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Outcome Following Repair of Sinus Venosus Atrial Septal Defects in Children

Division of Cardiovascular and Thoracic Surgery Children’s and Women’s Health Centre of British Columbia Vancouver, British Columbia, Canada
For reprint information contact: Jacques G LeBlanc, MD Tel: 1 604 875 3165 Fax: 1 604 875 3159 email: jleblanc{at}cw.bc.ca Division of Cardiovascular and Thoracic Surgery, Children’s and Women’s Health Centre of British Columbia, 4480 Oak Street, Suite 3G63, Vancouver, British Columbia V6H 3V4, Canada.

	ABSTRACT

TOP ABSTRACT INTRODUCTION PATIENTS AND METHODS RESULTS DISCUSSION REFERENCES

 
The outcome of surgical repair of sinus venosus atrial septal defect was reviewed retrospectively. The operation was performed on 44 children aged 8 to 163 months, between April 1985 and November 1998. Median cardiopulmonary bypass and aortic crossclamp times were 58 minutes (range, 29 to 141 minutes) and 29 minutes (range, 4 to 67 minutes), respectively. Use of blood products decreased from 4.5 units per patient in the first period (1985 to 1989) to 0.6 units in the last period (1995 to 1998). Median intensive care and hospital stays were 2 days (range, 1 to 12 days) and 6 days (range, 4 to 16 days), respectively. There was 1 early death (2.3%). Complications included reexploration for bleeding in 2 patients (4.5%) and for superior vena cava obstruction in 1 (2.3%), and arrhythmias in 3 (6.8%), which required a pacemaker in one. During follow-up of 15 to 176 months, 83.8% of patients were in sinus rhythm. One required angioplasty for superior vena cava stenosis, hemodynamically insignificant residual shunt was found in 3, and mild superior vena cava stenosis in 3. Repair of sinus venosus atrial septal defect carries a low mortality and morbidity, but long-term follow-up is needed to monitor potential sinus node dysfunction and superior vena cava stenosis.

	INTRODUCTION

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Sinus venosus defects account for approximately 10% of interatrial communications.¹ Sinus venosus atrial septal defect (ASD) occurs above the fossa ovalis immediately beneath the orifice of the superior vena cava (SVC) that typically overrides the atrial defect. As there is an absence of the superior margin of the defect, there is frequently associated anomalous pulmonary venous return from the right lung to the SVC or the right atrium (80% to 90%). Surgical repair of this defect was first described by Kirklin and colleagues² in 1956. Results have remained suboptimal due to a significant incidence of SVC obstruction, pulmonary vein obstruction, residual left-to-right shunts, and arrhythmias with sinus node (SN) dysfunction. This has led to the development of many alternative surgical techniques in order to minimize the occurrence of these late complications.^3–7 This study retrospectively reviewed the outcome in children undergoing surgical repair of this defect.

	PATIENTS AND METHODS

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Between April 1985 and November 1998, surgical repair of sinus venosus ASD was performed in 44 patients. There were 30 males (68.2%) and 14 females (31.8%). The median age at operation was 50 months (range, 8 to 163 months). Other associated cardiac defects included pulmonic stenosis in 6 (13.6%), an abnormal aortic valve in 3 (6.8%), a left SVC in 3 (6.8%), and an abnormal mitral valve in 3 (6.8%). Associated syndromes included Down syndrome in 2 patients (4.5%), and fetal alcohol syndrome in 1 (2.3%). Patients requiring concurrent procedures other than pulmonary valvotomy were excluded. Of the 44 patients, 29 (65.9%) were asymp-tomatic, 10 (22.7%) had shortness of breath on exertion, 3 (6.8%) had failure to thrive, and 2 (4.5%) had frequent respiratory tract infections. Preoperative electrocardio-grams were performed in all patients; there was first-degree atrioventricular block in 2, low atrial rhythm in 1, wandering atrial pacemaker in 1, and right bundle branch block in 3. All patients had a preoperative echocardiogram demonstrating the presence of a left-to-right shunt and right-sided chamber enlargement. Nine patients (20.5%) underwent preoperative cardiac catheterization for hemodynamic assessment. Partial anomalous pulmonary venous return was present in 40 patients (90.9%).

