Papers by Lisa Hornberger
ABSTRACTBackgroundDistances between delivery centers and cardiac services can make the care of fe... more ABSTRACTBackgroundDistances between delivery centers and cardiac services can make the care of fetuses with cardiac disease(CD) at risk of acute cardiorespiratory instability(ACRI) at birth a challenge. In 2013 we implemented a fetal echocardiography(FE)-based algorithm targeting fetuses considered high-risk for ACRI at ≤2 hours of birth for Caesarian section(CS) delivery in our pediatric cardiac operating room(PCOR) of our children’s hospital. We examine the experience and outcomes of affected newborns.MethodsWe reviewed maternal and postnatal medical records of all fetuses with CD at high-risk for ACRI encountered January 2013-March 2022. Secondary analysis was performed including all fetuses with diagnoses of d-transposition of the great arteries/intact ventricular septum(d-TGA/IVS) and hypoplastic left heart syndrome(HLHS) encountered over the study period.ResultsForty fetuses were considered high-risk for ACRI: 15 d-TGA/IVS and 7 HLHS with restrictive atrial septum(RAS), 4 abse...
Journal of the American College of Cardiology, 2000
The purpose of this study was to describe the clinical characteristics and outcome and to elucida... more The purpose of this study was to describe the clinical characteristics and outcome and to elucidate the pathogenesis of ductus arteriosus aneurysm (DAA). BACKGROUND Ductus arteriosus aneurysm is a rare lesion that can be associated with severe complications including thromboembolism, rupture and death. METHOD We reviewed the clinical records, diagnostic imaging studies and available histology of 24 cases of DAA, diagnosed postnatally (PD) in 15 and antenatally (AD) in 9 encountered in five institutions. RESULTS Of PD cases, 13 presented at Ͻ2 months, and all AD cases were detected incidentally after 33 weeks of gestation during a late trimester fetal ultrasound study. Of the 24, only 4 had DAA-related symptoms and 6 had associated syndromes: Marfan, Smith-Lemli-Opitz, trisomies 21 and 13 and one possible Ehlers-Danlos. Three had complications related to the DAA: thrombus extension into the pulmonary artery, spontaneous rupture, and asymptomatic cerebral infarction. Six underwent uncomplicated DAA resection for ductal patency, DAA size or extension of thrombus. In the four examined, there was histologic evidence of reduced intimal cushions in two and abnormal elastin expression in two. Five of the 24 died, with only one death due to DAA. Of 19 survivors, all but one remain clinically asymptomatic at a median follow-up of 35 months; however, two have developed other cardiac lesions that suggest Marfan syndrome. A review of 200 consecutive third trimester fetal ultrasounds suggests an incidence of DAA of 1.5%. CONCLUSIONS Ductus arteriosus aneurysm likely develops in the third trimester perhaps due to abnormal intimal cushion formation or elastin expression. Although it can be associated with syndromes and severe complications, many affected infants have a benign course. Given the potential for development of other cardiac lesions associated with connective tissue disease, follow-up is warranted. (J Am Coll Cardiol 2000;36:262-9) © 2000 by the American College of Cardiology Ductus arteriosus aneurysm (DAA) is characterized by a localized saccular or tubular dilation of the ductus arteriosus. Although congenital DAA may be identified in infants, children and adults, published case reports suggest the most common age of diagnosis is less than two months (1-24). Ductus arteriosus aneurysm may be observed in patients with connective tissue abnormalities such as Marfan, Ehlers-Danlos and Larsen syndromes (2,20,21). Previous reports suggest that congenital DAA encountered postnatally may be associated with a high rate of serious complications (2,7-9,15). This probably represents presentation bias of only symptomatic patients with congenital DAA. Spontaneous rupture, erosion, thromboembolism, infection and compression of adjacent structures are some of the reported complications.
