I worldwide [ 11. The overall number of transplantations

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1 Expanding Applicability of Transplantation After Multiple Prior Palliative Procedures Alan H. Menkis, MD, F. Neil McKenzie, MD, Richard J. Novick, MD, William J. Kostuk, MD, Peter W. Pflugfelder, MD, Martin Goldbach, MD, Hershel1 Rosenberg, MD, and The Paediatric Heart Transplant Group University Hospital and Children s Hospital of Western Ontario, University of Western Ontario, London, Ontario, Canada The number of heart transplantations performed over the past 3 years has plateaued. However, the number of pediatric transplantations continues to slowly increase. Unlike adult heart transplantation, for which cardiomyopathy remains the most frequent indication, structural congenital heart disease is the primary indication in children. This report reviews our experience with orthotopic heart transplantation in the presence of structural congenital heart disease with and without prior palliative repair. The diagnoses included transposition of the great arteries, common atrium, left superior vena cava with and without a bridging innominate vein, dextrocar- dia, and univentricular configurations. The palliative repairs included Blalock-Taussig shunt, bilateral Glenn shunt, Fontan repair, and Mustard and Rastelli procedure. There were no early deaths. Two rejection-related late deaths have occurred at 8 months and at 3 years postoperatively. Extended use of donor tissue and modifications to surgical technique allowed for successful orthotopic heart transplantation in these patients who had structural congenital heart disease with and without prior surgical palliation. ( 1991;52:722-6) n 1989, 2,437 heart transplantations were performed I worldwide [ 11. The overall number of transplantations has plateaued over the past 3 years, but the number of heart transplantations in the pediatric age group (0 to 18 years) is increasing. Since 1984 when approximately 50 transplantations were done in this age group worldwide, there has been a gradual increase to more than 200. Although acquired heart disease continues to be the most frequent indication for heart transplantation in adults, congenital heart disease has become the primary indication for heart transplantation in children. Much of this is due to the dramatic increase in newborns having transplantation for hypoplastic left heart syndrome. There are some congenital heart defects where the morphological right ventricle serves as the systemic ventricle (21. It has been suggested that the right ventricle cannot sustain systemic performance over the long term, which may lead to end-stage heart failure [3], resulting in referral for consideration of transplantation. As many as 10% to 20% of patients now alive with anatomical congenital heart lesions may undergo transplantation [4]. This report describes our experience with orthotopic heart transplantation for structural congenital heart disease with and without prior palliation. The group of patients with congenital heart defects who underwent subsequent orthotopic heart transplantation underscores the extended use of donor tissue to create a normal Presented in part at the Current Controversies and Techniques in Congenital Heart Surgery Meeting, Baltimore, MD, Sep S9, Address reprint requests to Dr Menkis, University Hospital, 339 Windermere Rd, London, Ont, Canada N6A 5A5. anatomical configuration that will make orthotopic transplantation possible. Material and Methods Patient Population To date, our experience includes 9 patients with congenital heart anomalies who have had orthotopic heart transplantation. Three patients, 2 girls 8 and 11 years old and a 6-year-old boy, had had prior palliative procedures. Several different anatomical defects were present in the 9 patients. Of particular importance were defects of systemic venous drainage, abnormalities of the atria, and abnormalities of the great vessels. Similarly, palliative repairs affecting systemic venous drainage, the great arteries, or the atria were relevant and required modifications of the standard transplantation technique to accommodate the anatomical varjations in the structural defects. The anatomical variations encountered included transposition of the great arteries in 3 patients, a common atrium or large atrial septa1 defect in 3 patients, a left superior vena cava with and without a bridging innominate vein in 2 patients, a Blalock-Taussig shunt in 1 patient, bilateral Glenn shunts in 1 patient, and a Fontan repair as well as a Mustard repair and a Rastelli repair in 1 patient. Anatomical Conditions and Operative Techniques ABNORMALITIES OF SYSTEMIC VENOUS DRAINAGE. When there was a persistent left superior vena cava with a bridging innominate vein, the confluence of the vein and atrium was simply oversewn once the existence and patency of the bridging vein had been assured (Fig 1) by The Society of Thoracic Surgeons /91/$3.50

