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Objectives The goals of this study were to: 1) evaluate the cardiac magnetic resonance (CMR) characteristics of pediatric cardiac masses from a large international cohort; 2) test the accuracy of previously developed CMR-based diagnostic criteria; and 3) expand diagnostic criteria using new information. Background After diagnosis of a cardiac mass, clinicians must weigh the benefits and risks of ascertaining a tissue diagnosis. Limited data are available on the accuracy of previously developed noninvasive pediatric CMR-based diagnostic criteria. Methods CMR studies (children 0-18 years of age) with confirmatory histological and/or genetic diagnosis were analyzed by 2 reviewers, without knowledge of prior diagnosis. Diagnostic accuracy was graded as: 1) single correct diagnosis; 2) correct diagnosis among a differential; or 3) incorrect diagnosis. Results Of 213 cases, 174 (82%) had diagnoses that were represented in the previously published diagnostic criteria. In 70% of 174 cases, both reviewers achieved a single correct diagnosis (94% of fibromas, 71% of rhabdomyomas, and 50% of myxomas). When ≤2 differential diagnoses were included, both reviewers reached a correct diagnosis in 86% of cases. Of 29 malignant tumors, both reviewers indicated malignancy as a single diagnosis in 52% of cases. Including ≤2 differential diagnoses, both reviewers indicated malignancy in 83% of cases. Of 6 CMR sequences examined, acquisition of first-pass perfusion and late gadolinium enhancement were independently associated with a higher likelihood of a single correct diagnosis. Conclusions CMR of cardiac masses in children leads to an accurate diagnosis in most cases. A comprehensive imaging protocol is associated with higher diagnostic accuracy.

Background In patients with hypoplastic subpulmonary ventricles, the one and one-half ventricle (1.5V) repair is an alternative to the Fontan procedure. However, in 1.5V-treated patients with pulsatile pulmonary blood flow, superior vena cava (SVC) hypertension or right atrial hypertension may develop. This study aimed to (1) describe patient outcomes after 1.5V repair and (2) determine whether pulmonary artery septation at 1.5V repair confers a lower risk of SVC or right atrial hypertension. Methods This study retrospectively reviewed patients who underwent a 1.5V repair between 1989 and 2020. The primary outcome was transplant-free survival. Secondary outcomes were postoperative SVC hypertension (defined by mean Glenn pressures greater than 17 mm Hg, SVC flow reversal or pulsatility, venovenous collateral vessels, or SVC syndrome) and right atrial hypertension (defined as mean right atrial pressures greater than 10 mm Hg with inferior vena cava and hepatic vein dilation or flow reversal). Results A total of 74 patients underwent 1.5V repair at a median age of 29.6 months (interquartile range, 8.9 to 45.5 months). Median follow-up time was 39.9 months (interquartile range, 11.4 to 178.1 months). Transplant-free survival at 10 years was 92.4%. Among survivors, 12% (8 of 69) had right atrial hypertension and 39% (27 of 69) had SVC hypertension on follow-up. Survivors with unseptated pulmonary arteries had a greater risk of SVC hypertension compared with patients with septated pulmonary arteries (44% vs 10%; P = .04). No difference was found in right atrial hypertension between the 2 groups. Conclusions Patients with 1.5V repair avoid Fontan-associated complications with favorable transplant-free survival. However, SVC hypertension remains a significant long-term complication. Pulmonary artery septation at 1.5V repair may reduce the risk of SVC hypertension.