Journal of the American Heart Association, Jun 6, 2023
Background Fetal diagnosis of congenitally corrected transposition of the great arteries (ccTGA) ... more Background Fetal diagnosis of congenitally corrected transposition of the great arteries (ccTGA) has been increasingly reported; however, predictors of clinical outcomes remain underexplored. We undertook a multicenter, retrospective study to investigate natural history, associated anomalies, and outcomes of fetal ccTGA. Methods and Results Fetuses with ccTGA diagnosed from January 2004 to July 2020 within 20 North American programs were included. Fetuses with severe ventricular hypoplasia thought to definitively preclude biventricular repair were excluded. We included 205 fetuses diagnosed with ccTGA at a median gestational age of 23 (interquartile range, 21–27) weeks. Genetic abnormalities were found in 5.9% tested, with extracardiac anomalies in 6.3%. Associated cardiac defects were diagnosed in 161 (78.5%), with atrioventricular block in 23 (11.3%). On serial fetal echocardiogram, 39% demonstrated a functional or anatomic change, most commonly increased tricuspid regurgitation (6.7%) or pulmonary outflow obstruction (11.1%). Of 194 fetuses with follow‐up, 26 were terminated, 3 experienced fetal death (2 with atrioventricular block), and 165 were live‐born. Of 158 with postnatal data (median follow‐up 3.7 years), 10 (6.6%) had death/transplant before 1 year. On univariable analysis, fetal factors associated with fetal death or death/transplant by 1 year included ≥ mild tricuspid regurgitation, pulmonary atresia, aortic obstruction, fetal arrhythmia, and worsening hemodynamics on serial fetal echocardiogram (defined as worse right ventricular function, tricuspid regurgitation, or effusion). Conclusions Associated cardiac lesions and arrhythmias are common in fetal ccTGA, and functional changes commonly occur through gestation. Worse outcomes are associated with fetal tricuspid regurgitation (≥mild), any arrhythmia, pulmonary atresia, aortic obstruction, and worsening hemodynamics on serial echocardiograms. These findings can inform prenatal counseling and perinatal management planning.
American Journal of Obstetrics and Gynecology, Jul 1, 2023
Journal of the American Heart Association, Jul 5, 2022
Ultrasound in Obstetrics & Gynecology, Sep 1, 2012
Echocardiography-a Journal of Cardiovascular Ultrasound and Allied Techniques, Sep 11, 2012
Objective: We sought to evaluate the completeness of echocardiographic diagnosis of fetal tetralo... more Objective: We sought to evaluate the completeness of echocardiographic diagnosis of fetal tetralogy of Fallot (fTOF) at 12-17 weeks gestation, and compare assessment and clinical outcomes to diagnoses made at >17 weeks gestation. Methods: We identified all fTOF diagnoses made in our experience from 2003 to 2008. Referral indication, anatomic detail by echocardiography and pregnancy outcomes were compared between fetuses diagnosed at 17 weeks (Group I) and >17 weeks gestation (Group II). A 10-point scoring tool was applied retrospectively to the echocardiograms at initial diagnosis (1 point each was ascribed to visualization of right ventricular outflow obstruction, pulmonary valve, pulmonary arteries including dimensions, pulmonary arterial flow, systemic and pulmonary venous anatomy, atrioventricular valves, ductus arteriosus, ductus flow, aortic arch morphology, sidedness and flow). Results: There were 10 pregnancies in Group I (12-17 weeks) and 25 in Group II (mean gestation at diagnosis 23.5 ± 5.7). The most common reason for referral was extracardiac pathology in Group I (80%) and suspected fetal heart disease on obstetric ultrasound in Group II (64%). Transabdominal imaging was adequate in about half of Group I studies. Mean anatomic diagnosis score in Group I was 6.1(range 2.5-9) and Group II was 8.4 (range 6.5-10). Elective pregnancy termination occurred in 80% in Group I and 33% in Group II. Conclusions: fTOF can be diagnosed in first and early second trimesters with detailed anatomic assessment possible in most. Referral indication and pregnancy outcome differ considerably between early and later prenatal diagnosis of fTOF.
