The American Association for Thoracic Surgery (AATS) 2024 expert consensus document: Management of neonates and infants with Ebstein anomaly.

OBJECTIVES: Symptomatic neonates and infants with Ebstein anomaly (EA) require complex management. A group of experts was commissioned by the American Association for Thoracic Surgery to provide a framework on this topic focusing on risk stratification and management. METHODS: The EA Clinical Congenital Practice Standards Committee is a multinational and multidisciplinary group of surgeons and cardiologists with expertise in EA. A citation search in PubMed, Embase, Scopus, and Web of Science was performed using key words related to EA. The search was restricted to the English language and the year 2000 or later and yielded 455 results, of which 71 were related to neonates and infants. Expert consensus statements with class of recommendation and level of evidence were developed using a modified Delphi method, requiring 80% of members votes with at least 75% agreement on each statement. RESULTS: When evaluating fetuses with EA, those with severe cardiomegaly, retrograde or bidirectional shunt at the ductal level, pulmonary valve atresia, circular shunt, left ventricular dysfunction, or fetal hydrops should be considered high risk for intrauterine demise and postnatal morbidity and mortality. Neonates with EA and severe cardiomegaly, prematurity (<32 weeks), intrauterine growth restriction, pulmonary valve atresia, circular shunt, left ventricular dysfunction, or cardiogenic shock should be considered high risk for morbidity and mortality. Hemodynamically unstable neonates with a circular shunt should have emergent interruption of the circular shunt. Neonates in refractory cardiogenic shock may be palliated with the Starnes procedure. Children may be assessed for later biventricular repair after the Starnes procedure. Neonates without high-risk features of EA may be monitored for spontaneous closure of the patent ductus arteriosus (PDA). Hemodynamically stable neonates with significant pulmonary regurgitation at risk for circular shunt with normal right ventricular systolic pressure should have an attempt at medical closure of the PDA. A medical trial of PDA closure in neonates with functional pulmonary atresia and normal right ventricular systolic pressure (>20-25 mm Hg) should be performed. Neonates who are hemodynamically stable without pulmonary regurgitation but inadequate antegrade pulmonary blood flow may be considered for a PDA stent or systemic to pulmonary artery shunt. CONCLUSIONS: Risk stratification is essential in neonates and infants with EA. Palliative comfort care may be reasonable in neonates with associated risk factors that may include prematurity, genetic syndromes, other major medical comorbidities, ventricular dysfunction, or sepsis. Neonates who are unstable with a circular shunt should have emergent interruption of the circular shunt. Neonates who are unstable are most commonly palliated with the Starnes procedure. Neonates who are stable should undergo ductal closure. Neonates who are stable with inadequate pulmonary flow may have ductal stenting or a systemic-to-pulmonary artery shunt. Subsequent procedures after Starnes palliation include either single-ventricle palliation or biventricular repair strategies.

Predicting High-Risk Fetal Cardiac Disease Anticipated to Need Immediate Postnatal Stabilization and Intervention with Planned Pediatric Cardiac Operating Room Delivery.

BACKGROUND: Distances between delivery and cardiac services can make the care of fetuses with cardiac disease at risk of acute cardiorespiratory instability at birth a challenge. In 2013 we implemented a fetal echocardiography-based algorithm targeting fetuses considered high risk for acute cardiorespiratory instability at ≤2 hours of birth for delivery in our pediatric cardiac operating room of our children's hospital, and, herein, examine our experience. METHODS AND RESULTS: We reviewed maternal and postnatal medical records of all fetuses with cardiac disease encountered January 2013 to March 2022 considered high risk for acute cardiorespiratory instability. 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. Forty fetuses were considered high risk for acute cardiorespiratory instability: 15 with d-TGA/IVS and 7 with HLHS with restrictive atrial septum, 4 with absent pulmonary valve syndrome, 3 with obstructed anomalous pulmonary veins, 2 with severe Ebstein anomaly, 2 with thoracic/intracardiac tumors, and 7 others. Pediatric cardiac operating room delivery occurred for 33 but not for 7 (5 with d-TGA/IVS, 2 with HLHS with restrictive atrial septum). For high-risk cases, fetal echocardiography had a positive predictive value of 50% for intervention/extracorporeal membrane oxygenation/death at ≤2 hours and 70% at ≤24 hours. Of "low-risk" cases, 6/46 with d-TGA/IVS and 0/45 with HLHS required intervention at ≤2 hours. Fetal echocardiography for predicting intervention/extracorporeal membrane oxygenation/death at ≤2 hours had a sensitivity of 67%, specificity 93%, and positive and negative predictive values of 80% and 87%, respectively, for d-TGA/IVS, and 100%, 95%, 71%, and 100% for HLHS, respectively. CONCLUSIONS: Fetal echocardiography can predict the need for urgent intervention in a majority with d-TGA/IVS and HLHS and in half of the entire spectrum of high-risk cardiac disease.

