H the past 5 years [l]. A major cause of this expansion. Pediatric Cardiac Transplantation Despite Atrial and Venous Return Anomalies

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1 Pediatric Cardiac Transplantation Despite Atrial and Venous Return Anomalies Claude Chartrand, MD Department of Cardiovascular Surgery, Sainte-Justine Hospital, University of Montreal, Montreal, Quebec, Canada Congenital anomalies of the atrium, pulmonary venous return, and systemic venous return are often regarded as anatomical contraindications to orthotopic cardiac transplantation. Among our pediatric transplant patients, 10 children aged 3 to 15 years, weighing 9 to 45 kg, and all previously operated on for a total of 18 interventions had 32 anomalies needing correction at the time of transplantation. Besides the 18 instances of great vessel abnormalities, 14 anomalies of the atrium and of the venous return were encountered either alone or in combination: single atrium or previous septectomy (4), hypoplastic left atrium (21, previous Mustard procedure (l), cor triatriatum (11, anomalous pulmonary venous return (3), and anomalous systemic venous return (3). The preparation of the donor heart was modified in four ways: right atrial paraseptal incision, left atrial flap technique, full-length mobilization of the pulmonary arteries, and aortic arch incision. Correction of the atrial and venous return anomalies was carried out at the time of orthotopic transplantation with the following techniques: atrial septation, atrial enlargement, superior systemic venous return reroofing, inferior systemic venous return reroofing, double venous rerouting (pulmonary and systemic), and septa1 realignment. One child died of pulmonary hypertension in the early postoperative period. After a follow-up ranging from 1 month to 52 months, all survivors are asymptomatic. Based on echocardiography, heart catheterization, and angiography, there are no stenoses and no shunts, and the atrial dimensions are good. Based on the results achieved with these surgical techniques, we conclude that most atrial lesions, anomalous pulmonary venous returns, and anomalous systemic venous returns are correctable at the time of orthotopic transplantation and do not preclude a successful outcome in children. (Ann Thorac Surg 1991;52:716-21) eart transplantation in children has expanded during H the past 5 years [l]. A major cause of this expansion is the increasing number of transplantations performed for cor,genital heart diseases. Within this group of patients, infant orthotopic transplantation accounts for most of tho growth [2]. However, according to the 1990 report of the Registry of the International Society for Heart Transplantation [l], the number of transplantations performed for congenital diseases in other age groups has not changed significantly. One explanation for this plateau is that congenital anomalies of the atrium, pulmonary venous return, systemic venous return, and the great vessels are often regarded as anatomical contraindications to orthotopic transplantation. My associates and I [3] have recently documented the feasibility of surgical correction of these congenital lesions at the time of orthotopic transplantation in our initial group of pediatric patients [3]. In this report, I will update our data on pediatric transplantation performed despite atrial and venous return anomalies. Presented in part at the Current Controversies and Techniques in Congenital Heart Surgery Meeting, Baltimore, MD, Sep b9, Address reprint requests to Dr Chartrand, Ste-Justine Hospital, 3175 Ch. CBte Ste-Catherine, Montreal, Quebec H3T 1C5, Canada. Material and Methods Among our pediatric transplant patients, 10 children with congenital heart diseases were selected for transplantation because of profound ventricular failure and prediction of early death. There were 2 girls and 8 boys. They ranged in age from 3 to 15 years and weighed between 9 and 45 kg. All patients had been operated on previously for a total of 18 corrective or palliative interventions. This group of patients had 