Pulmonary Valve Replacement for Tetralogy of Fallot

Right ventricular outflow tract (RVOT) dysfunction is common following surgical repair of tetralogy of Fallot and other forms of complex congenital heart disease. This results in pulmonary stenosis or regurgitation and may ultimately lead to RV failure and dysrhythmias. Transcatheter valve technologies are now available to treat certain patients with RVOT dysfunction. Current devices include the Medtronic Melody valve and the Edwards Lifesciences SAPIEN XT. Although these valves are approved for use in dysfunctional circumferential RVOT conduits, they are increasingly being used off label for nonconduit outflow tracts. Procedural complications include but are not limited to conduit rupture and coronary compression. Longer-term complications include stent fracture and endocarditis. Outcomes with these valves have demonstrated durable relief of stenosis and regurgitation. The Medtronic Harmony valve and the Alterra Prestent from Edwards Lifesciences are investigational devices that are intended to treat the patulous RVOT that is too large to accommodate currently available valves. This review will focus on current indications to treat RVOT dysfunction, existing transcatheter valve technologies, and investigational devices undergoing clinical trials. Hopefully, within the not-too-distant future, transcatheter pulmonary valve implantation will be feasible in the vast majority of patients with RVOT dysfunction following surgical repair of congenital heart disease.