BackgroundVentricular dyssynchrony and its relationship to clinical outcomes is not well characterized in patients following Fontan palliation.MethodsSingle-center retrospective analysis of cardiac magnetic resonance (CMR) imaging of patients with a Fontan circulation and age-matched healthy comparison cohort as controls. Feature tracking was performed on all slices of a ventricular short-axis cine stack. Circumferential and radial strain, strain rate, and displacement were measured; and multiple dyssynchrony metrics were calculated based on timing of these measurements (including standard deviation of time-to-peak, maximum opposing wall delay, and maximum base-to-apex delay). Primary endpoint was a composite measure including time to death or heart transplant listing (D/HTx); secondary outcomes were the presence of atrial or ventricular arrhythmias.ResultsA total of 503 cases (15y; IQR 10, 21) and 42 controls (16y; IQR 11, 20) were analyzed. Compared to controls, Fontan patients had increased dyssynchrony metrics, longer QRS duration, larger ventricular volumes, and worse systolic function. Dyssynchrony metrics were higher in patients with right ventricular (RV) or mixed morphology compared to those with LV morphology. At median follow-up of 4.3 years, 11% had D/HTx, 7% ventricular arrhythmia, and 38% atrial arrhythmia. Multiple risk factors for D/HTx were identified, including RV morphology, ventricular dilation, dysfunction, QRS prolongation, and dyssynchrony. Ventricular dilation and RV morphology were independently associated with D/HTx; ventricular dilation and global circumferential strain were independently associated with ventricular and atrial arrhythmias.ConclusionsMechanical dyssynchrony is highly prevalent in functional single ventricles palliated to the Fontan circulation and is more pronounced in hearts with RV or mixed ventricular morphology compared to those with LV morphology. Dyssynchrony is associated with death or need for heart transplantation and cardiac arrhythmias. These data add to the growing understanding regarding factors that can be used to risk-stratify patients with the Fontan circulation.

Objectives The Super Glenn procedure involves targeted increased in blood flow to left sided heart structures with fenestrated atrial septation. The objective of this study was to examine the outcomes of patients who had this procedure as a part of biventricular staging and specifically evaluate the effect on dimensions of left heart structures. Methods Data for patients who had this procedure between 2005 and 2019 were retrospectively identified. Results Thirty-seven patients were identified. Most common diagnosis was hypoplastic left heart syndrome in 40% (n = 15). On echocardiography, the median mitral valve z score was −2.26. On cardiac magnetic resonance imaging, median indexed left ventricular end-diastolic volume was 31.5 ml/m2 and mitral/tricuspid inflow ratio was 0.35. The median age at Super Glenn was 2.3 years (interquartile range 1.5–3.6) while median weight was 12 kg (interquartile range 9.8–14). There were no early/hospital deaths. The median intensive care unit length of stay was 4 days, and median hospital length of stay was 10 days. Median follow-up for the entire cohort was 3 years (range 15 days to 13.2 years). There was a statistically significant increase in indexed left ventricular dimensions. There were 5 deaths (14%). Three patients (8%) underwent heart transplant. Freedom from death/transplant was 79% at 5 years. Seven patients (19%) needed a reoperation. Twenty-three patients (62%) underwent biventricular conversion after a median of 11.3 months after Super Glenn. Conclusions The Super Glenn procedure achieves consistent increase in left ventricular dimensions. This may be a useful strategy to help achieve a successful biventricular circulation in patients with borderline left ventricle. The superiority/non-inferiority of this approach over the conventional Fontan pathway is unclear.

Cardiac tumors in children are rare and the majority are benign. The most common cardiac tumor in children is rhabdomyoma, usually associated with tuberous sclerosis complex. Other benign cardiac masses include fibromas, myxomas, hemangiomas, and teratomas. Primary malignant cardiac tumors are exceedingly rare, with the most common pathology being soft tissue sarcomas. This paper provides consensus-based imaging recommendations for the evaluation of patients with cardiac tumors at diagnosis and follow-up, including during and after therapy.