Impact of Neonatal Intervention on Left Ventricular Performance in Ebstein's Anomaly and Tricuspid Valve Dysplasia.

BACKGROUND: Severe neonatal Ebstein's anomaly (EA) and tricuspid valve dysplasia (TVD) are associated with high perinatal morbidity and mortality. The authors recently demonstrated left ventricular (LV) dysfunction and dyssynchrony to be prevalent in affected newborns and to contribute to poor outcomes. The aim of this study was to investigate the impact of patent ductus arteriosus (PDA) closure, spontaneous or surgical ligation, or right ventricular exclusion (Starnes procedure) on LV performance in neonatal EA and TVD. METHODS: Neonates with EA or TVD encountered from 2004 to 2018 at three institutions were identified. Pre- and postoperative LV function was assessed using two-dimensional, Doppler-derived deformation (six-segment vector velocity imaging) and two measures of mechanical dyssynchrony (the SD of time to peak and global dyssynchrony index), and values were compared using paired t test analysis or the Wilcoxon rank sum test. RESULTS: Before the intervention, LV function was impaired in the PDA (n = 18) and Starnes (n = 6) groups and was similar between groups. After PDA closure, LV performance did not change. After the Starnes procedure, however, LV function, including synchrony, improved significantly: fractional area change from 45 ± 5% to 58 ± 8% (P = .003), global circumferential strain from -18.2 ± 5.0% to -32.5 ± 5.5% (P = .01), cardiac index from 1.9 ± 0.3 to 3.9 ± 1.5 L/min/m2 (P = .05), and circumferential strain dyssynchrony (dyssynchrony index from 0.19 ± 0.09 to 0.04 ± 0.02 [P = .009] and SD of time to peak from 59.8 ± 18.5 to 29.9 ± 8.2 [P = .02]). CONCLUSION: The Starnes procedure results in early improvements in LV dysfunction and dyssynchrony, not observed after PDA closure in neonatal severe EA and TVD, which may benefit critically unwell neonates.

Left Ventricular Dysfunction in Neonatal Ebstein's Anomaly and Tricuspid Valve Dysplasia.

BACKGROUND: The mechanisms and prognostic importance of left ventricular (LV) dysfunction in neonatal Ebstein's anomaly (EA) and tricuspid valve dysplasia (TVD) are not well understood. The authors recently demonstrated reduced cardiac output and dyssynchrony to be common in fetal EA/TVD and therefore hypothesized that LV dysfunction may be associated with worse outcomes in neonatal EA/TVD. METHODS: A multicenter retrospective case-control study was conducted among neonatal patients with EA/TVD (n = 32) and a healthy control cohort (n = 17) encountered from 2004 to 2019. The left ventricle was assessed in the first 48 hours after birth using two-dimensional, Doppler-derived, six-segment global and segmental longitudinal strain and circumferential strain (CS) and dyssynchrony indices (the SD of time-to-peak strain and a novel global dyssynchrony index [DI], calculated as [peak segmental average - peak global average]/peak segmental average). RESULTS: Neonates with EA/TVD demonstrated reduced combined cardiac index (4.2 ± 1.5 L/min/m2 vs 6.5 ± 2.2 L/min/m2 in control subjects, P < .001), impaired LV CS (-15.4 ± 6.9 vs -26.2 ± 5.8, P < .001), and increased circumferential dyssynchrony (CS DI 0.20 ± 0.16 vs 0.09 ± 0.04 [P = .019]; SD of time-to-peak CS 63 ± 25 vs 40 ± 15 [P = .003]). Transplantation-free survival occurred in 20 of 32 patients (63%) at 6 months. Increased CS DI and absence of pulmonary valve flow (PVF) were most predictive of mortality; CS DI > 0.2 was associated with 25% survival in subjects without PVF, whereas all patients with CS DI < 0.1 survived. CONCLUSIONS: In neonates with EA/TVD and absence of PVF, there is abnormal LV deformation and compromised cardiac output in association with increased dyssynchrony. Increased CS DI is associated with increased risk for mortality in EA/TVD with no forward PVF.

