Abstract 2089: Surgical Repair of Primary Pulmonary Vein Stenosis: A Single-Centre, 20 Years Experience
Background: Primary pulmonary vein stenosis is often associated with relentless restenosis and early death. Over the last two decades we have developed a sutureless repair to improve prognosis.
Population and Methods: Clinical courses of patients undergoing repair of primary pulmonary vein stenosis from 1989 to 2008 were reviewed. Stenosis was diagnosed by echocardiography, angiography, operative reports and pathology specimens. Each vein was assigned a pulmonary vein stenosis (PVS) score (from 0 to 3) by two independent observers and the sum of the single scores provided the total score per patient before and after the repair. Survival was determined using Kaplan-Meier analysis.
Results: Twenty three patients underwent surgical repair of primary pulmonary vein stenosis. Mean age at diagnosis and first surgical repair were 23.3 ± 45.6 and 24.1 ± 40.9 months respectively. Systemic or supra-systemic pulmonary artery pressures were present in 13/18 (72%) pts. Seven (31%) had single ventricle circulation. A sutureless technique was employed in 18/23 (78%) and 12/18 had a unilateral and 6/18 had bilateral repairs. Other types of repair were used in 5/23 (22%). Survival was 64, 47 and 31% at 1, 5 and 10 years, respectively. All recorded deaths (11 patients) occurred within 12 months of initial repair (average survival 4.6 ± 4.5 months). In this subgroup all but one patient had a high PVS score at the time of death. Among all patients, 5/23 (22%) pts required one re-intervention, and 2/23 (9%) were re-operated twice, regardless of the technique used at first repair. Surgical repair significantly reduced the total PVS score (5.6 ± 2.10 before repair, 2.6 ± 2.72 after repair, 0.0057). The preoperative PVS score was the only identified independent predictor of mortality (hazard ratio 1.732, p <0.01). A pre-operative PVS score of greater than 4 was a poor prognostic indicator (AUC = 0.83).
Conclusions: Mortality and restenosis rates remain high in this population, despite the adoption of a sutureless technique and despite a significant reduction in PVS score at the time of initial repair. A preoperative PVS score of greater than 4 was found to be a strong predictor of postoperative mortality.