After diagnosis of a cardiac mass, clinicians must weigh the benefits and risks of ascertaining a tissue diagnosis. Limited data are available on the accuracy of previously developed noninvasive pediatric cardiac magnetic resonance (CMR)-based diagnostic criteria.The goals of this study were to: 1) evaluate the CMR characteristics of pediatric cardiac masses from a large international cohort; 2) test the accuracy of previously developed CMR-based diagnostic criteria; and 3) expand diagnostic criteria using new information.CMR studies (children 0-18 years of age) with confirmatory histological and/or genetic diagnosis were analyzed by 2 reviewers, without knowledge of prior diagnosis. Diagnostic accuracy was graded as: 1) single correct diagnosis; 2) correct diagnosis among a differential; or 3) incorrect diagnosis.Of 213 cases, 174 (82%) had diagnoses that were represented in the previously published diagnostic criteria. In 70% of 174 cases, both reviewers achieved a single correct diagnosis (94% of fibromas, 71% of rhabdomyomas, and 50% of myxomas). When ≤2 differential diagnoses were included, both reviewers reached a correct diagnosis in 86% of cases. Of 29 malignant tumors, both reviewers indicated malignancy as a single diagnosis in 52% of cases. Including ≤2 differential diagnoses, both reviewers indicated malignancy in 83% of cases. Of 6 CMR sequences examined, acquisition of first-pass perfusion and late gadolinium enhancement were independently associated with a higher likelihood of a single correct diagnosis.CMR of cardiac masses in children leads to an accurate diagnosis in most cases. A comprehensive imaging protocol is associated with higher diagnostic accuracy.

Background: Surgical resection of cardiac fibromas in children reduces hemodynamic and arrhythmia burden; however, little is known about postoperative left ventricular (LV) function. We aimed to evaluate factors associated with postoperative LV dysfunction. Methods: In this retrospective observational cohort study, imaging data were reviewed from 41 patients who had undergone surgical resection of a cardiac fibroma. Tumor volume was indexed to body surface area (tumor volume index). Right ventricular tumors were excluded from analysis of postoperative ventricular function. Postoperative regional wall motion abnormality score was defined as number of wall segments with regional wall motion abnormality, and LV dysfunction was defined as LV ejection fraction Results: Of 41 patients who underwent resection at a median age of 2.1 years (range, 0.5–19), 37 fibromas were in the LV, (29 free wall and 8 septal), and 4 in the right ventricle. Preoperative median tumor volume index was 66 mL/m 2 (range, 11–376). Of 37 patients with LV tumors, younger patients had larger tumor volume index and higher grades of preoperative mitral regurgitation ( P P 2 , P =0.011), with decreased LV ejection fraction (mean 60% versus 55%, P =0.014), a higher prevalence of low global circumferential strain (36% versus 64%, P =0.045), and decreased cardiac index (mean 4.8 versus 3.9 L/[min·m 2 ], P =0.039). More than mild preoperative mitral regurgitation was the only independent predictor of predischarge LV dysfunction (odds ratio, 22 [95% CI, 2.8–179], P =0.008). Conclusions: Surgical resection of LV fibroma is associated with regional wall motion abnormality, increased LV volume, and reduced systolic function. Children with significant preoperative mitral regurgitation are at highest risk for LV dysfunction and warrant ongoing close surveillance.

Hypothesis: Biventricular conversion (BiVC) following takedown of Fontan circulation is feasible and results in improved hemodynamics. Methods: Retrospective analysis of patients who had takedown of Fontan circulation and conversion to BiV circulation at a single center from September 2007 to April 2020. Failing Fontan physiology was defined as Fontan circulation pressure >15 mmHg. Results: There were 23 patients (median age: 10.0 (7.5-13.0) years); 15 (65%) had failing Fontan physiology and 8 (35%) underwent elective takedown of their Fontan circulation. Of the 15 patients with failing Fontan physiology, 4 had exercise intolerance or cyanosis, 3 had hepatic congestion or cirrhosis, 3 had end-organ damage and 1 patient had protein losing enteropathy; the rest 4 patients had no other sign of SVP complications. A subset of patients (n=6) underwent recruitment of the non-dominant ventricle prior to conversion. HLHS (p Conclusions: BiVC is feasible in patients with failing Fontans, and has promising outcomes after elective takedown of Fontan circulation. A staged approach for ventricular recruitment does not seem inferior to primary BiVC. The optimal timing for BiVC in Fontan patients needs further evaluation.

