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Original Articles: Cardiovascular

Evolving Surgical Strategy for Sinus Venosus Atrial Septal Defect: Effect on Sinus Node Function and Late Venous Obstruction

^a Divisions of Cardiovascular Thoracic Surgery and Cardiology, Children’s Memorial Hospital, Northwestern University Feinberg School of Medicine, Chicago, Illinois
^b Departments of Surgery and Pediatrics, Northwestern University Feinberg School of Medicine, Chicago, Illinois

Accepted for publication April 11, 2007.

^_* Address correspondence to Dr Mavroudis, Division of Cardiovascular Thoracic Surgery, M/C 22, Children’s Memorial Hospital, 2300 Children’s Plaza, Chicago, IL 60614 (Email: cmavroudist{at}childrensmemorial.org).

Presented at the Fifty-third Annual Meeting of the Southern Thoracic Surgical Association, Tucson, AZ, Nov 8–11, 2006.

	Abstract

Top Abstract Introduction Patients and Methods Results Comment Discussion References

 
Background: Our surgical strategy for repair of sinus venosus atrial septal defect has evolved chiefly to avoid sinus node dysfunction. We reviewed our experience with the single-patch, two-patch, and Warden repairs.

Methods: We identified 54 patients with repair of sinus venosus atrial septal defect from 1990 to 2006. Mean age was 9.5 ± 12.6 years; median age was 4.2 years. Partial anomalous pulmonary venous connection was found in 52 patients (96%); drainage was to the right atrium in 8, right atrial–superior vena cava (SVC) junction in 17, and directly to the SVC in 27. Techniques were single-patch repair (24), two-patch repair (25), and Warden repair (5). Autologous pericardium was used in all patients. Echocardiogram and electrocardiogram follow-up were available for 48 patients (89%).

Results: There were no early or late deaths and no reoperations. No patient had pulmonary vein stenosis. Five patients had SVC stenosis: 2 mild after two-patch repair; 1 moderate and 1 mild after single-patch repair; and 1 severe stenosis after Warden procedure (p = 0.3). The incidence of rhythm change from sinus to low atrial or junctional rhythm was 35% and was significantly greater among patients with two-patch repair (12 of 22, 55%) compared with single-patch repair (5 of 21, 24%), or the Warden repair (0 of 5, p = 0.02).

Conclusions: Repair of sinus venosus atrial septal defect with autologous pericardium is associated with a low incidence of late SVC or pulmonary vein stenosis with all techniques. Use of the two-patch technique, however, is associated with a significantly greater incidence of sinus node dysfunction. For the patients with partial anomalous pulmonary venous connection entering the SVC, the Warden procedure avoids interfering with the sinus node and should be used preferentially. The single-patch technique remains the procedure of choice for sinus venosus atrial septal defect with partial anomalous pulmonary venous connection entering the right atrium or right atrium–SVC junction.

	Introduction

Top Abstract Introduction Patients and Methods Results Comment Discussion References

 
Sinus venosus atrial septal defects represent 10% of all atrial septal defects, are most commonly located at the superior vena cava (SVC) to right atrial (RA) junction, and are frequently associated with partial anomalous pulmonary venous connections (PAPVC). The PAPVC may insert into the RA-SVC junction or more superiorly into the SVC. The complexity of the connections precludes closure by catheter intervention and has been addressed by numerous surgical procedures [1–13]. Historically, the most common operation for sinus venosus atrial septal defect with PAPVC to the RA or RA-SVC junction consists of closing the atrial septal defect with a single atrial patch that includes the PAPVC, thereby baffling the pulmonary venous drainage to the left atrium. However, if the PAPVC is committed to the SVC, baffling by a single patch can result in obstruction to the pulmonary veins or the SVC, or both. A second patch across the SVC-RA junction minimizes the risk of SVC obstruction but may result in sinoatrial node dysfunction by disrupting the sinoatrial node or the sinoatrial node artery. In 1984, Warden and colleagues [1] described a procedure used in 15 patients with PAPVC into the SVC. The SVC is transected above the PAPVC and anastomosed to the RA appendage, followed by atrial septal defect patch closure that includes the entire SVC orifice. The now eponymed "Warden procedure" has gained popularity for avoiding both pulmonary venous and SVC obstruction as well as sinoatrial node dysfunction.

