Pulmonary Artery Stenosis in Tetralogy of Fallot

Tetralogy of Fallot (TOF) is the most common cyanotic congenital cardiac defect. The survival rate after primary complete repair is high (98-100%); however, pulmonary artery stenosis (PAS) is not uncommon after TOF repair, and severe PAS aggravates pulmonary regurgitation, resulting in right ventricle dilation, ventricular arrhythmia, and possibly death. PAS in TOF can be congenital due to hypoplasia or coarctation or can be acquired secondary to a surgical procedure. The latter may be caused by an exogenous conduit implant, compression from the adjacent enlarged ascending aorta, or outflow tract dilation after transannular patch repair. PAS can also be caused by the pulmonary artery plasty strategy itself. Here, the intrinsic mechanisms underlying PAS and pulmonary artery plasty techniques and strategies are reviewed to provide guidance for surgeons.