Aortic Implantation Is Possible in All Cases of Anomalous Origin of the Left Coronary Artery From the Pulmonary Artery

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1 Aortic Implantation Is Possible in All Cases of Anomalous Origin of the Left Coronary Artery From the Pulmonary Artery Kevin Turley, MD, Robert J. Szarnicki, MD, Keith D. Flachsbart, MD, Richard C. Richter, MD, Robert W. Popper, MD, and Harold Tarnoff, MD California Pacific Medical Center, Kaiser Perrnanente Medical Center, and University of California, San Francisco, California Background. Anomalous origin of the left coronary artery from the pulmonary artery (PA) optimally is treated by creation of a multiple coronary system. This study explores the use of aortic implantation employing alternative methods to achieve coronary transfer in all patients, regardless of the site of origin of the anomalous coronary artery, avoiding the problems of bypass grafts and tunnel procedures. Methods. During the period 1986 to 1994, 11 patients aged 6 months to 8 years (mean age, 2.6 years) underwent repair. Coronary artery origin from the PA included left sinus in 3, posterior in 2, right sinus in 2, intramural aorta with its orifice at the bifurcation of the main and right PA in 1, high left main PA in 1, high at the bifurcation of main and right PA in 1, and anterior in 1. Findings included angina in 4, prior infarctions in 3, ischemia in 7, left ventricular dysfunction in 6, mitral regurgitation in 5, atrial septal defect in 2, and echocardiograms suggestive of endocardial fibrosis in 4. One patient had prior ligation with ventricular dysfunction and collateralization and recanalization. A single patient was asymptomatic. Repair was accomplished by direct transfer using the PA sinus of Valsalva as a button in only 6; tubular reconstruction was used in 4 when the distance was too great to avoid tension; 2 short tubes were constructed with PA wall in 2 of the 3 left sinus origins, whereas 2 long tubes of PA wall were used (1 high on the left side of the main PA and 1 with left anterior descending origin from the anterior sinus of Valsalva in a patient with malrotation [end neo-artery to side aortic reconstruction]); finally, in situ transfer and intraaortic reconstruction (unroofing and anastomosis) was performed in 1 intramural coronary artery. Division of the PA, mobilization of the distal PA, division of the ductus, and direct reanastomosis of the PA was performed in 3 tubular reconstructions, as well as all 6 direct coronary transfers. Results. There were no operative or late deaths. Follow-up of 2 to 100 months (mean, 46 months) revealed no new angina or infarctions, improved function and decreased mitral regurgitation. Echocardiographic and angiographic studies demonstrated patency and prograde flow in the new coronary systems. Conclusions. Aortic implantation is the treatment of choice for anomalous origin of the left coronary artery. Methods such as direct transfer, tubular reconstruction, and in situ transfer make such implantation possible in all patients regardless of the site of coronary origin, distance from the aorta, or coronary artery configuration. (Ann Thorac Surg 1995;60:84-9) A nomalous origin of the left coronary artery from the pulmonary artery is a rare congenital anomaly that often presents insidiously in early infancy with infarction. First described in 1885, the clinical syndrome was explored by Bland, White, and Garland in 1933 [1]. They demonstrated the combined effects of absence of a normal coronary flow with a coronary steal and the profound ischemia that can result. Early attempts to treat this problem with a simple ligation resulted in a high mortality, and construction of a two coronary artery system has been recognized as the treatment of choice [2, 3]. The optimal method of such reconstruction remains contro- Presented at the Thirty-First Annual Meeting of The Society of Thoracic Surgeons, Palm Springs, CA, Jan 30-Feb 1, Address reprint requests to Dr Turley, Pediatric Cardiovascular Surgery, California Pacific Medical Center, 2100 Webster St, #332, San Francisco, CA versial. Methods used in the past to achieve this goal include bypass graft procedures with the potential for distal anastomotic stenosis and tunnel procedures, in which tunnel obstruction and pulmonary arterial obstruction have been reported [2-8]. Coronary transfer, popularized by experience with neonatal arterial switch procedures, currently represents the most attractive method of creating a multiple coronary system and has been employed successfully in many such patients [3, 9-12]. However, anatomic variations of the anomalous coronary origins often preclude direct transfer [3, 12]. The current study explores methods of aortic implantation including both standard transfer techniques and alternate methods of arterial transfer regardless of the site of origin of anomalous coronary to provide arterial reconstruction of a multiple coronary artery system in all patients by The Society of Thoracic Surgeons /95/$ (95) O

