pediatrics
Congenitally Corrected Transposition of the Great Arteries
Treatment of CCTGA
Medications Surgery Rhythm devices Medications may be needed for patients with pressure or volume overload or ventricular dysfunction resulting in congestive heart failure. Surgical treatment is often necessary to repair associated defects such as ventricular defects, pulmonary outflow tract obstruction or tricuspid regurgitation. Because of the inevitability of progressive cardiac dysfunction in the adult years, many clinicians consider the "double switch" as a surgical option in childhood. This procedure involves switching the aorta and pulmonary arteries to the morphologically appropriate ventricles and switching the venous return with an intraatrial baffle, which directs vena caval return to the left-sided RV and the pulmonary venous return to the right-sided left ventricle. This complex procedure eliminates the inherent disadvantage of the CCTGA circulation, namely having a morphologic RV to supply the systemic circulation. Pacemakers are generally required if patients have AV block, and adults with moderate to severe RV dysfunction and risk for ventricular arrhythmias may receive implantable cardioverter defibrillators. Patients with severe manifestations, including progressive cardiomyopathy due to RV dysfunction, may require Heart Transplantation . 