Adult Anomalous Left Coronary Artery Arising From the Pulmonary Artery (ALCAPA) Syndrome as First Presentation With Atrial Fibrillation in a Marathon Runner
Ocala Regional Medical Center
congenital cardiac anomalies, stroke, anomalous origin of the left coronary artery from pulmonary artery (alcapa), mitral valve insufficiency, atrial fibrillation
Cardiovascular Diseases | Congenital, Hereditary, and Neonatal Diseases and Abnormalities | Internal Medicine
Anomalous left coronary artery arising from the pulmonary artery (ALCAPA) syndrome is a very rare congenital heart disease with an incidence of one in 300,000 and a high rate of mortality early in life if left untreated. Adult-type ALCAPA presents when significant collaterals develop from the right coronary artery (RCA) to the left coronary artery (LCA). Even with the collaterals, chronic sub-endocardial ischemia occurs in most cases, and patients die from sudden cardiac death.
Here we present a case of a 38-year-old female who lived an active and healthy life and presented with chest pain and palpitations. Initial electrocardiography (EKG) showed atrial fibrillation with rapid ventricular response. Although initial cardiac enzymes were negative and there were no ischemic EKG changes, troponins became elevated over the course of the hospital stay and the patient underwent a left heart catheterization. Results revealed a dilated RCA extending to the left ventricle and an anomalous left main originating from the pulmonary artery with complete occlusion. The patient received medical management for acute coronary syndrome, including heparin infusion for 48 hours, aspirin, metoprolol, and atorvastatin. She was referred to a tertiary care facility for surgical correction of anomaly of the coronary arteries. The next day, the patient arrived in the emergency department with an acute onset of speech difficulty and left-sided weakness. A brain CT without contrast showed hematoma in the right frontal lobe. The patient underwent surgical evacuation of the hematoma with marked improvement of her weakness. The patient recovered after a successful surgical repair involving translocation of the left main coronary artery to the aorta.
It has been reported that ALCAPA should be considered in a young adult with dilated cardiomyopathy and mitral regurgitation (MR). Other common presentations include acute myocardial infarction, angina, and dyspnea on exertion. Sudden cardiac death is not uncommon; however, it tends to decrease with age of diagnosis. Interestingly, our patient was known to have MR with regular follow-up at the cardiology clinic for years. Echocardiogram never showed any abnormalities other than MR. She never received further workup to address the reason of MR, although she has no underlying chronic conditions that can explain it. In relatively young patients with a healthy lifestyle presenting with chest pain, a broader look at etiologies should be considered. We would like to emphasize the importance of looking up for possible coronary artery disease, especially in young individuals.
Publisher or Conference
Alsamman M, Tuna K, Dunn S, et al. Adult Anomalous Left Coronary Artery Arising From the Pulmonary Artery (ALCAPA) Syndrome as First Presentation With Atrial Fibrillation in a Marathon Runner. Cureus. 2021;13(5):e15354. doi:10.7759/cureus.15354