Over the last four decades, the Fontan operation has been the treatment of choice for children born with complex congenital heart diseases and a single-ventricle physiology. However, therapeutic options remain limited and despite ongoing improvements in initial surgical repair, patients still experience a multiplicity of cardiovascular complications. The causes for cardiovascular failure are multifactorial and include systemic ventricular dysfunction, pulmonary vascular resistance, atrioventricular valve regurgitation, arrhythmia, development of collaterals, protein-losing enteropathy, hepatic dysfunction, and plastic bronchitis, among others.
View Article and Find Full Text PDFBackground: Ventricular arrhythmias and sudden death are recognized complications in tetralogy of Fallot. Electrophysiological studies (EPS) before pulmonary valve replacement (PVR), the most common reintervention in tetralogy of Fallot, could potentially inform therapy to improve arrhythmic outcomes.
Methods: A prospective multicenter study was conducted to systematically assess EPS with programmed ventricular stimulation in patients with tetralogy of Fallot referred for PVR from January 2020 to December 2021.
Ventriculocoronary arterial connections are typically found in patients with pulmonary atresia with an intact ventricular septum. This report describes a case of ventriculocoronary arterial connections in a patient with pulmonary atresia with a ventricular septal defect. Our case supports recent data suggesting a primary coronary artery developmental anomaly in pulmonary atresia.
View Article and Find Full Text PDFObjectives: The aim of the study was to report the outcome of cardiac catheterisation in low-weight patients.
Background: Data regarding cardiac catheterisation in infants weighing <2500 g are scarce.
Methods: We reviewed all cardiac catheterisations performed in infants weighing <2500 g between January 2000 and May 2016.
Objectives: Repair of tetralogy of Fallot (ToF) can be challenging in the presence of an abnormal coronary artery (CA) in 5-12% of cases. The aim of this study was to report our experience with ToF repair without the systematic use of a right ventricle-to-pulmonary artery (RV-PA) conduit.
Methods: We conducted a monocentric retrospective study from 2000 to 2016, including 943 patients with ToF who underwent biventricular repair, of whom 8% (n = 76) presented with an abnormal CA.