pockets of fluid in different
line complication) assist in institution of specific treatments (e.g., locations
 Aiding management of fetal discontinue infusion, emergency
hydrops at birth pericardiocentesis or pleurocentesis, as needed)
 Identify compartment with most urgent need for
drainage in newborn with multiple effusions to aid
resuscitation efforts
Suspected hypovolemia  Qualitative identification of underfilled cardiac  Fetal shunts may complicate
chambers may guide volume resuscitation assessment of volume status
 May assist in decisions to alter ventilator  Relationship of IVC diameter and
strategies if affecting venous return collapsibility and volume changes not
well established in neonates,
particularly with invasive ventilation
Suspected underperfused states  Qualitative findings such as grossly impaired  Scope limited by need to confirm
 Hypotension myocardial systolic performance, normal cardiac anatomy, particularly
 Lactic acidosis hypercontractile myocardial function (e.g., for first few weeks of age
 Metabolic acidosis vasodilatory physiology in sepsis) may aid clinical  TNE required to delineate specific
decisions (e.g., alter ventilator strategies, pathophysiology, interplay between
inotrope vs vasopressor and dose titration). LV pulmonary and systemic
fractional shortening may be taught at some hemodynamics and shunts
centers.
IVC, Inferior vena cava; RA, right atrium.

Shunts. An in-depth discussion on the evaluation of hemodynamic Other Considerations. TNE evaluation of central lines is
significance of the PDA will follow in Section 3. When measuring important as thrombi and pericardial tamponade may all cause sud-
diameter, it is important to note (particularly after treatment) that den and unexpected acute deterioration; specifically, cardiogenic
there are differences in transductal size depending on the plane of in- shock or pulmonary embolism may ensue. Having a high index of sus-
sonation. A sweep from the aortic arch to the branch PAs from a high picion for these complications is crucial and can be lifesaving.
parasternal view has the greatest potential of demonstrating entire
Recommendation
PDA length (Table 3, Figure 18). Left-to-right flow is exclusively
from the aorta to the PA and right-to-left flow is the opposite (exclu- All standard TNE evaluations should include an assess-
sively from PA to aorta). When there is bidirectional flow, flow should ment of the position of any central lines (Table 3,
be further qualified as mostly left to right or right to left. PAP may be Figures 21 and 22), an exclusion of pericardial effusion
considered suprasystemic if $60% of transductal systolic flow is right (Table 3, Figure 23), and surveillance for potential com-
to left63 (Table 3, Figure 19). Branch PA diastolic velocity may be plications such as thrombosis and/or vegetations
measured as an alternative marker of ductal shunt56 (Table 3, (Table 3, Figure 24).
Figure 20). Atrial communications are very common in the neonatal
period and their size and shunt direction are optimally viewed from Advanced Imaging and Measurements
the subcostal window. Assessment of atrial shunt direction using The use of Doppler tissue imaging to obtain supplementary data
pulsed-wave Doppler may be challenging because of frequent move- about myocardial performance may have advantages over conven-
ment of the atrial septum.64 It is also important to note that the atrial tional Doppler methods. Conventional pulsed-wave Doppler cap-
jet may be eccentric and, when directed toward the ostium of the su- tures the velocity of low amplitude, fast-moving signals and
perior vena cava, may appear blue on color Doppler but still reflect therefore reflects the velocity of blood. In contrast, Doppler tissue
left-to-right flow. Atrial shunt direction is primarily reflective of atrial imaging captures the velocity of high amplitude, slower moving
and ventricular compliance and should not be used independently signals.65 Higher temporal resolution, and therefore higher frame
to adjudicate pulmonary pressure relative to systemic.64 rate, is achievable when a very narrow sector is used. This makes
it possible to capture time intervals, such as the isovolumetric
Recommendation contraction time and IVRT, with greater accuracy and enables cal-
culations such as the systolic to diastolic duration ratio (Figure 25).
All standard TNE should include an assessment for the
This is particularly useful in neonates given the tendency toward
presence and directionality of shunts. Evaluation of
high heart rates. There is limited neonatal literature; however, in
the PDA should include measures of LA volume loading
adults the ratio of conventional Doppler velocity (e.g., MV E) to
and SBF as detailed in Section 3.
tissue Doppler velocity (e.g., LV lateral wall e’) have been used
178 McNamara et al Journal of the American Society of Echocardiography
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Table 3 Images and measurements that should be included in any targeted neonatal echocardiogram

Formula/
measurement
Measurement View (if applicable) Measurement performance Other points

LV systolic function
Area-length method Apical and PSAX Figure 1  Calculated using a long Assumptions
Also known as axis length (L) and cross-  Base = cylindrical
hemicylindrical sectional area (A) of an  Apex = ellipsoid
hemiellipsoid model orthogonal short-axis
view at the midpapillary
muscle
Simpson biplane Apical four-chamber/ Figure 2  Trace LV endocardial–  ECG can help identify
Normal: 55%- 70% apical two-chamber blood pool interface end of diastole, but
LV-focused view that (between cavity and end-systole is less
maximizes LV area and compacted reliable. Estimating
ensures clear myocardium); end- the minimum
endocardial border systole and end-diastole chamber size on 2D
definition  Papillary muscles should evaluation is
be excluded from the preferable.
cavity tracing and MV
closed
 When approaching the
MV plane, the contour is
closed by connecting the
two opposite sections of
the MV ring (from the
valve hinge point) with a
straight line
Fractional shortening PLAX or PSAX Figure 3  Linear, internal  ECG can help identify
Normal: 30%-45% dimensions may be end-systole and end-
obtained either on 2D or diastole.
M-mode images of the  M-mode imaging:
left ventricle in PLAX or myocardial border of
PSAX view LV posterior wall
 M-mode imaging: line of (location of caliper
interrogation should be application for
applied at the level of the measurements) may
MV leaflet tips51 be denoted as the
 2D: measurements line of greatest slope
should be obtained from during systole
PSAX or PLAX view at
the level of the MV leaflet
tips
LV diastolic function/LA loading
Pulmonary vein S, D, and Apical four-chamber Figure 4  Align RLPV and place the  Venous dilation may
A velocities sample volume parallel affect flow velocity,
to flow inside the vein particularly when
shunt is chronic
Mitral E, A, and E/A Apical four-chamber Figure 5  PW Doppler at level of  If E and A wave
Normal: GA dependent tips of MV leaflets appear fused (cannot
 Estimate ratio of velocity be distinctly
of early (E) and late (or identified),
atrial, A) waves measurement should
not be performed
IVRT Apical four-chamber Figure 6  Open LVOT with anterior  Increase sweep
angulation and/or slight speed to spread out
clockwise rotation the waveform to
 PW Doppler with the facilitate IVRT
sample volume at the measurement
crossing of LV inflow and
outflow
(Continued )
Journal of the American Society of Echocardiography McNamara et al 179
Volume 37 Number 2

Table 3 (Continued )
Formula/
measurement
Measurement View (if applicable) Measurement performance Other points

LA/Ao ratio PLAX Figure 7  M-mode measurement  Relatively poor


Normal <1.552 using leading edge of Ao performance as a
to leading of LA end- PDA marker in
diastole51 isolation53
 Make sure two leaflets
can be seen
SBF
LVO Apical five-chamber, Figure 8  VTI: PW Doppler with  Angle of insonation
Normal 150-300 mL/min/kg PLAX sample volume at level of as close to 0 as
aortic valve annulus possible. Angle
 Annulus: from hinge correction should not
point (not leaflet) to hinge be used.
point of annulus
Aortic diastolic flow Suprasternal or Figure 9  Image aortic arch and  Diastolic flow
Normal: absent flow abdominal use color Doppler to reversal in the
throughout diastole identify DAo (scale 60-70 postductal DAo and
cm/sec) abdominal Ao
 PW Doppler with sample reversal is a marker of
volume in DAo at the hemodynamically
level of the diaphragm significant PDA.
 Decrease ‘‘low-velocity Rarely this can
reject’’ to <0.1 m/sec to denote
visualize lower velocity hemodynamically
flow important aortic valve
incompetence in
neonates.
 Antegrade flow may
suggest aortic arch
obstruction or
coarctation
 Retrograde flow in
the preductal arch
may suggest cerebral
vein of Galen
malformation or
severe LV disease
 Suprasternal view
preferred as easier to
align parallel to flow
Celiac artery and SMA Subcostal LAX view Figure 10  Image abdominal Ao in  Angle of insonation
diastolic flow velocity long axis and identify as close to 0 as
Normal: antegrade flow celiac trunk possible. Angle
throughout  PW Doppler with sample correction should not
volume in proximal celiac be used.
artery or SMA  Poor angle is
common for SMA
measurements
RV function
RV FAC RV three-chamber Figure 11  RV-focused view that  Identifying the
Normal or maximizes RV area endocardial–blood
RV three-chamber: $0.35 RV-focused apical four-  Trace RV endocardial– pool interface may be
RV four-chamber: $0.35 chamber blood pool interface challenging because
(between compacted of RV trabeculations
myocardium and the or papillary muscle
cavity) at end-systole
and end-diastole on
images with clear
(Continued )
180 McNamara et al Journal of the American Society of Echocardiography
February 2024

Table 3 (Continued )
Formula/
measurement
Measurement View (if applicable) Measurement performance Other points

endocardial border
definition
 RV trabeculations should
be excluded from the
cavity tracing
 When approaching the
TV plane, the contour is
closed by connecting the
two opposite sections of
the TV ring (from the
valve hinge point)
TAPSE Apical four-chamber Figure 12  DTI enhanced M-mode  Measurement should
Normal: GA dependent54 (RV focused) imaging is used with be performed from
sector narrowed (if ‘‘leading edge to
needed) such that frame leading edge’’ or
rate is >200 frames/sec ‘‘outer edge to outer
 Line of interrogation edge’’
should pass through the  TAPSE is calculated
apex and through the RV as the differnce from
base at the lateral end diastole to end
tricuspid annulus systole
 Cursor perpendicular to
the TV annulus
RVO PLAX or PSAX Figure 13  Narrow window on the  Angle of insonation
Normal 150-300 mL/min/kg PA as close to 0 as
 PW Doppler with sample possible
volume at the level of the  PV should be well
PV annulus seen throughout the
 PV diameter estimated entire cardiac cycle
as the distance between  VTI and annulus
valve hinge points in late should be from the
systole same plane
Pulmonary hemodynamics
PAP (PI jet) PLAX or PSAX Figure 14  CW Doppler through the  PI jet velocity can be
pulmonary regurgitation used to calculate
jet components of PAP
RV systolic pressure (TR Various Figure 15  CW Doppler of tricuspid  Falsely low TR jet
jet) regurgitation jet velocity may occur in
 Measure peak TR jet setting of reduced RV
velocity from a complete systolic function
Doppler envelope
Systolic EI PSAX Figure 16  Ratio of left-right and AP  Ensure RV overlies
Normal #1.3 diameter of LV at end- LV
systole that  Ventricular wall can
quantitatively estimates be differentiated from
‘‘interventricular septal papillary muscle by
flattening’’55 its smooth interface
 Level of papillary muscle
PVR index PLAX or PSAX Figure 17  PW sample volume must  Estimate of RVET
May be expressed as either be within the main PA at may be difficult in
RVET/PAAT (normal <4) or the tip of the PV leaflets setting of PDA shunt
PAAT/RVET (normal <0.25) when open because of
 Peak velocity of the obscuration of the
Doppler envelope may Doppler envelope at
appear ‘‘rounded,’’ such end-systole
(Continued )
Journal of the American Society of Echocardiography McNamara et al 181
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Table 3 (Continued )
Formula/
measurement
Measurement View (if applicable) Measurement performance Other points

that one distinct peak is


difficult to identify. In this
scenario, PAAT should
be measured at the
earliest aspect of the
peak, rather than at
midpeak.
PDA
Ductal diameter Suprasternal/high PS Figure 18  With probe at 12 o’clock  Among neonates on
position, image the MPA mechanical
and DAo visualizing the ventilation, the high
full length of the PDA PS view may not
 Measure diameter at the provide optimal
narrowest point along image quality; an
the ductal length, alternative view is low
typically near the PS by angling
pulmonary end, when posteriorly from a
the shunt is at its peak branch PA view
during cardiac cycle
Ductal Doppler Suprasternal/high PS Figure 19  Sample volume within  When using CW
the PDA at pulmonary Doppler, ensure that
end, distal to the Doppler beam is
narrowest diameter. Use placed at the
PW if there is no aliasing narrowest point of the
(typically peak velocity < PDA parallel to the
2 m/sec) and CW if direction of flow
aliasing occurs despite
increasing the PW scale
to the maximum.
 Measuring shunt
gradient: perform a VTI
trace of the PDA gradient
to obtain the (1) peak
systolic pressure
gradient and (2) mean
pressure gradient
Branch PA velocity High PS Figure 20  Narrow window on the  Left PA is typically
Normal diastolic velocity PA and angle anteriorly easier to align and is
<0.3 m/sec to bring branch PAs into therefore the
view preferred site for
 PW Doppler with sample Doppler interrogation
volume within the
proximal branch PA
Other core elements of TNE
UVC position Subcostal modified Figure 21  Identify the UVC in long  Eustachian valve
PSAX axis in the ductus appears as a thin,
venosus and the tip of linear echogenic fold
the UVC at the junction of the
IVC and RA and may
be mistaken for a
central line
UE PICC position Sagittal high PS Figure 22  Central to peripheral  Identifying the
sequential evaluation catheter tip may be
technique:
(Continued )
182 McNamara et al Journal of the American Society of Echocardiography
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Table 3 (Continued )
Formula/
measurement
Measurement View (if applicable) Measurement performance Other points