Journal of Ultrasound in Medicine, Feb 1, 2010
The purpose of this series was to identify vascular rings prenatally on fetal echocardiograms. Me... more The purpose of this series was to identify vascular rings prenatally on fetal echocardiograms. Methods. We reviewed the fetal echocardiograms and clinical histories of 9 patients with a diagnosis of vascular rings at our institution from 2004 to 2009. Eight patients had a prenatal diagnosis by fetal echocardiography. One other patient who had undergone fetal echocardiography had a diagnosis of a vascular ring and a cervical arch only postnatally. Results. Among the 8 patients with a prenatal diagnosis (4 with a double aortic arch and 4 with a right aortic arch [RAA], an aberrant left subclavian artery, and a left ductus arteriosus [LDA]), the vascular ring was isolated in 4 and associated with other structural congenital heart disease in 4. In all 8 patients with a prenatal diagnosis, the vascular ring was identified by cephalad transducer sweeps from a 3-vessel view (3VV) with and without color Doppler imaging, which revealed vascular structures coursing around the trachea. Seven of these patients had postnatal confirmation of the abnormality (there was 1 pregnancy termination without autopsy). The only known vascular ring missed at fetal echocardiography but diagnosed after birth was that associated with a cervical RAA (which could not be visualized in cross-sectional sweeps) and an LDA evaluated only late in pregnancy. Only 3 of the 8 postnatally treated neonates had clinical symptoms of the vascular ring, and 5 underwent surgical division of the ring. Conclusions. Vascular rings can be largely identified before birth with cephalad sweeps from the 3VV with attention to the relationship of the aortic and ductal arches to the trachea on fetal echocardiograms.
Journal of the American College of Cardiology, 2002
OBJECTIVES We reviewed our institution's experience with isolated (congenital) third-degree atrio... more OBJECTIVES We reviewed our institution's experience with isolated (congenital) third-degree atrioventricular block (CAVB) to identify pre-and post-natal predictors of mortality and the requirement for pacemakers in infancy and childhood. BACKGROUND Because of the relative rarity of the disease, there is a paucity of data concerning the outcome of fetuses and children with isolated CAVB. METHODS The medical records of all cases of CAVB encountered at our institution from January 1965 to December 1998 were analyzed. RESULTS Of 102 cases identified, 29 were diagnosed in utero (F) at 26.1 Ϯ 5.6 weeks gestation, 33 as neonates (N; Յ28 days), and 40 as children (C) at 5.7 Ϯ 4.8 years of age. Anti-Ro and/or anti-La were present in 95% of F and 90% of N, but only in 5% of C mothers tested (p Ͻ 0.0001). Patients with CAVB having F, N and C diagnosis had a mortality of 43%, 6% and 0%, respectively, in the first two decades of life. Increased mortality risk was associated with a fetal diagnosis of CAVB (13/15 deaths; p Ͻ 0.05), fetal hydrops (6/6 cases; p Ͻ 0.0001), endocardial fibroelastosis (5/5 cases; p Ͻ 0.0001) and delivery at Յ32 weeks (4/6 cases; p Ͻ 0.05). Timing of pacemaker implantation differed significantly among F versus N (p Ͻ 0.05) and N versus C (p Ͻ 0.001) cases. At 20 years of age only 11% and 12% of CAVB patients with N and C diagnosis, respectively, were not paced. CONCLUSIONS Pre-natal diagnosis of CAVB is associated with high fetal and neonatal mortality. Among survivors, whether the diagnosis is made before or after birth, most undergo pacemaker implantation by adulthood, with earlier intervention and a significantly greater need for reintervention among those diagnosed in utero. (
Journal of The American Society of Echocardiography, Jun 1, 2017
Background: In severe right heart obstruction (RHO), redistribution of cardiac output to the left... more Background: In severe right heart obstruction (RHO), redistribution of cardiac output to the left ventricle (LV) is well tolerated by the fetal circulation. Although the same should be true of severely regurgitant tricuspid valve disease (rTVD) with reduced or no output from the right ventricle, affected fetuses more frequently develop hydrops or suffer intrauterine demise. We hypothesized that right atrium (RA) function is altered in rTVD but not in RHO, which could contribute to differences in outcomes. Methods: Multi-institutional retrospective review of fetal echocardiograms performed over a 10-year period on fetuses with rTVD (Ebstein's anomaly, tricuspid valve dysplasia) or RHO (pulmonary atresia/intact ventricular septum, tricuspid atresia) and a healthy fetal control group. Offline velocity vector imaging and Doppler measurements of RA size and function and LV function were made. Results: Thirty-four fetuses with rTVD, 40 with RHO, and 79 controls were compared. The rTVD fetuses had the largest RA size and lowest RA expansion index, fractional area of change, and RA indexed filling and emptying rates compared with fetuses with RHO and controls. The rTVD fetuses had the shortest LV ejection time and increased Tei index with a normal LV ejection fraction. RA dilation (odds ratio, 1.27; 95% CI, 1.05-1.54) and reduced indexed emptying rate (odds ratio, 2.49; 95% CI, 1.07-5.81) were associated with fetal or neonatal demise. Conclusions: Fetal rTVD is characterized by more severe RA dilation and dysfunction compared with fetal RHO and control groups. RA dysfunction may be an important contributor to reduced ventricular filling and output, potentially playing a critical role in the worsened outcomes observed in fetal rTVD.