Influence of extreme prematurity and bronchopulmonary dysplasia on cardiac function.

OBJECTIVE: Prematurity and bronchopulmonary dysplasia (BPD) are associated with poorly understood abnormalities of ventricular function. We therefore comprehensively compared biventricular function in infants with and without BPD. METHODS: Prospective observational study in extremely preterm infants with (n = 20) and without (n = 38) BPD using conventional and advanced echocardiography at 28 days (T1) and near-term (T2). RESULTS: Infants with BPD had lower birth gestational age (26.7±1.9 vs 27.4±1.1 weeks, p = 0.047) and weight (884±207 vs 1108±190 g, p = 0.0001). BPD was associated with larger right ventricles (RV) and reduced RV systolic strain rate at T1 and pulmonary hypertensive indicators at T2 (pulmonary artery acceleration time BPD 51±17 vs no BPD 63±12 ms, p = 0.017). At T1/T2, infants with BPD had lower RV tissue Doppler velocities (e', a' and s) and higher E/e' ratios (T1: BPD 10.4±2.4 vs no BPD 6.2±3.1 cm/sec, p = 0.001; T2: BPD 8.0±3.1 vs no BPD 5.6±2.6 cm/sec, p = 0.02), altered LV diastolic function (apical circumferential T1 early diastolic strain rate BPD 2.8±0.8 vs no BPD 3.6±1.0 /sec, p = 0.04; T2 late diastolic strain rate, BPD 2.29 ± 0.99 vs no BPD 1.67±0.84 /sec, p = 0.03) and LV rotational mechanics (T1: twist rate BPD 90±16 vs no BPD 130±48 deg/sec, p = 0.008; untwist rate (UTR) BPD -69±90 vs no BPD -147±68 deg/sec, p = 0.008; torsion BPD 2.78±0.56 vs no BPD 4.48±1.74 deg/cm, p = 0.009; and T2: UTR BPD -132±69 vs no BPD -179±57 deg/sec, p = 0.013). CONCLUSION: BPD is associated with altered RV diastolic function that persists near term, with elevated pulmonary vascular resistance, and with persistent alterations in LV apical strain rate and rotational mechanics.

Quantitative Assessment of Left Ventricular Dysfunction in Fetal Ebstein's Anomaly and Tricuspid Valve Dysplasia.

BACKGROUND: Fetal Ebstein's anomaly and tricuspid valve dysplasia (EA/TVD) are associated with high perinatal mortality relative to pulmonary atresia with intact ventricular septum (PAIVS), despite both requiring redistribution of the cardiac output (CO) to the left ventricle (LV). LV dysfunction is suspected to contribute to adverse outcomes in EA/TVD. OBJECTIVE: We sought to examine global and segmental LV function in fetal EA/TVD with comparison to normal controls and PAIVS. We hypothesized that LV dysfunction in EA/TVD is associated with abnormal LV remodeling and interventricular mechanics. METHODS: We retrospectively identified 63 cases of fetal EA/TVD (40 with retrograde ductal flow) and 22 cases of PAIVS encountered from 2004 to 2015 and compared findings to 77 controls of comparable gestational age. We measured the combined CO and global LV function using two-dimensional, Doppler-derived, deformational (six-segmental vector velocity imaging) and dyssynchrony indices (DIs; SD of time to peak), and a novel global DI. RESULTS: EA/TVD fetuses demonstrated abnormal LV global systolic function with reduced ejection fraction, fractional area change, and CO, while in PAIVS we observed a normal ejection fraction, fractional area change, and CO. PAIVS, but not EA/TVD, demonstrated increased LV sphericity, suggestive of remodeling, and associated enhanced radial function in the third trimester. In contrast, while EA/TVD fetuses had normal LV segmental longitudinal strain, there was abnormal radial segmental deformation and LV dyssynchrony with increased SD of time to peak and DI. CONCLUSIONS: Fetal EA/TVD is associated with a lack of spherical remodeling and presence of mechanical dyssynchrony, which likely contribute to reduced CO and ejection fraction. Clinical monitoring of LV function is warranted in fetal EA/TVD. Further studies incorporating quantification of LV function into prediction models for adverse outcomes are required.

