Management of adults with Tetralogy of Fallot

Abstract

Tetralogy of Fallot is the most common form of cyanotic congenital cardiac disease. Patients with previously repaired tetralogy of Fallot are the most common patients seen in the Program for Adults with Congenital Heart Disease at The Johns Hopkins All Children's Heart Institute. Guidelines for the management of these patients are available from multiple sources including The American College of Cardiology (ACC) and The American Heart Association (AHA), The Canadian Cardiovascular Society, and The European Society of Cardiology (ESC). These guidelines describe multiple components related to the care for these patients including strategies for medical follow-up, the management of arrhythmias and electrophysiological diseases, and the treatment of chronic pulmonary insufficiency and stenosis. Several new strategies are available for replacement of the pulmonary valve including transcatheter replacement of the pulmonary valve and replacement of the pulmonary valve with a self-manufactured bicuspid polytetrafluoroethylene pulmonary valve.