Caval Division Technique for Sinus Venosus Atrial Septal Defect With Partial Anomalous Pulmonary Venous Connection

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1 Caval Division Technique for Sinus Venosus Atrial Septal Defect With Partial Anomalous Pulmonary Venous Connection Ali Shahriari, MD, Mark D. Rodefeld, MD, Mark W. Turrentine, MD, and John W. Brown, MD Section of Cardiothoracic Surgery, Department of Surgery, Indiana University School of Medicine, James Whitcomb Riley Hospital for Children, Indianapolis, Indiana Background. Repair of sinus venosus atrial septal defect (ASD) with high partial anomalous pulmonary venous connection (PAPVC) using an internal patch may be complicated by obstruction of the superior vena cava (SVC) or pulmonary veins, or both, and sinus node dysfunction. In cases in which the anomalous veins insert more than 2 cm above the cavoatrial junction, we have adopted the technique of caval division in which the SVC is divided and the proximal end is anastomosed to the right atrial appendage, and the distal SVC serves as a conduit for pulmonary venous drainage to the left atrium through the ASD. We retrospectively compare the results of the internal patch repair versus the Warden technique. Methods. Between 1991 and 2004, 54 patients diagnosed with sinus venosus ASD and PAPVC have undergone repair at our institution. Mean age was 13.4 years (range, 1.5 to 58). Thirteen patients (24%) had high insertion of anomalous veins and underwent the Warden technique. Follow-up averages 4.3 years (range, 1 to 13). Results. There were no early or late deaths. All patients remain in normal sinus rhythm. Twelve of the 13 patients with Warden procedure have had postoperative echocardiograms, and 11 of these patients showed no evidence of SVC or pulmonary venous obstruction. In 1 patient, symptomatic pulmonary venous obstruction developed and required revision of a contracted intra-atrial pericardial baffle. Conclusions. Caval division for treatment of high PAPVC appears to be safe and is associated with low morbidity and mortality. The Warden procedure is an effective surgical option for patients undergoing correction of high PAPVC. (Ann Thorac Surg 2006;81:224 30) 2006 by The Society of Thoracic Surgeons Partial anomalous pulmonary venous connection (PAPVC) is present in approximately 90% of patients with sinus venosus type atrial septal defect (ASD) [1, 2]. This represents a physiologic left-to-right shunt with subsequent risk for pulmonary vascular disease, Eisenmenger syndrome, and biventricular failure [3, 4]. Some cases are detected because of the symptomatic status of the patient, but occasionally they may go unrecognized until detected for unrelated reasons. Most commonly, surgical correction of these lesions is performed using the internal patch technique, or a modification thereof, to redirect the anomalous pulmonary venous return through the sinus vensous defect by baffling these structures with a pericardial or synthetic patch with or without performing a patch cavoplasty as needed. Occasionally, however, the anomalous right pulmonary veins may enter the high superior vena cava (SVC). Internal patch repair of PAPVC with high insertion of anomalous veins Accepted for publication July 5, Presented at the Forty-first Annual Meeting of The Society of Thoracic Surgeons, Tampa, FL, Jan 24 26, Address correspondence to Dr Brown, Section of Cardiothoracic Surgery, EH 205, Indiana University School of Medicine, Indianapolis, IN 46202; jobrown@iupui.edu. has the disadvantage of requiring an extensive SVC patch. The potential of distortion or obstruction of the pliable structures in this region, as well as the difficulty of patch placement in the high SVC make the internal patch repair less attractive. Using the internal patch technique without SVC enlargement for repair of high insertion of the pulmonary veins has been reported to be complicated by obstruction of the SVC, the pulmonary veins, sick sinus syndrome, and supraventricular arrhythmias [5 7]. The technique of caval division and atriocaval anastomosis was first described by Warden and colleagues [8] in We have adopted this technique for its ease and its potential to reduce postoperative complications that may occur with internal patch repair of sinus venosus ASD with high anomalous pulmonary venous connection. Material and Methods Between January 1991 and January 2004, 54 patients were diagnosed with sinus venosus ASD and PAPVC. Fortyone of 54 (76%, group A) had low PAPVC, defined by pulmonary venous connection to the lower half of the 2006 by The Society of Thoracic Surgeons /06/$32.00 Published by Elsevier Inc doi: /j.athoracsur

