A 53-year-old man was admitted because of exertional dyspnea. Transthoracic echocardiography revealed aortic valve (AV) deformity. The aortic cusps.
Images in Cardiovascular Medicine
Quadricuspid Aortic Valve with Anomalous Coronary Artery Comprehensive Evaluation with Multidetector Computed Tomography
Na Wang, MD Chuanchen Zhang, MD Zhaoqi Zhang, MD Buxing Chen, MD Fenghe Du, MD
A
53-year-old man was admitted because of exertional dyspnea. Transthoracic echocardiography revealed aortic valve (AV) deformity. The aortic cusps were not clearly defined. Doppler examination revealed severe aortic regurgitation. It was decided to use cardiac multidetector computed tomography (MDCT) to clarify the AV configuration and to rule out coronary stenosis before operation. The MDCT images showed a quadricuspid AV with 4 equal-sized cusps, a central coaptation defect, and central aortic regurgitation (Fig. 1). The ostia of the left anterior descending coronary artery (LAD) and the right coronary artery (RCA) were juxtaposed at the right coronary sinus (Fig. 2). The proximal segment of the LAD coursed
Section Editor: Raymond F. Stainback, MD, Department of Adult Cardiology, Texas Heart Institute at St. Luke’s Episcopal Hospital, 6624 Fannin St., Suite 2480, Houston, TX 77030 From: Division of Cardiology (Drs. Chen, Du, and Wang), Capital Medical University Beijing Tiantan Hospital, Beijing 100050; and Division of Radiology (Drs. Wang, C. Zhang, and Z. Zhang), Capital Medical University Beijing Anzhen Hospital, Beijing Institute of Heart, Lung & Blood Vessel Diseases, Beijing 100029; People’s Republic of China Address for reprints: Fenghe Du, MD, Division of Cardiology, Capital Medical University Beijing Tiantan Hospital, No. 6 Tiantanxili, Chongwen District, Beijing 100050, PRC E-mail: fenghedu2008@ yahoo.com.cn © 2012 by the Texas Heart ® Institute, Houston
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Fig. 1 Multidetector computed tomogram (short-axis view) of the aortic valve during diastole shows 4 equal-sized cusps and lack of coaptation of the valve leaflets, with a regurgitation orifice.
Fig. 2 Multidetector computed tomogram (oblique axial view) reveals that the ostia of the left anterior descending coronary artery (arrowhead) and the right coronary artery (arrow) are juxtaposed at the right coronary sinus. Calcium is also found at the ostium of the right coronary artery.
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between the aortic root and the right ventricular outf low tract (Fig. 3). The left circumflex coronary artery originated from the proximal segment of the RCA (Fig. 4) and coursed posterior to the aortic root, and then into the atrioventricular groove (Fig. 5). The ascending aorta was mildly enlarged. No significant coronary stenosis was found. The MDCT findings of a quadricuspid AV
Fig. 5 Volume-rendered multidetector computed tomogram shows that the proximal segment of the left circumflex coronary artery (LCx) courses posterior to the aortic root and then into the atrioventricular groove. Ao = aorta; LA = left atrium; RCA = right coronary artery; RVOT = right ventricular outflow tract
and anomalous coronary arteries were all confirmed during the subsequent surgery.
Comment
Fig. 3 Volume-rendered multidetector computed tomogram reveals that the left anterior descending coronary artery (LAD) originates from the right aortic sinus and courses between the aortic root and the right ventricular outflow tract.
Fig. 4 Multidetector computed tomogram (oblique axial view) reveals that the left circumflex coronary artery (arrowhead) originates from the proximal segment of the right coronary artery (arrow).
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Quadricuspid Aortic Valve with Anomalous Coronary Artery
Quadricuspid AV is a very rare congenital anomaly.1 Anomalous coronary artery origin in addition to quadricuspid AV is even rarer. The combined phenomenon often prompts surgery because of severe aortic regurgitation or the risk of sudden death.2-4 Comprehensive evaluation of the valves and coronary arteries, and their relation with adjacent structures, is essential to preoperative planning. In this patient, MDCT—with its high temporal and spatial resolution—provided clear images of the quadricuspid AV, mildly dilated ascending aorta, anomalous location of the coronary ostia, abnormal courses of the coronary arteries, and absence of significant coronary stenosis. The last 3 of these were not visible on echocardiography. Furthermore, transthoracic echocardiography is hard to use in patients with emphysema and thoracic deformities. Such patients physically tolerate MDCT more easily than transesophageal echocardiography. Early identification and comprehensive evaluation of quadricuspid AV and anomalous coronary artery origin are crucial because of the risk of sudden death. Multidetector computed tomographic imaging can provide detailed information for the diagnosis of this complex situation and the selection of appropriate interventions. This approach can be especially useful in improving preoperative planning. Volume 39, Number 2, 2012
References 1. Feldman BJ, Khandheria BK, Warnes CA, Seward JB, Taylor CL, Tajik AJ. Incidence, description and functional assessment of isolated quadricuspid aortic valves. Am J Cardiol 1990;65(13):937-8. 2. Basso C, Maron BJ, Corrado D, Thiene G. Clinical profile of congenital coronary artery anomalies with origin from the wrong aortic sinus leading to sudden death in young competitive athletes. J Am Coll Cardiol 2000;35(6):1493-501. 3. Mutsuga M, Tamaki S, Yokoyama Y, Kato N, Yokote J, Ohata N, Suzuki T. Acute occlusion of left coronary ostium associated with congenital quadricuspid aortic valve. Ann Thorac Surg 2005;79(5):1760-1. 4. Kurosawa H, Wagenaar SS, Becker AE. Sudden death in a youth. A case of quadricuspid aortic valve with isolation of origin of left coronary artery. Br Heart J 1981;46(2):211-5.
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