Pulmonary Arterial Hypertension After Repair of Classical Tetralogy Of Fallot

Tetralogy of Fallot (TOF), the most common cyanotic congenital heart disease, is classically associated with decreased pulmonary blood flow. Classical TOF is associated with antegrade pulmonary blood flow and right ventricular outflow tract obstruction at varying levels. In the modern era, most children with TOF undergo definitive intracardiac repair in infancy and pulmonary artery hypertension (PAH) is practically unheard of in postoperative TOF in the absence of major aortopulmonary collaterals. We report the circumstances where PAH can occur after definitive repair of classical TOF in the modern era.