I. Evaluate for intracardiac challenging for PICC


placement: from multiple in the UE
planes, sweep the right  Injection of a small
ventricle and right volume of saline (and
atrium; if no catheter visualization of the
identified, go to II saline exiting the
II. Evaluate for SVC catheter tip) is the
placement: from most reliable method
modified PS long-axis of ascertaining
view, interrogate SVC catheter tip position
for the presence of the
catheter; if no catheter
identified, go to III
III. From high PS view,
interrogate the
subclavian and
innominate veins for the
presence of the
catheter
Pericardial effusion/ Various Figure 23  Pericardial effusion may  Tamponade occurs
tamponade be circumferential (larger when intrapericardial
volume) or focal (smaller pressure exceeds RA
volume, typically pressure
dependent areas)  RA collapse during
 Unilateral/localized ventricular systole,
effusions may also be which reflects
located in the pleural reduced atrial filling,
space or abdomen is the earliest
 Tension pneumothorax echocardiography
may cause tamponade indicator of
without a pericardial tamponade
effusion
Thrombus/vegetation Various Figure 24  Interrogate suspicious  Echogenic foci within
echogenic foci from at cardiac chambers
least two different are common and
planes, using continuous should be
sweeps if possible distinguished from
adjacent cardiac
endocardium/
myocardium
 Pedunculated or
mobile echogenic
foci should elicit a
high index of
suspicion for
thrombus or
vegetation

2D, Two-dimensional; Ao, aorta; AP, anteroposterior; CW, continuous-wave; DAo, descending aorta; ECG, electrocardiography; GA, gestational
age; LAX, long-axis; LVOT, left ventricular outflow tract; PLAX, parasternal long-axis; PS, parasternal; PSAX, parasternal short-axis; PV, pulmonary
valve; PW, pulsed-wave; RA, right atrium; RLPV, right lower pulmonary vein; SMA, superior mesenteric artery; SVC, superior vena cava; TV,
tricuspid valve; UE, upper extremity; VTI, velocity-time integral.

as a relatively load independent measure of filling pressure and parallel with the sample volume placed just below the annulus.
diastolic performance.66 Similar calculations can be performed at In neonates a standard Doppler gate size of 2 mm is recommen-
the tricuspid valve free wall; however, normative data in neonates ded to minimize contamination by atrial signal. Additionally,
are limited. Pulse-wave Doppler tissue imaging may also be used Doppler tissue imaging is a point measurement and may not be
to measure ventricular deformation/strain.67 As in conventional reflective of global myocardial performance in the presence of
Doppler, it is essential that the angle of insonation of the wall is regional wall motion abnormalities.
Journal of the American Society of Echocardiography McNamara et al 183
Volume 37 Number 2

Recommendation strain, the strain task force recommends tracing the left atrium to
extrapolate across pulmonary veins and LA appendage orifices.72
Where normative data exist, Doppler tissue imaging pro-
vides additional information on myocardial perfor-
Recommendation
mance and should be included in a standard TNE When performing standard TNE, STE may provide ancil-
assessment as part of a multiparametric appraisal of heart lary data regarding systolic performance, segmental ab-
function. Myocardial velocities should be considered normalities, and load dependency; however, natural
when there is suspected heart dysfunction and disagree- history data are limited to date in the neonate, and
ment between other modalities (e.g., TAPSE, RV FAC). further research is needed.

The First Complete Echocardiogram


Research Tools and Emerging Measurements
If standard TNE constitutes the first neonatal echocardiogram, for pa-
Speckle-Tracking Echocardiography. Speckle-tracking echo- tients with low suspicion for CHD, it should include the essential
cardiography (STE) is a method of function assessment that is used views and sweeps to enable a comprehensive anatomical and func-
primarily to measure tissue deformation by tracking the motion of tional assessment. The study should be performed by a sonographer
two-dimensional ‘‘speckles’’ within the myocardium throughout the with proficiency in performing a complete study to screen for critical
cardiac cycle. Strain analysis involves the absolute deformation CHD.73,74 Although the study may be performed and interpreted
from baseline and is expressed as a percentage change.67 Strain rate preliminarily by a neonatologist with advanced TNE experience, it
is the rate at which that deformation occurs and is thought to be should also be reviewed by a pediatric cardiologist in a timely fashion
less load dependent than strain, making it a potentially superior mea- (i.e., within 24 hours or a reasonable time frame on the basis of local
sure of contractility.67 Both strain and strain rate may be measured for standards).8 Data from a single high-volume center reports high
the left and right ventricles. Global longitudinal strain is increasingly concordance of the impression from a first study, performed and in-
recognized as a more effective technique than conventional EF in de- terpreted by a trained neonatologist, with the results of the formal pe-
tecting subtle changes in LV function, and normative data for STE pa- diatric cardiology evaluation.75 In addition, the need for reimaging
rameters have been published in select populations68-70 (Figure 26). was also low. Of note, the rate of major CHD was low, which shows
Because the processes of LV systolic performance are complex and compliance with the guidelines. It is also important to highlight that
include three directions according to myocardial fiber alignment, these results are reflective of a high-volume neonatal hemodynamics
strain may be measured using STE in three orientations: longitudinal, program which is closely integrated with the pediatric echocardiogra-
circumferential, and radial. RV systolic performance, in contrast, is phy laboratory; therefore, the results may not be applicable to low-
limited to longitudinal strain which may be measured either in an volume or rural centers. Telemedicine may be an option for NICUs
RV-focused four-chamber or RV three-chamber view. Segmental without pediatric cardiologists on staff.
RV strain should be measured at the free wall only and should not The first complete echocardiogram must include standardized im-
include the septum in the four-chamber view. STE has also been ages and sweeps (Table 4) sufficient to exclude critical cyanotic lesions
used to demonstrate the three phases of LA mechanics: (1) the reser- (e.g., d-transposition of the great arteries). Outflow tract obstruction
voir phase (mitral closure to opening), which encompasses the LV iso- must be assessed to exclude ductal dependency for SBF (for a
volumetric contraction time, ejection, and IVRT; (2) the conduit neonate with shock) or pulmonary blood flow (for a neonate with
phase, from MV opening through diastasis until the onset of LA cyanosis). Certain ductal-dependent lesions, such as coarctation of
contraction; and (3) the contraction phase, from the onset of LA the aorta, may be difficult to assess without sufficient expertise and
contraction until MV closure. The clinical utility of LA strain remains experience. Both atrioventricular valves and the ventricles should
an area of active research. be evaluated for size and morphology to differentiate functional vs
Finally, STE can be used in conjunction with other measurements. structural pathology, such as congenital valve dysplasia or primary car-
For example, the load dependency of strain may be used in conjunc- diomyopathy. Sweeping in all planes is important to understand the
tion with systemic BP to produce a measurement of LV myocardial anatomy in three dimensions and, using color Doppler, to assess for
work, which has been validated as a measure of myocardial ener- septal defects. Pulmonary venous return must be assessed critically,
getics. Similarly, more comprehensive evaluation of the entirety of particularly for a cyanotic neonate with or without respiratory distress.
LV contractility may be obtained using a measurement of LV Twist, As the pulmonary veins are small structures and various forms of
which is defined as the net difference of LV rotation between apical anomalous drainage may occur, some more life-threatening than
and basal short-axis planes. ‘‘Torsion’’ is the term used to describe others in the neonatal period, careful review by a pediatric cardiolo-
LV twist indexed to its length and enables the comparison of LV twist gist is essential.
across differing LV sizes. Rotational strain is performed at the basal Other elements of the first-time anatomic evaluation may be per-
(which rotates clockwise) and apical (which rotates counterclockwise) formed in specific situations. Systemic venous anomalies are unlikely
levels. Software is used to plot apical and basal rotation during one to be critical but may have important implications for central line ac-
cardiac cycle to determine twist and then indexed to LV length to cess. Arch sidedness and branching should be evaluated for neonates
calculate LV torsion, which accounts for differences in LV size.71 with upper airway obstruction to exclude a vascular ring or PA sling.
To optimize STE, it is essential that the imaging plane includes the Aortic arch sidedness may also be necessary if surgical PDA ligation or
entirety of the wall(s) of interest throughout the cardiac cycle. Air other intrathoracic interventions are warranted. Finally, although cor-
interference is often a limitation of STE, particularly among babies onary artery anomalies rarely present in the neonatal period, their
with hyperinflated lungs or pneumomediastinum. Images that are evaluation is a standard part of first-time congenital heart studies.
going to be used for STE should be taken at similar heart rates such Complete evaluation of coronary artery anatomy and physiology
that it is possible for the software to integrate the images. For LA should be performed during the hospitalization. Interpretation by
184 McNamara et al Journal of the American Society of Echocardiography
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Parasternal Short Axis View Apical Four Chamber View

Area

Length

Figure 1 LV volume measurement using the area-length method from the apical four-chamber view. L, Length; r, radius.

Four Chamber View Two Chamber View

Diastole Diastole

Systole Systole

Figure 2 Simpson biplane measurement of EF from the apical four-chamber and two-chamber views. EDV, End-diastolic volume;
ESV, end-systolic volume.

Parasternal Long Axis View

Parasternal Short Axis View

Figure 3 Shortening fraction measurement from the parasternal long-axis view. FS, Fractional shortening; LVEDD, LV end-diastolic
diameter; LVESD, LV end-systolic diameter.
Journal of the American Society of Echocardiography McNamara et al 185
Volume 37 Number 2

Apical View of the Pulmonary Veins

S
D

Figure 4 Pulmonary vein S, D, and A velocities using pulsed-wave Doppler.

echocardiographers with expertise is important, and follow-up imag- or chronic PH, or quantitative evaluation of heart function are not rec-
ing may be performed as recommended. ommended indications for cPOCUS. A standard POCUS assessment
Recommendation may involve evaluation of multiple organ systems; therefore, as the
emphasis on cardiac imaging is limited, the training requirements
A neonatologist with advanced training in TNE may are less demanding than for standard TNE.
perform standard TNE as the first study in patients
with a low index of suspicion for CHD, but the study
Recommendation
should include the essential views and sweeps to enable A neonatologist-performed cPOCUS evaluation may
anatomic assessment. At centers with on-site pediatric include evaluation of central catheter tip location, iden-
echocardiography laboratories, these studies should be tification of pericardial or pleural effusions, subjective
reviewed within a timely manner on the basis of local (‘‘eyeballing’’) evaluation of inferior vena caval collaps-
standards. At centers without on-site pediatric cardiol- ibility as a surrogate of hypovolemia, and subjective
ogy, when significant CHD is suspected or diagnosed, evaluation of myocardial systolic performance. If a cPO-
transfer to a site with pediatric cardiology or remote pe- CUS study is the first patient evaluation, a timely stan-
diatric cardiology study review should occur. dard TNE evaluation or complete pediatric cardiology
echocardiography evaluation is recommended.
The cPOCUS Evaluation
Scope of cPOCUS Evaluation. The recent ASE guidelines and TNE: APPLICATION OF IMAGING
recommendations for cPOCUS focused on use in children and adults,
Disease-Based Screening
but neonates were excluded.15 A recent technical report by the
American Academy of Pediatrics, however, provided additional guid- PDA in Premature Infants. Scope of the Problem. PDA is the
ance on the use of POCUS, including cPOCUS, in neonates.76 The most common cardiovascular abnormality in premature infants
role of cPOCUS in the evaluation of the acutely unstable neonate with >70% of infants <28 weeks’ gestation demonstrating persistent
has also been suggested.77 Therefore, the writing group felt it was ductal patency beyond the first week of age.23 Although accurate
important to delineate the scope of cPOCUS and how it differs echocardiography determination of shunt volume is not feasible,
from a standard TNE evaluation. cPOCUS is a brief, limited cardiovas- surrogate markers of pulmonary overcirculation and systemic hypo-
cular imaging evaluation in specific clinical situations. The typical in- perfusion are used to estimate the degree of hemodynamic signifi-
dications for cPOCUS include evaluation of central catheter cance.24 Adjudicating hemodynamic significance requires
(arterial or venous) tip location, identification of pericardial or pleural integration of echocardiographic markers of PDA shunt volume
effusions, and differentiation of hypovolemia vs myocardial dysfunc- and clinical factors such as gestational age or confounding treat-
tion in an acutely unstable neonate (Table 2). At some centers, LV ments (e.g., mechanical ventilation). This may result in improved
fractional shortening may be used to quantify the severity of heart risk prediction facilitating a more accurate and targeted selection
dysfunction. Assessment of hemodynamic significance of PDA, acute of infants that are more likely to benefit from treatment.78 Those
186 McNamara et al Journal of the American Society of Echocardiography
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Apical Four Chamber View – Mitral Inflow Doppler

EA
A
E

Preterm Infant Term Infant

Figure 5 MV pulsed-wave Doppler measurement showing passive (E-wave) and active (A-wave) flow in preterm and term infants
from the apical four-chamber view.

IVCT IVRT

Aor c
Ejec on
Time

Figure 6 Estimation of isovolumetric measurements using pulsed-wave Doppler from the apical four-chamber view. IVCT, Isovolu-
metric contraction time.

Parasternal
Long Axis View

Figure 7 LA–to–aortic root ratio measurement from the parasternal long-axis view. Ao, Aorta.
Journal of the American Society of Echocardiography McNamara et al 187
Volume 37 Number 2

Apical Four Chamber View

Parasternal Long Axis View

LVOT
VTI

Figure 8 LVO measurement on the basis of pulsed-wave Doppler from the apical five-chamber view. HR, Heart rate; r, radius; VTI,
velocity-time integral.

Aor c Arch
Forward Flow in Diastole

PDA Reversed Flow in Diastole

Figure 9 Pulsed-wave Doppler interrogation of aortic diastolic flow from the suprasternal arch view.

