A large aortopulmonary window found in the setting of a right aortic arch with discontinuous pulmonary arteries.

Abstract

Aortopulmonary window (APW) is a rare but serious congenital cardiac malformation, most patients with APW will die from congestive heart failure within one year after birth. In fact, patients with large APW is rarely seen in childhood or adult life. However, we report an older child with a large anomalous "window" on the ascending aorta, with discontinuous pulmonary arteries, and the left pulmonary artery (LPA) arising via a left-sided arterial duct in the presence of a right aortic arch. Preoperative diagnosis made by echocardiography and chest computerized tomography revealed anatomical futures clearly. Cardiac catheterization indicated that the pulmonary resistances indices were 2.92 Wood unit/m2 in LPA and 3.35 Wood unit/m2 in RPA, was 3.26. This patient underwent surgical correction at the age of 10 and successfully survived.