Prenatal Detection, Comorbidities, and Management of Vascular Rings.

The 3-vessel and trachea view is now integrated into obstetrical screening and facilitates prenatal detection of vascular rings. We examined trends in prenatal detection, associated cardiac and extracardiac anomalies, and surgical management in this population. We reviewed a population-based cohort of pediatric vascular ring patients diagnosed prenatally and postnatally between 2002 and 2017 in Alberta, Canada. Of 106 cases, 28 (26%) had a prenatal diagnosis. Prenatal detection increased over time: 0/29 from 2002 to 2009, 4/28 (14%) from 2009 to 2011, 7/23 (30%) from 2012 to 2014, and 17/26 (65%) from 2015 to 2017 (p <0.01). The prenatal group more commonly had right aortic arch/left ductus/aberrant left subclavian artery (24/28vs 53/78, p = 0.04) and associated cardiac pathology (18/28vs 33/78, p = 0.05). The rate of genetic anomalies was overall higher than previously reported (34%) and did not differ between groups (11/28vs 25/78, p = 0.48). Those with a prenatal diagnosis were less likely to require cross-sectional imaging (9/28vs 48/78, p <0.01), modifying the vascular ring subtype diagnosis in 2 patients. Surgical intervention was common and did not differ between groups (24/28vs 66/78, p = 0.89). In conclusion, prenatal detection of vascular rings has increased. Despite differences in vascular ring subtype and associated cardiac pathology, the incidence of genetic anomalies and need for surgical intervention is not associated with timing of diagnosis. Genetic counseling should be universally offered. The diagnostic accuracy of echocardiography suggests additional imaging may not be routinely required.

Initial shunt type at the Norwood operation impacts myocardial function in hypoplastic left heart syndrome.

OBJECTIVES: We investigated the impact of initial shunt type, a Blalock-Taussig (BT) shunt versus a right ventricle to pulmonary artery conduit (RV-PA) on myocardial function at different stages of surgical palliation in patients with hypoplastic left heart syndrome (HLHS). METHODS: A population-based cohort of 63 Finnish children with HLHS (BT n = 23, RV-PA n = 40) born between 2003 and 2010 were studied retrospectively by echocardiography prior to Stages 1, 2 and 3 palliation and 0.5-3 years after Stage 3. For comparison of systolic myocardial function, we evaluated the RV fractional area change (FAC), strain, strain rate and mechanical synchrony from the apical 4-chamber view by velocity vector imaging. RESULTS: There were no intergroup differences in demographics during the study period. At baseline, no intergroup differences were detected in RV systolic myocardial function. Before Stage 2, RV FAC was higher ( P = 0.03) in the RV-PA conduit group. At Stage 3, an increase in all systolic myocardial functional parameters was observed in the BT shunt group. After Stage 3, the BT shunt group had better RV systolic function. In multiple regression analysis, the shunt type and the stage of palliation had an impact on myocardial function. CONCLUSIONS: Although patients with HLHS initially palliated with a BT shunt demonstrate lower RV FAC after Stage I, RV FAC improves after Stage 2 with better systolic performance after Stage 3 compared with those initially palliated with an RV-PA conduit.

Right Atrial Dysfunction in the Fetus with Severely Regurgitant Tricuspid Valve Disease: A Potential Source of Cardiovascular Compromise.

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.

Diagnosis and treatment of fetal cardiac disease: a scientific statement from the American Heart Association.