Surgery was performed in all cases using a median sternotomy incision and cardiopulmonary bypass with moderate systemic hypothermia (28°C to 32°C). Cold blood potassium cardioplegia was used. The SVC and right pulmonary veins were dissected free. In almost all patients, the SVC was cannulated through the right atrial appendage, and the inferior vena cava was cannulated through the lower part of the right atrium. Separate cannulation of a left SVC was required in 3 patients. Repair of the defect was carried out through a standard longitudinal right atriotomy starting at the atrial appendage and extending toward the inferior vena cava. The defect was repaired with a pericardial patch bringing the superior aspect of the patch along the superior margin of the highest pulmonary vein (Figure 1). A high small pulmonary vein was left draining into the SVC in 3 patients. Care was taken to ensure that the stitches along the upper lateral aspect of the right atrium were not full wall thickness, in order to avoid the SN area. Direct cavoatrial appendage anastomosis was not used in these patients. Five patients underwent enlargement of the medial aspect of the SVC-right atrial junction with a pericardial patch because of intraoperative SVC stenosis. Recently, this approach has been replaced by an incision on the low lateral aspect of the SVC where the anomalous pulmonary veins enter. This facilitates the repair and enlargement whenever needed.

View larger version (143K): [in this window] [in a new window]   Figure 1 (A & B). Patch repair of sinus venosus with partial anomalous pulmonary venous return.

	RESULTS

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The median cardiopulmonary bypass and aortic cross-clamp times were 58 minutes (range, 29 to 141 minutes) and 29 minutes (range, 4 to 67 minutes), respectively. The median defect size measured at surgery was 20 mm (range, 10 to 60 mm). Patch closure was used in 41 patients (93.2%) and primary repair in 3 (6.8%). Five patients (11.4%) had patch enlargement of the SVC-right atrial junction. The median intensive care and hospital stays were 2 days (range, 1 to 12 days) and 6 days (range, 4 to 16 days), respectively. Use of blood products including red cells, fresh frozen plasma, and platelets was reviewed. Autologous donation was not included as it was only used in 1 patient. The volume of blood products decreased from a median of 4.5 units per patient (range, 0 to 12) in the early period (1985 to 1990) to 0.6 units per patient (range, 0 to 2) between 1995 and 1998.

Postoperative complications included bleeding in 2 patients (4.5%), both of whom required reexploration. One patient required reoperation on day 3 for SVC stenosis; angiography showed narrowing at the area of SVC cannulation, well above the ASD sinus venosus repair. Transient perioperative atrioventricular block occurred in 3 patients (6.8%), of whom 1 (2.3%) required pacemaker insertion during the hospitalization. Post-pericardiotomy syndrome with documented pericardial effusion was diagnosed in 4 patients (9.1%), and 1 required drainage. There was 1 perioperative death (2.3%) in a patient who had undergone an uncomplicated repair and was discharged on postoperative day 8. The patient died on postoperative day 23 at a remote hospital, due to pericardial tamponade secondary to an undiagnosed pericardial effusion.

Follow-up (median, 105 months; range, 15 to 176 months) was carried out by our cardiologists, and details of 40 patients were available. There were no late deaths. Follow-up electrocardiograms were available in 37 patients: 31 (83.8%) were in sinus rhythm; 3 (8.1%) demonstrated new onset of low atrial rhythm at 2 months, 5 months, and 84 months postoperatively; and one patient had persistence of low atrial rhythm. One patient was found to have SN dysfunction at 66 months postoperatively, which was asymptomatic and did not require therapy. Late reintervention was necessary in 2 patients (4.5%): balloon angioplasty for SVC stenosis in 1 at 12 months following initial repair; and reoperation for constrictive pericarditis 84 months after ASD closure in the other. Follow-up echocardiograms were available in 35 patients (79.5%), of whom 3 (8.6%) had a hemodynamically insignificant residual shunt; these patients had a small pulmonary vein left draining into the SVC. Three patients (8.6%) had mild SVC stenosis, including the patient who had undergone balloon angioplasty. None of these had undergone SVC patch enlargement at the initial operation, and one had a left SVC; all were asymptomatic. Two patients (5.7%) were noted to have mild flow turbulence in the right pulmonary veins.