Celiac Trunk and SMA Diastole

Forward
Diastolic Flow
Diastole
Absent
Diastolic Flow
Diastole
Reversed
Diastolic Flow

Middle Cerebral Artery Diastole


Forward
Diastolic Flow

Absent Diastole
Diastolic Flow
Diastole
Reversed
Diastolic Flow

Figure 10 Pulsed-wave Doppler interrogation of celiac trunk and superior mesenteric artery flow from the abdominal view and middle
cerebral artery from the axial mastoid view.
188 McNamara et al Journal of the American Society of Echocardiography
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RV Three
Chamber View

RV Four
Chamber View

=

Figure 11 RV FAC measurement from the RV three-chamber and RV four-chamber views.

elements have been described at length elsewhere but include the premature infants.79 There are several risk factors associated
following (Table 5): PDA shunt volume assessment and its impact with developing BPD-associated PH. These include fetal growth
on the systemic and pulmonary circulations (see the previous sec- restriction, oligohydramnios, prolonged rupture of membranes,
tion), myocardial function evaluation (including LV diastolic perfor- preeclampsia, prolonged exposure to PDA, length of mechanical
mance), and antenatal and perinatal characteristics that can act as ventilation and oxygen supplementation, and extreme prematu-
effect modifiers to either mitigate or exacerbate potential detri- rity.80 BPD-associated PH is associated with an increased risk
mental consequences of a shunt. for mortality and morbidity in premature infants; therefore,
prompt recognition and diagnosis are essential for ongoing man-
Indications for and Suggested Timing of Echocardiography. All agement.81
premature infants with clinical features of pulmonary overcirculation
(oxygenation or ventilation impairment) and/or systemic hypoperfu- Indications for and Suggested Timing of Echocardiography.
sion (postductal hypotension, metabolic acidosis) should have timely Screening for BPD-associated PH should be considered for all prema-
echocardiography assessment. In addition, because of the imprecision ture infants requiring ventilator support beyond the first week of age.
of clinical symptoms, early screening echocardiogram within 72 hours A screening echocardiogram is recommended for all preterm infants
after birth may be considered in extremely premature infants born before 29 weeks’ gestation, who require ongoing oxygen
<28 weeks of gestation. requirement or assisted ventilator support, at 8 postnatal weeks or
36 weeks (whichever is sooner) postmenstrual age to screen for the
Guidance on Clinical Decision-Making. Currently there is no
presence of BPD-associated PH.
consensus on the need for, or timing of PDA treatment in preterm in-
fants. Ascribing hemodynamic significance should be based on a mul- Guidance on Clinical Decision-Making. Standard TNE should be
tiparametric echocardiography approach including ductal size and the used to estimate PAP, RV function, and the adequacy of SBF
pattern of transductal flow, markers of pulmonary over circulation (Table 5). TNE can be used to guide the initiation of therapy,
and systemic hypoperfusion. assess treatment response, and finally to guide the weaning of
treatment. Assessment of PAP should include the interrogation
Recommendations of the TR jet and the shunt velocity across the PDA, if present,
In every neonate with clinical suspicion for PDA, or to estimate RVSP and PAP. However, those markers may not
those <28 weeks’ gestation, the first standard TNE study be present in all infants and can underestimate the degree of
to characterize hemodynamic significance of PDA PH in the presence of RV dysfunction. Assessment of septal
should be sufficiently comprehensive to exclude major wall morphology from the parasternal short-axis view and the
CHD, especially ductal-dependent systemic or pulmo- calculation of the systolic EI may provide an objective measure
nary blood flow lesions. Subsequent TNE may be useful of the degree of septal flattening observed in the presence of
in follow-up to document spontaneous closure or the elevated RV pressure. PAAT and PVR index are validated and
effect of treatment. Patients suspected of an additional reproducible markers that can be used to detect the presence
cardiovascular malformation should be referred for of elevated PAP in premature infants. Subjective assessment of
pediatric cardiology review, or transfer if indicated, in a RV function is highly unreliable and should be avoided.55 RV
timely manner. function should be objectively assessed using validated measure-
ments including FAC, Doppler tissue imaging, TAPSE, and defor-
mation imaging. Appraisal of pulmonary vein flow (all visualized
PH in Infants with Bronchopulmonary Dysplasia. Scope of vessels) to detect high pulmonary vein velocity (>1.0 m/sec)
the Problem. Bronchopulmonary dysplasia (BPD)–associated PH should be a mandatory component of all studies for BPD-
is an important morbidity occurring up to 20% of high-risk associated PH, with timely referral to a pediatric cardiologist.
Journal of the American Society of Echocardiography McNamara et al 189
Volume 37 Number 2

RV Focussed Four Chamber View


Diastole Systole

TAPSE

Figure 12 TAPSE measurement from the apical four-chamber view.

Recommendation 900,000 neonatal deaths each year.82 Therapeutic hypothermia has


become standard of care therapy in the developed world, with evi-
Preterm infants with persistent need for respiratory sup- dence from randomized trials suggesting both a morbidity and mortal-
port (continuous positive airway pressure or mechanical ity benefit.83 Observational studies have suggested that deranged
ventilation) and/or prolonged oxygen need should be cerebral blood flow during the first 3 to 4 postnatal days may be asso-
considered for standard TNE evaluation to screen for ciated with a greater likelihood of abnormal brain outcomes.60,84-86
the presence of PH and rule out CHD. Infants born Although the mechanism by which this may occur is uncertain,
before 29 weeks’ gestation should be considered for a ischemia/reperfusion disease in the setting of impaired cerebral
screening TNE assessment at 8 postnatal weeks or autoregulation is a biologically plausible mechanism. RV
36 weeks’ postmenstrual age (whichever is sooner) to dysfunction is a common co-traveler with HIE, in part because of a
assess for the presence of BPD-associated PH. TNE allows common mechanistic origin. In utero adaptation to impaired
assessment of the effect of treatment on PAP, RV func- placental substrate delivery results in redirection of blood away
tion, shunt direction at the atrial and ductal levels, and from the lungs (via increasing PVR) to the most vulnerable organs
screening for pulmonary vein stenosis. which include the adrenal gland, coronary circulation and the
brain.87 This results in two primary issues of postnatal adaptation.
First, the pulmonary vasculature is primed to constrict, which impedes
Infants with HIE. Scope of the Problem. HIE is one of the leading the rapid postnatal PVR decline; this is further exacerbated by the
causes of neonatal mortality worldwide and contributes to nearly presence of acidosis, hypercarbia and hypoxia and places a substantial

Parasternal Long Axis View

Figure 13 RVO measurement on the basis of pulsed-wave Doppler from the parasternal long-axis view. HR, Heart rate; r, radius; VTI,
velocity-time integral.
190 McNamara et al Journal of the American Society of Echocardiography
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Parasternal Long Axis View


mPAP = 1/3 RVSp + 2/3 PADp
PADP = 4 (PADV)2 + RAp*
RAp* es mated as 5mmHg in neonates

End diastolic pulmonary


regurgita on velocity

Figure 14 PAP measurement on the basis of pulsed-wave Doppler from the parasternal long-axis view. mPAP, Mean pulmonary ar-
tery pressure; PADP, PA diastolic pressure; PADV, PA diastolic velocity; RAp, right atrial pressure; RVSp, RV systolic pressure.

afterload stress on the right ventricle. Second, the right ventricle is results in peripheral vasoconstriction, which may mask hypoperfu-
vulnerable to simultaneous ischemic injury because of its prominent sion. In addition, other biochemical markers of cardiovascular health
role in the transitional circulation88 and has a greater ratio of circum- are difficult to interpret following a hypoxic-ischemic event91 because
ferential radius to wall thickness89 and lower coronary perfusion pres- of the primary hypoxic-ischemic insult. RV dysfunction and PH may
sure in the presence of high central venous pressure.90 In addition to be present despite relatively subtle clinical symptoms.
some degree of primary injury, the left ventricle may be compromised
because of impaired preload, ventricular interdependence (a second- Indications for Echocardiography. All infants with HIE who pre-
ary impact due to shared muscle fibers), or via ventriculoventricular sent with cardiovascular instability or hypoxemic respiratory failure,
interaction (impact of a pressure- and volume-loaded right ventricle and/or require vasoactive agents, should undergo timely standard
on LV compliance, filling, and size). The ductal shunt may be an TNE. Screening echocardiography may be beneficial because of the
important modulator of either systemic or pulmonary blood flow in poor reliability of common markers of systemic perfusion (e.g.,
patients with univentricular dysfunction. Therapeutic hypothermia elevated lactate, decreased urinary output).92-94 It is essential to

Parasternal Long Axis View

RAp* es mated as 5mmHg in neonates

Apical Subcostal View

Peak TR velocity

Figure 15 RV systolic pressure measurement on the basis of continuous-wave Doppler from the apical four-chamber view. RA, Right
atrial; RVSp, RV systolic pressure.
Journal of the American Society of Echocardiography McNamara et al 191
Volume 37 Number 2

LV Parasternal Short Axis View


LV Eccentricity index = D2 ÷ D1
(Measured during end systole)
FLAT
FL
LAT IVSS EI
EI

D1

D2 D1

D2

Normal RV Pressure Elevated RV Pressure


Figure 16 LV EI from the parasternal short-axis view at the level of the papillary muscle just distal to MV leaflets.

Pulmonary Artery Doppler


Right Ventricular
Ejec on Time

RV ejection time
PA acceleration time

Pulmonary Artery
Accelera on me

Pulmonary Artery
Doppler Notching In
the se ng of elevated
PA pressure

Figure 17 PVR index (PVRi) on the basis of pulsed-wave Doppler from the parasternal long-axis view. PVRi may be calculated as
either RVET:/PAAT (normal, <4.0) or PAAT/ RVET (normal, <0.25) according to institutional standards.

PDA Diameter
Measurement
PDA Anterior Wall

PDA Diameter is measured


perpendicular to the
anterior wall at the
pulmonary end in 2D

Figure 18 PDA diameter measurement estimated from the suprasternal view.


192 McNamara et al Journal of the American Society of Echocardiography
February 2024

Non-restric ve Le to Right Flow

Restric ve Le to Right Flow PDA Maximum


Systolic Velocity

Bidirec onal Flow

Right to Le Flow PDA Doppler

Figure 19 Pulsed-wave Doppler interrogation of PDA flow from the suprasternal view.

rule out duct-dependent CHD, in addition to evaluating for the Recommendations


consequences of perinatal hypoxia-ischemia.
Infants with HIE and hemodynamic instability and/or
Imaging Techniques and Guidance of Clinical Decision-Making. oxygenation failure should undergo standard TNE as
It is preferable that TNE evaluation take place as early in the hos- soon as feasible to appraise pulmonary pressures,
pitalization as is feasible. Multiple reassessments may be required to myocardial function, and cardiac output. Hemodynam-
guide therapy in acutely unstable patients. Particular attention to ically stable patients with moderate to severe HIE may
measures of RV performance, PAP and ductal shunt directionality benefit from screening to evaluate for subclinical disease
are recommended (Table 5). A right-to-left ductal shunt should and support prognostication.
also prompt consideration of duct-dependent SBF (e.g., LV dysfunc-
tion). The presence of a left-to-right atrial shunt is further suggestive
of a left heart systolic dysfunction. Comprehensive evaluation Infant of Diabetic Mother. Pathophysiology and Mechanistic
including objective metrics of RV performance should be per- Phenotypes. Infants of diabetic mothers (IDMs) may present with
formed. different cardiac phenotypes, including CHD, cardiac muscle

Laminar Flow – No diastolic Component


High Parasternal View
Pulmonary Branches

Turbulent Flow – Diastolic Runoff

Figure 20 Pulmonary branch artery velocity from the high parasternal view.
Journal of the American Society of Echocardiography McNamara et al 193
Volume 37 Number 2

Umbilical Venous
Catheter in Le Atrium Right Ventricle Pulmonary
Artery
Right
Atrium Aorta

Le
Atrium

Figure 21 Interrogation of UVC tip position from the subcostal short-axis view.

PICC in the SVC/RA


Right
Atrium

SVC

Figure 22 Interrogation of PICC tip from the high parasternal view. RA, Right atrium; SVC, superior vena cava.

RA LA
Pericardial Effusion
RV LV

External Compression
LV
(not RA collapse) RV

Figure 23 Pericardial effusion and tamponade.