32 congenital anomalies requiring additional surgical procedures at the time of transplantation. Besides the 18 lesions of the great vessels, 14 anomalies of the atrium, pulmonary venous return, or systemic venous return were corrected at the time of orthotopic transplantation. The anomalies, encountered singly or in association with other lesions, were as follows: single atrium or previous septectomy (4 patients), a previous Mustard operation (1 patient), hypoplastic left atrium (2), an abortive type of cor triatriatum (l), anomalous pulmonary venous return (3), and anomalous systemic venous drainage (3). Surgical Techniques The general approach including timing of the operation, patient preparation, dissection, and native heart excision has been presented previously [3]. Before graft implantation, the identified anomalies were corrected according to by The Society of Thoracic Surgeons /91/$3.50

2 Ann Thorac Surg CONGENITAL HEART CHARTRAND 717 PEDIATRIC CARDIAC TRANSPLANTATION When the recipient pulmonary artery or branches need to be reconstructed and when the great vessels are in D- or L-transposition or in malposition, the donor right and left pulmonary arteries are completely freed and divided beyond the pericardial reflection, and the aorta is divided at the brachiocephalic trunk. Atrial Septation A Mustard procedure had previously been performed in 1 patient. At transplantation, the baffle was totally resected, and a rectangular synthetic patch was used for septation. In 2 patients with single atrium and in 2 other children in whom total septectomy had previously been done, straight atrial septation was carried out using a Dacron patch or autogenous pericardium. In all patients, aided by the straight paraseptal incision, a single septal anastomosis was performed (Fig 2). Donor pericardium could have been used as well for septation. Postoperative angiography showed normal septal orientation and good left and right atrial sizes. Atrial Enlargement In a 5-year-old boy, the left atrium was somewhat small, and we encountered an unusual type of anomalous pul- Fig 1. Donor heart preparation. (Reprinted from Chartrand C, Guerin R, Kangah M, Stanley P. Pediatric heart transplantation: surgical considerations for congenital heart diseases. J Heart Transplant 1990;9:608-17, by permission of Mosby-Year Book, lnc.) techniques that will be described later. Methods of myocardial protection, ventricular venting, hemostasis, and heart rate control have been previously described [4-71. Preparation of the Donor Heart The donors were aged from 4 to 16 years (mean age, 9 years) and weighed 18 to 48 kg (mean weight, 30.2 kg). The grafts were distantly procured eight times and on site twice. To adapt the graft structures to the complex recipient anatomy or to enable correction of some of the recipient's anomalies, the usual donor heart preparation has to be modified (Fig 1). To facilitate the atrial-septa1 anastomosis when atrial septation or double venous rerooting needs to be done, instead of the classic Barnard incision, we do a straight paraseptal incision at the junction of the right atrial wall and the posterior interatrial septum starting at the orifice of the inferior vena cava and ending 1 cm below the junction of the superior vena cava and the atrium [3, 81. This incision is simple, it obviates trauma to the sinus node, and it allows a single septal anastomosis; we now routinely make this incision in all orthotopic transplantations. When the recipient left atrium is known to be hypoplastic or when there is some form of cor triatriatum that will necessitate atrial enlargement, a graft (donor) left atrial flap is fashioned by making an incision from the left to the Fig 2. Atrial septation. (Reprinted from Chartrand C, Guerin R, right superior Pulmonary vein, down to the right inferior Kangah M, Stanley P. Pediatric heart transplantation: surgical con- Pulmonary vein, and across to the left inferior pulmonary siderations for congenital heart diseases. \ Heart Transplant 1990;g: vein (see Fig 1) , by permission of Mosby-Year Book, lnc.)