2 Ann Thorac Surg SHAHRIARI ET AL 2006;81: SINUS VENOSUS ATRIAL SEPTAL DEFECT REPAIR 225 muscles cut to provide a wide opening, and anastomosed to the proximal SVC. Upon completion of these anastomoses, the ascending aorta is cross-clamped and cold blood cardioplegia administered. A right atriotomy is made close to the atrioventricular groove, to avoid injury to the sinus node, its arterial supply, or the crista terminalis. A synthetic patch (PTFE in 10 patients, Dacron [C.R. Bard, Haverhill, Pennsylvania] in 2, and untreated autologous pericardial patch in 1) is sutured to the margin of the sinus venosus defect, effectively baffling the SVC orifice to direct the blood flow from the anomalous pulmonary veins, through the SVC conduit, through the sinus venosus defect into the left atrium. The suture line avoids the area of sulcus terminalis. It is important to ensure that the baffle is not redundant to prevent it from bulging, creating a functional stenosis of the SVC. The atriotomy is then closed, the aortic clamp removed, and deairing maneuvers are performed. The patient is weaned off bypass, and the sternotomy is closed (Figs 3 through 6). CARDIOVASCULAR Fig 1. Internal patch technique. Proposed site of incision distal to the atriocaval junction. SVC or directly to the right atrium. Included in this group were 2 patients diagnosed with Scimitar syndrome. All of these patients were treated by baffling the anomalous pulmonary veins to the left atrium through the sinus venosus defect using the internal patch technique (Figs 1 and 2). In 39 of 41 cases (95%), a polytetrafluoroethylene (PTFE) patch was used, and in 2 of 41 patients (5%) an untreated autologous pericardial patch was used. Twelve patients (12 of 41, 29%) had their PAPVC repaired through a superior caval venotomy, sparing the atriocaval junction. In all the cases where a caval venotomy was created, the superior vena cava was enlarged with a PTFE or untreated autologous pericardial patch. Thirteen of 54 patients (24%, group B), had high anomalous pulmonary venous connection, defined by insertion of the anomalous pulmonary veins more than 2 cm above the cavoatrial junction. These patients underwent the Warden procedure. The SVC is cannulated as high as possible, close to the innominate vein. Cardiopulmonary bypass is initiated and the patient cooled to 30 C. During the cooling process, the SVC is occluded, divided, and the distal stump carefully oversewn above the highest anomalous pulmonary vein making sure to avoid stenosis or distortion of the anomalous vein. The apex of the right atrial appendage is then amputated, the pectinate Fig 2. The intra-atrial baffle is in place, and the anomalous pulmonary venous return is redirected to the left atrium through the sinus venosus defect.

3 226 SHAHRIARI ET AL Ann Thorac Surg SINUS VENOSUS ATRIAL SEPTAL DEFECT REPAIR 2006;81: were performed in 13 patients (13 of 41, 32%). These were performed at an average of 2.8 years after the operation (range, 0.1 to 8.5). One patient (2.4%) 2.6 years postoperatively had mild pulmonary venous obstruction (gradient 6 mm Hg). A PTFE patch was used in this repair. Clinical follow-up in this group was 100% and averages 2.8 years (range, 1.0 to 8.5). In group B, 13 of 54 patients (24%), the mean age at operation was 13.4 years (range, 1.5 to 43). There were no early or late deaths. All patients remained in normal sinus rhythm postoperatively. This was assessed by obtaining an electrocardiogram at each visit for the duration of the follow-up. The difference in the incidence of supraventricular arrhythmias in group A and B was not statistically significant (p 0.6). Twelve of 13 patients (92%) have had postoperative echocardiograms. These studies were performed at an average of 3.9 years postoperatively (range, 0.1 to 12.8). Ten of these patients showed no evidence of SVC or pulmonary venous obstruction. One patient with symptoms of pulmonary congestion was shown by echocardiography to have pulmonary venous obstruction. One patient was lost to Fig 3. Proposed transposition of the right atrial appendage to the superior vena cava (arrow). Statistical Analysis The Mann-Whitney rank sum test was used for analysis. A p value less than 0.05 was considered a statistically significant difference. Results In group A, the mean age at operation was 11.1 years (range, 1.0 to 59). There were no early or late deaths (Table 1). Four patients (9.6%) had supraventricular arrhythmias. One patient who was treated for Scimitar syndrome, concomitant with coronary artery bypass grafting at the age of 59 years, had chronic atrial fibrillation requiring anticoagulation and rate control. Another patient had transient junctional rhythm early postoperatively; this spontaneously converted to sinus rhythm before discharge. One patient with a superior caval venotomy had sick sinus syndrome, and another child with repair through SVC venotomy had paroxysmal supraventricular tachycardia and is well controlled with a beta blocker. None of these patients has required insertion of a pacemaker. Postoperative echocardiograms Fig 4. The right atriotomy is created well below the cavoatrial junction.

