Usually diagnosed post-mortem, aortopulmonary artery fistula is an exceptionally rare and often highly lethal condition. Here, we present a unique case of a 29-year-old woman with no history of aortic aneurysm or previous aortic surgery, referred for surgical management of Laubry-Pezzi syndrome in whom an aorto-pulmonary artery fistula bridging the ascending aorta and the main pulmonary artery was discovered intra operatively. The patient was successfully managed with an uneventful postoperative recovery. Our case outlines the inaugural reported occurrence of an aortopulmonary artery fistula coinciding with Laubry-Pezzi syndrome. Unique features include subtle onset of heart failure over months due to occult left ventricular volume overload, challenging diagnosis alongside known intracardiac shunts, emphasizing the need for vigilance, and demonstration of successful surgical outcomes despite high morbidity and mortality.
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