3 718 COK CI NlTAL HTAI<T CHARIKASD I F1)IAlRIC CARDIAC I KANSPLAN ratlon Ann Thorac Surg monary venous return called an abortive form of cor triatriatum. At the midpoint of the lateral wall, the left atrium including the anomalous ring was incised vertically, and the incision was extended between the left pulmonary veins (Fig 3). The tip of the donor atrial flap was brought into the incision between the left pulmonary veins, and the donor and recipient atria were anastomosed. In 2 other patients, enlargement of the hypoplastic left atrium was easily performed using the donor left atrial flap technique. Postoperative angiography showed that the reconstructed left atria had good dimensions (Fig 4). lnferior and Superior Reroofing When a left superior vena cava drains into the left atrium, two options are available. In 2 patients, an inferior septostomy was made, and a tunnel was created from the inlet of the left superior vena cava down to the septa1 defect and sutured to the left of the left pulmonary veins (Fig SA). Synthetic material can be used, but we prefer autologous pericardium because of easier modeling. After transplantation, angiography demonstrated an adequate inferior reroofing in both patients (Fig 6). In the third patient with superior vena cava-left atrial drainage, the other procedure, applicable solely during transplantation because the recipient ventricles have been excised, was performed. After a defect was made high in Ficy 3. Atrial enlargetnent. (A) An unusual type of anomalous pulmonary venous return. (B) Vertical incision in left atrium is extended betumti pulmonary veins. (C) Anastomosis of donor and recipient atria. (Reprinted from Chartrand C, Guerin R, Kangah M. Stanley P. Pediatric heart transplantation: surgical considerations for congenifa! heart diseases. Heart Transplant 1990;9:608-17, by permission of Mosby-Year Book, 11tc.) Fig 4. Postoperative angiogram showing result of left atrial enlargement. (LA = ieft atrium.) (Reprinted from Chartrand C, Giierin R, Kangah M, Stanley P. Pediatric heart transplantation: surgical considerations for congenital heart diseases. ] Heart Transplant 1990;9: , b.y permission of Mosby-Year Book, lnc.) Fig 5. (A) inferior and (B) superior reroofing. (Reprinted from Chartrand C, Guerin R, Kangah M, Stanley P. Pediatric heart transplantation: surgical considerations for congenital heart diseases. Heart Transplant 1990;9:608-17, by permission of Mosby-Year Book, lnc.)

4 Ann Thorac Surg COYGENITAL IILAR I CHAR I RXUD 71Y I tdia I RIC CARDIAC TRANSI I AN I A I ION Fig 6. Postoperative angiogram showing adequate inferior reroofing. (Reprinted from Chartrand C, Gnerin R, Kangah M, Stanley P. Pediatric heart transphtation: surgical considerations for congenital heart diseases. Heart Transplant 1990;9:608-17, by permission of MoSh- Year Book, lnc.) Fig 7. Postoperative alzgiograrn showing adequate superior reroofing. (Reprinted frorll Chartrand C, GLlerin R, Kangah M, Stanley p. Pediatric heart transplantation: surgical considerations for congenital heart diseases. 1 Heart Transplant 1990;9:608-17, by permission of Mosby- Year Book, lnc.) Fig 8. Double venous rerouting. (A) Single atrium and absent coronary sinus plus anomalous pulmonary and systemic Venous return. (B) Inferior reroofing. (C) Synthetic patch tailored for double uenous rerouting. (D) Completion of double uenous rerouting. (Reprinted from Chartrand C, Guerin R, Kangah M, Stanley P. Pediatric heart transplantation: surgical considerations for congenital heart diseases. ] Heart Transplant 1990;9:608-17, by permission of Mosby- Year Book, Inc.)

5 720 CONGENITAL HEART CHARTRAND PEDIATRIC CARDIAC TRANSPLANTATION Ann Thorac Surg Fig 9. Septnl realipnrnent. (A) V-shaped incisio~ is rmde in center of free edge of patch to allozc, straightening. (B) The two cut edges hazw been sutured togc&er, arid donor to recipient sing/e septa/ anastom~sis can be perfornred. (Reprinted from Chartrand C, Guerin R, Kun#~ M, Stank!/ P. Pediatric heart transplantation: surgical considerations for congenital heart diseases. / Heart Transplunt 1990;9:608-17, b!/ pertnission of Moshy-Yur Book, Irzc.) the interatrial septum, the cephalic wall of the large left atrium was rolled down and sutured inside the atrium up to the newly created atrial septa1 defect (Fig 5B). The FiS 10. Angiogram niude after double uenous rerouting. (IVC = infcrior wia caua; LSVC = left superior ziena caua; SHV = supraheptrc zieins.) (Reprinted from Chartratid C, Guerin R, Kanph M, Stanley P. Pediatric heart transplnntntio,~: surgical considrrnfioizs for c-onp~ital heart diseases. Hmrt Transplant 1990;9:608-17, by permissioii of MoslyYear Book, Inc.) Fig 11. Angiograru rnade affer double uenoiis rerouting. (RA = right afriiiin; RSVC = ri'yht superior uena cam.) (Reprinted from Charfrond C, Giicriii R, Kangah M, Stanley P. Pediatric heart transplantation: siirgic-ul considerations for congenital heart diseases. Heart Trunsplant 1990;9: , b!