BACKGROUND: The goal of this statement is to review available literature and to put forth a scientific statement on the current practice of fetal cardiac medicine, including the diagnosis and management of fetal cardiovascular disease. METHODS AND RESULTS: A writing group appointed by the American Heart Association reviewed the available literature pertaining to topics relevant to fetal cardiac medicine, including the diagnosis of congenital heart disease and arrhythmias, assessment of cardiac function and the cardiovascular system, and available treatment options. The American College of Cardiology/American Heart Association classification of recommendations and level of evidence for practice guidelines were applied to the current practice of fetal cardiac medicine. Recommendations relating to the specifics of fetal diagnosis, including the timing of referral for study, indications for referral, and experience suggested for performance and interpretation of studies, are presented. The components of a fetal echocardiogram are described in detail, including descriptions of the assessment of cardiac anatomy, cardiac function, and rhythm. Complementary modalities for fetal cardiac assessment are reviewed, including the use of advanced ultrasound techniques, fetal magnetic resonance imaging, and fetal magnetocardiography and electrocardiography for rhythm assessment. Models for parental counseling and a discussion of parental stress and depression assessments are reviewed. Available fetal therapies, including medical management for arrhythmias or heart failure and closed or open intervention for diseases affecting the cardiovascular system such as twin-twin transfusion syndrome, lung masses, and vascular tumors, are highlighted. Catheter-based intervention strategies to prevent the progression of disease in utero are also discussed. Recommendations for delivery planning strategies for fetuses with congenital heart disease including models based on classification of disease severity and delivery room treatment will be highlighted. Outcome assessment is reviewed to show the benefit of prenatal diagnosis and management as they affect outcome for babies with congenital heart disease. CONCLUSIONS: Fetal cardiac medicine has evolved considerably over the past 2 decades, predominantly in response to advances in imaging technology and innovations in therapies. The diagnosis of cardiac disease in the fetus is mostly made with ultrasound; however, new technologies, including 3- and 4-dimensional echocardiography, magnetic resonance imaging, and fetal electrocardiography and magnetocardiography, are available. Medical and interventional treatments for select diseases and strategies for delivery room care enable stabilization of high-risk fetuses and contribute to improved outcomes. This statement highlights what is currently known and recommended on the basis of evidence and experience in the rapidly advancing and highly specialized field of fetal cardiac care.

Effectiveness of prenatal screening for congenital heart disease: assessment in a jurisdiction with universal access to health care.

BACKGROUND: Neonates with certain forms of severe congenital heart disease (CHD) diagnosed prenatally might have better outcomes in comparison with those diagnosed after birth. The proportion of prenatally detected neonates with severe CHD and the effect of prenatal diagnosis on clinical outcomes have not been previously investigated in Canada. METHODS: We retrospectively studied infants in Alberta, Canada, who required surgical or catheter intervention for CHD at younger than 1 year of age, between January 2007 and December 2010, and pregnancy terminations affected by CHD. RESULTS: Of the 374 subjects identified (327 infants, 47 pregnancies with termination), 188 (50%) were detected prenatally. Failure of prenatal diagnosis was associated with anomalies not involving the 4-chamber view on ultrasound (odds ratio, 1.86; 95% confidence interval, 1.48-2.35; P < 0.001) and region of residence (P = 0.04). Prenatal detection was associated with fewer days to hospital admission (P < 0.001), fewer days to surgery (P = 0.003), and greater use of prostaglandins (P = 0.001). Infants diagnosed prenatally who underwent surgery within 15 days of age had higher preductal O2 saturations (P = 0.04), fewer days to admission (P = 0.03), and less frequently required preoperative intubation (P = 0.004), and inotropes (P = 0.001). Pregnancy termination occurred among 49% of fetuses detected before 24 weeks' gestation. CONCLUSIONS: Only 50% of fetuses and/or neonates with severe CHD managed in Alberta have a prenatal diagnosis. The likelihood of prenatal detection is influenced by the status of the 4-chamber view on ultrasound and the region of maternal residence indicating heterogeneous access to fetal echocardiography within Alberta. Prenatal detection might improve clinical outcomes for neonates with severe CHD.

Can a complete fetal echocardiogram be performed at 12 to 16 weeks' gestation?

