Moruno Tirado Antonio, Santos De Soto José, Grueso Montero Josefina, Gavilán Camacho José L, Alvarez Madrid Antonio, Gil Fournier Mauro, Descalzo Señorans Alfonso
Secciones de Cardiología y Hemodinámica, Hospital Infantil Virgen del Rocío, Sevilla.
Rev Esp Cardiol. 2002 Mar;55(3):266-70. doi: 10.1016/s0300-8932(02)76595-0.
Aortopulmonary septal defect is an uncommon congenital cardiac anomaly. To date, approximately 300 cases have been reported. We present our experience, emphasizing the importance of early correction to avoid irreversible pulmonary hypertension.
Between 1979 and 2000, seven patients underwent surgical repair of this heart defect in our hospital. Two had type I (proximal), 4 had type II (distal) and 1 had type III (complete). Complex associated cardiac anomalies were present in 4 cases: type A interruption of the aortic arch in 2 cases, hypoplastic aortic arch in 1 and transposition of great arteries with ventricular septal defect in 1. Four cases (57%) were diagnosed by echocardiography. In all patients diagnoses were confirmed by cardiac catheterization. Patient records were reviewed retrospectively, with special attention to clinical, echocardiographic and hemodynamic data as well as surgical characteristics.
No intraoperative deaths occurred. The patient with associated transposition of great arteries died 22 days after surgery as a result of severe pulmonary hypertension. The remaining patients are asymptomatic without treatment after a mean follow-up period of 69 months.
Even though aortopulmonary septal defect is a rare anomaly, it should be considered whenever the course of complex congenital heart disease includes early cardiac failure and pulmonary hypertension. Repair before 6 months will prevent irreversible damage of pulmonary vessels.
