Pulmonary valve replacement is being performed with increasing frequency in patients with various congenital heart diseases. Chronic pulmonary regurgitation after repair of tetralogy of Fallot is a typical situation that requires pulmonary valve replacement. Chronic pulmonary regurgitation after repair of tetralogy of Fallot can lead to right ventricular dilatation, biventricular dysfunction, heart failure symptoms, arrhythmias, and sudden death. Although pulmonary valve replacement can lead to improvement in functional class and a substantial decrease or normalisation of right ventricular volumes, the optimal timing of pulmonary valve replacement in patients with chronic pulmonary regurgitation is still unknown. There are several options for surgical pulmonary valve replacement. However, no ideal pulmonary valve substitute exists currently and most of the surgically implanted pulmonary valves will eventually require re-replacement. This article will review options and timing of surgical pulmonary valve insertion in patients with chronic pulmonary regurgitation after repair of tetralogy of Fallot.

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.