Pediatr Cardiol (2010) 31:1119–1121 DOI 10.1007/s00246-010-9780-z
IMAGES IN PEDIATRIC CARDIOLOGY
Anomalous Right Coronary Artery Arising from the Pulmonary Artery in a 36-Year-Old Woman: Diagnosis by Coronary Computed Tomographic Angiography Helina Kassahun • Gina Larocca • James K. Min
Received: 5 July 2010 / Accepted: 11 August 2010 / Published online: 29 August 2010 Ó Springer Science+Business Media, LLC 2010
A 36-year-old woman with congenital absence of the uterus presented to our cardiology clinic reporting progressive shortness of breath for 6 months. She was referred for exercise nuclear stress testing. The patient exercised to 7 min and 40 s of a Bruce protocol, reaching 88% of the predicted maximum heart rate. During exercise, the patient experienced chest pain, and an electrocardiogram (ECG) demonstrated 1.5-mm horizontal ST-segment depressions. Myocardial perfusion scintigraphy (MPS) demonstrated normal myocardial perfusion. Given the discrepancy between the ECG, symptoms, and MPS results, the patient was referred for prospective ECG-triggered 64-detector row coronary computed tomographic angiography (CCTA). The CCTA demonstrated an anomalous right coronary artery arising from the pulmonary artery (ARCAPA) (Figs. 1, 2). Furthermore, collateralization of the right coronary artery from the left anterior
H. Kassahun G. Larocca J. K. Min (&) Department of Medicine, Greenberg Division of Cardiology, Weill Cornell Medical College, New York Presbyterian Hospital, 520 E. 70th Street, Starr Pavilion, K-415, New York, NY 10021, USA e-mail:
[email protected] J. K. Min Department of Radiology, Weill Cornell Medical College, New York Presbyterian Hospital, New York, NY, USA
descending artery and left circumflex artery was noted on the CCTA (Fig. 3). The patient was referred for right heart catheterization, which showed normal cardiac output and normal rightsided filling pressures but left-to-right-sided shunt physiology (oxygen saturation of 66% in the superior vena cava, 76% in the right atrium, and 80% in the pulmonary artery). An aortogram demonstrated early contrast filling of the left coronary artery system without contrast filling of the right coronary artery system (Fig. 4). A left ventriculogram demonstrated contrast filling of the right coronary artery from the pulmonary artery (Fig. 5). Selective left coronary catheterization showed substantial retrograde collateralization of the right coronary artery from the left anterior descending and left circumflex arteries (Fig. 6). The patient was referred for surgery and underwent transposition of the anomalous right coronary artery onto the ascending aorta with autologous pericardial patch repair of the main pulmonary artery. Her postoperative course was uneventful, and she was discharged home 7 days after admission. Anomalous origin and course of coronary arteries are found in less than 1% of the general population and may be associated with myocardial infarction and sudden death [1]. Anomalous right coronary artery arising from the pulmonary artery is a very rare entity, typically diagnosed in childhood. Unlike anomalous left coronary artery arising from the pulmonary artery (ALCAPA), patients with ARCAPA may be asymptomatic up to adulthood [2]. The treatment of choice is transaction of the right coronary artery from the pulmonary trunk and direct anastomosis to the aorta [3]. The reported case represents the oldest case presentation of ARCAPA and the first in which CCTA was used for the initial diagnosis.
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1120 Fig. 1 Anomalous right coronary artery arising from the pulmonary artery (white arrow). a Volume-rendered image of the heart and coronary arteries. b Volume-rendered image of the coronary arteries. Note the left-to-right collaterals (double white arrow)
Fig. 2 Anomalous right coronary artery arising from the pulmonary artery (white arrow). a Axial maximum intensity projection of right coronary artery. b Two-dimensional oblique maximum intensity projection of the right coronary artery
Fig. 3 a Left-to-right collaterals from the distal left anterior descending artery to the right ventricular marginal branch of the right coronary artery (white arrow). b Left-toright collaterals from the distal left anterior descending artery (single arrow) and the left circumflex artery (double arrow) to the posterior descending artery
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Pediatr Cardiol (2010) 31:1119–1121
Pediatr Cardiol (2010) 31:1119–1121
Fig. 4 Aortogram demonstrating flow in the left coronary artery system, with no evident flow in the right coronary artery system
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Fig. 6 Left coronary angiogram with robust left-to-right collaterals to the right coronary artery
References 1. Davis JA, Cecchin F, Jones TK et al (2001) Major coronary artery anomalies in a pediatric population: incidence and clinical importance. J Am Coll Cardiol 37:593–597 2. Roberts WC (1986) Major anomalies of coronary arterial origin seen in adulthood. Am Heart J 111:945–963 3. Warnes CA et al (2008) ACC/AHA 2008 ACC/AHA 2008 guidelines for the management of adults with congenital heart disease. J Am Coll Cardiol 52:e1–e121
Fig. 5 Left ventriculogram with late filling of the right coronary artery (arrow)
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