international perspectives

The Neonatologist as an
Echocardiographer
Alan M. Groves, MBChB, MRCPCH,* heart disease in newborns presenting
Carl A. Kuschel, MBChB, FRACP,† with cyanosis or a cardiac murmur.
Jonathan R. Skinner, MD, FRACP‡ This review describes the principal
applications of echocardiography by
the neonatologist, with some practi-
Introduction
cal guidance on method of assess-
When echocardiography is necessary
ment and interpretation of findings.
in the newborn period, it often is
needed urgently. (1) However, staff-
ing, organizational, and geographic Limitations on Practice
limitations make it unlikely for pedi- Echocardiography is highly user-
atric cardiologists to provide 24- dependent. (8) The primary concern
hour coverage for echocardiographic about neonatologists performing
assessment of sick newborns in most echocardiography has been the po-
Author Disclosure centers. (2) Pediatric cardiology ser- tential for misdiagnosis, particularly
vices also could be overwhelmed failure to recognize cardiac disease.
Drs Groves, Kuschel, and Skinner did
with requests for assessments of duc- There is scope for error in the diag-
not disclose any financial tal shunting in preterm infants who nosis of both structural and func-
relationships relevant to this article. have yet to develop clinical signs. (3) tional cardiac pathology. Although
In many parts of the world, litera- errors in assessment of structural car-
ture on the value of early hemody- diac disease usually become evident
namic assessment and increased ac- over time, cardiac function changes
cess to ultrasonography equipment dramatically during the neonatal pe-
with improving image quality has riod, so it is more difficult to assess
motivated neonatologists to develop whether interoperator discrepancies
echocardiographic skills. As a result, in functional assessment are due to
echocardiography is increasingly observer error or true functional
considered an integral component of change.
the assessment of the critically ill new- Some reports have suggested an
born. (2)(4)(5) An increasing number unacceptably high error rate in the
of neonatologists are undertaking as- diagnosis of structural cardiac disease
sessments of functional hemody- when scans are performed by person-
namic status by echocardiography, nel other than pediatric cardiologists.
with particular interest in volume of (8)(9) However, these assessed diag-
ductal shunt, (3) severity of persis- noses were made by radiologists and
tent pulmonary hypertension, (6) echocardiographers of adults rather
and detection of low systemic blood than neonatologists. (10)
flow. (7) In addition, some neonatol- Others have supported the prac-
ogists have assumed the responsibil- tice of neonatologists performing
ity of excluding structural congenital echocardiography with appropriate
cardiology support. (1)(2)(11) These
*Neonatal Specialist Registrar, Queen Charlotte’s opinions are supported by the results
and Chelsea Hospital, London, United Kingdom.
†
Neonatologist, Newborn Services, Auckland District of a prospective analysis in which
Health Board, Auckland, New Zealand. echocardiography performed by
‡
Paediatric Cardiologist, Paediatric Congenital and neonatologists showed minimal dis-
Cardiac Services, Starship Children’s Health,
Auckland District Health Board, Park Road, crepancies from that carried out by a
Auckland, New Zealand. pediatric cardiologist. (4)

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However, there is no excuse for

