Outcomes after surgical treatment of children with partial anomalous pulmonary venous connection.

BACKGROUND: We explore early results and time-related morbidity after surgical repair of partial anomalous pulmonary venous connection (PAPVC) at our institution.

METHODS: Between 1982 and 2006, 306 consecutive patients underwent surgery for PAPVC; of these, 236 (77%) were children with a median age of 5.3 years (range, 0.47 to 18 years). Clinical and echocardiographic follow-up was obtained.

RESULTS: PAPVC was right-sided in 214 patients (90%), left-sided in 17 (7%), and bilateral in 5 (2%). Anomalous veins were partial in 186 patients (79%) and involved the entire lung in 50 (21%). The most common type was right PAPVC into the superior vena cava in 175 (74%), with 87% associated with sinus-venosus atrial septal defect; followed by right PAPVC into the right atrium in 29 (12%), left PAPVC into the innominate vein in 22 (9%), and scimitar syndrome in 15 (6%). Repair strategy included intracardiac baffle in 203, pulmonary vein implantation in 22, and SVC division with reimplantation on the right atrial appendage in 14. There was no early or late mortality. Freedom from reoperation, vena cava obstruction, pulmonary vein obstruction, and pacemaker implantation at 15 years was 97%, 97.8%, 86%, and 99.1%, respectively. Pulmonary vein obstruction was significantly more common in patients with scimitar compared with others (22.4% versus 98.3%, p < 0.0001). Postoperative quantitative lung perfusion scans in 13 of 15 scimitar patients showed decreased right lung perfusion (mean, 22.5%).

CONCLUSIONS: Surgical treatment of PAPVC is associated with excellent outcomes and low time-related morbidity. However, management of children with scimitar syndrome is complicated by an exceptionally high incidence of postoperative pulmonary venous obstruction and abnormally diminished perfusion of the right lung.