Williams Syndrome and Neonatal Cardiac Surgery for Congenital Single Ventricle.

Williams syndrome (WS) is an arteriopathic derangement associated with supravalvular aortic stenosis and branch pulmonary stenosis. We describe double-outlet right ventricle with mitral atresia and aortic arch hypoplasia in an infant with WS. This case demonstrates the difficulty in managing patients with WS with complex cardiac defects.

Outcomes related to immediate extubation after stage 1 Norwood palliation for hypoplastic left heart syndrome.

Objective: Immediate extubation may have outcome benefits when judiciously instituted after neonatal congenital cardiac surgery. We sought to evaluate the outcomes of immediate extubation specifically in neonates undergoing stage 1 Norwood palliation of hypoplastic left heart syndrome.

Methods: Consecutive neonates undergoing stage 1 Norwood (January 2010 to December 2016) for hypoplastic left heart syndrome were retrospectively studied.

An Analysis of Left Ventricular Retraining in Patients With Dextro- and Levo-Transposition of the Great Arteries.

Background: Patients with dextro (D)-transposition of the great arteries (TGA) who have undergone a previous atrial switch and for some patients with levo (L)-TGA (ie, no ventricular septal defect or outflow tract obstruction), the left ventricle (LV) may require retraining before late arterial switch. The purpose of this study was to analyze the results of LV retraining for these two entities.

Methods: This was a retrospective review of 51 patients enrolled in an LV retraining program.

Left Ventricular Retraining and Double Switch in Patients With Congenitally Corrected Transposition of the Great Arteries.

Purpose: Congenitally corrected transposition of the great arteries (CC-TGA) is a complex form of congenital heart defect with numerous anatomic subgroups. The majority of patients with CC-TGA are excellent candidates for a double-switch procedure. However, in the absence of an unrestrictive ventricular septal defect or subpulmonary stenosis, the left ventricle (LV) may undergo involution and require retraining prior to double switch.

Surgical Technique for Repair of Peripheral Pulmonary Artery Stenosis and Other Complex Peripheral Reconstructions.

Surgical reconstruction of peripheral pulmonary artery stenosis is a technically challenging procedure due to the need to access all lobar and segmental branches. This paper describes our surgical approach that entails division of the main pulmonary and separation of the branch pulmonary arteries. This surgical approach can also be utilized for other complex peripheral pulmonary artery reconstructions.

Early Complete Atrioventricular Canal Repair Yields Outcomes Equivalent to Late Repair.

Background: Repair of complete atrioventricular canal early in infancy has traditionally carried greater morbidity and mortality than repair performed later. However, an individualized anatomy-based repair may give young infants outcomes that are equivalent to older patients.

Methods: We retrospectively reviewed 139 patients who underwent complete atrioventricular canal repair from January 2005 to December 2012.

Use of bicaval dual-lumen catheter for adult venovenous extracorporeal membrane oxygenation.

Background: Extracorporeal membrane oxygenation (ECMO) provides supplementary oxygenation and carbon dioxide removal for selected patients on mechanical ventilatory support. Venovenous ECMO is traditionally established by dual cannulation of the internal jugular and femoral veins. We report our institutional experience using single-site, dual-lumen cannula for venovenous ECMO as an alternative to the 2-catheter approach.

Post-heart transplant survival is inferior at low-volume centers across all risk strata.

Background: Previous studies have demonstrated a relationship between increasing center volume and cardiac transplant outcomes. The purpose of this study was to confirm a relationship between post-heart transplant outcomes and center experience and to determine whether this relationship persists among low- and high-risk heart transplant recipient-donor pairs.

Methods And Results: The United Network for Organ Sharing (UNOS) provided deidentified patient-level data.