Early Outcomes for Management of Atrioventricular Septal Defect-Tetralogy of Fallot in the Last Decade: A Congenital Heart Surgeons' Society Study.

Background: We sought to determine the management and early outcomes of complete atrioventricular septal defect-tetralogy of Fallot (AVSD-TOF) for a contemporary multicenter cohort.

Methods: Of 739 participants in the Congenital Heart Surgeons' Society AVSD cohort (January 2012-May 2021), 40 had AVSD-TOF. We first compared survival differences for patients with AVSD-TOF versus those with isolated AVSD using propensity matching.

Hybrid palliation versus nonhybrid management for a multi-institutional cohort of infants with critical left heart obstruction.

Objective: To compare patient characteristics and overall survival for infants with critical left heart obstruction after hybrid palliation (bilateral pulmonary artery banding with or without ductal stenting) versus nonhybrid management (eg, Norwood, primary transplantation, biventricular repair, or transcatheter/surgical aortic valvotomy).

Methods: From 2005 to 2019, 1045 infants in the Congenital Heart Surgeons' Society critical left heart obstruction cohort underwent interventions across 28 institutions. Using a balancing score propensity analysis, 214 infants who underwent hybrid palliation and 831 infants who underwent nonhybrid management were proportionately matched regarding variables significantly associated with mortality and variables noted to significantly differ between groups.

Time-Related Risk of Pulmonary Conduit Re-replacement: A Congenital Heart Surgeons' Society Study.

Background: Patients receiving a right ventricle to pulmonary artery conduit (PC) in infancy will require successive procedures or replacements, each with variable longevity. We sought to identify factors associated with time-related risk of a subsequent surgical replacement (PC3) or transcatheter pulmonary valve insertion (TPVI) after a second surgically placed PC (PC2).

Methods: From 2002 to 2016, 630 patients from 29 Congenital Heart Surgeons' Society member institutions survived to discharge after initial valved PC insertion (PC1) at age ≤ 2 years.

Partial Anomalous Pulmonary Venous Connection Repair: Customized Approach and Outcomes.

Alternative options for the correction of partial anomalous pulmonary venous connection (PAPVC) have been proposed. Each can be associated with variable risk for dysrhythmias, caval or pulmonary venous (PV) obstruction. A selective customized strategy to address PAPVC taking into account atrial shunt (AS) and growth potential was pursued.

Central Vascular Thrombosis in Neonates with Congenital Heart Disease Awaiting Cardiac Intervention.

Central vascular thrombosis (CVT) in critically ill neonates carries significant clinical implications. Neonates with congenital heart disease (CHD) awaiting cardiac intervention might be associated with increased risk of thrombosis. Outcome analysis was undertaken.

Surgical Management of Aorto-Ventricular Tunnel. A Multicenter Study.

Aorto-ventricular tunnel (AoVT), a rare congenital anomaly, is a channel originating in the ascending aorta just above the sinotubular junction and leading to the cavity of the left ventricle (AoLVT), or, rarely, the right (AoRVT). This study reviews our collective 30-year experience with the surgical treatment of AoVT. Data were submitted by 15 participating centers on 42 patients who underwent correction of AoVT between 1987 and 2018.

Right Ventricular Outflow Tract Reintervention in the Transcatheter Era: Outcomes and Cost Analysis.

Surgical pulmonary valve insertion (SPVI) for re-entry right ventricular outflow tract intervention (RVOTI) remains an established and reproducible approach. Fast-track in patients undergoing RVOTI of the comprehensive valve program targets early ICU and hospital discharge (Hd). Feasibility study for outcome and cost analysis was undertaken.

Standardized Approach to Intervention for Intestinal Malrotation in Single Ventricle Patients with Heterotaxy Syndrome: Impact on Interstage Attrition and Time to Superior Cavopulmonary Connection.

Heterotaxy syndrome (HS) is a significant determinant of outcome in single ventricle (SV) physiology. Attrition rate and time-related events associated with intestinal malrotation (IM) are, yet, to be determined. We sought to evaluate hospital and interstage outcomes in relation with operative intervention for IM (IMO).

Myocardial Ischemia and Anomalous Origin of the Right Coronary Artery From the Pulmonary Artery in the Adult: Management Implications and Follow-Up.

Anomalous origin of the right coronary artery from the pulmonary artery (ARCAPA) is a very rare and potentially fatal congenital heart defect due to myocardial ischemia secondary to coronary steal phenomenon. We present a case of an adult presenting with a myocardial infarction who was found to have ARCAPA. Three-dimensional (3D) reconstructed computed tomography angiography (CTA) was utilized preoperatively for surgical planning.

Surgery for anomalous aortic origin of coronary arteries: a multicentre study from the European Congenital Heart Surgeons Association†.

Objectives: We sought to describe early and late outcomes in a large surgical series of patients with anomalous aortic origin of coronary arteries.

Methods: We performed a retrospective multicentre study including surgical patients with anomalous aortic origin of coronary arteries since 1991. Patients with isolated high coronary takeoff and associated major congenital heart disease were excluded.

Anomalous aortic origin of coronary arteries: Early results on clinical management from an international multicenter study.

Background: Anomalous aortic origin of coronary arteries (AAOCA) is a rare abnormality, whose optimal management is still undefined. We describe early outcomes in patients treated with different management strategies.

Methods: This is a retrospective clinical multicenter study including patients with AAOCA, undergoing or not surgical treatment.

Hammock Mitral Valve Repair in Infancy: Operative Steps Toward a Customized Reconstruction After Preoperative Planning.

