A RARE CASE OF ADULT TYPE ALCAPA SYNDROME: TEN YEARS AFTER SUCCESSFUL SURGICAL REPAIR A Gaddam MD, B Swirsky MD Department of Medicine, Baton Rouge General Medical Center, Baton Rouge, LA INTRODUCTION Anomalous left coronary artery from the pulmonary artery (ALCAPA) Syndrome is a very rare congenital heart disease where the left coronary artery (LCA) originates from the pulmonary artery. It affects 1 in every 300,000 live births in the USA and if left untreated, has a 90% mortality within the first year of life. CASE DESCRIPTION: A 32-year-old woman with a past medical history of hypertension, non-insulin-dependent type 2 diabetes mellitus, hyperlipidemia, with no family history of heart disease presented with complaints of exertional angina since 2 years and had a normal physical examination. Further work-up with coronary angiography showed an anomalous left coronary artery arising from the pulmonary artery. Coronary angiographic findings also revealed a large voluminous right coronary artery (8 mm in diameter) with extensive collaterals to the left coronary artery. Transthoracic Echocardiogram revealed an ejection fraction of 40%withmild left ventricular hypertrophy. EKG showed nonspecific T wave abnormality in lateral leads. Our patient underwent surgical repair with coronary button transfer procedure without any perioperative complications. After 10 years of post repair, our patient is asymptomatic with and ejection fraction of 45% on Transthoracic Echo. DISCUSSION: In extremely rare cases of adult type ALCAPA Syndrome, those who live to adulthood have extensive collateral circulation from the right coronary artery and develop chronic myocardial ischemia resulting in arrhythmias, congestive heart failure, and sudden cardiac death. The extent of myocardial ischemia is determined by the development of collaterals between the right and left coronary artery. Diagnosis is usually established with coronary angiography, cardiac MR imaging, multi detector CT angiogram. Surgical correction with coronary button transfer procedure has excellent long-term results.
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