We had previously used the two-patch technique for sinus venosus atrial septal defects with PAPVC to the SVC. We have recently (2002) adopted the Warden procedure for sinus venosus atrial septal defects with PAPVC entering the SVC. The purpose of this review is to assess our experience with single-patch, two-patch, and Warden repairs to determine whether the incidence of obstruction of the pulmonary venous drainage, SVC drainage, and sinoatrial node dysfunction warrants our change in technique.

	Patients and Methods

Top Abstract Introduction Patients and Methods Results Comment Discussion References

 
Institutional Review Board approval was obtained for the conduct of this study, and the Board waived the need for patient consent.

We identified 54 patients from our computerized cardiovascular database who underwent primary surgical repair of sinus venosus atrial septal defect between April 1990 and February 2006. Mean age was 9.5 ± 12.6 years (range, 5 months to 55 years); median age was 4.2 years. There were 26 males and 28 females. Patients who had PAPVC without a sinus venosus atrial septal defect were not included in this review.

Fifty-one patients had typical defects at the SVC-RA junction, and 3 patients had inferior vena cava–type defects. Two patients had completely normally draining pulmonary veins, and the remaining 52 had PAPVC (96%). Drainage of the PAPVC was directly to the RA in 8 patients (15%), to the RA-SVC junction in 17 patients (33%), and to the SVC at least 1 cm above the SVC-RA junction in 27 patients (52%).

All procedures were performed using cardiopulmonary bypass with bicaval venous cannulation and systemic hypothermia to 32°C. Cardioplegic arrest was achieved with cold blood cardioplegia and topical slush saline. A left ventricular vent was not routinely used. All patches were of fresh autologous pericardium. Twenty-four patients (44%) had a single-patch repair of the sinus venosus atrial septal defect with inclusion of the PAPVC with the atrial septal defect closure. Twenty-five patients (46%) had a two-patch repair with the use of a second pericardial patch to enlarge the SVC-RA junction. Five patients (10%) underwent a Warden procedure. Among the 27 patients with PAPVC to the SVC, 7 had single-patch repairs (26%), 15 two-patch repairs (56%), and 5 Warden repairs (18%).

The Warden procedure is illustrated in Figures 1 through 4. Important details of our technique include high cannulation of the SVC (right-angle cannula) with division of the SVC immediately above the PAPVC to save proximal SVC length (Fig 1). A pericardial patch is used to patch the cardiac stump of the SVC to prevent stenosis or obstruction of the pulmonary venous return (Fig 1). The RA appendage is opened with an incision in the tip and care is taken to excise all trabeculations to avoid late stenosis (Fig 2). The SVC orifice in the RA is baffled to the left atrium through the sinus venosus atrial septal defect with a fresh autologous pericardial patch that includes the entire orifice of the SVC (Fig 3). Finally, the cut SVC is anastomosed to the RA appendage using absorbable interrupted sutures to prevent purse-stringing and to allow growth (Fig 4). The azygos vein was divided in 2 patients and preserved in 3 patients.

View larger version (80K): [in this window] [in a new window]   Fig 1. Details of the Warden technique. High cannulation of the superior vena cava (SVC) is performed with a right-angle cannula with division of the SVC immediately above the partial anomalous pulmonary venous connections to save proximal SVC length. A pericardial patch is used to patch the cardiac orifice of the SVC to prevent stenosis or obstruction of the pulmonary venous return. (SA = sinoatrial).

View larger version (52K): [in this window] [in a new window]   Fig 2. The right atrial appendage is opened with an incision in the tip, and care is taken to excise all trabeculations to avoid late stenosis. The completed pericardial patch on the superior vena cava is shown.

View larger version (60K): [in this window] [in a new window]   Fig 3. The sinus venosus atrial septal defect is not "closed" but is used as an orifice through which the superior vena cava is baffled to the left atrium. The partial anomalous pulmonary venous connection blood flow goes through the remnant of superior vena cava through the sinus venosus atrial septal defect to the left atrium.