complacency. Errors can exact a high Guidelines for Safe Practice for
Table 1.
cost from the patient. For any neona-
tologist performing echocardiogra-
Neonatologists Performing
phy, a few guidelines for safe practice Echocardiography
are shown in Table 1.
Another concern for neonatolo- Training and Audit
gists performing echocardiography is ● Consider whether you will perform this procedure often enough to acquire
the handling involved, which poten- and maintain a basic set of skills.
● Attend a formal course in echocardiography and decide on the level of
tially conflicts with the principles of expertise you wish to acquire.
minimal handling of preterm infants. ● If you wish to diagnose heart disease accurately, seek a training fellowship
(12) When performed carefully, echo- or placement within a pediatric cardiac center.
cardiography can be undertaken in the ● When you begin practice, keep a log of your scans, and ask a mentor to
preterm infant without significantly discuss and audit your progress.
disturbing cardiorespiratory status. Examination
(13) However, the number and du- ● Perform a clinical examination prior to echocardiography. If the
ration of scans should be minimized echocardiographic findings do not fit the clinical picture, request a
and directed by clinical necessity. cardiology review. Always check the femoral or foot pulses.
● Refer any child who has suspected or confirmed structural congenital heart
disease for expert cardiology review.
Training/Teaching ● It may be safer to initiate prostaglandin therapy on the basis of clinical
As demand for the technique in- suspicion rather than delaying treatment while a noncardiologist performs
creases, familiarity with echocardiog- echocardiography.
raphy may become a recommended ● Do not hesitate to rescan with an open mind if an infant’s clinical signs
part of the training for neonatolo- unexpectedly persist or change.
● Record all scans, audit your performance, and reassess any diagnostic
gists, at least in Europe. However, at inaccuracies.
present we are not aware of any for- ● Know your limits: If you are not certain of the diagnosis, obtain a second
malized training process or accredita- opinion before altering management on the basis of the scan.
tion, despite suggestions that such Reporting Scans
guidelines are required. (2)(4)(5)
● Establish a standard pro forma (if you forget to check something, go back
A number of courses on pediatric
and check!). Title the report “Neonatal Cardiac or Hemodynamic
and neonatal echocardiography are Assessment” or similar to avoid the impression that pediatric cardiologist
available and serve as a useful intro- echocardiography has been performed, which could delay referral later for
duction to the technique. A textbook clinical signs of cardiac disease.
aimed exclusively at the neonatal echo- ● Always write a full report and be careful to state if you consider heart
disease has been excluded.
cardiographer is available. (14) Inter-
active DVDs that provide a compre-
hensive guide to assessment of the
normal as well as the structurally or with supervision, especially while train- provides scope for cardiologists to re-
functionally abnormal heart also have ing. This experience may be obtained view scan findings without either the
been produced and are extremely valu- on the neonatal unit and in outpatient infant or the specialist having to travel.
able. (15) For neonatologists who an- clinics. Because the recognition of nor- Those who wish to exclude congenital
ticipate echocardiography becoming a mal anatomy rather than the delinea- heart disease confidently should seek a
significant component of their prac- tion of precise abnormality is a priority training fellowship or placement
tices, a detailed pediatric cardiology in neonatal scanning, it is helpful to within a pediatric cardiology unit.
text is another worthwhile investment. perform most early scans on infants
However, there is no substitute who have normal anatomy. Routine Structural
for performing scans under the su- Throughout a neonatologist’s Echocardiographic Views
pervision of a pediatric cardiologist scanning career, it is vitally important It is beyond the scope of this review
or experienced neonatal echocardi- to maintain close contact with pediat- to offer comprehensive instruction
ographer. Every opportunity should ric cardiologists. The use of recorded on the technique of echocardiogra-
be taken to observe and perform scans scans or teleechocardiography also phy. Rather, we examine standard

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views obtained in neonatal echocar- Standard Views in Neonatal

