Alcapa of adult hood.

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Conference Proceeding

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J Gen Intern Med


Learning Objective #1: Diagnosis of congenital heart disease in adults. Learning Objective #2: Anomalous origin of the left coronary artery from the pulmonary artery usually manifests and causes death in infancy, however, with significant collateral blood supply to the left coronary artery patients may survive into adulthood. CASE: This is a case of a 33 year old male who presented to the emergency room with chest pain that started shortly after having an altercation with his boss at work. He described it as a squeezing left sided chest pain radiating to the left arm. On presentation the patient had stable vital signs, physical exam was unremarkable. Electrocardiogram revealed new T wave inversions in leads I and aVL, and the patient had a troponin elevation up to 0.9 (normal < 0.04). The patient was diagnosed with a non-ST elevation myocardial infarction (NSTEMI) and admitted for coronary angiography. Coronary angiography revealed a large caliber ectatic right coronary artery filling many arteriovenous fistulae flowing into the left coronary system which in turn appeared to drain into the pulmonary artery. No coronary artery disease was seen. The patient then underwent computed tomography angiogram which confirmed a left coronary artery arising form the main pulmonary artery posteriorly at the superior aspect of the pulmonary valve. Follow up echocardiogram revealed an ejection fraction 40% with anterior, anteroseptal and anterolateral wall hypokinesis as well as mitral valve regurgitation. The patient was referred to cardiothoracic surgery, and a few days later underwent tunneling from the main pulmonary artery-left coronary artery origin to the aorta using a pericardial patch. The patient did well after surgery and was discharged a few days later with minimal symptoms. IMPACT: This case shows us that we need to consider congenital heart disease even when adults present with common cardiac syndromes. Most patients presenting with NSTEMI in adulthood are as a result of coronary artery disease, however in this vignette that was not the case. This also shows the rising diagnostic importance of imaging modalities such as CTand MR in cardiac patients, especially younger patients. DISCUSSION: Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) occurs in 0.25%-0.5% of all congenital heart diseases. 85% of patients present with heart failure symptoms within the first 1-2 months of life due to myocardial ischemia. Up to 90% of patients with ALCAPA die within the first year of life. A small subset of patients develop good collateral circulation and may live into childhood or adulthood. Despite this, blood supply to the left ventricle is insufficient and myocardial ischemia usually leads to mitral valve regurgitation, ischemic cardiomyopathy and sudden cardiac death. Diagnosis is usually confirmed by imaging modalities such as CTor MRI. Treatment is surgical correction consisting of reimplantation of the coronary artery to the aorta or tunneling of the coronary artery to the aorta.





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