View larger version (63K): [in this window] [in a new window]   Fig 4. The cut superior vena cava is anastomosed to the right atrial appendage using interrupted absorbable sutures to prevent pursestringing and to allow growth.

Data were compared with Fisher’s exact test using StatView statistical software (SAS Institute, Cary, North Carolina). Two-tailed p values are reported.

	Results

Top Abstract Introduction Patients and Methods Results Comment Discussion References

 
There were no early or late deaths. No patients in this series required reoperation. There were no residual atrial septal defects. None of the 25 patients with PAPVC to the RA or RA-SVC junction had late stenosis of either the SVC or pulmonary veins on follow-up echocardiography. Among the 27 patients with PAPVC to the SVC, 5 patients (18%) had some degree of stenosis in the SVC, and no patient had pulmonary vein stenosis. Three patients had mild SVC stenosis after two-patch repair (n = 2) and single-patch repair (n = 1). One patient had moderate SVC stenosis after single-patch repair, and 1 patient had severe stenosis of the SVC 2 years after Warden procedure. That patient had neck and facial edema and upper extremity edema. Magnetic resonance imaging showed near-total occlusion of the distal SVC at the junction of the SVC and RA appendage. Multiple collaterals had developed to decompress the SVC. The patient underwent successful balloon angioplasty with no residual obstruction.

Comparison of preoperative to late ECGs demonstrated a change from normal sinus rhythm to a low atrial rhythm or junctional rhythm in 17 of the 48 patients (35%). This change was significantly greater among patients with two-patch repair (12 of 22, 55%) compared with single-patch repair (5 of 21, 24%), or the Warden repair (0 of 5, p = 0.02; Fig 5). There were 3 patients in normal sinus rhythm preoperatively who converted to a low atrial rhythm on early ECG and then reverted to normal sinus rhythm on late ECG. No patients were on antiarrhythmic medications.

View larger version (88K): [in this window] [in a new window]   Fig 5. Comparison of preoperative to late electrocardiograms demonstrating a change from normal sinus rhythm (black bars) to a low atrial rhythm or junctional rhythm. (White bars = sinus node dysfunction.)

	Comment

Top Abstract Introduction Patients and Methods Results Comment Discussion References

The high incidence of sinoatrial node dysfunction in our two-patch patients is consistent with others’ observations that a repair with an incision that crosses the SVC-RA junction, whether on the anterior surface or laterally, puts the sinoatrial node at risk. DeLeon and associates [4] reported 40 patients repaired between 1979 and 1991, and found that the group that had been repaired with an RA appendage flap that included a continuous incision through the anterior RA-SVC junction had a higher incidence of sinoatrial node dysfunction than did patients who had either no incision onto the SVC or who had an end-to-side appendage to SVC anastomosis with avoidance of any incision near the SVC-RA junction. Stewart and coworkers [5] reported 15 patients with sinus venosus atrial septal defect and PAPVC to the SVC repaired with a single-patch technique, which included an incision from the atrial appendage superiorly along the SVC to the level of the PAPVC, thus directly across the sinoatrial node. They noted that 40% of the patients had early sinoatrial node dysfunction. Interestingly, they found that all of these postoperative rhythm problems "resolved" before hospital discharge. We found in 3 cases that early sinoatrial node dysfunction returned to normal sinus rhythm, but in all other cases, it persisted well past discharge, and in many cases beyond a year postoperatively. Although no patient has required a pacemaker, the long-term potential problems from sinus node dysfunction are a concern.