To assess the feasibility and outcomes of biventricular conversion following takedown of Fontan circulation.Retrospective analysis of patients who had takedown of Fontan circulation and conversion to biventricular circulation at a single center from September 2007 to April 2020. Failing Fontan physiology was defined as Fontan circulation pressure15 mm Hg and/or the presence of associated complications.Biventricular conversion was performed in 23 patients at a median age of 10.0 (7.5-13.0) years. Indications included failing Fontan physiology in 15 (65%) and elective takedown in 8 (35%) patients. A subset of patients (n = 6) underwent procedures for staged recruitment of the nondominant ventricle before conversion. Median z score of end-diastolic volume of borderline ventricle before takedown was -2.3 (-3.3, -1.3). Hypoplastic left heart syndrome (P .01) and sub-/aortic stenosis (P .01) were more common in these patients. Biventricular conversion with or without staged ventricular recruitment led to a significant increase in indexed end-diastolic volume (P .01), indexed end-systolic volume (P .01), and ventricular mass (P .01) of the nondominant ventricle (14 right, 9 left ventricle). There were 5 (22%) deaths (1 [4%] early death). All who underwent elective biventricular conversion survived, whereas 2-year survival rate for patients with a failing Fontan circulation was 72.7% (95% confidence interval, 37%-90%). The overall, 3-year reoperation-free survival was 86.7% (95% confidence interval, 56%-96%). Left dominant atrioventricular canal defect (P .01) and early era of biventricular conversion (P = .02) were significant predictors for mortality.A primary as well as a staged biventricular conversion is feasible in patients who have had previous Fontan procedure. Although this provides an alternative to transplantation in patients with failing Fontan, outcomes are worse in those with failing Fontan compared with elective takedown of Fontan circulation. Optimal timing needs further evaluation.

Background Stillbirth, defined as death of a fetus in utero after 20 weeks of gestation, occurs in 1 to 2% of pregnancies in the United States. Many of these stillborn infants have associated malformations, including chromosome abnormalities, neural tube defects, and malformation syndromes. Other causes are abnormalities of the placenta and maternal conditions, such as pre-eclampsia and obesity. A consecutive sample of malformed stillborn infants can establish the relative frequency and severity of the associated malformations. Methods Stillbirths were identified in the Active Malformations Surveillance Program at Brigham and Women's Hospital (1972-2012). The findings at autopsy, including the findings in the placenta and the results of diagnostic studies, were compiled. Results One hundred twenty-seven stillborn infants with malformations were identified at autopsy among 289,365 pregnancies, including trisomies 21, 18, and 13; 45,X; triploidy; anencephaly; lower urinary tract obstruction; holoprosencephaly and severe heart defects, such as hypoplastic left heart syndrome and tetralogy of Fallot with pulmonary atresia. The severity of the abnormalities in stillborn infants was more severe than the spectrum of abnormalities identified in live-born infants. Conclusion An autopsy of the stillborn fetus, including chromosome microarray and an examination of the placenta, can identify the underlying causes of the stillbirth. This review of stillborn fetuses with malformations showed that several different lethal malformations and heart defects are more common than among live-born infants. These postmortem examinations can improve the counseling of the parents about risks in future pregnancies. Birth Defects Research 110:114-121, 2018.© 2018 Wiley Periodicals, Inc.

Background In addition to tricuspid regurgitation (TR) and right ventricular (RV) enlargement, patients with Ebstein anomaly are at risk for left ventricular (LV) dysfunction and dyssynchrony. We studied the impact of the cone tricuspid valve reconstruction operation on LV size, function, and dyssynchrony. Methods All Ebstein anomaly patients who had both pre- and postoperative cardiovascular magnetic resonance (CMR) studies were retrospectively identified. From cine images, RV and LV volumes and ejection fractions (EF) were calculated, and LV circumferential and longitudinal strain were measured by feature tracking. To quantify LV dyssynchrony, temporal offsets (TOs) were computed among segmental circumferential strain versus time curves using cross-correlation analysis and patient-specific reference curves. An LV dyssynchrony index was calculated as the standard deviation of the TOs. Results Twenty patients (65% female) were included with a median age at cone operation of 16 years, and a median time between pre- and postoperative CMR of 2.8 years. Postoperatively, there was a decline in the TR fraction (56 ± 19% vs. 5 ± 4%, p

Cardiovascular magnetic resonance (CMR) plays an important role in the evaluation of children and adults with congenital heart disease. It provides complementary information to echocardiography and cardiac catheterization, and has unique diagnostic capabilities. Accordingly, data from CMR are increasingly being incorporated into disease management algorithms. This article critically reviews recently published reports related to pediatric and congenital CMR with sections on new techniques, congenital heart disease lesions, and cardiomyopathy.

