I veins draining into the superior vena cava proximal to. Surgical Techniques in Partial Anomalous Pulmonarv Veins to the SuDerior Vena Cava

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1 Surgical Techniques in Partial Anomalous Pulmonarv Veins to the SuDerior Vena Cava J I Serafin Y. DeLeon, MD, Jenny E. Freeman, MD, Michel N. Ilbawi, MD, Tarek S. Husayni, MD, Jose A. Quinones, MD, E. Phillip Ow, MD, Timothy J. Bell, MD, and Roque Pifarre, MD Departments of Cardiovascular-Thoracic Surgery and Pediatrics, Loyola University Medical Center, Maywood, Heart Institute for Children Oak Lawn, and The Children's Memorial Hospital, Chicago, Illinois Over a 12-year period, 40 patients underwent repair of partial anomalous pulmonary veins (PAPV) draining to the superior vena cava (SVC) proximal to the sinus node. Mean age was 6 f 2 years. In all patients, the SVC was cannulated superior to the PAPV, which were bafned with pericardium to left atrium. Six patients had associated defects repaired. In 18 patients (group I), an incision was made at the crest of the right atrial appendage (RAA) and extended upward through the sinus node and to the SVC. After rerouting of the PAPV, the SVC was enlarged using the RAA (atriocavoplasty). In 17 patients (group 11), rerouting of the PAPV was accomplished through a right atriotomy. Superior vena caval enlargement was not done. Drainage of the PAPV was close to the right atrium in 14 patients (low) and to the azygos vein (high) in 3. In 5 patients (group III), an incision was made on the SVC and RAA sparing the sinus node. After rerouting of the PAPV, the RAA was anastomosed to the SVC (end to side), providing another outlet for SVC flow. There was no early or late death. Two patients (10%) in group I had late sinus bradycardia. Obstruction of the SVC and PAPV developed in 1 patient in group I1 with high drainage. Intermittent complete heart block developed in 1 patient in group I11 who also had ventricular septal defect repair. We conclude that atriocavoplasty is effective for rerouting of the PAPV and enlarging the SVC, but may predispose to sinus node disease. Lack of provision for enlarging the SVC or alternate route for SVC flow in high drainage of the PAPV may predispose to venous obstruction. Sinus node dysfunction and venous obstruction are minimized with anastomosis of the RAA to the SVC and should provide another viable alternative in the management of high drainage of the PAPV. (Ann Thorac Surg 2993;55:1222-6) nappropriate operation in partial anomalous pulmonary I veins draining into the superior vena cava proximal to the sinus node can lead to permanent and disabling long-term sequelae. Sinus node dysfunction, residual shunts, and obstruction of the pulmonary veins or superior vena cava can occur [14]. Having encountered such complications and with the intent of minimizing their occurrences, we reviewed our experience. Material and Methods Over a 12-year period ending in 1991, 40 patients underwent repair of partial anomalous pulmonary veins draining to the superior vena cava proximal to the sinus node. Their clinical profile (age, associated defects), diagnosis, groups based on surgical techniques, and results were analyzed. Level of drainage of the anomalous pulmonary veins was categorized as low (close to the cavoatrial junction) and high (close to the azygos vein). Accepted for publication Sep 18, Address reprint requests to Dr DeLeon, Department of Cardiovascular- Thoracic Surgery, Loyola University Medical Center, 2160 S First Ave, Maywood, IL Clinical Profile and Diagnosis The age ranged from 7 months to 17 years with mean age of years. Eight patients had bilateral superior venae cavae. The presence of left to right shunt at the atrial level was established with a combination of clinical examination, electrocardiography, chest roentgenography, and echocardiography. Only 20 patients (50%) underwent cardiac catheterization, of whom 16 patients (80%) had the diagnosis of anomalous pulmonary veins. More recently, the ultrafast cine computed tomographic scan had been used and was helpful in determining levels of drainage of the anomalous pulmonary veins. The atrial septal defects were found to be sinus venosus in 35 patients, secundum in 1, and absent in 4. Surgical Techniques and Groups In all patients, the superior vena cava was cannulated superior to the anomalous veins, which were baffled with pericardium to the left atrium. The inferior vena cava was cannulated through the right atrium, and the operation was performed under cardiopulmonary bypass, moderate hypothermia (25" to 28 C rectally), and ventricular fibrillation. A ventricular sump was routinely used. Six patients had associated defects (ventricular septal defects, 3; complete canal, 1; partial canal and coarctation, 1; tetralogy, 1) repaired along with the partial anomalous pulmo by The Society of Thoracic Surgeons /93/$6,00