American Journal of Cardiology, Jun 1, 2012
Ultrasound in Obstetrics & Gynecology, Nov 10, 2022
Paediatrics and Child Health, May 1, 2003
unintentional injuries (82%) were similar pre and post equipment removal. Conclusions: We found a... more unintentional injuries (82%) were similar pre and post equipment removal. Conclusions: We found a decrease in injury rates related to equipment removal. There was no corresponding increase in intentional injuries. These results suggest that removal of non-compliant equipment is an effective strategy for the prevention of children's playground injuries. Comparisons of injury rates prior to equipment removal suggest that the CSA standards identified playgrounds with higher injury rates.
Journal of The American Society of Echocardiography, Dec 1, 2012
Background: The aim of this study was to determine the feasibility of performing complete early f... more Background: The aim of this study was to determine the feasibility of performing complete early fetal echocardiography (FE) at <17 weeks of gestation with comparison with standard FE in the midtrimester (17-23 weeks).
Springer eBooks, Sep 18, 2013
Congenital Heart Disease, Sep 1, 2008
A 13-month-old boy presented with acute onset of complete atrioventricular block and wide complex... more A 13-month-old boy presented with acute onset of complete atrioventricular block and wide complex tachycardia but normal hemodynamics. Endomyocardial biopsy disclosed active myocarditis with eosinophils, suggesting a hypersensitivity reaction. With no treatment, the rhythm disturbance resolved within days of onset. Our patient's presentation and self-limited illness is unique. To our knowledge, this is only the second reported case of eosinophilic myocarditis in a young child or infant.
The Journal of Pediatrics, Jul 1, 2009
To define current frequency of prenatal detection of congenital heart disease (CHD), factors affe... more To define current frequency of prenatal detection of congenital heart disease (CHD), factors affecting prenatal detection, and its influence on postnatal course. We prospectively identified all fetuses and infants &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt; or =6 months of age with major CHD at 3 referral centers in Northern California over 1 year; we obtained prenatal and demographic data, reviewed prenatal ultrasound (US) and postnatal records, and used logistic regression to analyze maternal, fetal, and prenatal-care provider risk factors for prenatal diagnosis. Ninety-eight of 309 infants with major CHD had prenatal diagnosis (36% accounting for 27 pregnancy terminations); 185 infant-families participated in the postnatal survey, and although 99% had prenatal US, only 28% were prenatally diagnosed. Anomalous pulmonary venous return (0%), transposition of the great arteries (19%), and left obstructive lesions (23%) had the lowest prenatal detection. Heterotaxy (82%), single ventricle (64%), and HLHS (61%) had the highest. Prenatal diagnosis was higher at university versus community practices (P = .001). Sociodemographics were not associated with prenatal diagnosis. Infants diagnosed prenatally were less frequently ventilated (P &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt; .01) or treated with prostaglandin (P &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt; .05). Prenatal detection of major CHD significantly alters postnatal course but remains low despite nearly universal US. CHD type and US practice type are important determinants of prenatal detection.
