Persistent Left Superior Vena Cava and Inferior Vena Cava Dual Drainage to Coronary Sinus: A Case Report.

Dilation of the coronary sinus is often a result of excessive volume overload from congenital anomalies of systemic venous return to the heart. These abnormalities are often discovered incidentally later in life when a patient requires cardiac imaging, cardiac catheterization, or thoracic surgery. The most common abnormality is a persistent left superior vena cava. Inferior vena cava malformation is less common, yet several different anomalies can arise. The presence of persistent left superior vena cava or inferior vena cava anomalies requires further evaluation to rule out congenital heart disease in infants. Knowledge of technically challenging systemic venous anatomy is beneficial prior to procedures necessitating central venous access such as a central line, cardiac catheterization, and intracardiac device implantation. We present an unusual case of persistent LSVC and IVC both draining directly into a severely dilated coronary sinus that was diagnosed by fetal echocardiogram and later confirmed postnatally by transthoracic echocardiogram and computed tomography angiography. To our knowledge this is the second reported case of IVC drainage into the CS and the first case that reports this as a prenatal diagnosis.

Palliative balloon pulmonary valvuloplasty for infants with unrestrictive ventricular septal defect or single ventricle associated with severe pulmonary stenosis.

BACKGROUND: Symptomatic infants with unrestrictive ventricular septal defect (VSD) or single ventricle with severe pulmonary stenosis (PS) are typically treated surgically. High surgical risk infants may benefit from catheter-based palliative pulmonary valvuloplasty. METHODS: We retrospectively reviewed all patients with severe PS associated with unrestrictive VSD or single ventricle who underwent palliative pulmonary valvuloplasty as initial management and describe outcomes. RESULTS: From 2000 to 2013, 16 patients met inclusion criteria and underwent valvuloplasty at a median age and weight of 25 (range 1-352) days and 3.65 (range 2.2-12) kg. Common diagnoses included tetralogy of Fallot (50%) and double-outlet right ventricle (25%). In all, the indication was cyanosis. Reasons to defer surgery included low weight (37.5%), preference for complex operation at older age (31.25%) and significant comorbidity (18.75%). Following valvuloplasty, significant increases in oxygen saturation (83.1±8.8 vs. 74.7±8.7%, P=0.008) and QP:QS (1.6±0.5 vs. 0.8±0.3, P=0.0005) occurred. All two ventricle patients (n=10) ultimately had complete operative repair at a median of 125 (range 27-382) days after valvuloplasty, during which time there was 20.3±9.2 g/day of weight gain without change in oxygen saturation. Single ventricle patients who underwent cavopulmonary connection (n=3) had similar weight gain and palliation time, but with a decrease in oxygen saturation. Two patients required reintervention to augment QP prior to definitive repair. CONCLUSIONS: In select infants with unrestrictive VSD or single ventricle associated with severe PS, palliative pulmonary valvuloplasty provides an effective and durable method of increasing oxygen saturation until definitive surgical treatment can be performed.

Health Equity and Policy Considerations for Pediatric and Adult Congenital Heart Disease Care among Minoritized Populations in the United States.

Achieving health equity in populations with congenital heart disease (CHD) requires recognizing existing disparities throughout the lifespan that negatively and disproportionately impact specific groups of individuals. These disparities occur at individual, institutional, or system levels and often result in increased morbidity and mortality for marginalized or racially minoritized populations (population subgroups (e.g., ethnic, racial, social, religious) with differential power compared to those deemed to hold the majority power in the population). Creating actionable strategies and solutions to address these health disparities in patients with CHD requires critically examining multilevel factors and health policies that continue to drive health inequities, including varying social determinants of health (SDOH), systemic inequities, and structural racism. In this comprehensive review article, we focus on health equity solutions and health policy considerations for minoritized and marginalized populations with CHD throughout their lifespan in the United States. We review unique challenges that these populations may face and strategies for mitigating disparities in lifelong CHD care. We assess ways to deliver culturally competent CHD care and to help lower-health-literacy populations navigate CHD care. Finally, we review system-level health policies that impact reimbursement and research funding, as well as institutional policies that impact leadership diversity and representation in the workforce.

Anomalous Origin of Left Coronary Artery From Right Pulmonary Artery in Association With Scimitar Syndrome.

This paper reports a unique case of anomalous origin of the left coronary artery from the right pulmonary artery associated with scimitar syndrome. The presence of pulmonary hypertension may have contributed to maintain coronary perfusion, which likely prevented early significant coronary steal. This study reports the clinical course, diagnosis challenges, and management strategy. (Level of Difficulty: Intermediate.).

Negative Impact of Obesity on Ventricular Size and Function and Exercise Performance in Children and Adolescents With Repaired Tetralogy of Fallot.

BACKGROUND: Up to 25% of children with congenital heart disease are obese, which may have negative physiologic consequences for patients with repaired tetralogy of Fallot (rTOF). METHODS: Patients with rTOF who underwent cardiac magnetic resonance (CMR) imaging and cardiopulmonary exercise testing from 2007 to 2018 were reviewed. Complex rTOF patients were excluded. Obese patients (body mass index [BMI] ≥ 95th percentile) were compared with normal-weight patients (BMI < 85th percentile). CMR data were indexed to actual body surface area (aBSA), height, and BSA assuming ideal body weight (iBSA). RESULTS: We compared 32 obese patients matched with 64 normal-weight patients. Obese vs normal-weight patients had significantly lower right (RV; median 45% [interquartile range 42%-48%] vs 52% [47%-55%]; P < 0.0001) and left (LV; 52% [47%-56%] vs 56% [54%-60%]; P < 0.0001) ventricular ejection fractions (EFs). There were no statistically significant differences regarding aBSA-indexed volumes of the RV or LV at either end-diastole (EDV) or end-systole (ESV). However, when indexed to either height or iBSA, obese patients had significantly greater RVEDV and LVEDV, greater LV mass, and higher RV and LV stroke volumes. Obese patients had lower peak oxygen consumption and oxygen consumption at anaerobic threshold. These results did not change after adjusting for degree of pulmonary regurgitation. CONCLUSIONS: Obesity is associated with increased biventricular size, decreased biventricular EFs, and impaired exercise performance after rTOF. These data suggest a potential role for cardiac rehabilitation for weight management and to optimize fitness.