Asian Cardiovasc Thorac Ann 2008;16:29-32
© 2008 Asia Publishing EXchange Ltd
Coronary Artery Fistulas in Pulmonary Atresia and Ventricular Septal Defect
Sathiakar P Collison, DNB,
Kulbhushan S Dagar, MCh,
Sunil K Kaushal, MCh,
Sitaraman Radhakrishanan, DM,
Savitri Shrivastava, DM,
Krishna S Iyer, MCh
Escorts Heart Institute and Research Center, New Delhi, India
For reprint information contact: Sathiakar P Collison, DNB, Tel: 91 11 2682 5000 Fax: 91 11 2682 5013 Email: spcollison{at}gmail.com, Escorts Heart Institute and Research Centre, Okhla Road, New Delhi 110 025, India.
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ABSTRACT
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Pulmonary atresia with ventricular septal defect is an anomaly with highly variable anatomy. Rarely, a coronary artery-to-pulmonary artery fistula may contribute to pulmonary blood flow. Since 1996, we have treated 4 patients with coronary-pulmonary fistula associated with pulmonary atresia and ventricular septal defect. Two fistulas originated from the left coronary, one from the right coronary, and one from a right-sided solitary coronary system. All terminated in the main pulmonary artery, which was adequate in all cases. The fistulas were managed by direct internal closure. Total intracardiac repair was then accomplished in all patients at the same sitting. There was one death. In children with favorable anatomy, direct closure of the fistula from the pulmonary artery is adequate and allows single-stage intracardiac repair.
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INTRODUCTION
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In the absence of right ventricular-to-pulmonary artery continuity, as in cases of pulmonary atresia (PA) with ventricular septal defect (VSD), the source of pulmonary blood flow can be variable. It may arise from different segments of the aorta and from many of its branches.1 It usually consists of major aortopulmonary collaterals arising from the descending aorta, abdominal aorta and less commonly from the subclavian artery and its branches.2 Equally often, pulmonary blood flow may be through a patent ductus arteriosus. Other rare sources include aortopulmonary window and persistent 5th aortic arch.3 Collaterals may also arise occasionally from the right or left coronary artery, and a number of cases have been reported.4–14 While the fistula is usually associated with additional collaterals, fistulas serving as the sole blood supply to the pulmonary circulation have been encountered.7,11,13 We describe our experience in managing such fistulas in patients with PA and VSD.
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PATIENTS AND METHODS
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From 1996 to 2005, 49 patients with PA and VSD were treated in this institution. Of these, 4 (8%) were found to have a coronary artery-to-pulmonary artery fistula. The case records, electrocardiograms, preoperative echocardiograms, preoperative cardiac catheterization data and angiograms were reviewed. All patients had detailed transthoracic echocardiograms preoperatively, and the adequacy of the main pulmonary artery (MPA) and branch pulmonary arteries was determined on the basis of Z scores. The nature of the blood supply to each segment of the lung was specifically examined at the time of angiography in patients with PA and native VSD. Catheterization was performed through the femoral artery and vein. Pressure and hemodynamic data were recorded, and a high descending aortogram was obtained using a pigtail catheter. Two patients underwent ascending aortography and selective coronary injection. The preoperative transthoracic echocardiography in both of them suggested an abnormal course of the coronary artery, and selective coronary injection was carried out to delineate the course of the vessel. In the other 2 patients, the coronary-pulmonary fistula was an unexpected intraoperative finding. When major aortopulmonary collaterals were found, they were entered and selective angiography was undertaken to assess their size and contribution to the pulmonary circulation. The operative record was used to determine the actual origin, course and termination of the coronary-pulmonary fistula, as well as the exact surgical technique employed in each case.
The mean age of the patients was 5.4 years, and the weight range was 7 to 40 kg. The salient features of each case are detailed in Table 1
. All patients had adequately sized and confluent central pulmonary arteries. In no case were the major aortopulmonary collaterals found to contribute the sole or predominant blood supply to any part of the lung. All preoperative electrocardiograms were normal. The origin, course and termination of the coronary-pulmonary fistulas varied considerably. The fistula arose from the left coronary artery in 2 patients, from the right coronary artery in one, and from a solitary coronary arterial system in the other. The fistula from the left coronary artery arose in both cases from the left anterior descending artery (Figure 1), took a tortuous course and terminated in the MPA. The fistula from the right coronary artery arose from the proximal segment, took a tortuous course across the right ventricular outflow tract, and terminated in the MPA. The fistula from the solitary coronary system was similar as it pursued a tortuous course across the right ventricular outflow tract and terminated in the MPA.
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Figure 1. Selective coronary angiogram demonstrating the main pulmonary artery (bold arrow) filling from the left coronary artery (black arrow).
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The operative techniques used to accomplish total correction of PA and VSD, and the means employed to manage the fistula are listed in Table 2. After sternotomy and pericardiotomy, all patients were put on standard cardiopulmonary bypass with core cooling to 28°C. During cooling, adequate dissection and control of the aorta, MPA, the right and left pulmonary arteries were achieved. The patent ductus arteriosus in patient no. 4 and the Blalock-Taussig shunt in patient no. 2 were ligated at this time. The left and right pulmonary arteries were temporarily occluded to prevent coronary steal into the pulmonary circulation and to ensure adequate distribution of cardioplegia to the myocardium. The aorta was cross clamped and cold blood cardioplegia was infused into the aortic root. On completion of cardioplegia delivery, a vertical arteriotomy was performed on the MPA, and the internal opening of the fistula was sought. This was found easily in all cases and closed directly with interrupted sutures of 5/0 polypropylene. The rest of the intracardiac repair was performed with incisions in the right atrium and right ventricle, appropriate to the individual anatomy (Table 2). All patients underwent epicardial echocardiography to confirm the adequacy of the repair before closure.