Table 2.
diography to aid understanding.
In all cases, it is essential to follow Echocardiography
a structured approach to echocardi-
View Demonstrates
ography. At every scan, standard
views (Table 2) should be obtained Transverse subcostal view ● Normal situs
Subcostal atrial and four- ● Superior vena cava drains into right atrium,
in sequence, with at least a minimum
chamber views pulmonary veins into left
“checklist” of normal anatomic fea- ● Intact intra-atrial septum
tures confirmed. The echocardio- ● Intact ventricular septum
gram should be performed in a logi- Apical four-chamber view ● Normal mitral and tricuspid valves, with
cal sequence, taking care to delineate tricuspid positioned closer to the apex of
the venous drainage, all four cardiac the heart. Establish atrioventricular
concordance
chambers, the septae, and all four ● Intact ventricular septum
valves. (14) ● Rotate to “five-chamber view” to identify
normal aortic valve from the left ventricle
Assessment of Structural ● Pulmonary artery from the right ventricle
Congenital Heart Disease crossing over aorta, excluding transposition
(ie, establishing ventriculo-arterial
The diagnosis and management of concordance)
structural congenital heart disease al- Parasternal long axis view ● Normal motion of mitral and aortic valves
ways will be the role of the pediatric ● Intact ventricular septum
cardiologist. In some cases, an ur- ● Normal pulmonary valve
Parasternal short axis view ● Intact ventricular septum
gent assessment of cardiac structure
● Normal pulmonary valve
may be carried out by a neonatolo- ● Drainage of pulmonary veins into left
gist, when the primary goal is to con- atrium
firm or exclude normal anatomy, Ductal view ● Ductal patency and direction of flow
rather than delineate a precise struc- Arch view ● Exclusion of coarctation
tural diagnosis. This can be of great
value, particularly in centers remote
from pediatric cardiology. In practi-
drome, coarctation of the aorta) This rapid circulatory adaptation
cal terms, the neonatologist is likely
must be differentiated from other can fail in both term and preterm
to have to exclude structural pathol-
causes of collapse such as sepsis or infants. Echocardiography allows de-
ogy in two primary scenarios.
metabolic disease. termination of the functional status
The first example involves an in-
fant who has cyanosis in which the of the transitional circulation and
Assessment of Functional permits exclusion of a structural
task is to differentiate cyanotic con-
Cardiovascular Status cause for any failure in transition.
genital heart disease (eg, transposi-
The central features of the transi- Because the transitional circulation
tion of the great arteries, pulmonary
tional circulation are: evolves rapidly, repeated echocardio-
atresia, tricuspid atresia, tetralogy
of Fallot) from persistent pulmo- ● An abrupt decrease in pulmo- graphic assessments may be required.
nary hypertension of the newborn nary vascular resistance leading Echocardiographic assessment may
(PPHN). Exclusion of total or partial to an increase in pulmonary be warranted when one or more of
anomalous pulmonary venous con- blood flow the principal components of the tran-
nection (TAPVC) can be particularly ● Removal of the low-resistance sitional circulation is failing. Failure
difficult, especially in infants who placental circulation by clamp- of pulmonary vascular resistance to
have evidence of PPHN and reduced ing of the umbilical cord, lead- decrease presents as PPHN, with cy-
pulmonary blood flow. ing to a sudden increase in pe- anosis resistant to adequate ventila-
The second example involves an ripheral vascular resistance tion and right-to-left shunting at
infant who has collapsed or has hy- ● Functional closure of the arte- atrial or ductal levels. Failure of the
potension in whom an obstructive rial duct and foramen ovale, myocardium to pump adequately
cardiac pathology (eg, critical aortic generally within 24 hours of may result in impaired systemic per-
stenosis, hypoplastic left heart syn- birth in the healthy term infant fusion.

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tent, ventilation-refractory hypoxia

