Transcaval repair of the sinus venosus syndrome

Background: Surgical correction of the sinus venosus syndrome has been asso ciated with sinus node dysfunction and venous obstruction postoperatively. We present the long-term follow-up of a lateral transcaval approach, which closes the atrial communication and corrects the partial anomalous pulmonar y venous connection to the superior vena cava with the use of a simple peri cardial patch. Methods: The records of 66 patients undergoing repair between April 1981 an d April 1997 were examined. Mean age at repair was 10.2 years (range, 1.5-6 5 years; median, 5 years). Six patients had a left superior vena cava, 4 ha d an additional atrial septal defect, and 2 had coronary artery bypass graf ts. Immediate and long-term follow-up included physical examination, electr ocardiography, transthoracic echocardiography, and use of a 24-hour ambulat ory Holter monitor. Sinus node function, incidence of significant arrhythmi a, and evidence of mechanical venous obstruction were assessed. Results: Follow-up data were available for 64 (97%) patients for a mean fol low-up of 4.1 years (range; 1-9 years). There were no deaths. No evidence o f residual atrial septal defect, superior vena cava, or venous obstruction were found by echocardiography. On electrocardiography all patients were in sinus rhythm, with no arrhythmia seen. Holter monitoring was performed at a mean of 7.3 years postoperatively. All patients had normal sinus node fun ction, and no sustained atrial arrhythmia was seen. Conclusion: Transcaval repair is a simple technique that does not interfere with sinus node function. There is no evidence to suggest that this approa ch leads to venous obstruction.