4 Ann Thorac Surg SHAHRIARI ET AL 2006;81: SINUS VENOSUS ATRIAL SEPTAL DEFECT REPAIR 227 CARDIOVASCULAR Fig 5. Interior view of the right atrium and the location of the sinus venosus defect. echocardiographic follow-up. Three patients (23%) had a left SVC draining to the coronary sinus. Most commonly, the anomalous pulmonary veins originated from the right upper and middle lobes. One of 13 patients (7.7%) had no associated atrial septal defect and 1 of 13 (7.7%) had a secundum defect in addition to a sinus venosus ASD, whereas 11 patients (84.6%) had an isolated sinus venosus defect. In 2 patients (15.4%), the defect was repaired with a Dacron patch, whereas an untreated autologous pericardium was used in 1 (7.7%). In the majority of patients (10 of 13, 77%), the repair was performed with an internal PTFE patch. Early in the series, 1 child whose pathology was corrected with an untreated pericardial baffle had symptomatic pulmonary venous obstruction and required revision 5 years later. At surgery, the pericardial patch was found to be contracted. Another patient with acquired pulmonary hypertension, secondary to longstanding left-to-right shunt, 9 years after her successful Warden procedure, had cardiopulmonary failure and died awaiting lung transplantation. Clinical follow-up was 100% and averages 4.3 years (range, 1.0 to 12.8). The patient characteristics for group B are shown in Table 2. Fig 6. Completed repair prior to the closure of the atriotomy. The arrows indicate the path of the blood flow in front and behind the patch. Comment Several different techniques have been described for repair of PAPVC. High insertion (more than 2 cm above Table 1. Patient Characteristics Group A (n 41) Group B (n 13) Mean age (range) 11.1 (1.0 59) 13.4 (1.5 43) Follow-up, years (range) 2.0 ( ) 3.7 ( ) 30-day mortality, n 0 0 Late mortality, n 0 1 a (7.7%) SVC stenosis 0 0 Pulmonary vein stenosis, n 1 (2.4%) 1 b (7.7%) Supraventricular dysrhythmias, n 4 (9.8%) 0 p 0.6 a Death secondary to acquired pulmonary hypertension. b Initially used untreated autologous pericardial patch that required revision owing to shrinkage. SVC superior vena cava.

5 228 SHAHRIARI ET AL Ann Thorac Surg SINUS VENOSUS ATRIAL SEPTAL DEFECT REPAIR 2006;81: Table 2. Group B Patient Demographics Sex Age Symptoms Number (APV) Origin APVs ASD LSVC Patch Postoperative Echocardiography F 36 Fatigue 1 RUL None None Dacron N/A M 4 Fatigue 6 N/A SV, None Vein N/A secundum F 4 Fatigue, chronic cough 3 RUL, Secundum None Dacron WNL, RLL, M 43 Fatigue, exercise intolerance 2 RUL, SV None PTFE N/A F 16 Asymptomatic 5 RUL, SV None PTFE WNL F 12 Asymptomatic 2 RUL, SV None PTFE N/A F 19 5 RUL, SV Yes PTFE N/A F 17 3 SV Yes PTFE WNL F 7 Asymptomatic 4 RUL, SV None PTFE WNL, RLL M 7 5 RUL, SV Yes PTFE WNL F 1.5 Failure to thrive 3 RUL, SV None Pericardium Small, L-R RLL shunt F 3 Asymptomatic 6 RUL, SV None PTFE Small L-R shunt M 5 Asymptomatic 5 RUL, SV None PTFE WNL APV anomalous pulmonary vein; ASD atrial septal defect; F female; L-R left-to-right; M male; N/A not applicable; PTFE polytetraflyarsethylene; RLL right lower lobe; right middle lobe; RUL right upper lobe; WNL within normal limits. the atriocaval junction) of one or several of the pulmonary veins adds to the complexity of the repair. Some of these techniques have been associated with significant morbidity including obstruction of the pulmonary vein orifices, SVC stenosis or obstruction, and atrial arrhythmias, including atrial flutter or atrial fibrillation [5 7]. For repair of high PAPVC, DeLeon and coworkers [10] have described an incision crossing the atriocaval junction. After placement of the internal patch, atriocaval continuity is restored by patching the incision using the right atrial appendage as a flap. Having recognized that the incision was carried across the region of sinoatrial node, their approach was subsequently modified and two separate incisions were created, one in the lateral atrial wall and one on the distal SVC, to avoid injury to the sinus node or its artery. They report 11% incidence of supraventricular arrhythmias. The Warden procedure has been our preferred approach to repair patients with high PAPVC. This operation is designed so that the atriotomy does not extend across the atriocaval junction, sinus node, or its arterial supply. Using this technique, Gustafson and colleagues [9] reported a series of 40 patients who underwent the Warden procedure, with only 1 case of SVC obstruction and sick sinus syndrome postoperatively. Similarly, Gaynor and associates [11] published