requires a pediatric cardiology re-
view. Tricuspid and pulmonary atre-
sia, Ebstein anomaly, and tetralogy
of Fallot can be excluded by the dem-
onstration of four normally function-
ing cardiac valves. Transposition of
the great vessels can be excluded by
demonstrating normal ventriculo-
arterial connection of the two great
arteries. The primary difficulty echo-
cardiographically in the infant who
has persistent cyanosis is excluding
the relatively rare – but treatable –
TAPVC (Fig. 1), which can be ex-
cluded only by seeing the pulmonary
veins draining into the left atrium.
Another trap is isolated valvar pul-
monary atresia. Because ductal flow
can swirl in the main pulmonary ar-
tery, suggesting that the pulmonary
valve is competent, it is important to
Figure 1. Subcostal view of TAPVC, with pulmonary veins failing to drain into the left
see that the valve opens well.
atrium (LA). Note that the flap of the foramen ovale (labeled ASD) bulges to the left,
consistent with pure right-to-left flow from right atrium (RA) to LA.
Signs of PPHN on echocardiogra-
phy include:
1. Distention of the right heart
Persistent Pulmonary Echocardiographic confirmation of chambers, with flattening of the
Hypertension of the Newborn the diagnosis allows exclusion of a interventricular septum (Fig. 2)
PPHN is a relatively common cause primary cardiac cause, focused ther- 2. Indirect evidence of raised
of morbidity and mortality in term apy, and assessment of response to right ventricular pressure from
infants. (16) The final common path- therapy. high-velocity tricuspid regur-
way in PPHN is failure of the pulmo- An essential step in echocardio- gitation. Most infants who
nary vascular resistance to decrease graphic assessment is exclusion of have PPHN have tricuspid re-
from intrauterine levels, leading to structural cardiac abnormality, which gurgitation, (6) and use of the
pulmonary-to-systemic shunting of can be more than can be expected Bernoulli equation (pressure⫽
blood, reduced pulmonary blood from the level of expertise of many 4⫻velocity2) allows calcula-
flow, and ventricular dysfunction. (6) neonatal echocardiographers. Persis- tion of the peak systolic pres-
sure gradient between the
right ventricle and the right
atrium (Fig. 3). This is the
most repeatable method of es-
timating pulmonary arterial
pressure. (17) The pulmonary
arterial pressure can be esti-
mated by adding a nominal
pressure value for the right
atrial pressure (usually taken as
5 to 10 mm Hg).
Figure 2. Parasternal short axis view. In healthy infants (A) the left ventricle is 3. Right-to-left shunting through
circular; in PPHN (or TAPVC) (B) the raised right ventricular pressure flattens the fetal channels. Although the
interventricular septum and displaces it into the left ventricle. arterial duct is not universally

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patent in PPHN, (6) clear vi-

sualization of pure right-to-
left ductal flow (Fig. 4) in the
presence of normal cardiac
anatomy confirms the diagno-
sis of PPHN, if left-heart ob-
struction (especially aortic co-
arctation) is ruled out. In some
cases, the shunt is bidirectional
or even pure left-to-right, al-
though with low velocity, again
demonstrating that the pul-
monary pressures are abnor-
mally high. (14) Shunt from
the right to the left atrium
through a patent foramen
ovale is also usual in infants
who have PPHN.
4. Low left ventricular output
(LVO) and right ventricular
Figure 3. Assessment of velocity of tricuspid regurgitation from the apical four- output (RVO) indicate a
chamber view. Application of the modified Bernoulli equation (Pⴝ4v2) allows poorer prognosis. (6)
estimation of the right ventricular/pulmonary arterial pressure. 5. Notching of the pulmonary ar-
tery waveform (due to a back-
ward traveling compression
wave “bouncing” back off the
constricted pulmonary vascu-
lar bed). (18)
In all cases, response to treatment,
particularly changes in cardiac out-
put and direction of ductal shunting,
can be assessed by repeated echocar-
diographic assessments. (6) If there is
any uncertainty about the cardiac
anatomy or if the pattern of PPHN is
not consistent with the clinical con-
dition of the infant, urgent referral to
a pediatric cardiologist should be
made. PPHN can coexist with struc-
tural congenital heart disease.