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). METHODS: Fetal echocardiograms obtained in pregnancies studied at <17 weeks at the University of California, San Francisco, over a 5-year period were retrospectively reviewed. FE was considered complete if anatomic details could be assessed (systemic and pulmonary venous connections and atrial, ventricular, and septal [four-chamber sweeps], outflow and great artery, branch pulmonary artery, and arch anatomy) and if color and pulsed Doppler evaluations of the inferior vena cava, pulmonary veins, ventricular inflows and outflows, umbilical artery and vein, and ductus venosus were demonstrated. RESULTS: One hundred thirty-nine pregnancies were assessed by early FE at <17 weeks transabdominally during the study period (median gestational age, 14.0 weeks; range, 12-0/7-16-6/7 weeks). Additional transvaginal imaging was performed in 14 of 139 (10%) of early fetal echocardiographic studies. One hundred thirteen pregnancies were assessed using both early and later, standard (>17 weeks) FE. Of these, complete fetal echocardiograms were obtained in 27 early (24%; 95% confidence interval [CI], 17%-33%) and 76 later (67%; 95% CI, 58%-75%) exams. In most early exams, color and pulsed Doppler interrogation of the pulmonary veins was unsuccessful. If pulmonary vein Doppler assessment was excluded, complete studies were performed in 80 early exams (71%; 95% CI, 62%-78%) and 97 standard midtrimester exams (86%; 95% CI, 78%-91%). On early FE, heart disease was suspected in 20 pregnancies, and although no major congenital heart disease was missed, in four pregnancies, ventricular septal defects were found only on later FE or after birth. CONCLUSIONS: Early FE yields nearly complete information (exclusive of pulmonary venous interrogation) in the majority of patients.

Neurodevelopmental outcomes after open heart operations before 3 months of age.

BACKGROUND: The purpose of this study was to monitor developmental progress and identify predictors of developmental outcomes at 2 years after operation in infants who underwent a surgical procedure with cardiopulmonary bypass (CPB) at less than 3 months of age. METHODS: Patients (N=131 enrolled; N=106 assessed) less than 3 months of age at the time of cardiac operation were prospectively enrolled (years 1999-2003) and assessed at 8, 12, and 24 months after operation. Patients with preexisting conditions independently associated with poor neurodevelopmental outcomes were excluded. Fine and gross motor development was formally assessed at all 3 visits, and parent ratings of development across several domains were obtained. Neurodevelopment was formally assessed at 24 months of age using the Bayley Scales of Infant Development, 2nd edition (BSID-II) Mental Development Index score (MDI). RESULTS: Significant gross motor difficulties were identified at 8 months of age (p<0.001) and, although improved by the 24-month assessment, remained lower than average. Fine motor skills showed a significant decrease from 8 to 24 months of age (p=0.001). Factors associated with poorer neurodevelopmental outcome (BSID-II MDI) at 24 months after operation included a diagnosis of univentricular anatomy or complex coarctation of the aorta, higher complexity of the surgical procedure, longer duration of hospital stay, and presence of complications in the postoperative period. CONCLUSIONS: Children undergoing repair of congenital heart disease (CHD) still have impaired development 2 years after the operation. Observed patterns of development were specific to the skill being assessed and related to both anatomic complexity and increased complexity of care received.

Progression of outflow tract obstruction in the fetus.

Fetal ventricular outflow tract obstruction (OTO) is congenital heart disease with significant potential for progression before birth as a consequence of the unique nature of the fetal circulation. The pattern of evolution depends upon the timing of development, severity of obstruction and the influence of the OTO on the fetal atrioventricular valve and myocardial function. Critical aortic (AS) or pulmonary (PS) valve stenosis, the two most common forms of fetal OTO, may be associated with progressive ventricular and great artery hypoplasia if presenting early in gestation or with normal ventricular and great artery growth if evolving later in gestation. In some affected fetuses, AS or PS may lead to the evolution of fetal heart failure. This article will review our current understanding of the natural history of fetal AS and PS, experience with fetal intervention and future directions of research.

Left ventricular aneurysm in a child with severe combined immunodeficiency syndrome.

We describe the case of a 23-month-old girl with combined immunodeficiency syndrome and a left ventricular aneurysm. Due to the size of the aneurysm and development of an intramural thrombus, repair was performed after confirmation of bone marrow transplant engraftment. Endoventricular circular patch plasty (Dor procedure) was performed, with excellent outcome and normalization of ventricular geometry and function.

The role of fetal echocardiography in fetal intervention: a symbiotic relationship.

In this review, the authors explore the role of noninvasive and invasive fetal interventions in fetal cardiovascular disease guided by observations at fetal echocardiography. They first review fetal cardiac lesions that may be ameliorated by fetal intervention and then review noncardiac fetal pathologic findings for which fetal echocardiography can provide important insight into the pathophysiology and aid in patient selection for and timing of intervention and postintervention surveillance.

Hypersensitivity myocarditis presenting as atrioventricular block and wide complex tachycardia in a toddler.

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.