a series of 11 patients undergoing the Warden procedure, with only 1 case of pulmonary venous obstruction and no evidence of sinus node dysfunction. Baron and associates [15] have also published their recent experience with this operation and have not reported any SVC, pulmonary venous, or sinoatrial complications. In our series, 13 patients underwent Warden repair, with only 1 case of pulmonary venous obstruction early in the series. In this patient, an untreated autologous pericardial patch was used, which subsequently contracted, requiring reoperation for stenosis. We now routinely use a PTFE patch for the diversion of the pulmonary venous return. Other authors have reported using untreated pericardial patches with no evidence of patch contraction and complications [9]; thus, in our series this likely represents a technical problem. No atrial arrhythmias have been identified in our 13 patients (Table 1). The incidence of supraventricular arrhythmias was less in the cohort treated with the

6 Ann Thorac Surg SHAHRIARI ET AL 2006;81: SINUS VENOSUS ATRIAL SEPTAL DEFECT REPAIR Warden procedure compared with the internal patch group. This finding is consistent with the experience of other authors using modifications of the internal patch technique in which the incisions or anastomosis have been placed in proximity of the sinus node or its arterial supply [10, 12, 14]. In our analysis, the difference between the incidence of supraventricular arrhythmias between the Warden versus the internal patch group was not statistically significant (p 0.6); however, this could be related to a small sample size and statistical power. The perceived lower incidence of atrial arrhythmias with the Warden procedure is likely related to less manipulation of the right atrium and the SVC, avoidance of the sinus node and its arterial supply, or alteration of the crista terminalis, which serves as a longitudinal rapid conduction pathway for atrial activation [15]. Although the trend in the published experience is toward lower incidence of supraventricular arrhthmias with the caval division technique, owing to the retrospective nature and the sample size in this study, we do not recommend the Warden procedure solely to avoid atrial arrhthmias. In conclusion, for correction of sinus venosus ASD with low insertion of the anomalous pulmonary veins, correction through a lateral right atriotomy, with or without a separate incision into the anterolateral aspect of the superior vena cava, offers appropriate exposure with minimal morbidity. However, we believe that it is inadvisable to extend the right atriotomy across the atriocaval junction. The Warden procedure is a safe and effective repair for PAPVR with high insertion into the SVC, and avoids long baffles and extensive SVC patching. This operation can be performed with low rates of morbidity and mortality [13, 16 18]. We thank Sharon Teal, Indiana University Visual Media Section, for assisting with illustrations. References 1. Cooley DA, Ellis PR, Bellizi ME. Atrial septal defects of the sinus venosus type: surgical considerations. Dis Chest 1961; 39: Lewis FJ. High defects of the atrial septum. J Thorac Cardiovasc Surg 1958;36: Campbell M. Natural history of atrial septal defect. Br Heart J 1970;32: Hickie JB, Gimlette TM, Bacon AP. Anomalous pulmonary venous drainage. Br Heart J 1956;18: Stewart S, Alexon 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: Freiedli B, Guerin R, Davignon A, Fouron JC, Stanley P. Surgical treatment of partial anomalous pulmonary venous drainage: a long-term follow-up study. Circulation 1972;45: Trusler GA, Kazenelson G, Freedom RM, Williams WG, Rowe RD. Late results following repair of partial anomalous pulmonary venous connection with sinus venosus atrial septal defect. J Thorac Cardiovasc Surg 1980;79: Warden HE, Gustafson RA, Tarnay TJ, Neal WA. An alternative method for repair of partial anomalous pulmonary venous connection to the superior vena cava. Ann Thorac Surg 1984;38: Gustafson RA, Warden HE, Murray GF. Partial anomalous pulmonary venous connection to the superior vena cava. Ann Thorac Surg 1995;60(Suppl): DeLeon SY, Freeman JE, Ilbawi MN, et al. Surgical techniques in partial anomalous pulmonary veins to the superior vena cava. Ann Thorac Surg 1993;55: Gaynor JW, Burch M, Dollery C, Sullivan ID, Deanfield JE, Elliott MJ. Repair of anomalous pulmonary venous connection to the superior vena cava. Ann Thorac Surg 1995;59: Nicholson IA, Chard RB, Nunn GR, Cartmill TB. Transcaval repair of the sinus venosus syndrome. J Thorac Cardiovasc