Pulmonary Agenesis with Congenital Heart Disease

The combined anomalies of pulmonary agenesis and congenital heart disease are exceedingly rare and a highly lethal association. We describe four cardiovascular operations in three patients with this association. Open-heart surgery was performed in two patients, and palliative heart surgery was performed in two patients. One patient died 100 days after the operation because of severe tracheal stenosis and renal failure, but the other two patients are doing well. Surgical access through a thoracotomy, instead of a median approach, was adequate in all patients. Careful evaluation and a precise understanding of the anatomy are necessary for the surgical management of these patients.