2 Ann Thorac Surg TURLEY ET AL ;60:84-9 AORTIC IMPLANTATION OF ANOMALOUS CORONARY uctus Fig 1. Site of coronary origin from the pulmonary artery (PA) in the current series: (a) right sinus of Valsalva in 2, (b) left sinus of Valsalva in 3, (c) anterior in 1 (malrotation of left anterior descending coronary artery), (d) posterior in 2, (e) left main pulmonary artery high in 1, and (f) right main pulmonary artery at bifurcation of the right pulmonary artery in 2. (Ao = aorta.) Material and Methods Patients During the period 1986 to 1994, 11 patients, aged 6 months to 8 years (mean age, 2.6 years) underwent aortic implantation. The site of coronary artery origin from the pulmonary artery included the left sinus of Valsalva in 3 patients, posterior in 2 patients, right sinus of Valsalva in 2 patients, intramural aorta with orifice at the bifurcation of the main and right pulmonary artery in I patient, high on the left main pulmonary artery in I patient, high at the bifurcation of the main pulmonary artery and right pulmonary artery in 1 patient, and anterior or facing sinus in 1 patient (the left anterior descending coronary in a patient with malrotation) (Fig 1). The pulmonary sinuses were designated as right, left, and facing according to the view from the posterior commissure looking anteriorly. Preoperative findings included angina in 4, prior infarctions in 3, ischemia in 7, left ventricular dysfunction in 6, mitral regurgitation in 5, atrial septal defects in 2, and echocardiograms suggestive of endocardial fibroelastosis (no biopsies were performed) in 4. One patient had a history of prior attempted ligation of the anomalous left coronary artery with ventricular dysfunction and subsequent collateralization and recanalization of the anomalous coronary artery. A single patient in the series was completely asymptomatic from the cardiovascular system (Table 1). Of note, 3 patients were asymptomatic from the coronary system, including this patient and 2 in whom an atrial septal defect with increased pulmonary arterial pressure, increased pulmonary arterial satura- tions, and increased pulmonary vascular resistance was present. Six other patients presented in early infancy including the 1 in whom ligation was performed at 2 months of age. Thus, only 2 of 11 patients presented late and symptomatic, I at age 3 years with angina manifested by chest pain with exertion and 1 at 8 years with supposed cardiomyopathy but excellent collaterals. Three additional patients presented with angina, one 11- month-old and one 6-month-old with pain on feeding, the classic presentation in the original description by Bland, White, and Garland [1]. A third patient had this as the original presentation at 2 months of age (attempted ligation) and in our series pain on exertion with dyspnea. Echocardiographic evaluation demonstrated the anomaly in 9 patients; however, despite the advances in color flow Doppler imaging, it failed to identify the abnormal coronary artery in 2. In both of these patients the diagnosis was entertained and the echocardiogram was performed in an attempt to define its presence. Preoperative catheterizations were performed in 11 patients, including 2 patients in whom prior echocardiography had failed to demonstrate the anomalous coronary artery. In 2 patients atrial septal defects were identified by two-dimensional color-flow echocardiography with significant left-to-right shunting, flattened septal motion, and right ventricular volume overload. Increase in pulmonary arterial saturations and pulmonary vascular resistance was noted at catheterization in each. Both patients were asymptomatic from their coronary lesions. A third patient, 2.8 years old, with left main coronary artery arising from the left sinus of Valsalva, was totally asymptomatic at the time of diagnosis. Surgical Technique Operation for all patients was performed via a median sternotomy and bicaval cannulation with cardiopulmonary bypass with