Abstract

BackgroundPulmonary artery to left atrium fistula is an unusual structural cause of silent cyanosis. Only less than 100 cases have been reported so far. A high index of clinical suspicion and proper evaluation with bubble contrast echocardiography and cardiac computed tomography (CT) will help to detect this treatable anomaly. The advent of safer percutaneous closure methods has replaced the need for more invasive surgical closure.Case presentationWe report an adolescent boy, who presented with long-standing cyanosis and progressive dyspnea with normal clinical cardiovascular examination. On evaluation, echocardiography and bubble contrast study revealed a large right pulmonary artery (RPA) to left atrium (LA) fistula. Cardiac CT confirmed the same with normal pulmonary venous drainage s/o a large 20 mm Type I RPA LA Fistula. He underwent successful percutaneous closure of the fistula tract with a 22 × 24 mm Cera™ duct occluder via transseptal approach uneventfully.ConclusionOur case enlightens the methodological approach to diagnosing this rare anomaly as well as the feasibility of percutaneous intervention in such cases as it is one of the largest fistula tracts closed percutaneously to date.

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