194 McNamara et al Journal of the American Society of Echocardiography
February 2024

LA
Intracardiac
Thrombus/Vegeta on
RA

Figure 24 Image of intracardiac thrombus and vegetation from the subcostal view. RA, Right atrium.

hypertrophy disorders, and/or disturbances of cardiovascular and pul- are evaluated as previously described. Note that left and right cardiac
monary adaptation after birth.95 The most common pathology is output calculations may be unreliable in the presence of outflow tract
asymmetrical septal hypertrophy (an anabolic result of fetal hyperin- obstruction and influenced by shunts. Torsion and LV longitudinal sys-
sulinemia triggered by maternal hyperglycemia).96 Myocardial hyper- tolic strain by STE may be impaired in IDMs during the transitional
trophy can extend beyond the septum and involve the free walls period,103 even with preserved EF, suggesting that rotational me-
symmetrically. As the right and left posterior walls can become thick- chanics may offer a more sensitive measure of ventricular function.104
ened diastolic dysfunction in the setting of normal systolic function Although septal wall thickness may normalize by 1 month of age,
may ensue.97 Further hypertrophy may obstruct the LV outflow tract abnormal global and segmental systolic and diastolic strain values
and leads to impaired muscle relaxation and diastolic filling, may persist.102
decreased SBF, and decreased cardiac output. The severe form of
the diabetic cardiomyopathy may also lead to decreased pulmonary Recommendations
blood flow and reduced pulmonary venous return presenting clini-
cally with hypoxemia. Additionally, fetal hyperinsulinemia can tran- In IDMs with clinical signs of low cardiac output or PH,
siently delay surfactant synthesis and secretion leading to persistent standard TNE should be performed to exclude CHD and
elevation of pulmonary pressures and vascular resistance during the evaluate the degree of dynamic obstruction to the
immediate postnatal period.95 Although most of the alterations in car- LV outflow tract, diastolic and systolic dysfunction,
diac morphology and systemic or pulmonary hemodynamics appear and impact on the pulmonary vasculature.
to resolve during the first 2 to 4 weeks after birth,98-101 some patients
have persistent functional abnormalities beyond 1 month.102 Twin-to-Twin Transfusion Syndrome. Pathophysiology and
Indications for Echocardiography. Clinical phenotypes relate Mechanistic Phenotypes. Twin-to-twin transfusion syndrome
directly to the degree of dynamic obstruction to the LV outflow (TTTS) affects approximately 10% to 15% of monochorionic-
tract, diastolic and eventual systolic dysfunction, and impact on diamniotic pregnancies and is a significant contributor to perinatal
the pulmonary vasculature. In the setting of a presumed low– morbidity and mortality.105 The resultant myocardial functional and
cardiac output state (cyanosis, tachypnoea, tachycardia, and cardi- structural phenotypes are poorly understood but may stem from
omegaly) or PH (increase oxygen requirement, BP lability), TNE is the presence of placental anastomoses leading to a distinct clinical
used to detect the different presentations of cardiopulmonary phenotype. Chronic hypovolemia and growth restriction complicates
compromise in IDM and facilitate appropriate therapeutic inter- the donor twin, while chronic fluid overload, hydrops, and an adverse
vention. afterload environment are hallmarks of the recipient twin.106,107 If left
untreated, the mortality rate can approach 100% in one or both fe-
Imaging Techniques and Guidance of Clinical Decision-Making. tuses with most survivors experiencing significant morbidity including
TNE assessment of an IDM with suspected hypertrophic cardiomy- adverse neurodevelopmental outcomes.108 Treatment of TTTS with
opathy and/or PH includes evaluation of chamber dimensions, heart selective laser photocoagulation of the communicating placental ves-
function, and pulmonary pressures, with special attention paid toward sels (SLPCV) improves survival and cardiovascular outcomes by alle-
the septum and its relationship to the LV outflow tract and degree of viating, or at least mitigating, the abnormal circulatory load and
obstruction (Table 5). The increase in basal septal thickness may lead cardiac morbidity.109,110 There is an increased incidence of structural
to apposition of the anterior leaflet of the MV to the interventricular valvular disease in affected fetuses including tricuspid, mitral, and
septum during systole leading to dynamic LV outflow tract obstruc- most commonly pulmonary valves occurring predominantly in the
tion. RV morphology is assessed by both linear dimensions and RV recipient twin.111 TTTS results in a cardiomyopathy, occurring pre-
areas acquired at end-diastole and end-systole from the RV-focused dominantly in recipient twins, that is only partially understood; specif-
apical four-chamber view. RV and LV systolic and diastolic function ically, recipient monochorionic-diamniotic twins with TTTS who do
Journal of the American Society of Echocardiography McNamara et al 195
Volume 37 Number 2

Pulsed
PulsedWave
WaveTDI
TDI Color TDI

Tricuspid Valve Septum Mitral Valve

Figure 25 Doppler tissue imaging. IVCT, Isovolumetric contraction time.

not undergo SLPCV exhibit persistent myocardial hypertrophy and normalities at discharge require long-term pediatric cardiology
systolic dysfunction, over the first postnatal week. A reduction in follow-up. This approach should be implemented in all TTTS infants,
both strain and strain rate STE measurements suggest a multifactorial regardless of SLPCV treatment, as it may prove beneficial to identify
etiology for the dysfunction including adverse loading conditions and the long-standing sequelae of cardiomyopathy induced by the syn-
impaired contractility.112 In addition, ventricular hypertrophy and pla- drome.
centally derived renin-angiotensin system effectors and discordant en-
dothelin are important contributors to diastolic dysfunction in Imaging Techniques and Guidance of Clinical Decision-Making.
recipient fetuses that can precede systolic function.113,114 This Standard TNE examination of infants with TTTS should include the
dysfunction can persist postnatally, especially in infants with TTTS essential views and sweeps to enable structural assessment with close
who did not undergo SLPCV. attention given to valvar disease, especially the pulmonary valve of
the recipient twin (Table 5). Repeat assessments are recommended
Indications for Echocardiography. Structural heart disease and the due to the dynamic and evolving nature of the condition. Special
acquired cardiomyopathy leading to adverse functional and morpho- attention should be paid to linear and morphologic measurements
logic changes are evolving processes that can extend into the post- of the left and right ventricles including cavity dimensions.
natal period. Detailed structural, functional, and morphologic Assessment of pulmonary hemodynamics and surrogates of RV after-
evaluation is therefore recommended to correlate findings with the load including PAAT or RVET: PAAT index, LV EI, tricuspid valve re-
clinical course, guide therapy, and monitor treatment. If gurgitant jet velocity (if present), and PDA shunt characteristics are
neonatologist-performed TNE is the first patient evaluation, the study helpful in characterizing pulmonary hemodynamics. Biventricular
should be reviewed by a pediatric cardiologist or a comprehensive pe- functional assessment is also recommended to include EF, Doppler
diatric echocardiography study should be obtained after to appraise tissue imaging, and deformation analysis. The use of both strain and
the anatomy. Infants with persistent functional and/or structural ab- strain rate techniques may aid in the determination of the underlying

Speckle Tracking Echocardiography

Figure 26 STE. AVO, Aortic valve opening; AVC, aortic valve closure; MVC, MV closure; MVO, MV opening.
196 McNamara et al Journal of the American Society of Echocardiography
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Table 4 Mandatory anatomic surveillance components of 700.116 Approximately 50% of babies with DS will have some form
comprehensive TNE scan of CHD, the most common being atrioventricular septal defects, fol-
lowed by ventricular septal defects. PH is also extremely common
Subsequent during the early neonatal period with recent literature suggesting an
Echocardiography view First study scans incidence approaching 40%; however, this is likely to be an underes-
Situs and position of the heart U
timate, as the data were obtained from retrospective studies using
in the thorax nonstandardized clinical and echocardiographic diagnostic criteria.117
PH in infants with DS is multifactorial and may be characterized by
Systemic venous return to the U
RA (IVC/SVC) reduced alveolarization, decreased vessel density, persistence of the
double-capillary network, hypertensive arterial remodeling, blunted
Left and right atrial size and U U
shape
response to nitric oxide, and the presence of CHD.118-120 In
addition, LV diastolic dysfunction and resultant increased LA
Interatrial septum, PFO, ASD, U U
pressure leads to pulmonary venous hypertension, which plays a
direction of shunting
role in the evolution of secondary arterial PH.121 Infants with DS
AV valve morphology U
are also at increased risk for LV and RV dysfunction which is evident
Atrioventricular concordance U during fetal life and persists throughout the neonatal period and into
Presence of AV regurgitation/ U U adulthood.121-125
flow acceleration
Indications for Echocardiography. All symptomatic infants with a
Ventricular chamber size and U U confirmed or suspected diagnosis of DS, and low clinical suspicion
shape
for CHD, may undergo comprehensive TNE evaluation to assess
Presence or absence of VSDs U U structural integrity, determine the presence of PH, and evaluate
(sweeps required)
myocardial performance.126 At centers with immediate access to pe-
LV and RV outflow tract U U diatric echocardiography laboratory services, a complete echocardi-
obstruction ography assessment and pediatric cardiology consultation is
Ventricular-arterial U recommended. Ongoing follow-up is determined by the findings on
concordance the initial TNE assessment; however, regular inpatient follow-up
Aortic and pulmonary valve U should be considered to assess the evolution of pulmonary hemody-
morphology namics.
Presence of aortic and U U
pulmonary valve Imaging Techniques and Guidance of Clinical Decision-Making.
regurgitation If a standard TNE examination of infants with DS is the first patient
AV leaflets and coronary U assessment, it should include the essential views and sweeps to enable
origins structural assessment with close attention given to the presence of
Branch PA size and flow U U atrioventricular septal defects (Table 5). If neonatologist-performed
Presence/absence of PDA and U U TNE is the first patient evaluation, the study should be reviewed by
shunt a pediatric cardiologist or a comprehensive pediatric echocardiogra-
Aortic arch sidedness U U*
phy study should be obtained after to appraise the anatomy.
Special attention should be paid to pulmonary hemodynamics,
Arch patency U U
indices of LV and RV function, and cavity dimensions. Assessment
Pulmonary venous drainage U of pulmonary hemodynamics and surrogates of RV afterload
into left atrium
including PAAT or RVET: PAAT index, LV EI, tricuspid valve regurgi-
ASD, Atrial septal defect; AV, aortic valve; IVC, inferior vena cava; tant jet velocity if present, and PDA shunt characteristics are helpful in
PFO, patent foramen ovale; RA, right atrium; SVC, superior vena characterizing pulmonary hemodynamics and guiding the initiation of
cava; VSD, ventricular septal defect. pulmonary vasodilator therapy. Follow-up scans to assess treatment
*Sidedness should be re-confirmed at the time of PDA surgical response and plan long-term care are essential.
ligation or percutaneous device closure.
Recommendations
etiology of dysfunction and contribution of loading conditions vs Infants with confirmed or suspected DS should undergo
intrinsic contractility.115 standard TNE assessment soon after delivery to assess
structural integrity, presence of PH, and adequacy of
Recommendations
myocardial performance. Postdischarge follow-up is rec-
Infants with TTTS, regardless of antenatal treatment ommended and should be determined on the basis of
with SLPCV, should undergo standard TNE assessments the initial findings.
to identify pulmonary or systemic hemodynamics,
characterize loading conditions and assess myocardial
performance. Clinical Scenario–Based Screening
Neonatal Hypotension. Definition and Scope of the Problem.
Infants with Down Syndrome. Pathophysiology and Mecha- Mean BP is the most common clinical parameter used to character-
nistic Phenotypes. Down syndrome (DS) is the most common chro- ized systemic hypotension, and is used as a surrogate of end-organ
mosomal abnormality of infants with a global incidence of 1 in perfusion to guide intervention.127 However, there is increasing
Journal of the American Society of Echocardiography McNamara et al 197
Volume 37 Number 2

Table 5 Disease-based screening and key measurements

Condition Principles of assessment Key measurements

PDA  Evaluate PDA characteristics  PDA size, pressure gradient, and shunt direction
 Identify pulmonary overcirculation  Pulmonary vein diastolic wave Vmax, LA/Ao, LVO
 Measure systemic hypoperfusion (or LVO/RVO), E/A ratio, IVRT, LVEDD
 Rule out moderate-severe RV or LV  Postductal aortic, celiac and middle cerebral
systolic dysfunction artery diastolic flow (absent, reversed)
 Rule out CHD and ductal-dependent  LVEF/RV FAC/TAPSE
lesions
PH/acute hypoxemia  Appraise pulmonary hemodynamics  PDA flow direction, peak TR jet velocity, PAATi or
(differentiate flow-driven from resistance- RVET/PAAT ratio, LV end-systolic EI, pulmonary
driven PH) vein systolic/diastolic velocity
 Assess RV systolic and diastolic function  RV FAC; TAPSE; RVO; DTI s0 , e0 , a0 (consider
 Assess LV systolic and diastolic function strain/SR)
 Exclude CHD and ductal-dependent  LVEF; LVO, E/A ratio; IVRT; DTI s0 , e0 , a0
lesions  Pulmonary vein Doppler in chronic PH
Systemic hypotension  Assess LV and RV systolic function  LVEF, LVO, IVRT, RV FAC, TAPSE, RVO
 Assessment of preload/afterload  IVC collapsibility, tissue Doppler–measured
 Characterize intra- and extracardiac systolic and diastolic time intervals
shunts  PFO, VSD, PDA assessment (see above)
 Assess LV and RV morphology  LVPWd and IVSd (M-mode imaging of LV)
 Rule out CHD  Images/views to exclude obstructive left heart
disease including coarctation are critical
HIE  Assess RV systolic function  RV FAC; TAPSE; RVO; DTI s0 , e0 , a0 (consider RV
 Assess LV systolic and diastolic function free wall strain)
 Assess pulmonary hemodynamics  LVEF; LVO; E/A ratio; IVRT; DTI s0 , e0 , a0 (consider
 Rule out CHD strain/SR)
 PDA flow direction, peak TR jet velocity, PAATi or
RVET/PAAT, LV end-systolic EI, pulmonary vein
peak systolic/diastolic velocity
 Images/views to exclude obstructive left heart
disease including coarctation are critical
IDM  Assessment of LV hypertrophy  LVPWd and IVSd (M-mode imaging of LV)
 Quantify obstructive left heart disease  LVOT flow velocity (continuous-wave Doppler)
 Appraise LV diastolic function  RV morphology (subjective appraisal)
 Assess pulmonary hemodynamics  LV E/A ratio, IVRT
 PDA flow direction, peak TR jet velocity, PAATi or
RVET/PAAT, LV end-systolic EI, pulmonary vein
peak systolic/diastolic velocity
TTTS  Cardiomyopathy/hypertrophy in recipient  LVPWd and IVSd (M-mode imaging of LV)
 Assess LV diastolic dysfunction in donor  LV E/A ratio, IVRT
 Assess pulmonary hemodynamics  PDA flow direction, peak TR jet velocity, PAATi or
 Valvular disease, especially pulmonary RVET/PAAT, LV end-systolic EI, pulmonary vein
stenosis peak systolic/diastolic velocity
 RV morphology (subjective appraisal)
 RVOT and LVOT flow velocity; requires serial
assessments
DS  Characterize CHD  Focus on interatrial/interventricular septal
 Assess pulmonary hemodynamics defects
 Assess LV and RV function  PDA flow direction, peak TR jet velocity, PAATi or
RVET/PAAT, LV end-systolic EI, pulmonary vein
peak systolic/diastolic velocity
 LVEF; LVO; E/A ratio; IVRT; DTI s0 , e0 , a0 (consider
strain/SR)
 RV FAC; TAPSE; RVO; DTI s0 , e0 , a0 (consider RV
free wall strain)

