Aortopulmonary window--a review of nine cases

Aortopulmonary window (APW) is a rare anomaly, accounting for 0.1% of congenital heart defects. It consists of a communication between the ascending aorta and the pulmonary artery in the presence of normal separate aortic and pulmonary valves. Early treatment is usually required in order to prevent the development of irreversible pulmonary hypertension.

To assess the results of treatment in all patients diagnosed at our institution between January 1994 and November 2007, based on a retrospective longitudinal study.

Nine patients treated for APW were identified. Their ages at diagnosis ranged from two days to 23 years; eight were infants aged 2 +/- 2.9 months. In this group clinical presentation was congestive heart failure in all cases. Five patients had associated lesions (interrupted aortic arch: 2; coarctation of the aorta: 2; VSD: 1; ASD: 3). In seven cases the diagnosis was made on the basis of echocardiography only. In the other two it was by cardiac catheterization, one patient with coarctation of the aorta and the other an adult patient with a smaller lesion who was initially misdiagnosed as having ductus arteriosus. All patients had corrective surgery, via a transaortic approach, with implantation of an autologous pericardial patch. All patients are alive and only one case has a small residual shunt at the correction site.

A diagnosis of APW should always be kept in mind in the differential diagnosis of a child with congestive heart failure. Associated congenital heart anomalies should be excluded. Surgical repair appears to provide good short- and long-term results.