moderate hypothermia. Both great arteries then were clamped in 7 patients with cold blood cardioplegic solution administered both to the aortic root and main pulmonary artery. In 4 patients aortic root administration alone occurred with digital occlusion of the anomalous coronary orifice. In the most recent cases, continuous retrograde cardioplegia has been added during the procedure. In addition venting of the left heart structures via the atrial septum, left atrial appendage, or right superior pulmonary vein was used to prevent distention in these compromised ventricles. The surgical technique involved division of the main pulmonary artery and mobilization of the distal pulmonary artery with division of the ductus in 10 of the 11 patients. In 6 of these patients the ostium of the left coronary artery was excised with a sizeable cuff of adjacent sinus of Valsalva and the left coronary artery was mobilized. A punch then was used to produce an opening in the left lateral side of the ascending aorta, care being taken to avoid injury to the aortic valve by a low incision. The anastomosis was performed using 7-0 absorbable continuous suture (polyglyconate) (Fig 2). In 4 patients, tubular reconstruction was used when the distance was too great to avoid tension. Two short tubes were constructed from the

3 86 TURLEY ET AL Ann Thorac Surg AORTIC IMPLANTATION OF ANOMALOUS CORONARY 1995;60:84-9 Table 1. Aortic Implantation in Patients With Artery Anomalous Coronary Age Site Findings Sinus Repair PA Repair 4.5 y LAD Dextrorotation, atrial septal Anterior Long tube (6 cm) Pericardial patch defect, Qp:Qs-3:l, PVR-1.7 facing Wood units 2.8 y Left main Asymptomatic Left Short tube (1.5 cm) Direct anastomosis 4.5 y Left main 2-month infarct, ligation Right high Transfer, resection of Direct anastomosis (2 too), calcification of scarred LAD endocardium, papillary muscle fibrosis, dyspnea angina, mitral regurgitation 6 mo Left main Angina (feedings), mitral RPA-MPA Unroofed, None regurgitation, dilated left junction anastomosis and ventricle entry site occlusion 8 y Left main Dilated left ventricle Posterior Transfer Direct anastomosis (cardiomyopathy) 9 mo Left main Infarct, echocardiogram High left Long tube (5 cm) Direct anastomosis suggestive of endocardial MPA fibroelastosis 6 mo Left main Infarct, mitral regurgitation, Right Transfer Direct anastomosis echocardiogram suggestive of endocardial fibroelastosis 3 y Left main Angina Right Transfer Direct anastomosis 11 mo Left main Angina Left Short tube (1.5 cm) Direct anastomosis 2 y Left main Atrial septal defect, Qp:Qs- Posterior Transfer Direct anastomosis 13 mo Left main 2.2:1, PVR-2.1 Wood units Dilated left ventricle, mitral regurgitation, echocardiogram suggestive of endocardial fibroelastosis Posterior left Transfer Direct anastomosis LAD left anterior descending coronary artery; PA = pulmonary artery; PVR = pulmonary vascular resistance; Qp:Qs - pulmonary-tosystemic shunt. pulmonary artery wall in 2 of the 3 patients with a left sinus of Valsalva origins of the anomalous coronary to avoid such tension. Two short lateral flaps were dissected and lateral suture lines constructed, again using absorbable 7-0 suture material. The proximal anastomosis was performed as with the standard direct transfer (Fig 3). In I patient the origin was high on the left side of the main pulmonary artery. In this case two long flaps of main pulmonary artery tissue were used to construct a long tube. This tube was then anastomosed to the aorta, again with 7-0 absorbable suture (Fig 4). In 1 patient with malrotation a left anterior descending coronary artery arose from the anterior or facing sinus of Valsalva (Fig 5); a long flap of pulmonary artery wall based to the left was used to fold around the orifice of the left anterior descending coronary and a single suture line of 7-0 absorbable suture was constructed to produce a tube, which then was anastomosed to the anterior aspect of the ascending aorta. Finally, in 1 patient, the coronary artery passed behind the main pulmonary artery and entered Fig 2. Coronary transfer. A cuff of pulmonary arterial wall is excised with the orij~'ce of the aberrant coronary and the artery is mobilized distally. A punch is used to create an orij~'ce in the aorta and anastomosis is performed. The main pulmonary artery, having been dissected to the hilum bilaterally and the ligamentum divided, then is anastomosed in an end-to-end fashion. Fig 3. Short tube reconstruction used in 2 patients.

