Fetal Characteristics and Perinatal Outcomes in Tetralogy of Fallot Without a Ductus Arteriosus.

Absence of the ductus arteriosus (DA) is common in tetralogy of Fallot (TOF), occurring in up to 30% of cases. Yet, the clinical course and fetal echocardiographic features are not well described, limiting prenatal counseling. This study examines the fetal echocardiographic characteristics and perinatal outcomes in children with TOF absent DA (TOF/ADA), comparing them to those with a DA (TOF/DA).

Health-Related Quality of Life After Neonatal Treatment of Symptomatic Tetralogy of Fallot: Insights from the Congenital Cardiac Research Collaborative.

To evaluate the association between initial management strategy of neonatal symptomatic Tetralogy of Fallot (sTOF) and later health-related quality of life (HRQOL) outcomes. We performed a multicenter, cross-sectional evaluation of a previously assembled cohort of infants with sTOF who underwent initial intervention at ≤ 30 days of age, between 2005 and 2017. Eligible patients' parents/guardians completed an age-appropriate Pediatric Quality of Life Inventory, a Pediatric Quality of Life Inventory Cardiac Module Heart Disease Symptoms Scale, and a parental survey.

Echocardiographic Predictors of Readiness for Double Switch Operation and Postoperative Ejection Fraction in Patients With Congenitally Corrected Transposition of the Great Arteries Undergoing Left Ventricular Retraining.

Background: In patients with congenitally corrected transposition of the great arteries (ccTGA), assessment of readiness for the double switch operation (DSO) after pulmonary arterial band (PAB) placement involves cardiac magnetic resonance imaging (cMRI) to measure left ventricular ejection fraction (LVEF) and mass and cardiac catheterization (catheterization) to assess the ratio of left ventricular to right ventricular pressure (LV:RVp). The aims of this study were to describe the relationships between echocardiographic and catheterization and cMRI measures of readiness for DSO and to develop risk factors for left ventricular (LV) dysfunction after DSO on the basis of echocardiographic measures of ventricular-arterial coupling (VAC).

Methods: Patients with ccTGA undergoing LV retraining at a DSO referral center were reviewed.

Left Ventricular Strain, Arch Angulation, and Velocity-Time Integral Ratio Improve Performance of a Clinical Pathway for Fetal Diagnosis of Neonatal Coarctation of the Aorta.

Introduction: Neonatal presentation of coarctation of the aorta (CoA) is a potentially life-threatening condition that is difficult to diagnose in fetal life. We therefore sought to validate and compare novel metrics that may add diagnostic value for fetal CoA, including the diastolic to systolic aortic isthmus VTI ratio (VTId:VTIs), ascending aorta to descending aorta angle (AAo-DAo), transverse aorta to descending aorta angle (TAo-DAo), and LV longitudinal strain (LVS), then to evaluate whether these novel metrics improve specificity to identify fetuses at the highest risk for postnatal CoA without compromising sensitivity.

Methods: Retrospective cohort study of fetuses followed a prospective clinical pathway and previously classified as mild, moderate, or high-risk for CoA based on standard fetal echo metrics.

Abbreviated cardiac magnetic resonance imaging versus echocardiography for interval assessment of systolic function in Duchenne muscular dystrophy: patient satisfaction, clinical utility, and image quality.

Purpose: Poor acoustic windows make interval assessment of systolic function in patients with (Duchenne Muscular Dystrophy) DMD by echocardiography (echo) difficult. Cardiac magnetic resonance imaging (CMR) can be challenging in DMD patients due to study duration and patient discomfort. We developed an abbreviated CMR (aCMR) protocol and hypothesized that aCMR would compare favorably to echo in image quality and clinical utility without significant differences in exam duration, patient satisfaction, and functional measurements.

Short- and Mid-Term Results of Pulmonary Valve Replacement with the Inspiris Valve.

Background: Various bioprosthetic valves are used off-label for pulmonary valve replacement (PVR), but there is no consensus on whether a particular valve is best for this application. Recently, the Inspiris Resilia valve (Edwards Lifesciences Inc) was approved for aortic valve replacement, and surgeons have begun using it for PVR. There is limited evidence on the performance of the Inspiris valve compared with other valves in the pulmonary position.

Unusual Case of Anomalous Coronary Origin and Myocardial Bridge to Right Ventricle Communication.

Anomalous coronary artery origin is rare and can occur with myocardial bridging. We describe a 28-year-old patient with symptomatic anomalous right coronary artery arising from the left sinus of Valsalva with concomitant myocardial bridging of the left anterior descending artery. It was discovered that the left anterior descending artery ran along the free right ventricular wall with trabeculated myocardium composing the bridge.

Impact of Management Strategy on Feeding and Somatic Growth in Neonates with Symptomatic Tetralogy of Fallot: Results from the Congenital Cardiac Research Collaborative.