/ perrnission of Mosby-Year Book, Inc.) upper part of the donor left atrium could then be sutured over that superior reroofing. The postoperative angiogram demonstrated patency of the superior reroofing (Fig 7). Double Venous Rerouting In 2 patients with single atrium and absent coronary sinus, we encountered a similar pattern of anomalous pulmonary and systemic venous return (Fig 8). As the left pulmonary veins were in their normal position, the right pulmonary veins drained very far to the right of the single atrium. A left superior vena cava, partially unroofed, drained to the left of the left pulmonary veins, and the suprahepatic veins emerged caudally and to the left of the single atrium. We elected first to perform an inferior reroofing, bringing the new left superior vena cava ostium confluent with the hepatic drainage (Fig 8B). Then a synthetic patch was tailored to permit the double venous rerouting (Fig 8C). As outlined in Figure 8D, the flat end of the patch was fixed to the inferior part of the atrial wall, and the inferior angle was anchored to the junction of the new superior vena cava ostium with the orifice of the suprahepatic veins. The patch was sutured over the inferior reroofing to avoid compression and then was anastomosed around the right border of the right pulmonary veins and up to the left of the right superior vena cava inflow. This double rerouting technique permitted reorientation of the systemic and pulmonary venous returns to the appropriate atria. Septa1 Realignment With these septation procedures, the free edge of the patch can become so curved that anastomosis with the

6 Ann Thorac Surg 1991; CONGENITAL HEART CHARTRASD 721 PEDIATRIC CAIWIAC TRANSPLANTA I'ION donor atrial septum is impossible. In this case, we make a V-shaped excision in the center of the patch and suture the two cut edges together (Fig 9). With this maneuver, septal realignment is achieved, and donor to recipient single septal anastomosis is easily performed. In both patients with double venous rerouting and septal realignment, the postoperative angiograms show that the venous returns from the left superior vena cava, suprahepatic veins, and inferior vena cava are reoriented to the right atrium (Fig 10) and that the right superior vena cava drains into a good-sized right atrium (Fig 11). Results One child died in the early postoperative period because of irreversible right heart failure secondary to underestimated preoperative pulmonary hypertension. All survivors made an uneventful recovery and were discharged from the hospital in very good clinical condition. Follow-up ranges from 1 month to 52 months. All survivors are asymptomatic and have been able to resume normal activities after the third postoperative month. The posttransplantation evaluation includes serial echocardiography and right heart catheterization in all patients and left heart catheterization and angiography in all but 2. Based on these studies, there is no evidence of venous, atrial, or great vessel stenosis. No residual shunt has been detected, the atrial cavities are of good size, and there is good orientation of the great vessels. Comment Based on our results, we conclude that, using the surgical techniques described here, most atrial, pulmonary venous return, and systemic venous return anomalies are correctable at the time of orthotopic transplantation. They do not preclude a successful outcome in 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% Bailey LL, Assaad NA, Franklin T, et al. Orthotopic transplantation during early infancy as therapy for incurable congenital heart disease. Ann Surg 1988;208: Chartrand C, Guerin R, Kangha M, et al. Pediatric cardiac transplantation: surgical considerations for congenital heart diseases. J Heart Transplant 1990;9:60% Chartrand C, Laroche B, Parent R, et al. Protection of the myocardial homograft. I. The cooling bag. Can J Surg 1981;24: Chartrand C, Stanley P. Expression hemodynamique du rejet cardiaque. Can J Surg 1976;19: Chartrand C, Dumont L, Stanley P. Value of fibrin sealant in heart transplantation. Transplant Proc 1989;21: Chartrand C, Angel1 WW, Dong E Jr, Shumway NE. Atrial pacing in the postoperative management of cardiac homotransplantation. Ann Thorac Surg 1969;8: Barnard CN. What we have learned about heart transplants. J Thorac Cardiovasc Surg 1968;56: