Tetralogy of Fallot Associated with Total Anomalous Pulmonary Venous Drainage

¹ Divisions of Cardiology and Cardiovascular-Thoracic Surgery (Willis J. Potts Children's Heart Center), The Children's Memorial Hospital and the Department of Pediatrics and Surgery, Northwestern University Medical School, Chicago

A previously unreported association of tetralogy of Fallot and total anomalous pulmonary venous drainage is described in two infants. The initial clinical manifestation was tetralogy, and both patients had systemic-pulmonary shunt surgery to increase the pulmonary blood flow. Postoperatively, one infant died of massive pulmonary edema, and at postmortem examination total anomalous pulmonary venous drainage below the diaphragm was found. The other patient had the association of tetralogy with anomalous pulmonary venous drainage into the coronary sinus diagnosed by cardiac catheterization. Following shunt surgery, prolonged continuous positive airway pressure was necessary to adequately ventilate the lungs, presumably the result of pulmonary venous congestion and low pulmonary compliance. The infant eventually died from tracheostomy complications. The diagnosis, clinical course, surgical implications and pathology of this association are discussed.