Ao, Aorta; DTI, Doppler tissue imaging; IVC, inferior vena cava; IVSd, interventricular septal wall thickness at end-diastole; LVEDD, LV end-diastolic
dimension; LVOT, LVOT outflow tract; LVPWd, LV posterior wall thickness at end-diastole; PAATi, pulmonary artery acceleration time corrected for
heart rate; PFO, patent foramen ovale; RVOT, right ventricular outflow tract; SR, strain rate; Vmax, peak velocity; VSD, ventricular septal defect.
198 McNamara et al Journal of the American Society of Echocardiography
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Table 6 Knowledge elements for training in neonatal hemodynamics and TNE

Domain Specific knowledge elements

1. Cardiovascular anatomy and 1. Normal and abnormal structure of the heart


physiology 2. Components and determinants of cardiac output
a. Determinants of preload, contractility, and afterload
b. Frank-Starling, stress-velocity, and force-frequency relationships
c. Systemic vascular function curves
d. Ventricular pressure-volume loops
3. Myocardial oxygen supply and demand
4. Physiology of intra- and extracardiac shunts
5. Peripheral circulation
a. BP and volume, including neuro-hormonal control, cardiac reflexes, and
baroreceptors
b. Mixed venous oxygen saturation and the relationship of venous oxygenation
and cellular metabolism
c. Fick principle and applications to mixed venous oxygen saturation
6. Regional circulation
a. Starling forces and fluid exchange in the microcirculation
b. Systemic and cerebral autoregulation in preterm and term neonates
2. Pulmonary physiology 1. Physiology of the pulmonary circulation in neonates
a. Normal transition from fetal to postnatal life including physiology of the
normal postnatal increase in pulmonary blood flow
b. Pathophysiology of impairment in postnatal pulmonary blood flow and
potential therapeutic targets
2. Influence of positive pressure ventilation on systemic and pulmonary
hemodynamics
3. Disease states: etiology and 1. PDA in preterm neonates, including post-PDA closure syndrome
pathophysiology 2. Shock (all types)
3. Acute PH secondary to
a. Parenchymal lung disease, including pulmonary hypoplasia
b. Pulmonary venous hypertension, including LV diastolic and/or systolic
dysfunction
c. Lesions with increased pulmonary blood flow, including cardiac shunts and
arteriovenous malformations
d. Idiopathic PA hypertension
4. Chronic PH, including due to left heart disease, pulmonary disease, or
increased pulmonary blood flow from cardiac shunts
5. Pericardial effusion and tamponade
6. Hemodynamic consequences of perinatal and postnatal HIE
7. Systemic hypertension and hypotension
4. Diagnostics and monitoring 1. Laboratory
a. Biochemical measures of end-organ perfusion
b. Biomarkers of cardiac volume and pressure loading
2. Non-sonographic measurements of cardiac output, including bioimpedance-
and bioreactance-based tools
3. Invasive catheter measurements, including central venous catheterization and
diagnostic and therapeutic cardiac catheterizations
4. Near-infrared spectroscopy
5. Principles of echocardiography in the 1. Biologic effects and safety of echocardiography
neonate 2. Principles of physics (including equations) and instrumentation of
echocardiography, including M-mode, two-dimensional, and blood and tissue
Doppler echocardiography
3. Indications, strengths, limitations, and clinical utility of transthoracic
echocardiography
4. Common ultrasound artifacts and their identifying echocardiographic features
5. Echocardiographic appearance and normal variants of cardiac structures,
including cardiac chambers, valves, pericardium, and major blood vessels
6. Echocardiographic appearance of abnormal cardiac structures and cardiac
function in disease states
7. Appearance and positioning of central arterial and venous catheters
(Continued )
Journal of the American Society of Echocardiography McNamara et al 199
Volume 37 Number 2

Table 6 (Continued )
Domain Specific knowledge elements

8. Maturity-based normative data for echocardiographic indices of cardiac


function in healthy neonates and within the spectrum of hemodynamic
disturbance
9. Diagnostic test characteristics of echocardiographic measurements, including
reliability, reproducibility, and measures of predictive accuracy
6. Therapeutics 1. Mechanism of action and indications for common hemodynamic treatments
including
a. Inotropic medications
b. Vasopressor medications
c. Systemic vasodilator medications
d. Prostaglandins
e. Pulmonary vasodilator medications (including inhaled nitric oxide)
2. Volume expanders
3. Diuretics
4. Management of PDA, including indications for and selection of conservative
management, medications, and device or surgical closure
5. Cardiopulmonary interactions and titration of mechanical ventilation and other
methods of respiratory support in the neonate with hemodynamic instability

recognition that BP, as the dependent variable defining organ perfu- sessments should include measures of areas (end-systolic and end-
sion, is only one of the end points of interest. Furthermore, there diastolic), cavity dimensions at the base, midcavity, and length of
are several recognized limitations with the use of BP to monitor the right ventricle from the apex to the middle of the base in the
and treat low–blood flow states in preterm and term infants, including RV-focused apical four-chamber view. RV outflow dimensions can
the lack of robust normative data sets,127 dissociation between BP and be obtained from either the parasternal long-axis or short-axis view
SBF,42 and overreliance on singular estimates of mean BP rather than to assess the proximal and distal components of the RV outflow tract.
systolic and diastolic BPs separately.128 The relationship between hy- For the left ventricle, morphologic assessment should include relative
potension, cerebral perfusion, and adverse neurodevelopmental wall thickness, LV mass, volume, and linear dimensions.
sequelae is therefore questionable. Characterization of the hemodynamic significance of a PDA is essen-
Neonatal sepsis is a common cause of both compensated (normal tial because of its contribution to transitional systemic hypotension.
BP) and uncompensated shock. Sepsis affects myocardial contractility
either directly or through ventricular-ventricular interaction with the Recommendations
left ventricle, contributing to further deterioration. Vasodilatory shock
and capillary leak may both be present in sepsis and contribute to low Standard TNE might provide additional diagnostic infor-
RV preload and low pulmonary blood flow which may mimic acute mation regarding causality and guide medical manage-
PH physiology.129 A similar pattern is observed in necrotizing entero- ment in hypotensive neonates or those with suspected
colitis, which can also result in vasoactive shock attributable to the low–cardiac output state. TNE should be considered in
release of cytokines and the alteration in endothelial function. in any neonate who presents with sepsis-like symptoms,
especially in the setting of a known maternal viral pro-
Indications for Echocardiography. In any neonate presenting with drome. These infants should be serially monitored for
signs of hypotension and clinical signs of a low–cardiac output state cardiomyopathy, arrhythmias, and potential circulatory
(especially in premature infants during the transitional period), stan- collapse.
dard TNE should be considered to characterize the underlying phys-
iology and recognize deviations from normal anatomy. In the absence
of structural heart disease, standard TNE can be used in the manage- The Hypoxemic Infant. Scope of the Problem and Differential
ment of hypotension and shock because it can be helpful in identi- Diagnosis. Neonatal hypoxemia can result in decreased tissue oxygen
fying the underlying mechanisms. delivery and metabolic acidosis. Hypoxemia can result from multiple
etiologies, including intracardiac right to left shunts and/or intrapulmo-
Imaging Techniques and Guidance of Clinical Decision-Making. nary shunt secondary to ventilation-perfusion mismatch.131 The differ-
Assessment of myocardial performance in neonates with low systolic ential diagnosis for hypoxemia in an infant includes CHD with
BP should include evaluation of left and right heart function and compromised pulmonary blood flow; lung disease due to conditions
morphology (Table 5). TNE can be used to measure cardiac output such as pneumonia, pneumothorax, and meconium aspiration syn-
in critically ill newborn infants,16-18,130 which may enhance the inter- drome; and acute PH due to sepsis, HIE, CDH, or diagnoses such as
pretation of BP. The calculations of RVO and LVO provide estimates trisomy 21 and IDM. In any neonate with hypoxemia, it is imperative
of pulmonary and SBF, respectively; however, in the presence of fetal to rule out CHD, especially conditions which require acute interven-
shunts, measurements of RVO and LVO are not a direct estimate of tion (e.g., transposition of the great vessels with intact atrial septum, ob-
pulmonary or SBF. Morphologic measures can provide diagnostic structed pulmonary venous return). In a structurally normal heart, TNE
clues for evidence of hypertrophy and dilation associated with cardio- evaluation of patients with presumed PH may aid quantification of the
myopathies unrelated to CHD.95 For the right ventricle, structural as- severity of PH, appraisal of RV function, and the adequacy of
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pulmonary and systemic hemodynamics. If no PDA is present, RV fail- tion, a congenital brain arteriovenous fistula, can lead to high-output
ure may result, as well as LV diastolic dysfunction through ventricular- cardiac failure in the neonate, with high morbidity and mortality.53 Of
ventricular interactions. In addition, exposure to prolonged hypoxia note, patients with vein of Galen malformation can develop PH which
may contribute to LV diastolic dysfunction. may either be due to elevated PVR secondary to pulmonary vascular
remodeling (‘‘resistance-driven physiology’’; pulmonary vasodilators
Role of Echocardiography in Aiding Clinical Assessment. are beneficial) or due to pulmonary over circulation with systemic
Standard TNE is indicated to differentiate the etiology of hypoxemia steal (‘‘flow-driven physiology’’; pulmonary vasodilators are
and to direct management in a patient with a structurally normal harmful).54 TNE may enable differentiation of the specific pheno-
heart. If standard TNE is the first patient evaluation it should include type, allowing treatment guidance.
the essential views and sweeps to rule out the presence of CHD.
Infants with cyanotic CHD will be hypoxemic but will not neces- Role of Echocardiography in Aiding Clinical Assessment. In pa-
sarily be hypoxic, and will continue to be closely monitored for signs tients with congenital anomalies, standard TNE with the essential
of suboptimal tissue oxygenation. Longitudinal TNE evaluation may views and sweeps can aid the identification of associated CHD or
be useful to monitor changes in PAP, RV or LV function, and indices pulmonary vascular disorders. If neonatologist-performed TNE is
of pulmonary or SBF after therapeutic intervention. the first patient evaluation, the study should be reviewed by a pedi-
atric cardiologist or a comprehensive pediatric echocardiography
Role of Echocardiography in Guiding Therapeutic Interventions. study should be obtained afterward to appraise the anatomy. In a
Once the etiology of hypoxemia is determined, echocardiography structurally normal heart, TNE augments bedside assessment to
can guide initial intervention as well as provide longitudinal assess- identify pathologic hemodynamic states such as PH and ventricular
ment as physiology changes. Assessment of PH by echocardiogra- dysfunction.
phy includes assessment of pulmonary pressures and PVR,
presence and direction of atrial and ductal shunt, as well as RV Role of Echocardiography in Guiding Therapeutic Interventions.
and LV functional assessment (Table 5). Elevated right-sided pres- TNE aids characterization of PH in malformations such as CDH, om-
sures can be evaluated through measurement of TR to estimate phalocele, and vein of Galen malformation and guides therapeutic in-
RV systolic pressure, PI jet to estimate mean PAP, and systolic EI terventions. Early screening echocardiography may be helpful in the
> 1.33 Systolic time intervals of the right ventricle may provide valu- CDH population because of the increased use of ECMO in patients
able information on PVR, with emerging literature on measure- with ventricular dysfunction.52 Longitudinal TNE assessment may
ments such as PAAT.62,132 Finally, measurements such as TAPSE allow disease or physiology specific changes in management that posi-
or RV FAC, myocardial performance index, MV inflow ratios, tively impact the hospital course. TNE evaluation of patients with
LVO, and RV and LV strain may enhance diagnostic capabilities. CDH, omphalocele, and vein of Galen should include assessment
Shunting patterns may aid diagnostic appraisal, especially in patients of PAP, biventricular size and function, arch anatomy, as well as the
with CDH; specifically, atrial shunting is reflective of RV and LV presence of shunts to appraise the severity of PH and its impact on
compliance, whereas a right-to-left or bidirectional (>30% right-to- heart function and systemic/pulmonary hemodynamics. PH is more
left) PDA shunt suggests elevated PAP. pronounced in patients with giant omphalocele (defects >5 cm and
including the liver) with higher mortality in patients with RV dysfunc-
tion on initial echocardiography.134 Thus, initial, and subsequent
Recommendation echocardiography should include assessment of pulmonary pressures
and RV function. In vein of Galen malformation, evaluation of LVO
In a patient with hypoxemia, standard TNE is important
and RVO is essential to ensure differentiation of ‘‘resistance’’ and
to facilitate diagnostic appraisal, guide the institution of
‘‘flow-driven’’ phenotypes because of the need for individualized
pulmonary vasodilators, inotropes or vasoactive agents,
treatment and the need for judiciousness in the use of selective pul-
and enable rapid triage of patients for pediatric cardiol-
monary vasodilators.
ogy review when anatomic abnormalities are unexpect-
edly identified. Longitudinal hemodynamic assessment Recommendation
with echocardiography can guide monitoring and
refinement of therapeutic intervention. Standard TNE, which includes the essential views and
sweeps to enable exclusion of major CHDs, should be
performed in patients with CDH, omphalocele, and
Noncardiac Congenital Anomalies. Definition and Scope of vein of Galen malformation. Longitudinal hemody-
the Problem. Noncardiac congenital anomalies comprise $10% of namic assessment through TNE can aid in characterizing
NICU admissions and can be categorized as malformations, deforma- the underlying physiology, defining phenotypes and
tions, and disruptions.133 Patients with congenital anomalies repre- guiding therapeutic intervention.
sent a subgroup with high rate of morbidity and mortality.51 There
is increasing evidence that patients with CDH, occurring in approxi-
mately 1 in 3,000 live births, may have a variable underlying pheno- Central Lines and ECMO Cannulation. Scope of the Prob-
type that affects morbidity and mortality; specifically, pulmonary lem. Infants in the NICU often need central access in the form of um-
hypoplasia with varying degrees of PH and RV dysfunction or LV hy- bilical venous catheters (UVCs) or umbilical arterial catheters or
poplasia leading to pulmonary venous hypertension have both been peripherally inserted central catheters (PICCs). Placement of PICC
reported.52 Early differentiation of the specific phenotype is impor- lines under ultrasound guidance has been strongly recommended
tant to ensure the basis for treatment is based on the underlying phys- for adults and children to reduce complications and increase proce-
iology. In patients with omphalocele, echocardiography aids the dural success.135 In addition, ultrasound is now increasingly being
identification of associated CHD and/or PH. Vein of Galen malforma- used to both place and confirm the UVC tip position. This has been
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Table 7 Enabling competencies for the performance of TNE