Objective: To evaluate early growth following primary or staged repair of neonatal symptomatic tetralogy of Fallot (sTOF).

Study Design: We performed a retrospective, multicenter cohort study of consecutive infants with sTOF who underwent initial intervention at age ≤30 days, from 2005 to 2017. Management strategies were either primary repair or staged repair (ie, initial palliation followed by complete repair).

Coarctation of the aorta: Prenatal assessment, postnatal management and neonatal outcomes.

Coarctation of the aorta (Coa) is a potentially life threatening diagnosis. It occurs in 0.3 per 1000 live births and accounts for 6-8% of all infants with congenital heart defects.

Impact of Maternal Hyperoxygenation on Myocardial Deformation and Loading Conditions in Fetuses with and without Left Heart Hypoplasia.

Background: Maternal hyperoxygenation (MHO) is used in a variety of clinical applications, but its impact on fetal cardiovascular physiology is poorly understood. Our aims were to describe the effects of MHO on myocardial deformation parameters and on ultrasound-based metrics of preload and afterload and to assess the differential effect of MHO on fetuses with left heart hypoplasia (LHH). We hypothesized that the effects of MHO would be modulated by loading conditions and that fetuses with LHH would be more sensitive to changes in preload and afterload induced by MHO.

Left Subclavian Artery Isolation with Right Aortic Arch and D-Transposition of the Great Arteries.

• In subclavian artery isolation, a subclavian artery arises from a pulmonary artery. • Subclavian artery isolation with d-TGA has unique findings on physical examination. • Subclavian artery isolation with d-TGA is often a missed diagnosis on echocardiogram.

Maternal Hyperoxygenation Testing in Fetuses with Hypoplastic Left-Heart Syndrome: Association with Postnatal Atrial Septal Restriction.

Introduction: In fetuses with hypoplastic left-heart syndrome (HLHS), maternal hyperoxygenation (MHO) may aid risk stratification. We hypothesized that pulmonary vein (Pvein) velocity time integral (VTI) change with MHO would more reliably identify neonates who undergo emergent atrial septoplasty (EAS) than changes in pulmonary arterial pulsatility index (PA PI).

Methods: Fetuses with HLHS who underwent MHO testing at our institution between 2014 and 2019 were identified.

COVID-19 Vaccination-Associated Myocarditis in Adolescents.

Objectives: In this study, we aimed to characterize the clinical presentation, short-term prognosis, and myocardial tissue changes as noted on cardiovascular magnetic resonance (CMR) or cardiac MRI in pediatric patients with coronavirus disease 2019 vaccination-associated myocarditis (C-VAM).

Methods: In this retrospective multicenter study across 16 US hospitals, patients <21 years of age with a diagnosis of C-VAM were included and compared with a cohort with multisystem inflammatory syndrome in children. Younger children with C-VAM were compared with older adolescents.

A Fetal Risk Stratification Pathway for Neonatal Aortic Coarctation Reduces Medical Exposure.

Objective: To test the hypothesis that a fetal stratification pathway will effectively discriminate between infants at different levels of risk for surgical coarctation and reduce unnecessary medicalization.

Study Design: We performed a pre-post nonrandomized study in which we prospectively assigned fetuses with prenatal concern for coarctation to 1 of 3 risk categories and implemented a clinical pathway for postnatal management. Postnatal clinical outcomes were compared with those in a historical control group that were not triaged based on the pathway.

Durability of Pulmonary Valve Replacement with Large Diameter Stented Porcine Bioprostheses.

There is limited information about durability of large diameter porcine bioprostheses implanted for pulmonary valve replacement (PVR). We studied patients who underwent surgical PVR from 2002-2019 with a stented porcine bioprosthetic valve (BPV) with a labeled size ≥27 mm. The primary outcome was freedom from reintervention.

Postpartum depression in mothers with pregnancies complicated by fetal cardiac anomaly.

Objective: To investigate factors associated with positive postpartum depression (PPD) screen in pregnancies complicated by fetal congenital cardiac anomaly.

Study Design: We reviewed all records of pregnancies complicated by fetal congenital cardiac anomaly receiving prenatal, intrapartum and postpartum care at our single center, October 2016-October 2019. Maternal, obstetric, and neonatal data were compared between women with and without a positive PPD screen at the 6-week postpartum visit.

Comparison of Management Strategies for Neonates With Symptomatic Tetralogy of Fallot.

Background: Neonates with tetralogy of Fallot and symptomatic cyanosis (sTOF) require early intervention.

Objectives: This study sought to perform a balanced multicenter comparison of staged repair (SR) (initial palliation [IP] and subsequent complete repair [CR]) versus primary repair (PR) treatment strategies.

Methods: Consecutive neonates with sTOF who underwent IP or PR at ≤30 days of age from 2005 to 2017 were retrospectively reviewed from the Congenital Cardiac Research Collaborative.