Ann Thorac Surg 1998;66:1797-1798
© 1998 The Society of Thoracic Surgeons
Case Reports
Anomalous right coronary artery from the pulmonary artery in Taussig-Bing anomaly
Benjamin W. Eidem, MDa,
Frank Cetta, MDa,
Patrick T. Roughneen, FRCSa,
Serafin Y. DeLeon, MDa,
Elizabeth A. Fisher, MDa
a Section of Pediatric Cardiology and Department of Thoracic and Cardiovascular Surgery, Loyola University Medical Center, Maywood, Illinois, USA
Accepted for publication May 14, 1998.
Address reprint requests to Dr Eidem, Section of Pediatric Cardiology, Loyola University Medical Center, 2160 First Ave, Maywood, IL 60153
e-mail: (beidem{at}wpo.it.luc.edu)
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Abstract
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The presence of associated anomalies in patients with double-outlet right ventricle can significantly alter surgical intervention. Preoperative delineation of these anomalies can facilitate surgical planning and improve outcome. We describe a case in which the right coronary artery and anterior descending coronary artery arose from the pulmonary artery in a patient with double-outlet right ventricle with subpulmonary ventricular septal defect (Taussig-Bing anomaly). Recognition of this important anomaly prevented significant intraoperative myocardial damage by altering techniques of cardioplegia administration for myocardial preservation.
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Introduction
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Patients with double-outlet right ventricle form a spectrum of lesions defined by the varying position of the ventricular septal defect and the relationship of the great arteries. More than half of patients with double-outlet right ventricle have additional cardiovascular anomalies that can significantly impact surgical timing and definitive correction. Coronary artery anomalies also vary considerably in these patients.
In 1949, Taussig and Bing described a patient with double-outlet right ventricle with side-by-side great arteries and a subpulmonary ventricular septal defect [1]. In subsequent series [2–4], patients with Taussig-Bing anomaly were noted to have normal coronary arterial anatomy or anomalies similar to patients with transposition of the great arteries. We describe a patient with typical anatomic features of Taussig-Bing Anomaly with unusual coronary artery anatomy. Recognition of this coronary anomaly was critical in successful operative repair.
An infant was born as the initial pregnancy of a 26-year-old woman at 40 weeks gestation. The initial newborn examination revealed the presence of multiple congenital anomalies, including coanal atresia, imperforate anus, urogenital fistula, and genital hypoplasia with hypospadias. Subsequent detailed evaluation revealed the presence of additional congenital anomalies suggestive of VACTERL syndrome [5]. Chromosomal studies documented a normal 46 XY karyotype.
An echocardiogram demonstrated double-outlet right ventricle with side-by-side great arteries (aorta to right). There was a single large ventricular septal defect located in the subpulmonary position consistent with a Taussig-Bing Anomaly [1]. No pulmonary stenosis was identified. The transverse aortic arch was significantly hypoplastic (measuring 3–4 mm) with a discrete area of coarctation in the juxtaductal region. Biventricular size and function were normal. The coronary artery anatomy was abnormal. A single coronary ostium was present in the posterior-facing aortic sinus, which gave rise to the circumflex coronary artery (Fig 1). No coronary artery was seen arising from the anterior-facing aortic sinus but rather the right coronary artery arose from the anterior-facing sinus of the pulmonary artery (Fig 2). The anterior descending coronary artery originated as a branch of the right coronary artery.
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Fig 1. Subcostal scan at the level of aortic and pulmonary valves. Left circumflex coronary artery seen arising from posterior-facing sinus of aortic valve and extending into the left atrioventricular groove. Ao = aortic valve; PA = pulmonary valve; LCX = left circumflex coronary artery.
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Fig 2. Parasternal short axis scan at level of aortic and pulmonary valves. Right coronary artery seen arising from anterior-facing sinus of pulmonary valve. Ao = aortic valve; PA = pulmonary valve; RCX = right coronary artery; LAD = left anterior descending coronary artery.