Circulation, Jul 18, 2023
Ultrasound in Obstetrics & Gynecology, May 1, 2023
ABSTRACTObjectiveWhile in‐utero treatment of sustained fetal supraventricular arrhythmia (SVA) is... more ABSTRACTObjectiveWhile in‐utero treatment of sustained fetal supraventricular arrhythmia (SVA) is standard practice in the previable and preterm fetus, data are limited on best practice for late preterm (34 + 0 to 36 + 6 weeks), early term (37 + 0 to 38 + 6 weeks) and term (&gt; 39 weeks) fetuses with SVA. We reviewed the delivery and postnatal outcomes of fetuses at ≥ 35 weeks of gestation undergoing treatment rather than immediate delivery.MethodsThis was a retrospective case series of fetuses presenting at ≥ 35 weeks of gestation with sustained SVA and treated transplacentally at six institutions between 2012 and 2022. Data were collected on gestational age at presentation and delivery, SVA diagnosis (short ventriculoatrial (VA) tachycardia, long VA tachycardia or atrial flutter), type of antiarrhythmic medication used, interval between treatment and conversion to sinus rhythm and postnatal SVA recurrence.ResultsOverall, 37 fetuses presented at a median gestational age of 35.7 (range, 35.0–39.7) weeks with short VA tachycardia (n = 20), long VA tachycardia (n = 7) or atrial flutter (n = 10). Four (11%) fetuses were hydropic. In‐utero treatment led to restoration of sinus rhythm in 35 (95%) fetuses at a median of 2 (range, 1–17) days; this included three of the four fetuses with hydrops. Antiarrhythmic medications included flecainide (n = 11), digoxin (n = 7), sotalol (n = 11) and dual therapy (n = 8). Neonates were liveborn at 36–41 weeks via spontaneous vaginal delivery (23/37 (62%)) or Cesarean delivery (14/37 (38%)). Cesarean delivery was indicated for fetal SVA in two fetuses, atrial ectopy or sinus bradycardia in three fetuses and obstetric reasons in nine fetuses that were in sinus rhythm at the time of delivery. Twenty‐one (57%) cases were treated for recurrent SVA after birth.ConclusionIn‐utero treatment of the near term and term (≥ 35‐week) SVA fetus is highly successful even in the presence of hydrops, with the majority of cases delivered vaginally closer to term, thereby avoiding unnecessary Cesarean section. © 2023 International Society of Ultrasound in Obstetrics and Gynecology.
Circulation, Nov 16, 2021
Introduction: Congenitally corrected transposition of the great arteries (ccTGA) is rare with var... more Introduction: Congenitally corrected transposition of the great arteries (ccTGA) is rare with varied associated cardiac defects and rhythm abnormalities. We aimed to describe the natural history, associated anomalies and prenatal outcome in a cohort of prenatally diagnosed patients in which biventricular repair is anticipated. Methods: A retrospective cohort study was conducted via the Fetal Heart Society Research Collaborative. All fetuses with ccTGA encountered at 15 North American cardiac centers between 1/2004-7/2020 were identified. Fetuses with a hypoplastic ventricle precluding biventricular repair were excluded. Data is presented as median (interquartile range). Results: Inclusion criteria were met in 139 fetuses who were diagnosed with ccTGA at 24 (21-29) weeks. There was a family history of congenital heart disease in 12%. Maternal diabetes was present in 10%. Prenatal genetic testing in 50 pregnancies was normal. Excluding 14 fetuses with heterotaxy, extracardiac anomalies were observed in 9/125 (7.1%). Associated cardiac/extracardiac defects are detailed in table 1. Fetal atrioventricular block (AVB) was present in 17 fetuses (12%), diagnosed at a median of 26 (23-28), range 20-36 weeks. Two fetuses had SVT; 1 self-resolved and 1 during labor. Change occurred during follow-up in 34 fetuses, most commonly in severity of tricuspid regurgitation (6 improved, 4 worsened) and worsening of pulmonary stenosis (n=5). There were 112 live births, 17 terminations, 2 fetal deaths (FD) and 8 lost to follow-up. Both fetuses with FD had AVB, making the intrauterine mortality associated with fetal AVB 11.8% (2/17). Conclusions: This study represents the largest cohort to date of fetuses with ccTGA. Fetal ccTGA is associated with a spectrum of cardiac defects that may evolve, with extracardiac pathology occurring in 7.1%. AVB is found in 12% of fetuses and is a risk factor for FD. This data informs fetal counseling and can aid in delivery planning and perinatal care.
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Papers by Lisa Hornberger