4 Ann Thorac Surg TURLEY ET AL ;60:84-9 AORTIC IMPLANTATION OF ANOMALOUS CORONARY l Fig 4. Long tube reconstruction with two lateral flaps used to construct the tube from high on the left side of the main pulmonary artery. the aortic wall, running intramurally, and then exited to find its origin at the bifurcation of the main and right pulmonary artery (Fig 6). In this case the pulmonary artery was not divided, but rather the aorta was incised. The intramural portion of the coronary was unroofed intraluminally, and its intima was attached to the aortic intima with interrupted 7-0 absorbable suture. The origin of the coronary artery at the main to right bifurcation then was ligated. In this case the aorta was closed directly and the cross-clamp removed. In the 10 patients in whom division of the pulmonary artery was performed, the cross-clamp was removed after completion of the coronary anastomosis to evaluate coronary perfusion. Main pulmonary artery continuity then was reestablished by direct anastomosis in 9. This was possible due to the aggressive mobilization of the distal pulmonary artery and division of the ductus as performed in mobilization for the LeCompte maneuver. In the single patient with the left anterior descending coronary arising anteriorly, a pericardial patch was used to reconstruct the longitudinal flap defect produced by mobilization of tissue for the tube reconstruction (see Table 1). Although mitral regurgitation was noted in 5 patients, none had severe (grade 3) insufficiency and no operative intervention was undertaken. Atrial septal defects were closed in 2. Primary closure of the chest was possible in Fig 5. Long tube reconstruction of the left anterior descending coronary artery arising anterior in a patient with malrotation. The orifice was in the sinus of Valsalva and eccentrically placed. Thus, the entire sinus was excised with a long flap of tissue superiorly. This was folded over with a single suture line laterally. The pulmonary artery was reconstructed using autologous pericardium. Fig 6. Unusual path of the intramural coronary artery with its origin at the bifurcation of the main and right pulmonary artery. Reconstruction was accomplished by unroofing the intramural portion of the coronary within the aorta, suturing the coronary intima to the aortic intima, and ligating the orifice of the aberrant coronary artery. all 11 patients with hemodynamic stability, and in no patient was extracorporeal membrane oxygenation or right or left ventricular assist used. Results There were no early or late deaths during the follow-up of 2 to 100 months (mean, 46 months). Echocardiographic evaluation was available in 11 and angiographic evaluation in 4. In each case, patency and prograde flow of the new coronary artery systems were demonstrated and no pulmonary artery obstructions were evident. No new angina has occurred and no new infarctions were demonstrated by electrocardiography. Improved left ventricular function was noted in 5 of 6 patients, although this became evident 3 to 6 months postoperatively in 2. Mitral regurgitation was improved in 4 of 5 patients with only mild insufficiency persistent in a patient with echocardiogram suggestive of endocardial fibroelastosis. Of the nine direct anastomoses, four demonstrated no valve insufficiencies and therefore right ventricular/pulmonary artery pressure could not be estimated. In 3 patients normal right ventricular/pulmonary artery pressures of 15, 12, and 14 mm Hg were observed. In the most recent patient a pulmonary artery gradient of 22 mm Hg was noted on initial postoperative examination, which has decreased to 16 mm Hg 6 months postoperatively. In 1 patient a 34 mm Hg estimated right ventricular pressure was recorded with a pulmonary artery gradient of 16 mm Hg. The right ventricular pressure decreased to 30 mm Hg 1 year later with gradient unchanged (mild stenosis). No residual shunts were noted in the patients with atrial septal defects, and reversal of the flattened septal motion in these patients was indicative of atrial septal closure rather than specific reversal of ischemia. Echocardiogram suggestive of endocardial fibroelastosis persisted in all 4 patients in whom it had been present preoperatively. Comment This study explores the methods available to create aortic implantation in all patients with anomalous left coronary