2 1991 :52:722-6 CONGENITAL HEART MENKIS ET AL W C Fig 1. Repair of persistent left superior vena cava (LSVC) with innominate vein by donor end-to-side anastomosis on recipient innominate vein. (RSVC = right superior vena cava.) (Reprinted from Menkis AH, McKenzie FN, Novick R], et al. Special considerations for heart transplantation in congenital heart disease. I Heart Transplant 1990;9:602-7.) When there was no bridging innominate vein, the donor innominate vein was anastomosed directly to the left superior vena cava (Fig 2) [5]. One patient also had an Fig 2. Repair of persistent left superior vena cava (LSVC) without bridging innominate vein using direct end-to-end anastomosis. (RSVC = right superior Venn cava.) (Reprinted from Menkis AH, McKenzie FN, Novick R], et a/. Special considerations for heart transplantation in congenital heart disease. ] Heart Transplant 1990;9:602-7.) interrupted inferior vena cava with azygos continuation. This was managed by placing the inferior venous cannula in the largest of the veins entering the atrium and using the cardiotomy suction intermittently for the rest of the hepatic drainage. These vessels were included within the right atrium during the creation of the interatrial septum. Fig 3. Orthotopic transplantation when common atrium or large atrial septa1 defect is present. (IVC = inferior vena cava; SVC = superior vena cava.) (Reprinted from Menkis AH, McKenzie FN, Novick R], et al. Special considerations for heart transplantation in congenital heart disease. 1 Heart Transplant 1990;9:602-7.)

3 724 CONGENITAL HEART MENKIS ET AL Fig 4. Closure of Blalock-Taussig shunt from within the pulmonary artery. (Reprinted from Menkis AH, McKenzie FN, Novick R], et al. Special considerations for heart transplantation in congenital heart disease. 1 Heart Transplant 1990;9:602-7.) PULMONARY * SUBCLAVIAN 1991;52:722-6 COMMON ATRIUM. In patients with a common atrium or very large atrial septa1 defect or in several patients with a fenestrated fossa ovalis, which was excised, the donor left atrial wall was minimally trimmed, and this excess tissue was sewn directly to the posterior wall of the common atrium, thereby creating an interatrial septum (Fig 3). The anastomosis was commenced at the most inferior portion of the posterior atrial wall corresponding to where the interatrial septum should be and proceeded superiorly from the left atrial side to the left superior pulmonary vein where the suture was brought out through the recipient atrial wall. The other end of the suture was used to complete the inferior portion of the left atrial anastomosis. The free wall was left untied, and a left atrial vent was placed through the suture line. The vent was removed after the aorta had been unclamped and effective ventricular function returned [5]. LEFT BLALOCK-TAUSSIG SHUNT. The left Blalock-Taussig shunt was oversewn from within the left main pulmonary artery after excision of the native heart (Fig 4). The orifice could be clearly seen within the stump of the pulmonary artery. Visualization was aided by a short period of circulatory arrest [5]. GLENN SHUNTS. The patient with bilateral Glenn shunts was placed on cardiopulmonary bypass through cannulation into the ascending aorta in the usual fashion and a single venous cannula in the pulmonary artery. The left and right pulmonary arteries were dissected to expose the Glenn shunts bilaterally. Cardiopulmonary bypass was then instituted. Excision of the recipient heart was carried out under deep hypothermia and circulatory arrest. The anastomoses of the Glenn shunts to the pulmonary arteries were cut, and the pulmonary arteries were repaired bilaterally (Fig 5). The donor innominate vein was anastomosed end-to-end to the left superior vena cava, and the donor right superior vena cava was anastornosed end-to-end to the recipient right superior vena cava. The donor and recipient inferior venae cavae were anastomosed end to end [5]. FONTAN REPAIR. The same patient who had the bilateral Glenn shunts underwent a Fontan repair. This was characterized by a pericardial conduit extending from the inferior vena cava through the common atrium to the pulmonary artery with end-to-end anastomoses on the I --DONOR Fig 5. Bilateral Glenn shunts repair of pulmonary artery and restoration of normal venous anatomy. (LSVC = left superior vena cava; RSVC = right superior vena cava.) (Reprinted from Menkis AH, McKenzie FN, Novick R1, et al. Special considerations for heart transplantation in congenital heart disease. J Heart Transplant 1990;9: )

4 -AORTA -AORTA I r - A B Y D Fig 6. Orthotopic transplantation after Fontan repair: (A) appearance after excision of native ventricle; (B) creation of left atrial chamber; (C) Opening of left atrial chamber for anastomosis; and (0) technique for left atrial anastomosis. (IVC = inferior vena cava; POST = posterior.) (Reprintedfrom Menkis AH, McKenzie FN, Novick RJ, et al. Special considerations for heart transplantation in congenital heart disease. J Heart Transplant 1990;9:602-7.)