Subtask Enabling competencies

1. Preparation 1. Prioritization/urgency of performance


2. Knowledge of machine operation, including selection of probes and techniques for image
optimization
2. Optimize physical 1. Consider patient temperature, infection prevention, stability, minimal handling, operator skill and/or
environment experience, and limited scan time
2. Recommend single use gel packets to minimize infection risk, especially in extremely premature
babies
3. Give appropriate attention to the neonate’s comfort
3. Image acquisition 1. Select the appropriate diagnostic mode (2D, color Doppler, pulsed-wave Doppler, continuous-wave
Doppler, M-mode imaging) and scanning protocol
2. Optimize image acquisition
3. Identify cardiac structures, including cardiac chambers, valves, pericardium, major blood vessels
and indwelling catheters
4. Measurement and analysis 1. Perform measurements and calculations
2. Use computer applications and postprocessing tools to optimize imaging analysis
5. Interpretation and report 1. Interpret measurements using appropriate normative ranges
2. Produce a comprehensive written report of the echocardiogram taking into account the complete
context of the patient

2D, Two-dimensional.

shown to be more accurate and reliable than the conventionally used the effusion does not always align with the degree of hemodynamic
chest radiographs.30,136 Similarly, ECMO cannulation can be significance; rather, rate of accumulation of the effusion may be a
routinely performed under ultrasound guidance, and ultrasound or more important determinant. Dilation of the inferior cava, RV dia-
echocardiography is a reliable and readily available modality to stolic collapse, and variability in atrioventricular valve inflow are addi-
confirm ECMO cannula tip position.137 tional signs of hemodynamic significance.

Guidance on Clinical Decision-Making. Ultrasound is the best mo- Recommendations


dality to assess the accuracy of UVC and PICC line tip position: it is
safe, reliable, noninvasive, and readily available and minimizes the Pericardial effusion should be ruled out in any infant
use of ionizing radiation. cPOCUS or TNE should be used to confirm with sudden unexplained deterioration with hemody-
tip position of UVCs, PICC lines, and ECMO cannulas. namic compromise, especially when a central line is in
situ. This should be differentiated from pleural effusion.
Recommendations Pericardiocentesis should be performed under ultra-
sound guidance, when the pericardial effusion results
cPOCUS or TNE should be routinely used to confirm
in hemodynamic compromise or tamponade physi-
UVC tip position after placement. Given the significant
ology, or occasionally for diagnostic purposes.
risk for migration with UVCs, it warrants surveillance
imaging (while the line is in situ) to determine the accu-
racy of the tip position and thrombus formation. Simi-
larly, PICC lines and ECMO cannulation should be TNE: TRAINING AND ACCREDITATION
performed under image guidance. When TNE is per-
formed for other indications, all imaging protocols Guidelines for training in TNE have historically been derived through
should include appraisal of indwelling catheters (where comparisons with, and extrapolations from, guidelines for training in
feasible) to document correct positioning. pediatric or adult echocardiography. Over the past decade, formal,
structured training and clinical programs in neonatal hemodynamics
have been developed that provide the foundation for accreditation
Screening for Pericardial Effusion. Scope of the Problem. of this subspecialty by regulatory bodies.2
Pericardial effusion is not uncommon in infants being treated in the
NICU. It could be part of the disease process, a sequela to cardiac sur- Previously Published Guidelines for Training in TNE
gery or a complication of central lines or catheters. Although most in-
fants tolerate mild to moderate pericardial effusion well, some may The previously published (2011) guidelines on TNE covered the indi-
have hemodynamic compromise, especially if the rate of accumula- cations for TNE, technical aspects, specific neonatal conditions and
tion is rapid, leading to cardiac tamponade. training requirements.8 In the United Kingdom, an expert consensus
statement was published in 2016 with representation from both
Guidance on Clinical Decision-Making. cPOCUS or standard TNE neonatology and pediatric cardiology.5 Implementation of both
should be routinely used to evaluate infants with suspected pericar- guidelines has proven to be challenging at some centers because of
dial effusion or tamponade physiology or those with sudden unex- the needs for training within a tertiary pediatric cardiology center.
plained deterioration with hemodynamic compromise. The size of In addition, concerns have been raised that there was an
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Table 8 Enabling competencies for the provision of NHTNE consultation to the NICU

Subtask Enabling competencies

1. Perform a patient-centered clinical 1. Determine the question to be answered by TNE consultation


assessment and establish a 2. Elicit a history, perform a physical examination, select appropriate investigations,
management plan and interpret the results for the purpose of diagnosis and management, disease
prevention, and health promotion
a. Perform TNE for the evaluation of
i. PDA, including post-PDA closure syndrome
ii. Hypotension or shock
iii. Suspected acute PH due to (1) parenchymal lung disease, including
pulmonary hypoplasia; (2) pulmonary venous hypertension; (3) lesions with
increased pulmonary blood flow; (4) idiopathic PA hypertension
iv. Suspected chronic PH
v. Pericardial effusion or tamponade
vi. HIE
vii. Systemic hypertension
viii. Position of central arterial or venous catheters
b. Integrate echocardiography findings with the clinical assessment and findings
of other hemodynamic studies and monitoring data
c. Formulate a differential and most likely diagnosis on the basis of relevant
findings
3. Establish a patient- and family-centered management plan
a. Integrate knowledge of neonatal hemodynamics, cardiovascular anatomy, and
imaging findings to provide consultative advice specific to the underlying
indication:
b. Provide recommendations for patient management, which may include
i. Initiation or titration of inotropic, vasopressor, or vasodilator medications,
prostaglandins, diuretics, or volume expanders
ii. Weaning and discontinuing medications
iii. Management of PDA (among preterm neonates), including conservative,
pharmacologic, and procedural/surgical closure
iv. Titration of respiratory support
2. Establish plans for ongoing care and, 1. Implement a patient-centered care plan that supports ongoing care, follow-up on
when appropriate, timely consultation investigations, response to treatment, and further consultation
a. Provide recommendations for the timing of TNE reassessment
b. Determine the need for and the timing of consultation with the pediatric
cardiology service
3. Multidisciplinary communication and 1. Actively contribute, as an individual and as a member of a team providing care, to
collaboration the continuous improvement of health care quality and patient safety
a. Recognize and respond to harm from health care delivery, including patient
safety incidents
b. Adopt strategies that promote patient safety and address human and system
factors
2. Communicate using a patient-centered and family-integrated approach that
encourages trust and is characterized by empathy, respect, and compassion
3. Participate, in the role of TNE consultant, in the sharing of health care information
and plans with families
4. Work effectively with physicians and other colleagues in the health care professions
a. Establish and maintain positive relationships and engage in respectful shared
decision-making with physicians and other colleagues in the health care
professions to support relationship-centered collaborative care
b. Negotiate overlapping and shared responsibilities with physicians and other
colleagues in the health care professions in episodic and ongoing care
c. Convey information from the TNE assessment to the referring physician in a
manner that enhances patient management
d. Work within the boundaries of the consultant role
5. Hand over the care of a patient to another TNE practitioner to facilitate continuity of
safe patient care
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Table 9 Enabling competencies for management of an NHTNE service

Subtask Enabling competencies

1. Equipment maintenance 1. Demonstrate an understanding of the factors affecting the lifetime of equipment and
recognize the need for replacement or additional equipment
2. Demonstrate an understanding of the selection of equipment and process of equipment
acquisition
2. Reporting and image archiving 1. Document and share written and electronic information about the medical encounter to
optimize clinical decision-making, patient safety, confidentiality, and privacy
a. Provide image capture and imaging documentation to facilitate reference to previous or
subsequent imaging
b. Store images that provide support for the diagnosis, treatment plan, and differential
diagnosis for the presenting symptoms and findings
c. Develop a written report, using appropriate terminology, summarizing all of the salient
positive and negative echocardiographic findings
d. Provide written clinical conclusions, integrating imaging and clinical data
3. Quality assurance 1. Contribute to the improvement of health care delivery in teams, organizations, and systems
a. Apply the science of quality improvement to contribute to improving the TNE system of
patient care
b. Participate in systemic quality process evaluation and improvement
c. Analyze patient safety incidents to enhance systems of care
2. Develop, implement, and maintain a quality assurance program for TNE
3. Participate in peer assessment and standard setting through the promotion of quality
assurance by discussing TNE studies and reports with other physicians, pediatric
cardiologists, and sonographers
4. Leadership in health care systems 1. Establish an effective collaborative model of care with the pediatric echocardiography
laboratory and/or medical imaging department, including mechanisms for ongoing dialogue,
shared imaging protocols, and clinical care strategies
2. Develop institutional policies and/or guidelines regarding the hemodynamic evaluation and
management of neonates and use of targeted echocardiography
3. Apply knowledge of health care financing, including physician remuneration, budgeting, and
organizational funding
4. Develop and rationalize NICU policy and infrastructure to support the process of referrals for
TNE consultation
a. Identify indications for TNE consultation and guidelines regarding the timeliness of
consultation
b. Demonstrate an understanding of the clinical and administrative infrastructure for
requesting TNE consultation and accessing TNE consultation reports and
recommendations

overemphasis on the training needs to exclude CHD, rather than di- and skill acquisition, and standards of accreditation for training pro-
recting the training toward meeting the specific competencies needed grams in NHTNE. The duration of training is 1 year, and successful
for each neonatal hemodynamic indication. completion is dependent upon acquisition of a comprehensive set
The Australasian Society for Ultrasound in Medicine guidelines for of competencies for clinical practice, without a prespecified minimum
clinician-performed cardiac ultrasound mandate training at an number of echocardiography studies performed.139 Currently, there
approved neonatal center only. The duration of basic training is is no nationally approved accreditation mechanism in the United
shorter and involves completion of a logbook of 50 echocardiograms, States. At some centers (e.g., the University of Iowa) the infrastructure,
which includes a requirement to ‘‘competently record a series of im- educational curriculum, and evaluative methods used for hemody-
ages to clearly demonstrate normal cardiac anatomy.’’ There is an op- namic training has received local graduate medical education office
tion for more advanced training including recognition of different accreditation. There are an insufficient number of programs presently
forms of CHD by echocardiography.138 to request Accreditation Council for Graduate Medical Education
approval, but this should be a long-term goal.
North American Guidelines and Accreditation: Neonatal He-
modynamics and TNE. In 2022, the Royal College of Physicians European Guidelines on NPE. In 2015, a European special inter-
and Surgeons of Canada established neonatal hemodynamics and est group was convened under the auspices of the European Society
TNE (NHTNE) as an area of focused competence diploma, represent- for Paediatric Research to produce a consensus statement on the
ing the first recognition of formal training of the subspecialty by a na- training requirements for NPE in Europe, because of the aforemen-
tional accrediting body. The prescribed training pathway included tioned challenges in implementing the 2011 TNE guidelines.6 The
guidelines specifying a comprehensive set of competency training re- group defined the training facilities and infrastructure necessary for
quirements (comprising knowledge elements and training experi- optimal training conditions in a European context and discussed
ences), elements of a portfolio for documentation of experiences some practical aspects including a suggested governance structure
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Table 10 Research opportunities and priorities for TNE and cPOCUS in neonates

Section and focus areas Research opportunities and priorities

Rationale for TNE  Develop high-quality evidence for the use of TNE to provide enhanced mechanistic insights
into common neonatal hemodynamic disease states and determine thresholds for
intervention.
 Develop prospective studies and clinical trials to characterize the impact of TNE-guided
care (monitoring, treatment) on patient outcomes.
Rationale for cPOCUS  Study the diagnostic reliability and safety of cPOCUS evaluations vs gold standard TNE or
pediatric cardiology evaluations.
 Develop high-quality evidence for use of cPOCUS-guided care on patient outcomes.
Image optimization and measurement  Develop standardized criteria for interpreting echocardiography data, including definitions
analyses of normal and abnormal findings.
 Design longer, time-to-event clinical trials to validate potential LV- and RV-based surrogate
end points that match the geometric shape, fiber orientation, and overall morphology with
estimations of contractility, quantification of function, and overall performance.
 Investigate imaging reliability and variance in measurements using commercial
echocardiography equipment.
 Improve the feasibility and reliability of deformation imaging, especially in preterm infants.
 Explore the use of artificial intelligence to optimize and potentially automate
echocardiography data analyses.
Use of TNE to guide care  Develop consensus on ‘‘physiologically acceptable’’ states of premature infants according
to maturation.
 Generation of robust data sets to define normative values with Z scores applicable for
neonates.
 Development and validation of standardized imaging protocols for neonatal hemodynamic
illnesses (e.g., PDA, PH [acute/chronic], RV/LV dysfunction, septic shock).
 Investigate and define disease-specific thresholds associated with adverse outcomes,
enabling intervention trials incorporating hemodynamic measures.
Training and accreditation  Generate evidence for adjudication and maintenance of competency for TNE and
cPOCUS.
 Study barriers to establishment of a high-functioning neonatal hemodynamics program.
 Investigate the impact of online platforms, simulation-based education.