5 88 TURLEY ET AL Ann Thorac Surg AORTIC IMPLANTATION OF ANOMALOUS CORONARY 1995;60:84-9 artery systems from the pulmonary artery. Backer and associates [3], Alexi-Meskishvili and co-workers [12], and Vouh6 and colleagues [10] clearly have demonstrated the efficacy of the creation of a multiple coronary system in such patients, and experience with arterial transfer in patients with transposition of the great arteries has made this modality standard for repair of the anomalous coronary artery. Other methods such as bypass grafting and tunnel procedures have been restricted to difficult sites of origin. Each of these methods is fraught with potential late complications, in the former distal anastomotic stenosis with obstruction and occlusion and in the latter, both obstruction of the tunnel itself and distal coronary occlusion, as well as pulmonary arterial obstruction [2, 7, 8]. Repair using the method described in our 5 patients in whom direct transfer was not possible affords alternatives to these methods avoiding their potential shortfalls. Armer and associates [13] first described the flap reconstruction approach in an autopsy reconstruction in We described our preliminary experience with this approach at the meeting of the European Chapter of the International Society for Cardiovascular Surgery in Subsequent to this report Tashiro and associates [14] described 2 adult patients in whom a tubular reconstruction was used with a side-to-side anastomosis posterior to the aorta. This technique, although providing an extravascular tunnel, has the potential for tunnel obstruction seen in the Takeuchi technique, because a side-to-side anastomosis with blind end and large saccular component results. The direct tubular approach used in the current study provides for linear flow from the aortic lumen to the distal coronary artery. In the current study no patient required delayed sternal closure or ventricular assist. The absence of a patient with acute infarction in the study group accounts in part for this, but also close observation to the principles of myocardial protection via both antegrade and retrograde cardioplegic administration, attention to adequate perfusion by prevention of pulmonary arterial run-off, and protecting the left heart structures from acute distention [10, 12]. Improvement in ventricular function has been demonstrated after reestablishment of normal coronary perfusion [10, 12, 15]. This may be present initially or may be delayed for several months. Although the Berlin group recommends a modified Kaye annuloplasty to improve mitral valve function, this was performed only in severe class 3 mitral incompetence in that series [12]. In the current series only mild to moderate regurgitation was noted and the response to myocardial revascularization, even in the face of endocardial fibroelastosis, produced excellent hemodynamics and avoided the need for mitral valve intervention. Finally, echocardiogram suggestive of fibroelastosis persisted in 4 patients, consistent with prior experience, reinforcing an aggressive approach to the identification and treatment of this anomaly at the earliest possible time [16]. It was hoped with the advent of color-flow Doppler two-dimensional echocardiography that early recognition of this problem could be achieved before infarction. However, recent experience has tempered our enthusiasm and must heighten our vigilance in identifying this insidious problem so that repair can occur before devastating infarction. The current approach represents such a method of repair. It can provide complete arterialization of a multiple coronary system, while avoiding the inherent problems associated with bypass and tunnel procedures. The method employs pulmonary arterial wall as in coronary transfers, where the cuff of the sinus of Valsalva is used to facilitate the transfer. No evidence of aneurysm formation has been encountered in using this technique in either the arterial switch or transfers in anomalous coronary arteries such as seen in this series. Thus, both short and long tubular reconstructions, which use the same material constructed into tubular extravascular structures providing linear flow from the aorta to the distal pulmonary artery bed, should