Partial anomalous pulmonary venous connection to the superior vena cava

Correction of partial anomalous pulmonary venous connection to the superior vena cava (SVC) is often complicated by sinus node dysfunction and occasional pacemaker insertion.

Between 1964 and 1994 40 patients, ranging from 14 months to 52 years old, underwent an operative approach designed to minimize trauma to the sinus node and its blood supply. The SVC was transected and oversewn above the highest anomalous vein. The anomalous pulmonary veins were redirected across a sinus venosus atrial septal defect (33 patients) or a surgically created atrial septal defect (7 patients) into the left atrium. The atrial septal defect rim was coapted to the intracardiac SVC orifice in 23 patients. An intracardiac baffle was used in 17 patients. The cephalad SVC stump was anastomosed to the right atrial appendage. The origin of the pulmonary veins draining into the SVC was right upper lobe (all patients), right middle lobe (33 patients), and the entire right lung (9 patients).

One 31-year-old woman died of severe pulmonary hypertension. An early technical error resulted in one symptomatic SVC obstruction. All patients remained well over follow-up (6 months to 30 years), without residual intracardiac defects. Sick sinus syndrome developed late in only 1 patient (2.5%). No patient has required a pacemaker.

The very low incidence of late arrhythmias with cavoatrial reconstruction is most encouraging.