Outcome of fetuses and infants with double inlet single left ventricle.

Double-inlet left ventricle (DILV) includes a diverse range of anatomic variables that affect the surgical strategy. The aim of this study was to determine the impact of anatomic subtype, associated anomalies, and fetal diagnosis on the management and outcomes of fetuses and infants with DILV. The outcomes of fetuses and infants with DILV diagnosed from 1990 to 2004 at 3 major referral centers were reviewed. Sixty-five cases of DILV were detected prenatally. Twenty-one of these pregnancies were terminated, including 17 of 37 (46%) in which the diagnoses were made at < or =24 weeks of gestation. An additional 106 patients were diagnosed with DILV within the first 3 months of life. The percentage of patients diagnosed prenatally increased significantly over the study period. Transplantation-free survival was 88%, 82%, 79%, and 76% at 1 month, 1 year, 5 years, and 10 years, respectively. Factors associated with improved survival in univariate analysis included year of birth after 1994, no neonatal Norwood or Damus procedure, and no neonatal surgery at all. In multivariate analysis, any neonatal surgery was the only factor associated with worse survival. Associated anomalies and prenatal diagnosis were not associated with postnatal outcome. In conclusion, although the frequency of prenatal diagnosis of DILV has increased significantly over the past 15 years, prenatal diagnosis is not associated with better postnatal survival. During this same period, postnatal survival has improved substantially. Neonatal surgery of any type was the only independent risk factor for worse survival.

Neonatal mitral and tricuspid valve repair for in utero papillary muscle rupture.

BACKGROUND: Papillary muscle rupture in the fetus and neonate is a rare event that leads to severe mitral or tricuspid insufficiency and is associated with high perinatal mortality. We undertook surgical repair of this lesion in the neonatal period and report on our midterm results. METHODS: Three neonates with tricuspid insufficiency and 1 infant with mitral insufficiency, all due to papillary muscle or chordal rupture, underwent surgical repair with artificial chordal replacement and a modification of the de Vega annuloplasty technique that allowed external adjustment of the annulus size under transesophageal echocardiographic guidance after separation from cardiopulmonary bypass. RESULTS: All patients recovered well from the operation. There have been no late deaths and no valve-related complications. On discharge, all 3 patients had evidence of trace to mild atrioventricular valve regurgitation. At a median follow-up of 33 months (range, 7 to 50; 123 patient-months), all 4 patients are growing normally. Three patients have had no change in the degree of tricuspid or mitral regurgitation. One patient required reoperation at 54 months postoperatively for acute mitral insufficiency secondary to separation of an artificial chorda from the ventricular wall. CONCLUSIONS: Surgical repair of critical neonatal tricuspid and mitral insufficiency associated with papillary muscle or chordal rupture is feasible and can result in good early and midterm results. Our modification of the De Vega annuloplasty technique with the ability to externally adjust the size of the annulus under echocardiographic guidance may improve the accuracy of the repair in the neonate.

Impact of selective laser ablation of placental anastomoses on the cardiovascular pathology of the recipient twin in severe twin-twin transfusion syndrome.

OBJECTIVES: We investigated the impact of selective laser ablation on the cardiovascular pathology of the recipient twin in twin-twin transfusion syndrome. STUDY DESIGN: Fetal echocardiograms and medical records were reviewed from 22 pregnancies with severe twin-twin transfusion syndrome where echocardiography was performed before and after laser. RESULTS: Before laser, cardiomegaly associated with right and/or left ventricular hypertrophy without ventricular dilatation, was observed in most cases. Right ventricular and left ventricular systolic dysfunction (shortening fraction <28%) was present in 59% and 27%, respectively, and diastolic dysfunction (based on inflow and venous Dopplers) in 73%. Shortly after laser, biventricular systolic function improved significantly and diastolic function tended to improve (50%, P = .06). Functional pulmonary atresia, secondary to right ventricular systolic dysfunction, resolved in 2 of 2 cases at post-laser echocardiography. On serial assessment, diastolic function was normal in 7 of 10, hydrops regressed in 4 of 5, and neither progressive myocardial hypertrophy nor anatomical right ventricular outflow obstruction were found. CONCLUSIONS: Selective laser ablation in severe twin-twin transfusion syndrome acutely improves biventricular systolic function and tends to improve diastolic function in the recipient twin.