Isolated partial anomalous pulmonary venous connections in adults: twenty-year experience

We sought to review our experience with isolated partial anomalous pulmonary veins as their management is controversial.

We reviewed all patients with isolated partial anomalous pulmonary veins >18 years attending the Congenital Clinic from 1988-2008.

Forty-three patients aged 20-73 years were included. Fifteen patients had no surgery, 11 of whom had a single anomalous pulmonary vein. Surgical repair was performed in 28 patients: 27 had right ventricular volume overload, 21 had more than one anomalous pulmonary vein, and 1 had stenosed pulmonary veins. Right ventricular systolic pressure was elevated (>35 mm Hg) in 12 patients. Eleven patients had anomalous left pulmonary veins: 10 had the pulmonary vein anastomosed to the left atrial appendage and one patient had anastomosis to the left lower pulmonary vein. One patient had bilateral anomalous pulmonary veins anastomosed directly to the left atrium. Sixteen patients had anomalous right pulmonary veins: fifteen had a baffle through a surgically-created atrial septal defect, while one had the anomalous pulmonary vein anastomosed to the left atrium. There was no early mortality. During mean echo follow-up of 2.7 years, 19 patients (68%) had reduced right ventricular size and five had improved right ventricular function. Seven patients with increased pulmonary pressure had a decrease post-operatively.

Partial anomalous pulmonary veins can cause symptoms, right ventricular volume overload, and pulmonary hypertension, particularly when more than one pulmonary vein is anomalous. Surgical repair can be accomplished with low morbidity and mortality, with improvement in the right ventricular size and pulmonary pressures in most.