2 Ann Thorac Surg 1%3;55: DELEON ET AL 1223 \ \ / Fig 1. Atriocavoplasty (group I). The right atrial appendage is used as a flap to enlarge the superior vena cava after creation of the venoatrial tunnel using pericardiuni nary veins. Cardioplegic arrest was used only when needed for the repair of the associated defects. In 18 patients (group I), an incision was made at the crest of the right atrial appendage and extended upward through the sinus node and to the superior vena cava (Fig 1). The drainage was high in 10 patients, low in 4, and not recorded in the remaining 4. Two patients had bilateral superior venae cavae. After rerouting of the anomalous pulmonary veins to the left atrium, the superior vena cava was enlarged using the right atrial appendage as a flap in the form of atriocavoplasty. In 17 patients (group 11), rerouting of the anomalous pulmonary veins was accomplished through a transverse atriotomy. The superior vena cava was not enlarged. Fourteen patients had low drainage and 3 had high drainage. Five patients had bilateral superior venae cavae, 2 of whom had high drainage. In 5 patients (group 111), an incision was made on the superior vena cava and crest of the right atrial appendage sparing the sinus node (Fig 2). All patients had high drainage. One patient had bilateral superior venae cavae. After rerouting of the anomalous pulmonary veins, the right atrial appendage was anastomosed end-to-side to the superior vena cava, providing another outlet for the vena caval flow. Results There was no early death, and all patients had an uneventful postoperative hospital stay. Thirty-four patients were followed up from 1 month to 12 years (mean, 3 * 3 years). There was no late death. Sinus bradycardia developed 5 and 9 years postoperatively in 2 patients (11%) in group I, who were 12 and 13 years old at operation. One patient (2 year old) in group I1 with high drainage and no bilateral superior venae cavae had development of obstruction of both superior vena cava and anomalous pulmonary veins (Fig 3) 1% years postoperatively and required reoperation. One patient in group I11 had development of intermittent heart block, probably related to ventricular septal defect closure, and eventually required insertion of a permanent cardiac pacemaker 2 years postoperatively. Comment The surgical management of partial anomalous pulmonary venous drainage to the superior vena cava has evolved over several decades. The operation ranged from partial repair with closure of the atrial septal defect and either leaving or ligating the anomalous veins to total repair with rerouting of the anomalous veins to the left atrium and providing unobstructed superior vena caval flow [5,6]. Lung resection was also performed in the early years. As in most congenital heart defects, surgeons have pursued total repair for partial anomalous pulmonary veins. For pulmonary veins draining to the right atrium or at the cavoatrial junction close to the atrial septal defect, successful repair was accomplished early on using either atrio-septo-pexy or patch connection of the anomalous veins with the left atrium [5, 71. However, for pulmonary

3 1224 DELEON ET AL Ann Thorac Surg 1993;55:12224 Fig 2. After creation of a venoatrial tunnel in group ZZZ, the right atrial appendage is anastornosed end-to-side to the superior vena cava, sparing the sinus node and providing another outlet for superior vena caval flow. veins draining high in the superior vena cava, surgical techniques employed early were not satisfactory. Compartmentalization of the superior vena cava combined with either atrio-septo-pexy or patch rerouting of the pulmonary veins to the left atrium and maintaining cavoatrial continuity were followed with superior vena caval obstruction and residual shunts [2, 5, 81. Although patch enlargement of the superior vena cava was subsequently added, caval obstruction remained a serious problem [2, 91. Additionally, incision through the cavoatrial junction with possible injury to the sinus node or artery might have contributed to the high incidence of atrial arrhythmia. Friedli and colleagues [2] reported a 42% incidence (6/14) of caval obstruction and a 35% incidence (5/14) of wandering pacemaker, coronary sinus rhythm, and nodal rhythm. Even if the incision did not go through the sinus node or artery itself, fibrosis in the area might have contributed to the sinus node dysfunction [lo]. Reimplantation of the anomalous veins to the right atrium generally was not performed because of technical difficulty due to venoatrial