Assessment of Systemic
Perfusion
Episodes of inadequate systemic per-
fusion in the newborn period may
occur in preterm infants due to im-
maturity (19) and in term infants due
Figure 4. A high parasternal view shows the characteristic “three-legged stool” to systemic illness. (7) Adequacy of
appearance of the duct (PDA) and left (LPA) and right pulmonary arteries (RPA). Color the circulation in most clinical set-
Doppler demonstrates blue flow in the duct (away from the transducer) from the tings is assessed using surrogate
pulmonary artery to the aorta. markers, which are imperfect predic-

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duced by Kluckow and associates

(unpublished observations).
Echocardiographic assessment of
cardiac filling as a guide to adequacy
of preload has not yet been shown to
be reliable. (35) However, multiple
sites of assessment (SVC, inferior
vena cava, atrial and ventricular di-
ameters) that are showing potential
in adult patients (36)(37) need to be
considered. An echocardiographic
assessment of cardiac filling in the
sick neonate is likely to be better than
no assessment at all, but in the sick
term infant, central venous pressure
should be measured. (38)
Echocardiographic assessment of
systemic perfusion clearly has limita-
tions, particularly in repeatability and
inaccuracies from shunt through fe-
Figure 5. Subcostal view of color Doppler assessment of SVC flow velocity (note that tal channels. Nevertheless, using
the flow velocity varies with respiration). Flow can be estimated by multiplying the multiple hemodynamic measure-
area under the curve by the cross-sectional area of the SVC. ments at each scan enables a single
clinician to gain a global assessment
tors of flow. (20) Blood pressure has proximates 20%. (28) Therefore, a of cardiac function and minimizes
an unreliable relationship with sys- 20% change is likely to represent a the impact of measurement error.
temic (21) and cerebral (22) perfu- genuine change in flow volume. (39) A global circulatory assessment,
sion. Prolonged capillary refill time is Doppler echocardiographic estima- including estimation of LVO, RVO,
associated with poor perfusion, (20) tion of RVO has been shown to have and SVC flow, fetal shunt patterns,
although the sensitivity is poor. Lac- similar repeatability to LVO. (29) and ventricular filling, can be
tate concentration (23) and urine However, in the presence of per- achieved in only 10 minutes. (13)
output (24) are generally markers of sistent shunting through a patent ar-
prior, rather than current, perfusion. terial duct or foramen ovale, neither Assessment of Ductal Shunt
Doppler echocardiography pro- LVO nor RVO represents true sys- Volume
vides a noninvasive method of assess- temic perfusion; both can overesti- Patency of the arterial duct is normal
ing cardiac output. (25) In the ab- mate systemic perfusion by at least in the first 48 hours of postnatal life
sence of shunting through fetal 50%. (30)(31) Superior vena cava in healthy term and preterm infants.
pathways, LVO and RVO are both (SVC) flow (Fig. 5) is unaffected by (3) Patency tends to be prolonged in
equivalent to systemic perfusion. shunting through fetal pathways and preterm infants who have respiratory
Doppler echocardiographic estima- has been shown to be a repeatable disease, leading to significant volume
tion of LVO has been shown to cor- marker of systemic perfusion. (32) of systemic-to-pulmonary shunting.
relate well with invasive estimates of Low flow in the neonatal period is Potentially this may lead to worsen-
flow in neonates. (26) Because aortic associated with subsequent periven- ing of respiratory status (40) and per-
diameter changes little in the new- tricular hemorrhage (33) and adverse haps systemic hypoperfusion. (41)
born period, (27) change in left ven- long-term neurodevelopmental out- However, trials of intervention have
tricular stroke volume over time may come. (34) Work by our own group failed to show significant benefit in
be assessed by monitoring aortic has confirmed that measurement of long-term outcomes from therapy
stroke distance (the area under the SVC flow volume is feasible in more aimed at ductal closure, (42) leaving
Doppler profile). In the first postna- than 95% of scans in preterm infants the possibility that the association
tal week, the intraobserver repeat- and suggested similar repeatability between prolonged ductal patency
ability of aortic stroke distance ap- and reference ranges to those pro- and adverse outcomes in preterm ne-

