Primary pulmonary vein stenosis: the impact of sutureless repair on survival.

BACKGROUND

Primary pulmonary vein stenosis is often associated with relentless restenosis and early death. During the last 2 decades, we have developed a sutureless repair to improve prognosis.

METHODS

Hospital records for patients undergoing repair of primary pulmonary vein stenosis from 1989 to 2008 were reviewed. Pulmonary vein stenosis was quantified with a pulmonary vein stenosis score. Survival was determined by Kaplan-Meier analysis.

RESULTS

Twenty-three patients underwent surgical repair. Mean ages at diagnosis and index repair were 23.3 ± 45.6 and 24.1 ± 40.9 months, respectively. Systemic or suprasystemic pulmonary artery pressures were present in 13 of 18 patients (72%). Seven (31%) had single-ventricle circulation. A sutureless technique was used in 19 of 23 cases (83%). Other types of repair were used in 4 of 23 (17%). There were 11 recorded deaths (47%). Survivals were 64%, 47%, and 31% at 1, 5, and 10 years, respectively. Five patients (22%) required 1 reintervention. Surgical repair significantly reduced the total pulmonary vein stenosis score (5.6 ± 2.10 before repair, 2.6 ± 2.72 after repair, P = .0057). The preoperative pulmonary vein stenosis score was the only independent predictor of mortality (hazard ratio, 1.732; P < .01). A preoperative pulmonary vein stenosis score of greater than 4 was a poor prognostic indicator (area under the curve, 0.83).

CONCLUSIONS

Mortality and restenosis rates remained high despite the adoption of a sutureless technique. A preoperative pulmonary vein stenosis score of greater than 4 was a strong predictor of poor prognosis.