provide a similar conduit. Care has been taken in the initial construction of these conduits to avoid any saccular configuration as in the Takeuchi or Tashiro procedure, and the approach itself avoids the potential for distal anastomotic obstruction inherent to bypass procedures. Aortic implantation is the treatment of choice for anomalous origin of the coronaries. Methods such as direct transfer, tubular reconstruction, and in situ transfer make such implantations possible in all patients regardless of the site of coronary artery origin, distance from the aorta, or coronary artery configuration. We thank Sophia E. Seto for research and preparation of the manuscript. References 1. Bland EF, White PD, Garland J. Congenital anomalies of the coronary arteries. Am Heart J 1933;8: Berdjis F, Takahashi M, Wells WJ, Stiles QR, Lindesmith GG. Anomalous left coronary artery from the pulmonary artery: significance of intercoronary collaterals. J Thorac Cardiovasc Surg 1994;108: Backer CL, Stout MJ, Zales VR, et al. Anomalous origin of the left coronary artery. A twenty-year review of surgical management. J Thorac Cardiovasc Surg 1992;103: Meyer BW, Stefanik G, Stiles QR, Lindesmith GG, Jones JC. A method of definitive surgical treatment of anomalous origin of left coronary artery: a case report. J Thorac Cardiovasc Surg 1968;56: Takeuchi S, Imamura H, Katsumoto K, et al. New surgical method for repair of anomalous left coronary artery from pulmonary artery. J Thorac Cardiovasc Surg 1979;78: Bunton R, Jonas RA, Lang P, Rein AJJT, Castaneda AR. Anomalous origin of the left coronary artery from pulmonary artery: ligation versus establishment of a two coronary artery system. J Thorac Cardiovasc Surg 1987;93: Midgley FM, Watson DC, Scott LP, et al. Repair of anomalous origin of the left coronary artery in the infant and small child. J Am Coll Cardiol 1984;4: Moises VA, Maciel BC, Swensson RE, et al. Left coronary artery-to-pulmonary artery communication (a late postoperative complication after the Takeuchi procedure for repair of anomalous origin of left coronary artery from the pulmonary artery) detected by color Doppler flow mapping. Am Heart J 1989;118: Necfies WH, Mathews RA, Park SC, et al. Anomalous origin of the left coronary artery from the pulmonary artery: a new method of surgical repair. Circulation 1974;50:582-7.

6 Ann Thorac Surg TURLEY ET AL ;60:84-9 AORTIC IMPLANTATION OF ANOMALOUS CORONARY 10. Vouh6 PR, Baillot-Venaant F, Trinquet F, et al. Anomalous left coronary artery from the pulmonary artery in infants: Which operation? When? J Thorac Cardiovasc Surg 1987;94: Neirotti R, Nijveld A, Ithuralde M, et al. Anomalous origin of the left coronary artery from the pulmonary artery: repair by aortic implantation. Eur J Cardiothorac Surg 1991;5: Alexi-Meskishvili V, Hetzer R, Weng Y, et al. Anomalous origin of the left coronary artery from the pulmonary artery. Early results with direct aortic reimplantation. J Thorac Cardiovasc Surg 1994;108: Armer RM, Shumaker HB, Lurie PR, Fisch C. Origin of the left coronary artery from the pulmonary artery without collateral circulation: report of a case with suggested surgical correction. Pediatrics 1965;32: Tashiro T, Todo K, Haruta Y, Yasunaga H, Nagata M, Nakamura M. Anomalous origin of the left coronary artery from the pulmonary artery. New operative technique. J Thorac Cardiovasc Surg 1993;106: Carvalho J, Redington AN, Oldershaw PJ, Shineburne EA, Lincoln CR. Analysis of left ventricular wall movement before and after reimplantation of anomalous left coronary artery in infancy. Br Heart J 1991;65: Dua R, Smith JA, Wilkinson JL, et al. Long-term follow-up after two coronary repair of anomalous left coronary artery from the pulmonary artery. J Card Surg 1993;8: DISCUSSION DR CHARLES B. HUDDLESTON (St. Louis, MO): I thank The Society for the opportunity to discuss the paper and compliment Dr Turley and his colleagues on their excellent series presented here. I think that most would agree now that a two-coronary type of reconstruction for this disease is the appropriate procedure, and most do it by coronary transfer rather than a tunnel technique. The uniqueness of this particular series is in the extensive use of pulmonary artery tissue to reconstruct by direct anastomosis the coronary artery that originates at a