	DISCUSSION

TOP ABSTRACT INTRODUCTION PATIENTS AND METHODS RESULTS DISCUSSION REFERENCES

 
Successful surgical repair of sinus venosus defects has been accomplished for many years. However, the incidence of late complications has led to the development of many alternative techniques to avoid SVC obstruction and SN dysfunction. This report describes a group of patients undergoing repair of sinus venosus ASD utilizing a relatively straightforward technique of patch diversion of the pulmonary venous drainage, and ASD closure with or without patch augmentation of the SVC. Methods designed to avoid SVC obstruction include patch closure of the SVC, rotation-advancement flap augmentation, transloca-tion of the SVC to the atrial appendage (atriocavoplasty), and end-to-side anastomosis of the right atrial appendage to the SVC as a second drainage pathway.^4,6,8 Trusler and colleagues⁹ reported that 6.9% of patients had cavoatrial gradients after patch augmentation, as assessed by cardiac catheterization, whereas 2.5% of patients had this complication after SVC translocation.⁴ None of the 5 patients with dual drainage pathways investigated by DeLeon and colleagues⁶ had postoperative SVC obstruction, but the cohort was small with a short follow-up. In this study, SVC stenosis occurred in 3 patients, reintervention was required in 2 (1 early at 3 days, and 1 late at 1 year).

SN dysfunction is the second most common complication in patients undergoing repair of a sinus venosus ASD. The incidence is much higher than in patients having repair of a secundum ASD (10% versus 0.3% in our experience). Early reports suggested a high incidence of SN dysfunction (27% to 40%).^9,10 Initially, this was felt to be related to incisions made through the cavoatrial junction, causing injury to the SN or its arterial supply.⁶ Subsequently, similar dysfunction has been described following SVC translocation, despite the avoidance of a cavoatrial incision.^4–11 Recently, Baskett and Ross¹² have described a superior vena caval transverse incision 1 cm above the right atrial junction, to avoid late arrhythmia. In the 10% of our patients demonstrating SN dysfunction at follow-up, none were symptomatic or required therapy. One patient required perioperative pacemaker insertion for Mobitz II block and remained paced at follow-up.

Residual shunts and pulmonary venous obstruction are the least common complications following repair. Residual shunting through a persistent interatrial communication or persistent anomalous pulmonary venous drainage to the right side was detected by radionuclide scanning in 7.1% of cases.⁹ We found an 8.6% incidence of hemodynamically insignificant residual left-to-right shunt by echocardiographic assessment. This includes the 3 patients in whom a small pulmonary vein was left draining into the SVC; none required reoperation. We also found a 5.7% incidence of turbulence in the right upper pulmonary venous flow by echocardiography, although this was clinically and hemodynamically insignificant. An incision extending into the SVC to provide access to the highest pulmonary venous orifice after closure, without an enlargement procedure, may lead to obstruction of SVC flow. Therefore, we left these small pulmonary veins draining into the SVC after repair to diminish the risk of SVC obstruction. We routinely measured the pressure gradient between the SVC and the right atrium intraoperatively, and if greater than 4 mm Hg, we enlarged the SVC-right atrial junction. Furthermore, our medial approach of SVC enlargement created sinus dysrhythmia in 2 patients and has been replaced recently by an incision in the low lateral aspect of the SVC where the anomalous pulmonary veins enter. This facilitates access to the high connecting pulmonary veins, and pericardial enlargement of this area.

Postpericardiotomy syndrome (PPS) has been reported as one of the most common complications following cardiac surgery (20% to 30%).^13–15 It may be accompanied by significant morbidity and rarely, by mortality. PPS was diagnosed clinically and confirmed by echocardiogram in 4 patients (9.1%); only one required drainage. Unfortunately, one patient developed PPS with tamponade 23 days postoperatively and died undiagnosed in a remote hospital. Although we have previously demonstrated a more rapid recovery in children treated with steroids, it is important to remember that PPS is typically a self-limiting illness that in many cases improves within a few days with minimal symptomatic treatment.¹⁴ Rarely, PPS may lead to the development of constrictive pericarditis as diagnosed in one of our patients investigated for decreased exercise tolerance 7 years after the primary repair.