Cardiac MRI can predict the likely tumor type in the majority of children with a cardiac mass. A comprehensive imaging protocol is essential for accurate diagnosis. However, histologic diagnosis remains the gold standard, and in some cases malignancy cannot be definitively excluded on the basis of cardiac MRI images alone.

Background— Multiple echocardiographic parameters have been identified to predict the severity of aortic regurgitation (AR) with variable reliability. This study was performed to identify which echocardiographic parameters best predict the severity of AR in a cohort of patients with congenital heart disease, using cardiovascular MRI quantification as a reference standard. Methods and Results— The study involved 2 phases. In phase 1, predictive models were developed on the basis of multivariable analysis of various morphometric and Doppler variables obtained from 174 echocardiograms that best predicted the severity of AR as defined by paired cardiovascular MRI examinations. A nonlinear estimate of regurgitation fraction, using the variables parasternal vena contracta-derived area divided by body surface area and abdominal aorta Doppler retrograde velocity-time integral divided by antegrade velocity-time integral, was identified through multivariable analysis as the best predictive model for AR fraction. In phase 2, the predictive models were prospectively tested on 43 echocardiographic examinations for which a paired cardiovascular MRI was performed. The agreement between the observed and predicted AR fraction was assessed using Bland-Altman analysis. For the 30 studies of the validation data set that had adequate quality images of both the parasternal vena contracta width and the abdominal aorta flow profile, the predicted AR values had a mean bias±SD of 0.4±7.3% ( P =0.80). Conclusions— A model using the 2 variables parasternal vena contracta-derived area divided by body surface area and abdominal aorta Doppler retrograde velocity-time integral divided by antegrade velocity-time integral can predict AR severity in patients with a wide variety of congenital heart disease.

To evaluate the feasibility of dobutamine stress magnetic resonance (DSMR) in pediatric patients.The medical records of all DSMR studies performed on patientsor =22 years old at a single institution were retrospectively reviewed. The DSMR protocol included dobutamine doses up to 40 microg/kg/minute and atropine to attain the target heart rate [0.85 . (220 - age)].Thirty-two DSMR studies were performed in 28 patients (median age = 7.3 years; range = 0.8-22 years). Twenty of the studies were performed under general anesthesia. The protocol was completed in 26 studies, technical problems and interruptions were few, and image quality scores (1-5) for all ventricular wall segments were high (mean = 4.2). A heart rateor =160 bpm was attained in 84% of the studies, a rate pressure productor =20,000 beats . mm Hg in 87%, and a heart rate greater than or equal to the target heart rate in 19%. No serious adverse events occurred. One patient had an inducible wall motion abnormality. Interobserver agreement was 100% (kappa = 1.0) for test positivity and 92% (kappa = 0.72) for wall motion scores.DSMR in pediatric patients is feasible and provides high-quality imaging of all ventricular wall segments with low interobserver variability. Further exploration of DSMR in pediatric patients is warranted, particularly for those children who are unable to cooperate sufficiently for exercise stress or have poor acoustic windows.

We describe 5 patients who presented with musculoskeletal abnormalities in the neonatal period. All patients were initially suspected to have Larsen syndrome or Beals syndrome but were subsequently diagnosed with a TGFBR2 mutation diagnostic of Loeys-Dietz syndrome. Patients had progressive aortic enlargement, which necessitated surgical intervention for 3 patients and resulted in the death of 1 patient. Delay in diagnosis of Loeys-Dietz syndrome may be associated with adverse prognosis.