We had no sinoatrial node dysfunction in the 5 patients who had the Warden procedure. Presumably that is because the procedure eliminates any incisions near the sinoatrial node or the sinoatrial node artery. This advantage has been shown by others. The Gustafson and Warden group has reported on their growing series of patients with the initial patient being operated on in 1967 [6, 7]. As of the latest report, they had performed 40 "Warden" procedures with only a single case of sinoatrial node dysfunction (2.5%) [7]. Two other patients had early sinoatrial node dysfunction, but returned to normal sinus rhythm by 5 days and 6 months. Shahriari and colleagues [8] reported the results of 54 patients with sinus venosus atrial septal defect repaired at Riley Children’s Hospital, of whom 27 had a single-patch technique, 12 had a two-patch technique, and 13 had a Warden procedure. Although there was a very low rate of arrhythmias in the entire series, 100% of the patients who had Warden procedures remained in normal sinus rhythm. Similarly, Gaynor and colleagues [9] reported a series of 11 patients who had a Warden procedure at Great Ormond Street between 1987 and 1995. There were no cases of sinus node dysfunction. DiBardino and associates [10] reported a single case of transient sinus bradycardia among 16 patients who had a Warden procedure at Texas Children’s Hospital between 1995 and 2004. In summary, the literature appears to corroborate the finding in this study that the Warden procedure essentially eliminates sinoatrial node injury, and thus sinoatrial node dysfunction.

The single patient in our small series who had severe obstruction 2 years after a Warden procedure is concerning, but almost certainly this outcome represents a technical problem. That was the second case in our series. Most likely this was a failure to adequately excise the trabeculations within the atrial appendage, as Warden reported in the original article [1]. In the combined series from Shariari, Gaynor, and DiBardino [8–10], there are only 2 patients with reported pulmonary vein stenoses and no SVC stenoses. Interestingly, in both cases, 1 from Great Ormond Street and 1 from Riley Children’s Hospital, the pulmonary vein stenosis appeared to be the result of shrinkage of an untreated autologous pericardial patch. Both of these groups recommend using a polytetrafluoroethylene intra-atrial baffle. We have used pericardium without any evidence of pulmonary vein stenosis due to patch shrinkage. Likewise, Warden and Gustafson use untreated pericardium without evidence of patch-related obstruction. Although we have not performed a patch augmentation of the SVC to atrial appendage anastomosis, some surgeons do this routinely to avoid SVC stenosis. Consideration for a patch at this anastomosis should be given if the anastomosis appears susceptible to tension-related narrowing.

Our review confirms that the single-patch technique remains the procedure of choice for patients with a sinus venosus atrial septal defect and PAPVC to the RA or SVC-RA junction. In that subgroup, we found no evidence of significant stenosis in either the SVC or pulmonary veins, and there was a low incidence of sinoatrial node dysfunction. For sinus venosus atrial septal defect with PAPVC to the SVC, the Warden procedure provides unobstructed drainage of the SVC and pulmonary veins and virtually eliminates the problem of sinus node dysfunction that was frequently seen with the two-patch repair. For these reasons, we believe the Warden procedure should be the procedure of choice for sinus venosus atrial septal defect with PAPVC to the SVC.

	Discussion

Top Abstract Introduction Patients and Methods Results Comment Discussion References

 
DR JOHN W. BROWN (Indianapolis, IN): I would like to congratulate Dr Stewart and his colleagues from Chicago on an excellent presentation and exquisite illustrations of not only the pathology but also the three techniques that they have employed to treat this type of ASD. Your manuscript is also extremely well written and illustrated, and it will be the reference article for any young surgeon who is going to treat a sinus venosus atrial septal defect with partial veins. I would like to thank the authors for providing me not only with a copy of their presentation but also of their manuscript.

My colleagues and I in Indianapolis agree with your rationale for adopting the Warden procedure for this subgroup with drainage to the SVC. We have largely abandoned the two-patch technique, as you are advocating, because of the concern of atrial arrhythmias. We continue to use, obviously, the simple one-patch technique for the simpler variety of this pathology.

I have two comments and one question. Occasionally fresh autologous pericardium will be unreliable, and we had 1 patient in the single-patch technique who had pulmonary vein obstruction because the patch shrank to about half of its original diameter, and for that reason, several years ago we started using Gore-Tex, and I noticed you used that one time in your series. We find Gore-Tex, first of all, doesn’t shrink and it is a lot easier to handle because the edges don’t roll up, and so we have sort of gone to using it. Obviously, it is a technical preference about what you want to use.