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likely to be associated with

high shunt volume. (44)
3. Ductal flow pattern, using
continuous wave Doppler, to
confirm direction of flow and
assess maximum and mini-
mum flow velocities. A mini-
mum flow velocity that is more
than 50% of the maximum ve-
locity is associated with a con-
stricting duct and suggests
lower shunt volume. (45)
4. Left atrial and ventricular dila-
tation. Increased left-to-right
ductal shunt creates a volume-
loaded appearance of both the
left atrium and ventricle. The
ratio of the left atrial to the
aortic diameter can be assessed,
Figure 6. High parasternal view of patent arterial duct with left-to-right shunting. using M-mode echocardiogra-
The duct is demonstrated by the red flow seen on color Doppler imaging. A duct phy from the parasternal long
diameter less than 1.5 mm at the point of maximal constriction is unlikely to be axis view. A ratio or more than
associated with high shunt volume. 1.5:1 suggests a higher-volume
shunt.
onates is not causative, but due to graphic examination cannot deter- 5. Descending aortic flow pat-
confounders such as the severity of mine whether a duct merits treatment; tern, which can be visualized
respiratory disease. (43) Some au- it merely can diagnose patency, ex- using pulsed wave Doppler
thors have recommended that phar- clude coexisting structural heart dis- from a high parasternal view.
maceutical closure not be considered ease, and establish whether the shunt In healthy infants, there is con-
standard care in the neonate. (43) volume through the duct is high, tinuous forward flow in the de-
Further discussion of the scenarios in moderate, or low. (3)(14) scending aorta, which is high
which treatment may be indicated is Echocardiographic assessment in velocity during cardiac systole
outside the scope of this review. ductal patency can be used for a and low velocity during dias-
Nevertheless, attempts at thera- number of conditions: tole (Fig. 7A). However, in in-
peutic ductal closure remain com- 1. Exclude structural congenital
fants who have high-volume
mon, particularly in infants who re- heart disease, particularly co-
ductal shunt, flow in the de-
main ventilator-dependent. The arctation of the aorta and pul-
scending aorta is reversed dur-
significance of an individual’s duct monary atresia. If this cannot
ing diastole, as blood is
remains a common clinical debate, be done with confidence, no
“sucked” back up the descend-
and this cannot be assessed reliably treatment aimed at ductal clo-
without echocardiographic examina- sure should be initiated until ing aorta and into the pulmo-
tion. However echocardiography the infant has been reviewed nary circulation (Fig. 7B). (46)
does not replace clinical examination by a cardiologist. 6. Persistent forward flow in pe-
for evidence of ductal shunt (eg, 2. Ductal diameter from the high ripheral pulmonary arteries.
bounding pulses, hyperactive precor- parasternal (“ductal”) view Blood traveling through the
dium), to exclude signs of structural (Fig. 6). With appropriate ductus creates persistent for-
congenital heart disease (cyanosis, color Doppler scale and gain ward flow through the periph-
lower limb pulses), and perhaps most settings, a duct diameter less eral pulmonary arteries, sug-
importantly, to assess the infant’s than 1.5 mm at the point of gesting high shunt volume.
overall clinical status. An echocardio- maximal constriction is un- (47)

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Figure 7. High parasternal views of (A) a healthy infant who has forward diastolic flow and (B) an infant who has reversed diastolic
flow that is associated with high-volume left-to-right ductal shunt.

Line Placements/Tamponade Increasing use of echocardiogra- preterm transitional circulatory compro-

Echocardiography also may have a phy on the neonatal unit has led to a mise. Early Hum Dev. 2005;81:413– 422
8. Ward CJ, Purdie J. Diagnostic accuracy
role in selected infants who do not greater awareness of cardiovascular
of paediatric echocardiograms interpreted
need the services of a pediatric cardi- function in the neonate. Minute-to- by individuals other than paediatric cardiol-
ologist in determining normal car- minute management can be greatly ogists. J Paediatr Child Health. 2001;37:
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clude infants in whom a sudden to improve function, increase vascu- 9. Stanger P, Silverman NH, Foster E. Di-
clinical deterioration could be due to agnostic accuracy of pediatric echocardio-
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