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.
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.
Authors: David B Frank; Philip T Levy; Corey A Stiver; Brian A Boe; Christopher W Baird; Ryan M Callahan; Charles V Smith; Rachel D Vanderlaan; Carl H Backes Journal: J Perinatol Date: 2021-03-05 Impact factor: 2.521
Authors: Edward Y Lee; Kathy J Jenkins; Muhammad Muneeb; Audrey C Marshall; Donald A Tracy; David Zurakowski; Phillip M Boiselle Journal: Pediatr Radiol Date: 2013-03-10