for oversight of training and continued quality assurance (and the effectiveness of the educational techniques used and to allow
mandated a minimum of 200 echocardiograms spanning a 12- to ongoing program improvement.142
18-month period). Follow-up articles published in 2018 (https:// The entry point for training in TNE is typically after successfully
www.nature.com/collections/pjlqbgkmwk) set the framework for a completing a fellowship in neonatal-perinatal medicine, which may
sustainable governance structure with the responsibility to provide be 3 years in the United States or 2 years in Canada. Performance
accreditation to NPE in Europe.140 of TNE involves multiple skill components, including image acquisi-
tion, image interpretation, measurement performance, and clinical
Knowledge Elements and Competencies for Clinical application. For individuals developing skills in TNE, each of these
Practice in TNE components may be attained and assessed using different methods.
Assessment should be both formative (during the learning process al-
Training programs in TNE should facilitate the acquisition of founda-
lowing for improvement) and summative (occurring at the conclusion
tional knowledge (Table 6) and the development of competence in
of the learning process determining competence) with a clear mile-
the major tasks of the discipline, which include (1) performance of
stone map for the trainee.141 The recommended duration of training
neonatal echocardiography (Table 7), (2) consultation to the NICU
is 1 year, which is dedicated exclusively to learning the required com-
(Table 8), and (3) management of the TNE service (Table 9).
petencies for TNE and neonatal hemodynamics.
Experience at centers with established TNE training programs has
Proposed Training and Evaluation in TNE prompted the development of a timeline template for trainee acqui-
Contemporary medical education has shifted to a competency-based sition of the knowledge and skills that culminate in clinical compe-
approach for trainee skill evaluation. In contrast to prior paradigms of tency in the discipline. The specific timing, trajectory, and intensity
training, time spent in a particular clinical experience is insufficient to of training in the development of competency in performing the ma-
determine successful acquisition of skills.141 Similarly, individual jor tasks occurs in an ordered fashion, with individual variability
learners may require different numbers of educational experiences (Figure 27).
to become proficient at a skill. Therefore, objective measures of pro-
ficiency are necessary to determine an individual clinician’s level of Training and Evaluation in Image Acquisition and
competence. Evaluation of a program is also important to determine Analysis. Training in image acquisition and interpretation should
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Figure 27 Depiction of the timing, trajectory, and intensity of training in the development of competency in performing the major tasks
of the discipline of targeted neonatal echocardiography. The timing of greater intensity or focus of training is depicted by the shade of
the horizontal bars. Darker areas of the bar correspond to the approximate time period of increased time and greater intensity of
training committed to development and acquisition of the competency. Lighter areas of a bar correspond to the timing of an
increasing or decreasing emphasis on the task. The length of each bar depicts the timing and duration typically allocated to compe-
tency acquisition. For example, early training is dedicated predominantly to developing the skills for echocardiography analysis and
image acquisition, with increasing emphasis on integration of clinical and echocardiography data in the latter two thirds of the training
period.

include a curriculum comprising competencies for which progress The evaluation of competence in image acquisition ultimately re-
is evaluated and tracked through regular assessments and defined quires direct assessment of image acquisition and review of images
milestones (Supplemental Table 1). A registered pediatric cardiac obtained by the learner. Each trainee should achieve competency in
sonographer, pediatric cardiologist, or TNE neonatologist should each parameter or milestone before moving on to the next major
be designated as the educator in charge of the implementation phase of training.151 Formal assessments of image acquisition and
and monitoring of progress throughout the training course.143 interpretation should be performed at defined intervals throughout
the course of training.
Image Analysis and Measurement. A didactic component of the Trainees should develop competence in performing standard TNE
program that provides instruction in image interpretation could be that will allow the identification of deviations from normal cardiac
achieved using previously recorded images available locally or in pub- anatomy, prompting pediatric cardiology review.73 At the end of
lic or private repositories (e.g., a database of teaching cases).144 Image training, the trainee should be able to independently obtain all
interpretation can be assessed on the basis of the content of those im- standard imaging views, identify structural heart disease and interpret
ages which should include a variety of clinical conditions typically neonatal TNE for the defined indications.
encountered during the practice of TNE. Exposure to cases of
CHD, which may be seen less frequently, is essential to ensure the Individualization of Training. Curriculums individualized to the insti-
learner is able to identify deviations from normal anatomy and appro- tution should be developed for the trainee group and then further
priately refer to a pediatric cardiologist. Skills in measurement perfor- customized for each individual trainee to ensure competency in ob-
mance could also be assessed using an examination based on stored taining the desired outcome.152 A milestone map (Supplemental
images. These examinations may be created in a virtual self- Table 1) should be shared with the trainee at the initiation of training
administered environment or with an instructor present as an objec- and reassessed at regular intervals. Support and evaluative methods
tive structured clinical examination.145,146 for trainees should be developed and shared with the trainee and pre-
ceptor (Table 6). Customized interventions should be developed to
Image Acquisition. Preliminary skills in image acquisition may be help trainees who are not meeting their image acquisition or interpre-
developed and/or evaluated using a simulator, if available.147,148 tive assessment goals.152 A portfolio highlighting studies performed
Neonatal echocardiography simulators can be used to develop basic by the learner should be considered as a tool to demonstrate skills
echocardiography and imaging skills, especially in hand-eye coordina- in acquiring images from a variety of patients.153 Although there is
tion, in the early phase of learning. However, live image acquisition no evidence to support a specific number of studies that would be
with patients is one of the most critical components necessary to necessary to determine that a learner is competent, the portfolio
develop proficiency in TNE. When possible, most (>75%) of the should include a broad range of clinical situations to document the
hands-on training should be completed on neonates to allow targeted types of experiences encountered.
and efficient training. This will allow the trainee in TNE to become
familiar with normal cardiac anatomy and recognize abnormal pat- Duration of Training Focusing on Image Acquisition. Trainees
terns suggesting the presence of structural heart disease. To appropri- should expect that it will take a minimum of 2 months and up to
ately develop competence, image acquisition should be practiced 6 months to achieve the competencies in the image acquisition phase
under the supervision of an expert (Supplemental Table 2).149 This of the training.
permits real-time feedback and adjustment that will facilitate develop-
ment of these skills. Newer automated technologies may be able to Training and Evaluation in Neonatal Hemodynamics
provide some of the same feedback necessary for effective Evaluating a learner’s ability to synthesize clinical, laboratory and im-
learning.150 aging information and formulate therapeutic recommendations in
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different clinical situations can be performed in several ways  Creation of a proposal and business plan for development of a new TNE ser-
(Supplemental Table 3).154 When a local TNE expert serves as the vice
instructor, both the learner and instructor should review cases  Completion of a physics of ultrasonography course, or equivalent
together in an apprenticeship style, an intensive format in which the
instructor develops a good understanding of the learner’s ability. Exposure to CHD. Guidelines for TNE training are directed to-
Programs could also use case presentations and discussions to assess ward the evaluation of patients with hemodynamic concern
a learner’s understanding of the clinical application of TNE findings as when there is low concern for CHD. Nevertheless, it is important
well as to reinforce physiologic principles. For a more objective mea- for TNE practitioners to have the skills to recognize deviation from
surement of skill, an objective structured clinical examination could normal anatomy. Therefore, exposure to a variety of cases of CHD
be used to assess a learner’s ability to apply the TNE data to clinical that are representative of right-sided, left-sided, and mixing lesions
situations in a standardized format. preferably cases in the newborn period. This exposure may be pro-
Traditionally, case logs or portfolios have been used to quantify a vided during dedicated rotations in the pediatric echocardiography
learner’s clinical experiences. Although these are not sufficient to laboratory (e.g., minimum 1-2 months) at a tertiary center (where
assess a learner’s hands-on skill, they do document the breadth of feasible) or using hands-on simulators which have a range of cases
cases a learner has experienced and therefore remain valuable. As of CHD.
an individual begins independent practice, case logs remain a valuable
method of tracking experience and ongoing use of the skills previ-
Practice Eligibility Route for Neonatologists With TNE
ously acquired. They are also useful for leaders of programs to track
Expertise
the studies and case mix performed by all members of the program.
Training programs in TNE should develop a plan for objective A practice eligibility route is a path to recognition of competence and
learner assessment. The evaluation plan should include a combination certification in TNE and neonatal hemodynamics for physicians who
of direct observation by an experienced clinician allowing formative are current TNE practitioners but who are ineligible for traditional cre-
feedback, and documented examination of knowledge, image acqui- dentials review from regulatory bodies as a result of completing unac-
sition, image interpretation, measurement, and application skills. credited training or no formal training in the subspecialty.
Suggested methods of objectively assessing these components of Certification through the practice eligibility route is most frequently
TNE are detailed in Tables 8 and 9. undertaken by accrediting bodies.
Requirements for Practice Eligibility Route Certification in TNE.
Required Training Experiences. A .Practice duration: Minimum 4 years in independent clinical practice
B. Practice profile
a. Setting: Tertiary or quaternary NICU
 Performance of comprehensive transthoracic echocardiography in the NICU,
b. Medical professional activity: At least 30% of professional activity dedi-
including M-mode, two-dimensional, and Doppler echocardiography
cated to clinical care, research, or teaching in NHTNE
 Performance and interpretation of targeted echocardiography in a level III or
c. Case mix: On an annual basis, the hemodynamics consultations per-
IV NICU
formed (and TNE performed or reviewed) reflects the breadth and
 Acting in the role of TNE consultant in the management of critically ill ne-
severity of disease states and pathophysiology of cardiorespiratory dis-
onates
ease as outlined in the competency training requirements (Table 6)
 Participation in the quality assurance activities of the TNE program
C .Evidence of competence: Multisource feedback including
 Attendance and participation in local TNE rounds or conferences
a. Candidate
 Participation in teaching and assessment of other trainees, sonographers,
i. Case descriptions demonstrating appropriate performance of a he-
and physicians
modynamics consultation (with TNE) in the NICU
 Scholarly activity related to TNE, which may include a research, education,
ii. Demonstration of leadership in the field of neonatal hemodynamics
or quality improvement project
and how these accomplishments have advanced the field
The recommended minimum duration of training to achieve iii. Scholarly activities in the field of NHTNE
expertise in TNE is 12 months, or longitudinal equivalent. As high- iv. Activities to further the education of peers, trainees, or learners in
the field of NHTNE
lighted earlier, it is recommended that training is commenced after
b. Referees, including department head, other NHTNE neonatologists
successful completion of a fellowship in neonatal-perinatal medicine.
both within and external to candidate’s institution, and non-TNE neona-
This may include 2 to 4 months in a pediatric echocardiography lab- tologists familiar with the candidate’s work in the field
oratory (with access to a NICU) and the remainder within the NICU.
Trainees should successfully complete (acquisition and interpretation)
a minimum of 250 scans that span the entire range of indications Requirements for Training Programs in TNE and Neonatal
(Section 3) to be deemed competent in the performance in TNE. Hemodynamics
Recommended Training Experiences. Program Organization. Programs providing training in TNE
should have appropriate organizational structure, with leadership
 Performance and interpretation of tissue Doppler, two-dimensional strain
and administrative personnel to support the program, program direc-
echocardiography, and STE
tor, affiliated teachers, and trainees effectively. Program organization
 Review of an image library of neonatal echocardiography cases, including
those with critical CHD and oversight may be facilitated by a program committee of key stake-
 Participation in educational activities by attendance at regional, national, or holders, which may include representatives from pediatric cardiology
international conferences with significant discipline-related components and/or neonatal critical care as appropriate for the setting. Finally, pro-
 Participation in the management and administration of the TNE service grams should have a system of continuous improvement of the
 Participation in the review and/or revision of scanning protocols educational experiences of the trainees.
Journal of the American Society of Echocardiography McNamara et al 207
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Education Program: Teaching, Evaluation, and equipment, image archiving capability, administration, teaching, qual-
Remediation. The training program should be designed, to facilitate ity assurance, and methods for both formative and summative trainee
trainees’ development of the required competencies of the discipline. evaluation (see Section 4).
Programs should have developed and implemented a curriculum plan The intensity and duration of training in cPOCUS should be
that is designed, on the basis of the ASE guidelines for TNE, and commensurate with the complexity of the anticipated future scope
includes regularly scheduled formal teaching. Programs should also of clinical practice of the trainee. There is currently a lack of standard-
have an effective, organized system of trainee assessment that includes ization of training regarding the clinical scope of practice for cPOCUS
a logbook for tracking of the clinical encounters, case mix, learning in the NICU. Until a formalized training curriculum is developed,
reflections and acquisition of knowledge elements. Programs should competency goals should therefore be individualized and formalized
have a system of assessment that includes regular, standardized review at the outset of training.
of trainee progress and portfolio, mechanisms for trainee engagement Although conclusion of training in cPOCUS should be determined
in performance review and support for trainees whose trajectory in on the basis of demonstrated competence, the typically shorter dura-
attaining competencies falls below expectations. tion of training (relative to TNE) and associated reduced case-mix
Training programs should perform evaluations of the effectiveness exposure implies a potential organizational benefit of recommending
of the educational experience through multiple different levels of a minimum number of training experiences. At present, a minimum
assessment.142 Learner surveys can determine the level of satisfaction number of scans to determine competency cannot be justified on
with the program and seek feedback on ways to improve the pro- the basis of the currently available evidence for training in neonatal
gram. The program should also track efficiency of training techniques cPOCUS. However, given the breadth of imaging findings one could
by noting how many trainees complete the program and are consid- encounter, a minimum of 75 performed scans (minimum of 25 for
ered competent at the conclusion. The program can also consider line and heart function evaluation) is recommended as a supplement
tracking how graduates use the skills in the future as a measure of to a competency-based portfolio, commensurate with recommenda-
effectiveness of the program. Finally, the ultimate measure of effec- tions for training in cPOCUS in adults.155 It must be highlighted that
tiveness of the program would be to determine the impact of this the actual number is an expert guide and should not replace the ‘‘in
training on patient care, though methodology to estimate this effect the moment’’ adjudication of competence in performing the required
is lacking. task. There is an urgent need to study the reliability or cPOCUS eval-
uations; in particular, the evaluation of line tip position may be partic-
Resources. Training programs in TNE should have the clinical, ularly challenging in some patients.
physical, technical, and financial resources to provide trainees with
the educational experiences needed to acquire all competencies.
The patient population should comprise preterm, extremely preterm, Quality Assurance for TNE and cPOCUS
and term neonates requiring neonatal intensive care. Training should All neonatal echocardiographers that have completed the advanced
occur at sites with clinical consultative services in NHTNE. Access to a training level in TNE should continue to perform/review a minimum
pediatric echocardiography suite and a pediatric cardiology consulta- of 100 echocardiographic studies per year to maintain their skills and
tion service is an asset, though may not be possible in all settings. The competence level. There are few data to inform a number for mainte-
program should have access to equipment capable of comprehensive nance of competency in cPOCUS, but a minimum of 50 studies per
neonatal echocardiography, systems for securely archiving and re- year is suggested. Maintenance of competence by regular participation
porting echocardiograms, and dedicated space and equipment for re- in echocardiographic conferences or training courses is strongly rec-
viewing and reporting echocardiograms. Finally, the program should ommended. A structured program for continued education should
have resources to facilitate training experiences across the breadth be developed. Crucial for both TNE and cPOCUS programs is that
and depth of cases, including simulation-based educational experi- they are organized according to current professional standards
ences in neonatal echocardiography, a teaching file of cases, or both. regarding image acquisition, image storage, and reporting. In hospitals
with pediatric echocardiography laboratories, this can best be
Instructors. In the program, instructors should appropriately imple-
achieved by integration of the TNE activity within the pediatric echo-
ment the curriculum, supervise, and assess trainees, contribute to the
cardiography laboratory. This includes standardization of imaging pro-
program, and model effective practices. The lead instructor (neonatol-
tocols, uniform reporting, and a single imaging archive within the same
ogist or pediatric cardiologist or codirectorship) in the program must
hospital. In hospitals in which direct access to pediatric
have demonstrated expertise in the discipline, including $3 years of
echocardiography laboratories is not available, the service should be
practice and/or completion of a formal period of TNE training. All
organized according to generally accepted standards for echocardiog-
other neonatology instructors should have completed formal TNE
raphy laboratories. This includes meeting operational standards
training and collaborating sonographers and/or pediatric sonogra-
(training of personnel, equipment, protocols, standards for storage,
phers should be familiar with the unique training requirements of
and reporting) as well as participation in quality improvement pro-
the neonatology TNE curriculum and the components of the stan-
cesses. Likewise, cPOCUS activity may be integrated either within
dardized TNE evaluation.
the TNE program and/or pediatric echocardiography. All echocardi-
ography (TNE or cPOCUS) studies should be recorded, and the im-
cPOCUS: Training Recommendations ages stored in a manner allowing immediate availability for review
Training in cPOCUS involves the acquisition of competence in a sub- and easy retrieval. The ultrasound systems must include the ability
set of the competencies required for clinical practice in TNE, often as to provide immediate playback with limited video degradation, stan-
a component of training alongside skill development for noncardiac dardized reports, and long-term storage. Digital storage is mandatory.
indications for ultrasound imaging in the neonate (e.g., cerebral hem- Reporting standards should comply with the recommendations of the
orrhage, vascular access). Like TNE, training in cPOCUS requires Intersocietal Commission for the Accreditation of Echocardiography
comparable program infrastructure, including quality sonographic Laboratories.156 In NICU programs with no direct access to pediatric
208 McNamara et al Journal of the American Society of Echocardiography
February 2024