Surg 2000;119: Boger AJ, Baak R, Lee PC, Boersma E, Meijboom FJ, Witsenburg M. Early results and long-term follow-up after corrective surgery for total anomalous pulmonary venous return. Eur J Cardiothorac Surg 1999;16: Baskett RJF, Ross DB. Superior vena cava approach to repair of sinus venosus atrial septal defect. J Thorac Cardiovasc Surg 2000;119: Rodefeld MD, Bromberg BI, Schuessler RB, Boineau JP, Cox JL, Huddleston CB. Atrial flutter after lateral tunnel construction in the modified Fontan operation: a canine model. J Thorac Cardiovasc Surg 1996;111: Baron O, Roussel JC, Videcoq M, Guerin P, Gournay V, Lefevre M. Partial anomalous pulmonary venous connection: correction by intra-atrial baffle and cavo-atrial anastomosis. J Card Surg 2002;17: Russell JL, LeBlanc JG, Deagle ML, Potts JE. Outcome following repair of sinus venosus atrial septal defects in children. Asian Cardiovasc Thorac Ann 2002;10: Jemielity M, Perek B, Paluszkiewicz L, Stachowiak W, Ponizynski A. Results of repair of partial anomalous pulmonary venous connection and sinus venosus atrial septal defect in adults. J Heart Valve Dis 1998;7: CARDIOVASCULAR DISCUSSION DR PETER B. MANNING (Cincinnati, OH): I think I saw something related to this flash up on one of your slides, but you didn t specifically address it. We found this technique particularly useful in a handful of kids who have bilateral vena cavae, where the right superior vena cava is particularly small. It doesn t matter whether the pulmonary veins come in high or low associated with the sinus venosis defect. You re still going to be at a higher risk for creating a cavoatrial stenosis with an internal patch technique. I think it s a good technique. We ve had 2 or 3 patients with bilateral SVCs. How many of these patients had LSVCs, and which technique was used? DR SHAHRIARI: We actually have 3 patients in the Warden group, and we successfully performed the Warden operation on these patients. DR ROBERT A. GUSTAFSON (Morgantown, WV): I would agree with all the conclusions you ve made. We have continued to do Dr Warden s operation since he first devised the operation. And the 1 patient with SVC obstruction, whom he had a number of years, is still the only patient we have had with an SVC stenosis, and we have yet to have to put in a pacemaker in any of these patients. We follow these patients in the group that we have done this procedure on yearly through the EP clinic,

7 230 SHAHRIARI ET AL Ann Thorac Surg SINUS VENOSUS ATRIAL SEPTAL DEFECT REPAIR 2006;81: hoping that we can show in the long term that we did not need pacemakers in this group. DR CARL L. BACKER (Chicago, IL): Gus, before you step down, the one question I have, because we ve done this very nicely in several patients, this is an excellent series, we ve used autologous pericardium for all of them, and I ve always felt autologous pericardium was better because it s more malleable. And I have had to actually reoperate on patients where a Gore-Tex patch was placed. Gus, do you want to comment about the choice of intra-atrial material? DR GUSTAFSON: The patch that we use inside the heart is autologous pericardium. DR RALPH S. MOSCA (New York, NY): Treated or untreated, Gus? DR GUSTAFSON: Untreated. DR EZZELDIN A. MOSTAFA (Cairo, Egypt): I have seen here that the mean age of your patients is That makes the technique of transcaval closure of the sinus venosus-type defects more or less applicable. My first question is, does the age affect your technique? My second actually is about the technique, the suturing technique, because some still use the interrupted one for the upper end and the continuous one for the lower end. Does the suture technique affect also the incidence of the obstruction of the SVC? DR SHAHRIARI: Well, the answer to your first question is we ve had a range of patients in different age groups, the youngest is 1 year of age. So I don t think that age of the patient is prohibitive to using this operation. In answer to your second question, we discussed this within our group, and I think that the suturing technique is very important. Number one, because you may distort the anatomy of this region of the cavoatrial region. Number two, we think that this may be more important in disrupting the impulse pathways in this region than creating a stenosis of the superior vena cava. If you look at Dr Nicholson s series as summarized in this slide, in 66 patients, they only used a superior vena cava incision and patch cavoplasty, with no incidences of superior vena cava obstruction or arrhythmias of any kind.