Hammock mitral valve (MV) repair is historically technically difficult with a guarded prognosis. Surgical experience is extremely limited and variable outcomes are reported. The perioperative strategy and technical details of hammock MV repair in an infant who presented with severe mitral stenosis are described and review of the existing literature was undertaken.

Selective Use of Temporary Epicardial Pacing Leads in Early Infancy Following Cardiac Surgery: Feasibility and Determinants of Clinical Application.

Use of temporary epicardial pacing (TEP) leads remains a routine perioperative strategy in congenital heart surgery. Selective use of TEP in neonates and infants undergoing cardiac intervention within the first 6 months of life has, yet, to be assessed. Outcome analysis was undertaken.

Neonatal Myocardial Perfusion in Right Ventricle Dependent Coronary Circulation: Clinical Surrogates and Role of Troponin-I in Postoperative Management Following Systemic-to-Pulmonary Shunt Physiology.

Right ventricle dependent coronary circulation (RVDCC) in pulmonary atresia with intact ventricular septum (PA/IVS) is associated with significant mortality risk in the immediate post-operative period following the initial stage of surgical palliation. Prognosis remains guarded during the interstage period towards conversion to the superior cavopulmonary shunt physiology. Current literature is scarce regarding this specific patient population.

Restoring Fetal Circulation as a Means of Bridging Treatment Prior to Surgical Repair of Anomalous Origin of the Right Pulmonary Artery from the Ascending Aorta with Persistent Pulmonary Hypertension of the Newborn.

Anomalous origin of one pulmonary artery from the ascending aorta is a rare congenital anomaly. Even more rarely reported is its presence in conjunction with persistent pulmonary hypertension of the newborn (PPHN). We present a case of a full-term infant, initially thought to have PPHN and later found to have anomalous origin of the right pulmonary artery from the ascending aorta.

The Challenge of Hammock Mitral Valve During Infancy: Precise Preoperative Advanced Imaging and Three-Dimensional Modeling Augments Customized Operative Valve Reconstruction.

Hammock mitral valve (MV), also known as anomalous mitral arcade, is a rare congenital anomaly. We report a case of a 10-month-old child who presented with congestive heart failure and was found to have severe mitral stenosis (MS) secondary to a hammock MV anomaly. Detailed advanced imaging with cardiac MRI and three-dimensional (3D) echocardiography favorably navigated a customized valve-sparing surgical reconstruction of the congenitally abnormal MV.

Anomalous Origin of Pulmonary Artery From Aorta: Neonatal Autologous Aortopulmonary Reconstruction.

Anomalous origin of a branch pulmonary artery (PA) from the ascending aorta is rather rare within the spectrum of congenital cardiovascular anomalies. In the scarce subset of patients with anomalous origin of right PA (RPA) from the ascending aorta originating from the lateral aspect of the distal ascending aorta, early neonatal repair by employing native aortic tissue for RPA reconstruction combined with translocation of the aorta posteriorly to the RPA can be an alternative option in the surgical armamentarium.

Critically Underdeveloped Left Heart Morphology Associated with Prematurity and Low Birth Weight: Conditional Staged Rehabilitation Towards Biventricular Repair and Time-Related Growth of Left Heart Structures.

This is a case report of premature low birth weight infant with hypoplasia of left heart structures and a large malaligned VSD who underwent successful staged approach of biventricular repair. We obtained qualitative and quantitative echocardiographic, MRI, and conventional catheterization data to support stepwise strategy towards LV rehabilitation to sustain adequate cardiac output. A thorough and intense follow-up has shown significant growth of left heart structures and favorable clinical status following staged biventricular repair.

Attrition in patients with single ventricle and trisomy 21: outcomes after a total cavopulmonary connection.

Objectives: Data are limited regarding the management of children with trisomy 21 (T21) syndrome and a functional single ventricle (FSV). We evaluated patients with T21 and a FSV who had a total cavopulmonary connection (TCPC).

Methods: From September 1999 to August 2012, 139 patients with a FSV underwent a TCPC.

Transposition of Great Arteries with Complex Coronary Artery Variants: Time-Related Events Following Arterial Switch Operation.

Coronary artery anatomy represents a challenging and, often, determining predictor of outcome in an arterial switch operation (ASO). Impact of specific coronary artery variants, such as single, intramural and inverted, on time-related events following ASO, is, yet, to be determined. We sought to compare early and late outcomes within the group of nonstandard coronary artery variants.

Post-cardiotomy Rescue Extracorporeal Cardiopulmonary Resuscitation in Neonates with Single Ventricle After Intractable Cardiac Arrest: Attrition After Hospital Discharge and Predictors of Outcome.

Extracorporeal cardiopulmonary resuscitation (ECPR) in children with cardiac arrest refractory to conventional cardiopulmonary resuscitation (CPR) has been reported with encouraging results. We reviewed outcomes of neonates with functional single ventricle (FSV) surviving post-cardiotomy ECPR after hospital discharge. Fifty-eight patients who required post-cardiotomy extracorporeal membrane oxygenation (ECMO) since the introduction of our ECPR protocol (January 2007-December 2011) were identified.

Intrapericardial NUT Midline Carcinoma: Unusual Presentation of a Rare Tumor and Literature Review with Management Considerations.

Rearrangements in the nuclear protein in testis (NUT) gene cause carcinomas that represent a rare but aggressive tumor type that often present at advanced stages in midline structures. Survival rarely exceeds 12 months from the time of diagnosis. There have been no reports of a primary cardiac presentation, and few studies have reported on the numerous treatment strategies.