Abstract
A 36-year-old woman was seen with complaints of exertional chest pain and shortness of breath. Her medical history included atrial fibrillation and diabetes. Physical examination was unremarkable except for an irregular cardiac rhythm. Myocardial perfusion imaging revealed the presence of a large area of infarction involving the entire anterior and apical walls and part of the anteroseptal wall with minimal periinfarct ischemia. Computed tomography coronary angiogram revealed an anomalous left main coronary artery arising from the main pulmonary artery. Right and left heart catheterizations demonstrated moderate pulmonary hypertension with a slight step-up in oxygen saturation between the right ventricle and main pulmonary artery. Coronary angiography showed a large tortuous right coronary artery with collaterals to the left anterior descending artery that drained into the main pulmonary artery. She was referred for surgery. This case demonstrates a rare coronary artery anomaly in an adult where survival is dependent on collateral circulation.
Highlights
Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital cardiac defect with an incidence of 1 in 300 000 live births.[1]
As the pulmonary artery resistance falls after birth, there is a “steal” of blood flow from the myocardium to the pulmonary artery in the direction of the pressure gradient.[2]
Coronary computed tomography (CT) angiogram revealed an anomalous origin of the left coronary artery system from the main pulmonary artery (Figures 1 and 2)
Summary
Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital cardiac defect with an incidence of 1 in 300 000 live births.[1] Most patients survive fetal life. A 36-year-old female was referred to a cardiology clinic following an abnormal treadmill stress test She had frequent episodes of exertional chest pain and shortness of breath with no palpitations, dizziness, or syncope. Coronary computed tomography (CT) angiogram revealed an anomalous origin of the left coronary artery system from the main pulmonary artery (Figures 1 and 2). The absence of a left coronary artery system arising from the left coronary sinus was confirmed with injection of contrast into the left coronary cusp (Figure 4) Given her ongoing symptoms, the patient was referred for surgical correction of her anomalous coronaries. At 6-month follow-up, her EF had significantly improved to 57%
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