distance from the aorta. The experience with the arterial switch procedure has indicated that we can mobilize coronaries when they are at a distance, but, again, the concept of using the pulmonary artery tissue to construct tubes, as performed by Dr Turley and associates, is really the uniqueness of this series, and for that reason I think the focus should be on the 4 patients in whom that was performed. I assume that the long-term patency of these coronaries would be good on the basis of using autologous tissue, but there is a very long suture line in the lumen of this newly constructed coronary artery. Echocardiography gives some indication of patency but by no means is the gold standard, and I think it may be a little bit presumptuous to say that there is 100% patency on the basis of the echocardiogram alone. I urge that a follow-up cardiac catheterization be performed not only to assess the patency but to see if there are any stenotic areas that have developed in these long tubes to get a better idea of their long-term results. A technical modification that we have used in this operation is do a transverse aortotomy to locate precisely the place for implantation of the coronary. That allows for the direct visualization of the aortic valve leaflets and avoids potential injury to those when a button of aorta is excised for the anastomosis, particularly in very small children. One difference in our series from Dr Turley and associates, is that 70% of our patients are less than 1 year of age at presentation and many of those, if not most, are in extremis, requiring inotropes and mechanical ventilation after presentation. Their series is somewhat different in that the majority were more than 1 year of age. Doctor Turley, would you comment about that? I congratulate Dr Turley and his associates on their excellent results and appreciate the opportunity to review the manuscript beforehand. DR CONSTANTINE E. ANAGNOSTOPOULOS (New York, NY): I enjoyed the presentation. The existence of another autologous tissue with a potential of growth should be noted, namely the rectus sheath, in addition to the pulmonary artery; it has been used in 7 patients with long-term follow-up showing potential for growth and absence of thromboembolism. The questions that I have are, first, what was the form of myocardial protection, particularly for your very advanced patients, and second, have you had any patients who were turned down and recommended for transplantation, and what was the indication for that? DR TURLEY: I thank the discussants for their questions. In answer to Dr Huddleston, in the 11 patients, 4 angiograms have been performed postoperatively, including in 2 of the 4 patients with tubular reconstruction. Both demonstrated patency without anastomotic stenosis. With the advent of extensive use of echocardiography, follow-up angiograms rarely are used unless there are questions of an anatomic problem. We agree, however, clearly it would be beneficial to follow up such patients with this more precise method of anatomic delineation. The age of the patients represented the distribution during this time frame. In our earlier experience we have noted, as did you, infants often with a recent infarction. Their absence in the current study is noted in the manuscript, and they clearly represent a difficult group. However, 6 of our 11 patients did present in the first year including the 1 with prior ligation and the most recent, treated at 13 months. In answer to Dr Anagnostopoulos, no patients during this period were refused operative intervention and none were recommended for transplantation. As to the question of myocardial protection, we used cold asanguineous cardioplegic solution in the first 4 patients and cold blood cardioplegic solution in the subsequent 7, with cold retrograde cardioplegia in the last 3. At the initiation of bypass in the first 4, occlusion of the coronary artery was performed at the time of injection into the aortic root. In subsequent patients, we have injected both the aorta and pulmonary artery with cardioplegic solution with cross-clamp of both vessels, ensuring delivery of the cardioplegia and preventing run-off through the pulmonary system. I thank the discussants for their kind remarks.

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