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Anomalous origin of left main coronary artery from the right sinus of Valsalva leading to sudden death


A 14-year-old female was brought to the emergency room with chest pain, shortness of breath and cyanosis. She was previously well with the exception of one previous post-exertion seizure-like event. On this day, she had been jogging when she complained of chest pain and collapsed. Her initial vital signs were heart rate 58/min, blood pressure 70/40 mmHg, respiratory rate 50/min, temperature 37 °C, and SaO2 68%. Electrocardiogram showed significant ST changes. She received multiple fluid boluses and dopamine was initiated (5–20 μg/kg/min). She was intubated and started on norepinephrine (0.05–0.5 μg/kg/min) for refractory hypotension. During the resuscitation, echocardiography showed poor left ventricular function with an ejection fraction of 38%. The coronary arteries could not be visualized clearly. To maintain cardiac output, epinephrine by infusion (0.1–3.0 μg/kg/min) was added, and she received multiple epinephrine boluses. Despite maximum ventilatory support and escalating inotropes, cardiac output rapidly deteriorated, and she developed an agonal rhythm with non-reactive pupils. Resuscitation was discontinued. Autopsy demonstrated an anomalous origin of left coronary artery from the right aortic sinus of Valsalva with acute myocardial ischemia. We describe the sudden coronary death of a young patient, and we review congenital coronary artery pathophysiology, screening difficulties and potential interventions.