Tetralogy of Fallot with Absent Pulmonary Valve

Absent or hypoplastic pulmonary valve is an uncommon condition usually associated with a ventricular septal defect and an obstructive pulmonary valve ring. Secondary abnormalities involving the pulmonary arteries and major bronchi are common, and many result in severe pulmonary complications. Eight patients with absent pulmonary valve, ventricular septal defect and obstructed pulmonary valve ring are herein described. The clinical features, cardiac catheterization, cineangiographic and postmortem findings are presented. Possible fetal hemodynamics and their influences are discussed, as are postnatal hemodynamics and their clinical consequences. Patients with this combination of lesions have been regarded as having a very poor prognosis, and recent publications have advocated early surgical intervention; however, most published series are small, and only 106 cases have been documented. These cases have been reviewed for clinical and anatomic features as well as an overview of prognosis and results of surgical intervention.