Abstract
WEBSITE FEATURE
A 35-year-old asymptomatic man was referred for evaluation of a systolic murmur. Transthoracic echocardiography with color-flow Doppler revealed a large network of dilated vessels anterior to the right ventricle. Contrast-enhanced, 64-slice multidetector computed tomography (MDCT) (LightSpeed™ VCT; General Electric Medical Systems; Milwaukee) was recommended for evaluation. The MDCT showed a grossly dilated right coronary artery (RCA) originating from the main pulmonary artery (Fig. 1). The left anterior descending coronary artery and diagonal branches were also diffusely enlarged and had multiple large collateral vessels (Fig. 2). No other structural anomalies were detected. Direct coronary angiography confirmed the anomalous origin of the RCA from the pulmonary artery (ARCAPA) (Fig. 3).
Fig. 1 Multidetector computed tomographic images (axial views) show a grossly dilated right coronary artery arising from the pulmonary artery (arrowhead) at the level of A) the main pulmonary artery bifurcation, B) the left main coronary artery, and C) the mid-left ventricle. D) Oblique coronal image shows the prominent, tortuous right coronary artery (arrow) and its anomalous origin from the pulmonary artery (arrowhead).
Ao = aorta; LMCA = left main coronary artery; LPA = left pulmonary artery; LV = left ventricle; MPA = main pulmonary artery; RPA = right pulmonary artery; RV = right ventricle
Fig. 2 A) Frontal volume-rendered and B) oblique lateral volume-rendered 3-dimensional multidetector computed tomographic images show a diffusely enlarged left anterior descending coronary artery (LAD) with multiple collateral vessels, and the anomalous right coronary artery (RCA) arising from the pulmonary artery (PA) (arrowheads).
Fig. 3 Direct coronary angiogram of the aortic root shows a dilated, tortuous proximal left coronary artery.
Real-time motion image is available at www.texasheart.org/journal.
Comment
Anomalous origin of the RCA from the pulmonary artery is a rare congenital coronary anomaly with an estimated prevalence of 0.002%.1 The first cases were described by Brooks in 1885,2 and since then only 100 cases have been reported. Most patients are asymptomatic, and their anomalies are detected incidentally.1,3 This is in contrast with anomalous origin of the left coronary artery from the pulmonary artery, which is characteristically fatal during early infancy without immediate surgical management. Clinical symptoms of ARCAPA depend upon the degree of collateralization between the RCA and the left coronary system. Anomalies such as aortopulmonary window, tetralogy of Fallot, and septal defects have been reported in 22% of patients.4 Definitive therapy requires surgical translocation of the anomalous RCA to the aortic root.5
As illustrated by this case, MDCT is well suited for evaluating ARCAPA because of its high spatial and temporal resolution and its flexible post-processing techniques for distinguishing complex coronary anatomy.1
Supplementary Material
Footnotes
Address for reprints: Christopher D. Maroules, MD, Department of Radiology, Naval Medical Center Portsmouth, 620 John Paul Jones Circle, Portsmouth, VA 23708
E-mail: Christopher.Maroules@gmail.com
References
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