distance and long-term concern regarding venoatrial anastomosis [ 111. As cardiopulmonary bypass techniques improved, more direct and meticulous creation of the venoatrial tunnel was performed using native tissues, pericardium, or synthetic grafts that decreased tunnel obstruction and residual shunts [l, 12-14]. Although some surgeons have not enlarged the superior vena cava, most generally do patch enlargement, usually with a piece of pericardium. The role of caval enlargement, however, is not clear in the prevention of either caval or venoatrial tunnel obstruc- tion. Stewart and colleagues [15] reported no obstruction in 15 patients who had pericardial venoatrial tunnel through a cavoatrial incision that was closed primarily. Trusler and colleagues [l], however, reported that 44% of patients (8/18) had some cavoatrial gradients at cardiac catheterization after repair using pericardial caval enlargement. These contrasting results probably could be due to difference in the level of drainage being dealt with. Our experience showed that when the anomalous pulmonary veins were draining close to the cavoatrial junction, caval enlargement was not required. However, when the level of drainage was high, obstruction of the superior vena cava and venoatrial tunnel occurred when caval enlargement was not performed. Additionally, Stewart and colleagues [15] and Trusler and colleagues [l] reported a 40% incidence (6/15) and 27% incidence (8/29), respectively, of development of early or late nodal rhythm and sinus bradycardia, probably related to the cavoatrial incision. Sinus node dysfunction did not develop in any of our patients who did not have cavoakial incision. Because of narrowing and obstruction of the superior vena cava from the compartmentalization procedure, Ehrenhaft and colleagues [ 111 transected the superior vena cava proximal to the anomalous pulmonary veins and created a cavo-right atrial appendage anastomosis either directly or with a homograft. The distal caval segment attached to the pulmonary veins was used for the venoatrial tunnel. Because the procedure had an advantage of not requiring an incision through the cavoatrial junction, it was subsequently used by several surgeons [6, 16, 171. Although rare, superior vena caval

4 Ann Thorac Surg 1993;55: DELEON ET AL 1225 A B c D E F Fig 3. (A-C) Ultrrifast computed tomographic scan with contrast of a patient zoho had a right atrial appendage to supcrior vena cava anastomosis (group 111) for high drainage, showing patency of the superior vena cava (VC) and the venoatrial tunnel (black arrows). (D-F) Similar study on a group 11 patient with high drainagc who did not have enlargement of the superior vena caua (VC), sho~~ing obstruction of both thl superior vena cam (white arrow) and the venoatrial tunnel. The azyxos vein (hollow arrow) decompressing the superior Venn cava is seen. (LA = left atriuni; PA = pulmonary artery; RA = right atrium.) obstruction has also been reported and, curiously, there is also a significant incidence of sinus node dysfunction. This could be related to proximity to the sinus node when sutures are being placed at the caval orifice for the venoatrial tunnel. Additionally, in the presence of high drainage of the anomalous veins, the superior vena cava might end up being so short that grafts with potential for late stenosis would be needed for the cavo-right atrial appendage anastomosis [ll]. Because of concern regarding superior vena caval obstruction from fibrosis and shrinkage of pericardial or synthetic patches, Long and colleagues [18] used the right atrial appendage in the form of atriocavoplasty for caval enlargement. They also used an atrial septa1 flap to reroute the anomalous veins to the left atrium. Atriocavoplasty is currently being used by other surgeons [ The main drawback of the procedure is the need to go through the cavoatrial junction with potential or actual injury to the sinus node or artery. A substantial incidence of junctional rhythm has been reported. We encountered an 11% incidence (2/18) of late sinus bradycardia. The ideal result in high partial anomalous pulmonary venous drainage to the superior vena cava should consist of normal sinus node function, absence of residual shunts, and unobstructed venoatrial tunnel and superior vena cava. Improvement in cardiopulmonary bypass and surgical techniques have minimized residual shunts and venoatrial tunnel obstruction. Sinus node dysfunction and superior vena caval obstruction, however, remain a problem. Patch enlargement of the superior vena cava and atriocavoplasty have reduced caval obstruction but, probably because of the cavoatrial incision, have been followed by serious sinus node dysfunction. Transection of the superior vena cava proximal to the anomalous veins and creation of a cavo-atrial appendage anastomosis avoided going through the cavoatrial junction. Unexpectedly, however, sinus node dysfunction still occurred, probably because of sutures at the caval orifice for the venoatrial tunnel. Additionally, there could be a substantial cavoatrial appendage distance that would require a tubular graft with potential for late fibrosis and shrinkage. Our approach using an anastomosis between the end of the right atrial appendage and the side of the superior vena cava has avoided cavoatrial incision and sinus node

