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Transcript
Unique echocardiographic findings in anomalous left coronary artery origin from the pulmonary artery (ALCAPA) Fereshteh Ghaderi, Arash Gholoobi, Ali Asghar Moeinipour ALCAPA is a rare congenital anomaly known as the Bland–White–Garland syndrome. Its usual clinical course is a severe left-sided heart failure with mitral valve insufficiency presenting during the first months of life. Age and severity of symptoms depend on the development of collateral coronary circulation and on the pulmonary vascular resistance. In some cases, collateral blood supply from the right coronary artery is sufficient and clinical symptoms may be subtle or even absent. However, ventricular arrhythmias or sudden cardiac death during exercise may be the first clinical presentation in patients with ALCAPA. Diagnosis can sometimes be achieved on the basis of echocardiography alone, but cardiac catheterization may also be required for confirmation. We report an 18-year-old male with no significant past medical history presenting with exertional syncope. He was referred for further evaluation of multiple muscular ventricular septal defects diagnosed on an outpatient echocardiogram. Transthoracic echocardiography showed mild left ventricular enlargement, systolic dysfunction (EF=45-50%) and multiple turbulent color flow regions in the ventricular septum. Coronary artery originating from the right aortic sinus was dilated (10mm). PW Doppler with sample volume in the coronary ostium showed systolic coronary flow predominancy. This finding is due to the left to right shunt in coronary arteries in the presence of collateral circulation. This unique finding is characteristic for ALCAPA and can differentiate it from other coronary anomalies. The patient underwent coronary angiography which revealed a large tortuous right coronary artery with a lot of collateral branches draining into the pulmonary artery via the left coronary artery in delayed sequences. Hence, the ALCAPA diagnosis was confirmed. Presenter Fereshteh Ghaderi, MD. Assistant Professor of Cardiology, Preventive Cardiovascular Care Research Center Imam Reza Hospital, School of Medicine Mashhad University of Medical Sciences Mashhad, IRAN P.O. Box: 09137913316 Tel: +989151615412 Fax: +985118544504 E-mail: [email protected]