Ann Thorac Surg 2001;72:1396-1398
© 2001 The Society of Thoracic Surgeons
Case report
Anomalous origin of the pulmonary artery from the right coronary artery
Qing-yu Wu, MDa,
Xiu-bin Yang, MDa
a Department of Cardiac Surgery, Cardiovascular Institute and Fu Wai Hospital, Chinese Academy of Medical Sciences, Beijing, People's Republic of China
Accepted for publication November 13, 2000.
Address reprint requests to Dr Wu, Department of Cardiac Surgery, Cardiovascular Institute and Fu Wai Hospital, Chinese Academy of Medical Sciences, Beijing 100037, People’s Republic of China
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Abstract
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A 5-year-old boy who had an anomalous origin of a stenotic pulmonary artery from the right coronary artery with ventricular septal defect and pulmonary atresia is reported. The diagnosis was made at operation. Successful repair was achieved for the complex anomaly by using a pulmonary artery homograft as a conduit and closing the ventricular septal defect.
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Introduction
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Anomalous origin of pulmonary artery (PA) trunk from the right coronary artery (RCA) in a patient with ventricular septal defect and pulmonary atresia is a rare entity and can evoke controversies in classification. We report the successful repair of such an anomaly.
A 5-year-old boy, weight 15 kg, was admitted with cyanosis in April 1995. Physical examination found mid-degree cyanosis, clubbed fingers and toes, no second sound, a grade 3/6 systolic murmur in the third and fourth intercostal space of the left sternal edge, and a grade 2/6 continuous machinelike murmur over the left second intercostal space. An electrocardiogram confirmed right ventricular hypertrophy and no myocardial ischemia. Chest radiography demonstrated absence of a PA shadow at the left upper cardiac border, reduced pulmonary vascularity, a boot-shaped heart, and right aortic arch. Echocardiography showed moderate right ventricular hypertrophy, ascending aorta enlargement and overriding (50%) of the ventricular septal defect (perimembranous infundibular defect, 15 mm in diameter), severe stenosis of the right ventricular outflow tract, and discontinuity between PA trunk and right ventricle. The proximal segment of the right coronary artery was enlarged. A patent ductus arteriosus was suspected.
Surgical correction was done through a median sternotomy. The heart was explored during the initial stages of the operation. The ascending aorta was enlarged and positioned anteriorly. The right ventricle was hypertrophied. The entire RCA was dilated but ran in its normal course. No PA trunk was found. After careful dissection, an unusual aspect of this patient’s anomaly was seen. The PA trunk originated from the RCA as the first branch (Fig 1). The proximal segment of the PA was severely stenotic and enlarged distally. The right and left pulmonary arteries and the bifurcation were nearly normal.
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Fig 1. The pulmonary artery (PA) trunk originated from the right coronary artery (RCA) as the first branch. The proximal segment of pulmonary artery was severely stenotic and enlarged distally.
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Hypothermic cardiopulmonary bypass was instituted by using routine ascending aortic and bicaval cannulations. The PA trunk was occluded temporarily to prevent blood flow into the pulmonary vasculature through the RCA and to ensure myocardial protection during crystalloid cardioplegia. The PA trunk was transected near the level of the anomalous RCA origin. A right-angled dissector that was passed through the stump of the coronary artery identified the RCA orifice (Fig 2). No pulmonary valve or attachment from the PA trunk to the right ventricle was found. There was no patent ductus arteriosus. The perimembranous infundibular ventricular septal defect was 20 mm in diameter, and a severely hypoplastic infundibular portion of right ventricle was confirmed through a right ventriculotomy. The surgical repairs were as follows: the stump of the RCA was oversewn using 5/0 prolene sutures without compromising the patency of the distal RCA; the ventricular septal defect was patched with Dacron through a right ventriculotomy; and a PA homograft was used to reconstruct the continuity between the PA trunk and right ventricle (Fig 3).
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Fig 2. The pulmonary artery (PA) trunk was transected near the anomalous origin, and a right ventriculotomy was done. (RCA = right coronary artery; RVI = right ventricular incision.)
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Fig 3. The stump of the right coronary artery side was oversewn. The ventricular septal defect was patched with Dacron through a right ventriculotomy. A pulmonary artery homograft was used to reconstruct continuity between the pulmonary artery trunk and right ventricle. A = the stump of the right coronary artery. B = the pulmonary artery homograft.
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The patient had a rapid and uncomplicated recovery. Presently, the patient is doing well more than 3 years later.
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Comment
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A similar entity, RCA-to-PA fistula in tetralogy of Fallot, has been reported [1–4]. The common pathophysiologic feature of these two types of anomalies is that the entire pulmonary blood flow is supplied from RCA. An important anatomic distinction differentiates these two anomalies. A fistula is a single, frequently tortuous, vessel with a single origin and a single termination. Therefore, RCA-to-PA fistula in tetralogy of Fallot terminates in the PA. In our case, however, the PA trunk originated from the RCA. There were no vestiges of a connection between the PA trunk and right ventricle. A coronary artery might be present.
A coronary artery "steal syndrome" might be present in patients with an anomalous origin of the left coronary artery from the PA [5]. However, the preoperative echocardiogram showed no evidence of ischemia, and there were no complaints of anginalike symptoms in this case or in patients with RCA-to-PA fistula [4]. Absence of steal syndrome may be related to severe stenosis in the proximal pulmonary trunk.
The correct diagnosis was not made preoperatively in this patient. Cardiac and selective coronary angiography could have made the precise diagnosis. During the operation careful surgical exploration facilitated the final diagnosis.
Pulmonary homograft interposition between the pulmonary artery and right ventricle currently is recommended for pulmonary hypoplasia or atresia in tetralogy of Fallot [5]. This procedure allegedly provides a competent pulmonary valve and reduced risk of pulmonary homograft calcification. Therefore, we selected a pulmonary artery homograft to reestablish continuity between the PA and right ventricle and obtained a satisfactory outcome. Successful repair also involves careful dissection and transection of the PA trunk and complete closure of the ventricular septal defect.
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References
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Krongrad E., Ritter D.G., Hawe A., Kincaid O.W., McGoon D.C. Pulmonary atresia or severe stenosis and coronary artery-to-pulmonary artery fistula. Circulation 1972;46:1005-1012.[Abstract/Free Full Text]
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Dark J.H., Pollock J.C.S. Coronary artery-pulmonary artery fistula in tetralogy of Fallot with pulmonary atresia. Eur Heart J 1985;6:714-716.[Abstract/Free Full Text]
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Bogers A.J.J.C., Rohmer J., Wolsky S.A.E., Quaegebeur J.M., Huysmans H.A. Coronary artery fistula as source of pulmonary circulation in pulmonary atresia with ventricular septal defect. J Thorac Cardiovasc Surg 1990;38:30-32.
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Solowiejczyk D.E., Coper M.M., Barst R.J., Quaegebeur J.M., Gersony W.M. Pulmonary atresia and ventricular septal defect with coronary artery to pulmonary artery fistula: case report and review of the literature. Pediatr Cardiol 1995;16:90-94.[Medline]
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Clarke D.R., Campbell D.N., Pappas G. Pulmonary allograft conduit repair of tetralogy of Fallot. An alternative to transannular patch repair. J Thorac Cardiovasc Surg 1989;98:730-736.[Abstract]
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Abstract
Introduction