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RESULTS
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There was one death (patient no. 1) who had undergone surgery at the age of 6 months. Although his anatomy was such that the coronary fistulous tract crossed the right ventricular outflow tract, at operation it was considered feasible to perform the right ventricular outflow reconstruction by direct anastomosis of the pulmonary trunk to the right ventriculotomy. Epicardial echocardiography confirmed the adequacy of the procedure. In the immediate postoperative period, he had evidence of left ventricular decompensation and required ventilation, inotropic support, and diuretics. By the 3rd postoperative day, he had developed manifestations of right ventricular dysfunction. He died on the 5th postoperative day. The postoperative period was uneventful in the other 3 patients. Their mean duration of intensive care unit stay was 4 days, and the mean length of hospital stay was 12 days. Postoperative transthoracic echocardiograms revealed no residual defects. The 3 survivors were active and well at a mean follow up of 30 months (range, 12 to 48 months).
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DISCUSSION
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In general, the pulmonary supply in patients with VSD and PA may be duct-dependent or depend on systemic-to-pulmonary collateral arteries, and the central pulmonary arteries may be confluent or discontinuous. It is essential to assess whether the systemic-pulmonary collaterals comprise redundant or dual supply to the lungs, as this determines the need to incorporate them into the pulmonary circulation. Three of our patients had PA and VSD with adequate sized and confluent central pulmonary arteries and systemic-pulmonary collaterals that were small, few in number, and did not constitute the predominant blood supply to any part of the lung. We were able to manage the systemic-pulmonary collaterals by preoperative coil occlusion, and the fistula by direct closure, without the need to incorporate them into the pulmonary circulation. The 4th patient, although having a duct-dependent PA with VSD that was physiologically different, could also be successfully managed by this technique.
The prevalence of coronary-pulmonary fistula in patients with PA and VSD is thought to be 10%.15 However, most previous reports have been single case studies, so the true prevalence is still uncertain.4–14 This stems mainly from the fact that until the series of Amin and colleagues,15 routine ascending aortography was not emphasized. Hence it is possible that small fistulas may have been missed in some patients. Although a coronary-pulmonary fistula may provide the sole supply to the pulmonary arterial system, most often it is associated with major aortopulmonary collaterals, both of which provide important pulmonary blood flow.7,11,13 It must be emphasized that these fistulas are not trivial connections; however, myocardial ischemia and coronary steal have not been reported with coronary-pulmonary fistula associated with PA and VSD. The physiological aspects of coronary-pulmonary fistula were discussed by Amin and colleagues.15
Coronary-pulmonary fistula with PA and VSD should be repaired by any technique that allows preservation of the coronary circulation, and with adequate connection of the right ventricle to the pulmonary artery. It is useful to consider such fistulas to be of 2 types: those (as in our cases) in which significant pulmonary supply originates from the fistula, but with additional sources of pulmonary blood flow; and those in which the fistula is the sole source of the pulmonary circulation. The optimal surgical management of the fistula in either of these circumstances is uncertain. When the fistula is the sole source of pulmonary blood flow, the following approaches have been described: transection of the fistula with oversewing of the aortic end, simple ligation of the fistula, multistage procedures ultimately dividing the fistula and using fistulous tissue in right ventricular outflow reconstruction, and unifocalization of the fistula. In these reports, total repair with establishment of right ventricle-pulmonary artery continuity could be accomplished, with palliative systemic-to-pulmonary shunts in some of the older reports.1,8,12,14,15 Amin and colleagues15 proposed that fistulas supplying the pulmonary arterial system along with major aortopulmonary collaterals should be unifocalized provided they do not constitute redundant supply or can facilitate native tissue-to-tissue reconstruction. They also suggested that redundant fistulas be ligated at the time of definitive surgery. Simple ligation has been the favored approach in other reports.
We advocate pulmonary arteriotomy and suture closure of the fistula from within the MPA. This avoids dissection of the fistula prior to ligation, which could be hazardous as it may be tortuous and thin walled. This technique also prevents impingement on the openings of the main branches of the coronary arteries, lessening the risk of iatrogenic injury to the coronary arteries. The death of one of our patients was probably related to an error in surgical judgment. Although the coronary vessel crossed the right ventricular outflow tract, we considered that it feasible to perform native tissue-to-native tissue repair by direct anastomosis of the pulmonary trunk to the right ventriculotomy. However, the subsequent clinical course was probably related to impingement on the coronary artery. The use of a right ventricle-to-pulmonary artery conduit would have been the ideal option.
Coronary-pulmonary fistula arising from a solitary coronary system is very rare. As far as we know, only 3 cases have been reported previously: 2 from a left-sided solitary coronary artery and one from a solitary right coronary artery.8,12 Palliative shunts were performed in 2 patients. In the other, the MPA was detached from the coronary artery and a valved conduit was inserted between it and the right ventricle. The defect in the coronary vessel was then repaired; this patient died of severe hemorrhage on the 2nd postoperative day. Our patient (no. 4) had a right-sided solitary coronary vessel and the fistula pursued a tortuous course across the right ventricle outflow tract. He also had a patent ductus arteriosus and major aortopulmonary collaterals. His postoperative period was uneventful, and he was well at follow-up after 4 years.
A high index of suspicion is needed to diagnose this condition. When present, direct ligation of the opening of the fistula from within the main pulmonary artery gives good results in the subset of patients like ours with favorable pulmonary anatomy.
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ABSTRACT
INTRODUCTION