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At the time of surgery both the intracardiac and extracardiac anatomy was confirmed. The anomalous right coronary artery could be seen arising from the anterior-facing sinus of the pulmonary artery and giving rise to the anterior descending coronary artery. The circumflex coronary artery was seen arising from the posterior-facing sinus of the aortic valve. Cardiopulmonary bypass was achieved with cannulation of both the ascending aorta and main pulmonary artery for perfusion of the descending aorta via the ductus arteriosus and venous cannulation of the atrial appendage. The area of coarctation was repaired by an extended arch reconstruction with Gore-tex (W.L. Gore and Assoc, Flagstaff, AZ) patch enlargement of the of the transverse arch and proximal descending aorta. After coarctation repair the heart was arrested, the ascending aorta and main pulmonary artery were transected, and antegrade cardioplegia was further infused into each coronary orifice. The subpulmonary ventricular septal defect was exposed through the pulmonary valve and was closed with a Gore-tex patch. A Jatene arterial switch was then begun by excising a button of aortic tissue around the left coronary ostia and implanting it into the stump of the native main pulmonary artery. Because the right coronary ostium was already located in the anterior-facing sinus of the pulmonary valve, no relocation was necessary. A Lecompte maneuver was then performed and the arterial switch was completed in routine fashion. The patient was weaned from cardiopulmonary bypass with excellent hemodynamics and normal biventricular function.
Before hospital discharge, transthoracic echocardiography revealed normal biventricular function with a mild degree of neoaortic and neopulmonary valve regurgitation. Bilateral proximal pulmonary artery branch stenoses were identified; however, no supravalvular aortic or pulmonary stenoses were present. The patient has continued to do well on serial follow-up.
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Comment
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In patients with double-outlet right ventricle of the Taussig-Bing variant [1], coronary artery anatomy is typically normal or has a pattern similar to those described in dextrotransposition of the great arteries [2–4]. To our knowledge, anomalous origin of the right coronary artery from the pulmonary artery has not been reported. Furthermore, the pre-operative noninvasive identification of this anomaly by two-dimensional echocardiography and its important surgical implications deserve close attention.
Current surgical management of patients with Taussig-Bing anomaly involves closure of the ventricular septal defect with concomitant arterial switch operation to direct left ventricular outflow into the aorta and right ventricular outflow into the pulmonary artery. Surgical cannulation techniques and intraoperative myocardial preservation involve infusion of cardioplegia directly into the aortic root and coronary arteries. Lack of adequate myocardial protection during cardiopulmonary bypass can lead to myocardial injury or death. Failure to identify coronary artery anomalies in which one of the coronary arteries arises from the pulmonary artery will result in lack of adequate myocardial protection for the affected myocardium with potential devastating results. In this patient with Taussig-Bing anomaly, recognition of anomalous origin of the right coronary artery necessitated administration of cardioplegia into both the aortic and pulmonary arterial roots to ensure adequate myocardial protection. In addition, this coronary artery anomaly necessitated the translocation of only one coronary artery from the aortic root to the pulmonary root as part of the arterial switch operation.
In the current surgical era, most congenital cardiac lesions can be successfully repaired or palliated with limited surgical mortality. In the majority of these cases, significant improvement in surgical risk is achieved with accurate preoperative description of complex congenital cardiac anatomy. This report emphasizes the need for complete preoperative evaluation of patients with congenital heart disease before surgical intervention. Failure to identify rare but correctable congenital lesions will lead to poor surgical outcomes in these patients [2–6].
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References
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- Taussig H.B., Bing R.J. Complete transposition of the aorta and a levoposition of the pulmonary artery: clinical, physiological, and pathological findings. Am Heart J 1949;37:551-559.[Medline]
- Sridaromont S., Ritter D.G., Feldt R.H., Davis G.D., Edwards J.E. Double-outlet right ventricle: anatomic and angiocardiographic correlations. Mayo Clin Proc 1978;53:555-577.[Medline]
- Elliott L.P., Amplatz K., Edwards J.E. Coronary arterial patterns in transposition complexes: anatomic and angiocardiographic studies. Am J Cardiol 1966;17:362-378.[Medline]
- Pasquini L., Sanders S.P., Parness I.A., et al. Coronary echocardiography in 406 patients with d-loop transposition of the great arteries. J Am Coll Cardiol 1994;24:763-768.[Abstract]
- Quan L., Smith D.W. The VATER association, vertebral defects, and atresia, T-E fistula with esophageal atresia, radial and renal dysplasia: a spectrum of the associated defects. J Pediatr 1973;82:104-107.[Medline]
- Kanter K., Anderson R., Lincoln C., Firmin R., Rigby M. Anatomic correction of double-outlet right ventricle with subpulmonary ventricular septal defect (the "Taussig-Bing" anomaly). Ann Thor Surg 1986;41:287-292.[Abstract]
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Abstract
Introduction