5 1226 DELEON ET AL Ann Thorac Surg 1993; dysfunction and has provided two outlets for the superior vena cava. Additionally, the superior vena cava is not shortened, therefore avoiding the use of tubular grafts. We believe that this approach should provide another viable alternative in the management of high partial anomalous pulmonary venous drainage. References 1. Trusler GA, Kazenelson G, Freedom RM, Williams WG, Rowe RD. Late results following repair of partial anomalous pulmonary venous connection with sinus venous atrial septal defect. J Thorac Cardiovasc Surg 1980;79776-! Friedli 8, Gherin R, Davignon A, Fouron JC, Stanley P. Surgical treatment of partial anomalous pulmonary venous drainage. A long-term follow-up study. Circulation 1972;45: Weber HS, Markowitz RI, Hellenbrand WE, Kleinman CS, Kopf GS. Pulmonary venous collaterals secondary to superior vena cava stenosis: a rare cause of right-to-left shunting following repair of a sinus venosus atrial septal defect. Pediatr Cardiol 1989;10: Anderson PD, Glaser SP, Czamecki S, Hopeman AR. Three unusual complications resulting from attempted repair of partial anomalous pulmonary venous drainage. Chest 1976; Kirklin JW, Ellis FH, Wood ED. Treatment of anomalous pulmonary venous connections in association with interatrial communications. Surgery 1956;39: Gustafson RA, Warden HE, Murray GF, Hill RC, Rozar GE. Partial anomalous pulmonary venous connection to the right side of the heart. J Thorac Cardiovasc Surg 1989;98: Bailey CP, Bolton HE, Jamison WL, Neptune WB. Atriosepto-pexy for interatrial septal defects. J Thorac Surg 1953; 26: Robicsek F, Daugherty HK, Cook JW, Selle JG. Sinus venosus type of atrial septal defect with partial anomalous pulmonary venous return. J Thorac Cardiovasc Surg 1979;78: Schuster SR, Gross RE, Colodny HH. Surgical management of anomalous right pulmonary venous drainage to the superior venacava associated with superior marginal defect of the atrial septum. Surgery 1962;51: Tung KSK, James TN, Effler DB, McCormack J. Injury of the sinus node in open-heart operations. J Thorac Cardiovasc Surg 1967; Ehrenhaft JL, Theilen EO, Lawrence MS. The surgical treatment of partial and total anomalous pulmonary venous connections. Ann Surg 1957;148: Puig-Massana M, Murtra M, Revuelta JM. A new technique in the correction of partial anomalous pulmonary venous drainage. J Thorac Cardiovasc Surg 1972;64:10% Kyger ER, Frazier OH, Cooley DA, et al. Sinus venous atrial septal defect: early and late results following closure in 109 patients. Ann Thorac Surg 1978;25& Ohmi M, Mohri H. A single pericardial patch technique for repair of partial anomalous pulmonary venous drainage associated with sinus venosus atrial septal defect. Ann Thorac Surg 1988;46:36& Stewart S, Alexson C, Manning J. Early and late results of repair of partial anomalous pulmonary venous connection to the superior vena cava with a pericardial baffle. Ann Thorac Surg 1986;41:49% Warden HE, Gustafson RA, Tamay TJ, Neal WA. An alternative method for repair of partial anomalous pulmonary venous connection to the superior vena cava. Ann Thorac Surg 1984; Williams WH, Zom-Chelton S, Raviele AA, et al. Extracardiac atrial pedicle conduit repair of partial anomalous pulmonary venous connection to the superior vena cava in children. Ann Thorac Surg 1984;38: Long DM, Rios MV, Elias DO, Meier MA, DuBrow IW. Parietal and septal atrioplasty for total correction of anomalous pulmonary venous connection with superior vena cava. Ann Thorac Surg 1974;18: Chartrand C, Payot M, Davignon A, Guerin R, Stanley P, Bruneau J. A new surgical approach for correction of partial anomalous pulmonary venous drainage into the superior venacava. J Thorac Cardiovasc Surg 1976;71: Lewin AN, Zavanella C, Subramanian S. Sinus venosus atrial septal defect associated with partial anomalous pulmonary venous drainage: surgical repair. Ann Thorac Surg 1978;26: Okabe H, Matsunaga H, Kawauchi M, et al. Rotationadvancement flap method for correction of partial anomalous pulmonary venous drainage into the superior vena cava. J Thorac Cardiovasc Surg 1990;99:3of?-ll.