cardiology services, telemedicine links between the NICU and the viewing by both neonatologists with TNE expertise and pediatric
central laboratory could be organized. It is possible to transmit com- cardiologists. Patients in whom TNE is performed may range in
plete digital echocardiograms rapidly over secure high bandwidth con- weight from 300 g to >5 kg, which requires the use of both
nections, including from high-volume level 3 NICUs.157 If TNE and phased-array and linear probes across a wide range of imaging fre-
cPOCUS are being performed in a facility without the availability of quencies (5-12 MHz).
a practitioner with at least advanced-level training in TNE as outlined
in this document, telemedicine capabilities should be considered a Recommendations
requirement. Each program should have a director who provides over-
sight to the operations, quality assurance and education. In addition, Programs in which standard TNE or cPOCUS assess-
there should be a structured program for review of clinical cases or ments are performed must have access to dedicated
archived studies, that occurs at least monthly. Wherever possible, the echocardiography machines, probes with a range suit-
establishment of joint educational rounds where neonatologists who able for neonatal studies, and a centralized storage sys-
perform TNE or cPOCUS evaluations, pediatric cardiologists, and so- tem that allows study retrieval and remote viewing.
nographers participate in review of challenging cases, archived imag-
ing studies, or new technologies should be welcomed.
Billing Considerations. Currently, there is no billing code which
Recommendation incorporates the diagnostic, interpretative and consultation elements
Programs in which standard TNE or cPOCUS assess- of the integrated TNE evaluation which is very work-intensive.
ments are performed must include a plan for ongoing Guidelines related to the scope of billing should be established based
quality assurance with oversight performed by an on local institutional standards.
appointed director. This should include regular review
of archived cases and challenging clinical dilemmas. Relationship Between Neonatal Hemodynamics Programs
and Pediatric Cardiology. Successful implementation of a hemo-
dynamics program requires close collaboration between neonatolo-
Program Establishment gists with TNE expertise and pediatric cardiologists. There are likely
Institutions should evaluate the need for cPOCUS and advanced to be differences in program setup among centers, according to pro-
neonatal hemodynamic assessments, ability to train learners, and pro- gram size, access to pediatric cardiology services, and the number of
cesses for quality assurance before program development. Common neonatologists with TNE training. First, all institutional policies for
to both cPOCUS and neonatal hemodynamics programs is the need TNE evaluation, including indications, should be mutually agreeable
for dedicated ultrasound equipment with the appropriate size and fre- and based on ASE (or equivalent) guidelines. These policies should
quency range of the probes, dedicated system for image storage, stan- stipulate those scenarios where pediatric cardiology consultation is
dardized reporting mechanism, and experienced program director. In advised (e.g., unexpected identification of CHD or persistent LV
addition, programs should develop clinical practice guidelines that dysfunction) and the required processes. The approach to interven-
clearly delineate the indications for either cPOCUS or TNE, scope tional PDA closure is an example of the importance of strong collabo-
of practice and the interface between the neonatal hemodynamics rative ties between the neonatologist with hemodynamics expertise
and pediatric cardiology services. As cPOCUS is only used to guide and the pediatric cardiology team, before, during and after interven-
decisions in a critical situation, these evaluations should be followed tion. Second, regular joint neonatal hemodynamics-pediatric cardiol-
by a comprehensive evaluation (TNE or pediatric echocardiography) ogy conferences to discuss clinical cases, ongoing research, or
within an agreed time frame specific to the institution. All TNE assess- scientific controversies are advisable. Third, a process for joint
ments should be completed by trained personnel that have morbidity and mortality discussion should be established. Fourth, a
completed formal training in TNE. At some centers it may be recom- rotation in neonatal hemodynamics for pediatric cardiology trainees
mended that the first echocardiogram be a comprehensive pediatric is advisable and, wherever possible, should be mandated to optimize
echocardiogram that is performed and reviewed by pediatric cardiol- exposure to neonatal hemodynamic problems. Fifth, research collab-
ogy staff. At other centers, the first assessment may be TNE either oration should be encouraged. Of note, much of the scientific
because pediatric cardiology services are not available or there is insti- advancement in the field of neonatal hemodynamics since the inau-
tutional agreement that TNE evaluation may be performed in patients gural guidelines for TNE has resulted from close collaboration be-
with low risk for structural heart disease. A process should be estab- tween neonatologists and pediatric cardiology imaging experts (e.g.,
lished to ensure these TNE studies are reviewed by a pediatric cardi- Toronto, Iowa). Finally, regular administrative meetings between the
ology expert to appraise the anatomy and/or arrange secondary director of the neonatal hemodynamics–TNE program and leaders
comprehensive pediatric echocardiography. Without governance, in pediatric cardiology is essential to discuss patient volumes, equip-
programs may develop in which universal access is prioritized over ment related matters, issues of quality, finances, and ongoing program
ensuring that practitioners have the necessary knowledge, expertise, challenges.
and critical volume of procedural exposure to optimize patient
care. This may contribute to diagnostic or therapeutic inaccuracy.
CONCLUSION
Equipment Standards. TNE programs require imaging equip-
ment which is designed for neonatal echocardiography and is dedi- Delineation of a clear boundary between the unique fields of
cated for exclusive NICU use. In addition, programs should have neonatal hemodynamics (primary expertise in physiology-based
access to a centralized storage system, which enables local or remote echocardiography) and pediatric cardiology (primary expertise in
Journal of the American Society of Echocardiography McNamara et al 209
Volume 37 Number 2

structural and functional echocardiography) has been clarified The following authors reported relationships with one or more
through published guidelines6,8 and supported through peer re- commercial interests: John Simpson, MB BCh, participated as a
viewed publication. This document provides clarification on the consultant for Canon Medical Systems and Philips Medical Systems.
scope of cPOCUS vs TNE to ensure that practitioners use these skills
in accordance with approved indications. The field of neonatal hemo-
dynamics, and the role of the neonatologist in performing echocardi-
ACKNOWLEDGMENTS
ography evaluations to delineate physiology, refine treatment, and
monitor response to intervention has flourished at centers at which
This document was reviewed by members of the 2023-2024 ASE
there is close collaboration between neonatologists with hemody-
Guidelines and Standards Committee, ASE Board of Directors, ASE
namic expertise and pediatric echocardiography laboratories.
Executive Committee, and designated reviewers (Piers Barker, MD,
Training programs should be structured to ensure trainees are
Kelly Boegle, ACS, RCCS, Lanqi Hua, ACS, RDCS, Jeff Jewell,
exposed to the broad range of patient sizes (<500 g to >5 kg) and he-
RDCS, Majd Makhoul, MD, David Orsinelli, MD, Anitha
modynamic scenarios in medical and surgical patients. Evaluation
Parthiban, MD, Alan Pearlman, MD, Andrew Pellet, PhD, RDCS,
should focus on the achievement of imaging and cognitive compe-
Gary Satou, MD, Kenan Stern, MD, and David H. Wiener, MD).
tencies, rather than an arbitrary number of assessments. The usual
The guidelines are dedicated to Dr Regan Giesinger, who
duration of training to reach competence in TNE should be at least
devoted her life to the field of neonatal hemodynamics but
1 year. Likewise, it is recommended that cPOCUS practitioners
who sadly passed away before their publication.
collaborate with local pediatric cardiology and/or neonatal hemody-
namic programs to support the establishment of training guidelines
and standards for practice to further enhance the care of neonates.
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214 McNamara et al Journal of the American Society of Echocardiography
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Supplemental Table 1 Example phased milestone map for NHTNE trainees

Phase 1 Phase 2 Phase 3 Phase 4

Basic principles of Advanced principles of Basic report writing Advanced report


echocardiography including echocardiography writing
ergonomics
Echocardiographic physics Basic CHD identification Advanced CHD identification
Operational processes Basic measurement performance Advanced measurement Advanced image
performance interpretation
Basic image acquisition Intermediate image acquisition Advanced image acquisition

Supplemental Table 2 Formative learning support for trainees in TNE

Support Description

Milestone map development Jointly developed with educator at the start of training
Preceptor training pods Avoid rotating trainee though a large number of sonographic preceptors
Training huddles and goal recalibration Periodic (weekly or monthly) team meetings to review and calibrate specific goals
Independent study assignments Identify and assign text or e-learning activities related to current phase of training
Technical assessments Direct observation of procedural skills (DOPS) assessment of image acquisition
on a neonate (live) or through use of a simulator
Interpretive assessments Observation of trainee interpretation of an echocardiogram; may be enhanced
with inclusion of clinical information and the formulation of a diagnostic evaluation
and therapeutic management plan

DOPS, Direct observation in practice study.


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Supplemental Table 3 Evaluation tools to assess learner competence in TNE

Skill Assessment tool Suggested use

Image interpretation OSCE Instructor provides standardized set of images with questions for learners
to answer.
Image acquisition Direct observation Instructor provides immediate feedback at the time of image acquisition.
Portfolio Instructor ensures adequate technique on standard set of views acquired
and provides postimaging feedback. Competence determined by need
for minimal feedback.
Measurement performance OSCE Learner performs measurements on images previously acquired. Findings
compared with instructor/standardized measurements.
Clinical application OSCE Learner reviews cases presented with clinical information and images
available and answers questions about management decisions.
Skill maintenance Case logs Track breadth of experiences over time.
Case conferences Regular discussion of clinical application with other TNE-skilled clinicians.
OSCE, Objective structured clinical assessment.

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