It is based on where pulmonary arteries arise from the common trunk. The Collett and Edwards system is the earliest form of classification, developed in 1949. Surgical repair consists of a Rastelli operation where the VSD is closed via a baffle patch, the branch pulmonary arteries are removed from the common trunk and connected to a right ventricle to pulmonary artery conduit and if needed (in the setting of truncal stenosis and/or regurgitation), repair of the truncal valve is performed. If left untreated, the majority (up to 88%) of patients die in infancy due to heart failure from truncal valve regurgitation or pulmonary overcirculation. Surgical repair is required due to likely development of congestive heart failure. Type I/A1 is the most common form, found in ~60% of patients with Truncus Arteriosus. There are four sub types of truncus arteriosus which are described using two different classification systems (outlined below). TA has a high association with 22q11 Deletion Syndrome (DiGeorge Syndrome) in ~20% of cases. A ventricular septal defect (VSD) is most commonly associated with TA, as well as anomalies with truncal valve, aortic arch and coronary arteries (outlined below). The common trunk gives rise to the pulmonary arteries, providing systemic, pulmonary and coronary perfusion. It is characterized by a single great artery arising from the heart with a single semilunar valve that overrides the right and left ventricles. Persistent truncus arteriosus results from incomplete or failed septation. During fetal development, the embryonic truncus arteriosus gives rise to the aorta and the pulmonary trunk. Truncus arteriosus (TA) is a rare form of congenital heart disease occurring in 1-3% of patients with congenital heart disease. Left Ventricular Non Compaction Cardiomyopathy (LVNC).Right Aortic Arch with an Aberrant Left Subclavian Artery (Vascular Ring).Infracardiac Total Anomalous Pulmonary Venous Return.Idiopathic Infantile Arterial Calcification.
Congenitally Corrected Transposition of the Great Arteries (ccTGA).Transposition of the Great Arteries (DTGA).Pulmonary Atresia with Intact Ventricular Septum (PA/IVS).Tetralogy of Fallot with Pulmonary Atresia.Tetralogy of Fallot with Absent Pulmonary Valve.Supracardiac Total Anomalous Pulmonary Venous Return.Total Anomalous Pulmonary Venous Return to the Coronary Sinus.Pulmonary Artery Sling (Vascular Sling).Anomalous Left Coronary Artery from the Right Coronary Artery.Anomalous Right Coronary Artery from the Left Coronary Artery.Transitional Atrioventricular Canal (AV Canal).Partial Atrioventricular Canal (AV Canal).Complete Atrioventricular Canal (AV Canal).
0 kommentar(er)
