Department of Thoracic and Cardiovascular Surgery, Southampton University Hospital, Southampton, United Kingdom.
J Thorac Cardiovasc Surg. 2011 Aug;142(2):344-50. doi: 10.1016/j.jtcvs.2010.12.004. Epub 2011 Feb 1.
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.
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.
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).
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.
原发性肺静脉狭窄常伴有进行性再狭窄和早期死亡。在过去的 20 年中，我们开发了一种无缝线修复技术来改善预后。
回顾了 1989 年至 2008 年接受原发性肺静脉狭窄修复的患者的住院记录。采用肺静脉狭窄评分量化肺静脉狭窄。通过 Kaplan-Meier 分析确定生存率。
23 例患者接受了手术修复。诊断和指数修复时的平均年龄分别为 23.3 ± 45.6 岁和 24.1 ± 40.9 岁。18 例患者中有 13 例（72%）存在系统性或超系统性肺动脉压。7 例（31%）有单心室循环。23 例中有 19 例（83%）采用了无缝线技术。23 例中有 4 例（17%）采用了其他类型的修复。有 11 例记录的死亡（47%）。1、5 和 10 年的生存率分别为 64%、47%和 31%。5 例（22%）需要 1 次再次干预。手术修复显著降低了总肺静脉狭窄评分（修复前为 5.6 ± 2.10，修复后为 2.6 ± 2.72，P =.0057）。术前肺静脉狭窄评分是死亡的唯一独立预测因素（危险比，1.732；P <.01）。术前肺静脉狭窄评分大于 4 是预后不良的指标（曲线下面积，0.83）。
尽管采用了无缝线技术，但死亡率和再狭窄率仍然很高。术前肺静脉狭窄评分大于 4 是预后不良的强烈预测指标。
