Partial anomalous pulmonary venous connection to the right side of the heart

Abstract

Partial anomalous pulmonary venous connection to the right side of the heart often complicates surgery for atrial septal defects. Between 1964 and 1987, 39 patients, ranging from 2 to 52 years old, underwent repair of partial anomalous pulmonary venous connection. At least one anomalous pulmonary vein arose from the right upper lobe in 38 patients and right middle lobe in 30 patients and connected to the superior vena cava in 28 patients and the right atrium only in 11 patients. An atrial septal defect was present in 32 patients (82%). Patients who had partial anomalous pulmonary venous connection to the superior vena cava-right atrium junction, the right atrium or both were treated by septal translocation (two patients) or patch redirection of the anomalous pulmonary venous flow to the left atrium through a native atrial septal defect (eight patients) or a surgically created atrial septal defect in two patients with intact atrial septum. For partial anomalous pulmonary venous connection to the high superior vena cava (27 patients), the superior vena cava was transected and oversewn above the anomalous veins. The anomalous pulmonary venous flow was redirected through the proximal superior vena cava into the left atrium across a sinus venous atrial septum defect (22 patients) or a surgically created atrial septal defect in five patients with intact atrial septum. The atrial septal defect was coapted to the intracardiac orifice of the superior vena cava, and the distal superior vena cava was anastomosed to the right atrial appendage. One 31-year-old woman with severe pulmonary hypertension died early and was the only death in the series. A technical error early in the series resulted in one symptomatic superior vena cava obstruction. Only one patient remains in sick sinus syndrome late. All patients remain well over long follow-up (1 to 24 years). Postoperative catheterization or echocardiography has revealed no intracardiac defects, pulmonary venous obstruction, or superior vena cava obstruction (except the one technical error). Correction of partial anomalous pulmonary venous connection should be individualized according to the site of connection of the anomalous pulmonary veins and the location of the atrial defect to minimize undesirable postoperative sequelae often associated with other methods of repair.