Lessons learned from a series of patients with missed aortopulmonary windows

Abstract

OBJECTIVES: To identify factors contributing to missed diagnosis of aortopulmonary windows on initial echocardiographic examination; and to analyze lesions associated with these malformations. DESIGN: Retrospective study where echocardiographic findings and per-operative findings of patients were correlated by reviewing records. SETTING: Tertiary-care paediatric cardiac centre. PATIENTS: From May 2002 to September 2007, we diagnosed 50 patients with aortopulmonary windows. The group included 31 boys and 19 girls. Mean age at intervention was 1.7 years, with a range from 3 months to 17 years. RESULTS: The diagnosis of aortopulmonary window was made correctly by echocardiography during initial evaluation in 46 of 50 patients (92%). The remaining 4 patients were diagnosed either during repeat echocardiographic evaluation, cardiac catheterization, or per-operatively. We describe the details of these patients. Of the 50 patients, 23 had associated malformations, including ventricular septal defects in 10 patients, patent arterial ducts in 9, atrial septal defects or patent oval foramens in 5, 3 of whom also had patent arterial ducts, interruption of the aortic arch at the isthmus in 4, or between the left subclavian and common carotid arteries in 2, tetralogy of Fallot in 2, double-outlet right ventricle in 2, discordant ventriculo-arterial connections in 2, 1 of whom also had anomalous origin of the left coronary artery from the pulmonary trunk, and superior-inferior ventricles in 1 patient. CONCLUSIONS: Even meticulous echocardiography may result in a false negative diagnosis of an aortopulmonary window. A high index of suspicion for this lesion is warranted in cases of unexplained severe pulmonary arterial hypertension, and/or unexplained cardiac dilation.