A weakness of our study relates to the completeness of follow-up. We did not have a follow-up protocol; therefore, electrocardiograms and echocardiograms were performed at the discretion of the cardiologist. Consequently, the incidence of postoperative complications may be underestimated. Follow-up protocols are now in place. Despite our conventional surgical approach, the incidence of residual shunt is low, but SN dysfunction remains a long-term problem in 10% of patients. Ongoing attention to surgical technique is required to minimize long-term SVC obstruction and SN dysfunction.

	REFERENCES

TOP ABSTRACT INTRODUCTION PATIENTS AND METHODS RESULTS DISCUSSION REFERENCES

 

1. Kyger ER III, Frazier OH, Cooley DA, Gillette PC, Reul GJ Jr, Sandiford FM, et al. Sinus venosus atrial septal defect: early and late results following closure in 109 patients. Ann Thorac Surg 1978;25:44–50.[Abstract]

2. Kirklin JW, Ellis FH Jr, Wood EH. Treatment of anomalous pulmonary venous connections in association with interatrial communications. Surgery 1956;39:389–98.

3. Lewin AN, Zavanella C, Subramanian S. Sinus venosus atrial septal defect associated with partial anomalous pulmonary venous drainage: surgical repair. Ann Thorac Surg 1978;26:185–8.[Abstract]

4. 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;38:601–5.[Abstract]

5. Hamilton JRL, Brooks SG, Walker DR. Alternative technique for repair of sinus venosus atrial septal defect. Ann Thorac Surg 1991;51:144–6.[Abstract]

6. DeLeon SY, Freeman JE, Ilbawi MN, Husayni TS, Quinones JA, Ow EP, et al. Surgical techniques in partial anomalous pulmonary veins to the superior vena cava. Ann Thorac Surg 1993;55:1222–6.[Abstract]

7. Pathi V, Guererro R, MacArthur KJ, Jamieson MP, Pollock JC. Sinus venosus defect: single-patch repair with caval enlargement. Ann Thorac Surg 1995;59:1588–9.[Abstract/Free Full Text]

8. Okabe H, Matsunaga H, Kawauchi M, Sekiguchi A, Naruse Y, Tanaka O, et al. Rotation-advancement flap method for correction of partial anomalous pulmonary venous drainage into the superior vena cava. J Thorac Cardiovasc Surg 1990;99:308–11.[Abstract]

9. 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;79:776–81.[Medline]

10. 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]

11. Gustafson RA, Warden HE, Murray GF. Partial anomalous pulmonary venous connection to the superior vena cava. Ann Thorac Surg 1995;60(Suppl 6):S614–7.

12. Baskett RJ, Ross DB. Superior vena cava approach to repair of sinus venosus atrial septal defect. J Thorac Cardiovasc Surg 2000;119:178–80.[Free Full Text]

13. Yip AS, Chau EM, Chow WH, Kwok OH, Cheung KL. Pericardial effusion in adults undergoing surgical repair of atrial septal defect. Am J Cardiol 1997;79:1706–8.[Medline]

14. Wilson NJ, Webber SA, Patterson MW, Sandor GG, Tipple M, LeBlanc J. Double-blind placebo-controlled trial of corticosteroids in children with postpericardiotomy syndrome. Pediatr Cardiol 1994;15:62–5.[Medline]

15. Engle MA, Zabriskie JB, Senterfit LB, Gay WA Jr, O’Loughlin JE Jr, Ehlers KH. Viral illness and the postpericardiotomy syndrome. A prospective study in children. Circulation 1980;62:1151–8.[Abstract/Free Full Text]

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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 Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Russell, J. L Articles by PhD Search for Related Content PubMed PubMed Citation Articles by Russell, J. L Articles by PhD, Related Collections Congenital - acyanotic