Background: While aortic valve repair (AoVR) is considered the most appropriate therapy in pediatric population, mid/long term outcomes have been disappointing from progressive dysfunction of currently available leaflet substitute material. We aim to compare outcomes of AoVR with autologous

Ebstein anomaly is a developmental abnormality of the tricuspid valve and right ventricle that results in tricuspid regurgitation and right heart enlargement. Because of the variation in clinical severity and associated findings, patients require a detailed, well-tailored evaluation. For these reasons, management of adults with Ebstein anomaly should take place in a center with expertise in adult congenital heart disease. In many patients, the decision regarding if and when to perform surgery remains controversial, largely because of a lack of published data demonstrating improved postoperative symptoms and survival compared to the natural history of the disease. Because standard two-dimensional echocardiography and cardiovascular magnetic resonance imaging planes do not provide the necessary data to preoperatively manage patients, comprehensive echocardiography and cardiovascular magnetic resonance imaging protocols by experts trained in congenital heart disease are essential in the preoperative management of patients with Ebstein anomaly. As patients may be unaware of their exercise limitations, and for prognostic value, serial cardiopulmonary exercise stress testing is very useful in the evaluation of Ebstein anomaly patients. Surgical tricuspid valve repair historically has not been highly successful because of the marked distortion of tricuspid valve leaflets and right ventricular pathology. Over the last several years, reports of newer surgical techniques to repair the valve, with concurrent advances in arrhythmia management of patients hold promise for improved long term outcomes of patients with Ebstein anomaly. However, because Ebstein anomaly is rare and tricuspid valve repair remains technically challenging, the newer valve repair techniques have not yet gained widespread acceptance throughout the adult congenital heart disease community.

Objectives To investigate the perinatal outcome of cases with a prenatal diagnosis of single-ventricle cardiac defects, single ventricle being defined as a dominant right ventricle (RV) or left ventricle (LV), in which biventricular circulation was not possible. Methods We reviewed patients with a prenatal diagnosis of single-ventricle cardiac defects, made at one institution between 1995 and 2008. Cases diagnosed with double-inlet LV, tricuspid atresia, pulmonary atresia with intact ventricular septum and severe RV hypoplasia and those with hypoplastic left heart syndrome (HLHS) were included in the study population. Patients with HLHS were identified prenatally as being standard risk or high risk (HLHS with highly restrictive or intact atrial septum, mitral stenosis with aortic atresia and/or LV coronary artery sinusoids). Patients with an address over 200 miles from the hospital, diagnosed with heterotaxy syndrome or referred for fetal intervention, were excluded. Results We identified 312 cases of single-ventricle cardiac defect (208 dominant RV; 104 dominant LV) that were diagnosed prenatally. Most (96%) patients with a dominant RV had HLHS. Among the total 312 cases there were 98 (31%) elective terminations of pregnancy (TOP), 12 (4%) cases of spontaneous fetal demise, 12 (4%) cases lost to prenatal follow-up and 190 (61%) live births. Among the 199 patients that underwent fetal echocardiography before 24 weeks' gestation, there were 97 (49%) cases of elective TOP. There was no difference in prenatal outcome between those with a dominant RV and those with a dominant LV (P = 0.98). Of the 190 live births, five received comfort care. With an average of 7 years' follow-up (to obtain data on the Fontan procedure), transplantation-free survival was lower in those with a dominant RV than in those with a dominant LV (standard-risk HLHS odds ratio (OR), 3.0 (P = 0.01); high-risk HLHS OR, 8.8 (P