5 726 CONGENITAL HEART MENKIS ET AL 1991;52:722-6 inferior vena cava and pulmonary artery. After the recipient ventricles had been carefully excised, the conduit came into view (Fig 6A). The anterior wall of the tube was incised and opened after the pulmonary artery had been disconnected. The opened walls of the conduit were then sutured to the free atrial wall, thereby creating a closed left atrial chamber (Fig 6B). The posterior wall of the conduit was then opened, creating an orifice in the newly created left atrium (Fig 6C). To this orifice, the donor left atrium was anastomosed in the usual fashion (Fig 6D). The systemic venous anastomoses were then fashioned as already described, followed by the aortic and pulmonary artery anastomoses (see Fig 5) [5]. DEXTROPOSITION OR DEXTROCARDIA. Two patients had dextropositioned hearts and 1 patient, the little girl with the Fontan repair, dextrocardia. Although the transplanted hearts in all 3 tended to assume a midline or right-sided position, there was no embarrassment of venous filling and no cardiac dysfunction attributable to obstruction to venous inflow. MUSTARD REPAIR. Since the presentation of our paper, another child with prior palliative repair has undergone transplantation. The patient was a 6-year-old boy with a diagnosis of complex congenital heart disease characterized by levotransposition and pulmonary atresia. When he was 5 days of age, a right-sided Blalock-Taussig shunt was performed. Two years ago, Mustard and Rastelli-type repairs were undertaken. Pericardium was used for the Mustard repair, and a 19-mm cryopreserved pulmonary homograft was used for the pulmonary artery conduit. Venous cannulas were placed in the superior and inferior venae cavae and the aortic cannula was inserted through the ascending aorta. After excision of the native heart, the pulmonary conduit was excised to the level of the pulmonary bifurcation because of moderate calcification of the conduit. The Mustard baffle was identified. Both caval orifices were of adequate size and were not encroached on by the baffle. A portion of the baffle could have been retained to aid in reconstruction of the interatrial septum; however, the entire baffle was removed and the interatrial septum reconstructed as in the technique for common atrium. It was necessary to use the full length of donor pulmonary artery including the bifurcation, which was opened and accommodated the much larger recipient pulmonary bifurcation. Results Orthotopic transplantation was possible in all patients. There were no early deaths (<90 days). There have been two late deaths, one at 8 months and one at 3 years. Both were rejection related. All other patients were in functional class I. Comment Heart transplantation remains the only viable option for children with end-stage myocardial failure. The presence of structural congenital heart disease or prior palliative surgical intervention necessitates modifications to orthotopic transplantation techniques. In addition to the techniques described in this report, procedures for successful orthotopic transplantation have been described for transposition of the great arteries [6, 71, atrial situs inversus [8], Senning repair [9], and hypoplastic left heart syndrome [lo]. The uniform feature in each of these reports is the retention of sufficient contiguous donor tissue to accommodate the technical modifications required. Specifically, excess aorta, pulmonary artery, venae cavae, and atrial wall are retained in continuity with the donor heart at the time of harvest. Orthotopic heart transplantation is feasible for any patient with end-stage myocardial failure associated with structural congenital heart disease or prior palliative surgical repair. Although the results of pediatric heart transplantation are not as good as those obtained for adult transplantation, we believe that this is a viable therapy that usually results in a good quality of life for the children. References 1. Kriett JM, Kaye MP. The Registry of the International Society for Heart Transplantation: seventh official report J Heart Transplant 1990;9:32> Addonizio LJ. Cardiac transplantation in the pediatric patient. Prog Cardiovasc Dis 1990;33: Dobell AR. Capability of the right ventricle. Can J Cardiol 1988;4: Penkoske PA, Rowe RD, Freedom RM, Trusler GA. The future of heart and heart-lung transplantation in children. Heart Transplant 1984;3:22> Menkis AH, McKenzie FN, Novick RJ, et al. Special considerations for heart transplantation in congenital heart disease. J Heart Transplant 1990;9: Reitz BA, Jamieson SW, Gaudiani VA, et al. Method for cardiac transplantation in corrected transposition. J Cardiovasc Surg (Torino) 1982;23: Harjula ALJ, Heikkila LJ, Nieminen MS, Kupari M, Keto P, Mattila SP. Heart transplantation in repaired transposition of the great arteries. 1988;46: Doty DB, Renlund DG, Caputo GR, Burton NA, Jones KW. Cardiac transplantation in situs inversus. J Thorac Cardiovasc Surg 1990;99:49>9. 9. Mayer JE Jr, Perry S, O'Brien P, et al. Orthotopic heart transplantation for complex congenital heart disease. J Thorac Cardiovasc Surg 1990;99: Bailey LL, Concepcion W, Shattuck H, Huang L. Method of heart transplantation for treatment of hypoplastic left heart syndrome. J Thorac Cardiovasc Surg 1986;92:1-5.