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Congenital aortic valve disease is a life-long condition that can require multiple interventions. It is one of the most common causes of congenital heart defect, with bicuspid aortic valve present in at least 1−2% of the general population. Surgical management of congenital aortic valve disease consists of either valve repair or replacement. While aortic valve replacement using the Ross procedure can be considered the gold standard management in the pediatric population, advancements in aortic valve repair techniques have proved its usefulness as an initial management approach as it prevents prosthesis-related complications and patient-prostheses mismatch while the patient grows. Overall, all techniques have their benefits and limitations in terms of growth potential, durability of repair, freedom from reoperation and anticoagulation, infection risk, and mortality. Each patient will require an individualized judiciously selected management plan to minimize the number of interventions over their lifetime. The aim of this review is to discuss the merits and drawbacks of the major techniques currently used in both aortic valve repair and replacement.

Background: Among the known risk factors for children undergoing surgery for congenital heart defect (CHD), temperature and blood flow during cardiopulmonary bypass (CPB), are two risk factors, which may be altered to improve outcomes. Moderate - to - deep hypothermia (MDH), traditionally used, has been associated with short/long-term neurologic sequelae, so there is a move towards mild hypothermia (MH) with selective antegrade cerebral perfusion (SACP). Aims and Objectives: To assess feasibility of mild hypothermia with selective antegrade cerebral perfusion during cardiopulmonary bypass as a cardiopulmonary bypass strategy in neonates and infants undergoing surgery for congenital heart defect. Materials and Methods: We conducted a retrospective chart review of neonates and infants who underwent SACP on CPB during CHD surgery at our tertiary care children's hospital between January 2014 and February 2017. We categorized all the patients into 2 groups - MH + SACP or MDH + SACP. We gathered data on preoperative, intra-operative and post-operative parameters. We analyzed comparative data using student t-test and chi-squared test with a significant p-value < 0.05. Results: There were 6 in the MH + SACP group and 7 in the MDH + SACP group, who underwent SACP on CPB during study period at our children's hospital. All the neonates and infants in the MH + SACP group survived at the hospital discharge with favorable neurologic outcomes. There was no statistically significant difference between the two groups for end organ dysfunction, ventilator days, hospital days, ICU and hospital mortality and PCPC/POPC at hospital discharge. Conclusions: Mild hypothermia with selective antegrade cerebral perfusion during cardiopulmonary bypass is a feasible cardiopulmonary bypass strategy in neonates and infants undergoing surgery for congenital heart defect.

Heterotaxy is a rare syndrome associated with cardiac complexity, anatomic variability and high morbidity and mortality. It is often challenging to visualize and provide an accurate diagnosis of the cardiac anatomy prior to surgery with the use of conventional imaging techniques. We report a unique case demonstrating how the use of three-dimensional (3D) cardiac printed model allowed us to better understand the anatomical complexity and plan a tailored surgical approach for successful biventricular repair in a patient with heterotaxy syndrome.

Anomalous origin of the left main coronary artery from the noncoronary sinus (LCANS) is a rare variant of anomalous aortic origin of a coronary artery that is not characterized by an interarterial course. Despite the absence of an interarterial course, there are rare reports of LCANS presenting with sudden death, life-threatening arrhythmia, exercise-induced myocardial ischemia, premature ventricular contractions, and syncope. We report a patient with LCANS presenting with acute myocardial infarction who recovered completely with emergent surgical unroofing of the coronary artery.

Objective: Reoperative procedures on the pulmonary valve are not uncommonly encountered in patients with congenital heart disease. For those patients who require cardiac reoperations, the novel INS...

Background The efficacy of extracorporeal membrane oxygenation (ECMO) in bridging children with unrepaired heart defects to a definitive or palliative surgical procedure has been rarely reported. The goal of this study is to report our institutional experience with ECMO used to provide preoperative stabilization after acute cardiac or respiratory failure in patients with congenital heart disease before cardiac surgery. Methods A retrospective review of the ECMO database at Children's Hospital Boston was undertaken. Children with unrepaired congenital heart disease supported with ECMO for acute cardiac or respiratory failure as bridge to a definitive or palliative cardiac surgical procedure were identified. Data collection included patient demographics, indication for ECMO, details regarding ECMO course and complications, and survival to hospital discharge. Results Twenty-six patients (18 male, 8 female) with congenital heart disease were bridged to surgical palliation or anatomic repair with ECMO. Median age and weight at ECMO cannulation were, respectively, 0.12 months (range, 0 to 193) and 4 kg (range, 1.8 to 67 kg). Sixteen patients (62%) survived to hospital discharge. Variables associated with mortality included inability to decannulate from ECMO after surgery (p = 0.02) and longer total duration of ECMO (p = 0.02). No difference in outcomes was found between patients with single and biventricular anatomy. Conclusions Extracorporeal membrane oxygenation, used as a bridge to surgery, represents a useful modality to rescue patients with failing circulation and unrepaired complex heart defects.