The aim of this study was to report the results of an international multicenter experience of cardiac magnetic resonance imaging (MRI) evaluation of cardiac tumors in children, each with histology correlation or a diagnosis of tuberous sclerosis, and to determine which characteristics are predictive of tumor type.Individual centers have relatively little experience with diagnostic imaging of cardiac tumors in children, because of their low prevalence. The accuracy of cardiac MRI diagnosis on the basis of a pre-defined set of criteria has not been tested.An international group of pediatric cardiac imaging centers was solicited for case contribution. Inclusion criteria comprised: 1) age at diagnosis ≤18 years; 2) cardiac MRI evaluation of cardiac tumor; and 3) histologic diagnosis or diagnosis of tuberous sclerosis. Data from the cardiac MRI images were analyzed for mass characteristics. On the basis of pre-defined cardiac MRI criteria derived from published data, 3 blinded investigators determined tumor type, and their consensus diagnoses were compared with histologic diagnoses.Cases (n = 78) submitted from 15 centers in 4 countries had the following diagnoses: fibroma (n = 30), rhabdomyoma (n = 14), malignant tumor (n = 12), hemangioma (n = 9), thrombus (n = 4), myxoma (n = 3), teratoma (n = 2), and paraganglioma, pericardial cyst, Purkinje cell tumor, and papillary fibroelastoma (n = 1, each). Reviewers who were blinded to the histologic diagnoses correctly diagnosed 97% of the cases but included a differential diagnosis in 42%. Better image quality grade and more complete examination were associated with higher diagnostic accuracy.Cardiac MRI can predict the likely tumor type in the majority of children with a cardiac mass. A comprehensive imaging protocol is essential for accurate diagnosis. However, histologic diagnosis remains the gold standard, and in some cases malignancy cannot be definitively excluded on the basis of cardiac MRI images alone.

Background— D-transposition of the great arteries (TGA) with left ventricular outflow tract obstruction (LVOTO) may be treated with arterial switch operation (ASO) with or without LVOT intervention, as well as non-ASO anatomic repairs, such as aortic translocation or Rastelli procedure. We evaluated midterm results of repair for TGA/LVOTO at our institution. Methods and Results— Eighty-eight patients with TGA/LVOTO who underwent anatomic repair were retrospectively reviewed. LVOTO was defined as pulmonary valve (PV) z-score ≤−2.0 or LVOT gradient ≥20 mm Hg in the presence of anatomic subvalvar stenosis. Risk factors for LVOT reintervention were determined by logistic regression. There was no hospital mortality and 1 late mortality. Patients undergoing Rastelli procedure were more likely to require surgical reintervention for LVOTO compared to the other groups ( P =0.015). Patients undergoing ASO alone had a higher rate of late LVOT reintervention compared to those who had concomitant ASO/LVOT intervention ( P =NS). In those undergoing Rastelli, a larger PV z-score was a predictor of LVOT reintervention ( P =0.012). PV z-scores significantly decreased before repair in patients undergoing delayed repair ( P =0.005); however, they increased significantly after neonatal ASO ( P Conclusions— Patients with TGA/LVOTO who undergo Rastelli repair have a high rate of LVOT reintervention. Higher preoperative PV z-score is a risk factor for reintervention in this group. Patients with mild/moderate LVOTO undergoing ASO alone without LVOT intervention may have an increased risk of LVOT reintervention. In neonates who are candidates for ASO, delay of repair is associated with diminution in size of PV, which may subsequently reduce their suitability for ASO.

Double-outlet right atrium is a rare congenital cardiac anomaly previously described in association with atrioventricular canal defect. Straddling tricuspid valve is another uncommon anomaly that is almost always associated with a ventricular septal defect. We report the echocardiographic features of a newborn with double-outlet right atrium associated with pulmonary atresia, intact ventricular septum, and right coronary artery ostial atresia. Alternatively, the anatomy can be interpreted as straddling tricuspid valve with intact ventricular septum. The echocardiographic findings were confirmed by cardiac catheterization.

We describe the case of a 3-year-old child with neonatal Marfan syndrome complicated by mitral valve prolapse with regurgitation, marked aortic root dilatation, and ventricular tachycardia. The patient had resolution of ventricular tachycardia following surgical intervention consisting of a valve-sparing aortic root replacement and mitral valve annuloplasty.

Marfan's syndrome (MS) and bicuspid aortic valves (BAVs) are associated with aortic dilation. Despite their histologic similarities, the 2 diseases differ with regard to the location of maximal aortic dilation. Echocardiographic analysis of aortic dimensions was performed in children with MS, children who had aortic dilation in the setting of nonstenotic BAVs (peak gradient