My other concern, and I think it is something that you have brought to everybody’s attention here, is the incidence of sinus node dysfunction, which was 36%. Now, I realize not in your series, not in our series, there is not a single patient I know of that is reported who has a pacemaker, but I am concerned about sinus node dysfunction, because in the old Mustard literature and in our experience, sinus node dysfunction leads to late requirements for medications or pacemakers. Do you think that your 15 or 20 patients who had the two-patch technique primarily will be at risk for requiring some sort of either medical treatment of their sinus node dysfunction or pacemakers in late follow-up? Thank you very much for allowing me to discuss this very important paper.

DR STEWART: Thank you very much, Dr Brown. Regarding the pericardium versus Gore-Tex, it clearly is a surgical preference. Both in your series and the series reported by Dr Gaynor out of Great Ormond Street—he is not there anymore, obviously—there was a single pulmonary vein stenosis, both attributed to pericardial patch shrinkage. We don’t believe that we have had that problem; however, it may not be an unreasonable thing to avoid it altogether by using Gore-Tex patches.

In reference to the sinus node dysfunction, I feel that there is probably a significant likelihood that several if not all of these patients are eventually going to go on to sick sinus syndrome later in life and possibly requiring pacemakers. There is a significant body of literature that shows that patients with sick sinus syndrome also develop atrial arrhythmias. And I don’t think this is quite the same substrate as having a Mustard, but I worry about these patients, and all the more reason to switch to the Warden procedure.

DR ROSS M. UNGERLEIDER (Portland, OR): Very nice, Bob. It was worth waiting around for. In case this does become the reference paper that John suggested it might, I just want to clarify something with you. Are you recommending the Warden procedure for patients who have sinus venosus ASDs with the veins draining into the atrial SVC junction—in other words, for all patients with sinus venosus ASDs?

DR STEWART: No, and we make this quite clear in our paper that we believe that when the veins drain into the right atrial SVC junction or the right atrium themselves, the single-patch technique has a relatively low incidence of any of those three major problems in terms of obstruction or sinus node dysfunction, and so we still recommend that operation.

DR UNGERLEIDER: We would agree. Through a simple oblique atriotomy, not extending laterally up the side of the SVC, it is really simple to close those defects with a single patch, and like John, we prefer Gore-Tex. And I was just wanting to be clear that we weren’t recommending that the Warden procedure be done for those patients. Thank you very much.

DR STEWART: Thank you, Dr Ungerleider, for that important clarification.

	References

Top Abstract Introduction Patients and Methods Results Comment Discussion References

 

1. 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;38:601-605.[Abstract]
2. Schuster SR, Gross RE, Colodny AH. Surgical management of anomalous right pulmonary venous drainage to the superior vena cava, associated with superior marginal defect of the atrial septum Surgery 1962;51:805-808.[Medline]
3. Kyger III ER, Frazier H, Cooley DA, et al. Sinus venosus atrial septal defect: early and late results following closure in 109 patients Ann Thorac Surg 1978;25:44-50.[Abstract]
4. DeLeon SY, Freeman JE, Ilbawi MN, et al. Surgical techniques in partial anomalous pulmonary veins to the superior vena cava Ann Thorac Surg 1993;55:1222-1226.[Abstract]
5. 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;41:498-501.[Abstract]
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7. Gustafson RA, Warden HE, Murray GF. Partial anomalous pulmonary venous connection to the superior vena cava Ann Thorac Surg 1995;60:5614-5617.
8. Shahriari A, Rodefeld, MD, Turrentine MW, Brown JW. Caval division technique for sinus venosus atrial septal defect with partial anomalous pulmonary venous connection Ann Thorac Surg 2006;81:224-230.[Abstract/Free Full Text]
9. 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;59:1471-1475.[Abstract/Free Full Text]
10. DiBardino DJ, McKenzie ED, Heinle JS, Su JT, Fraser Jr CD. The Warden procedure for partially anomalous pulmonary venous connection to the superior caval vein Cardiol Young 2004;14:64-67.[Medline]
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This Article Abstract Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to Personal Folders Download to citation manager Author home page(s): Robert D. Stewart Carl L. Backer Constantine Mavroudis Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Stewart, R. D. Articles by Mavroudis, C. Search for Related Content PubMed PubMed Citation Articles by Stewart, R. D. Articles by Mavroudis, C. Related Collections Congenital - cyanotic