Kalfa David, Belli Emre, Bacha Emile, Lambert Virginie, di Carlo Duccio, Kostolny Martin, Salminen Jukka, Nosal Matej, Poncelet Alain, Horer Jurgen, Berggren Hakan, Yemets Illya, Hazekamp Mark, Maruszewski Bohdan, Sarris George, Pozzi Marco, Ebels Tjark, Lacour-Gayet François
Department of Pediatric Cardiac Surgery, Morgan Stanley Children's Hospital of New York-Presbyterian, Columbia University Medical Center, New York, New York and Columbia University, New York, New York.
Department of Pediatric Cardiac Surgery, Marie Lannelongue Hospital, le Plessis Robinson, France.
Ann Thorac Surg. 2017 Jul;104(1):182-189. doi: 10.1016/j.athoracsur.2017.03.022.
Primary pulmonary vein stenosis (PPVS) still carries a poor prognosis, and prognostic factors remain controversial. The aim of this study was to determine outcomes and prognostic factors after PPVS repair in the current era.
Thirty patients with PPVS and a normal pulmonary vein (PV) connection operated on in 10 European/North American centers (2000-2012) were included retrospectively. A specific PVS severity score was developed based on the assessment of each PV. Studied end points were death, PV reoperation, and restenosis. A univariate and multivariate risk analysis was performed.
The mean number of affected PVs per patient was 2.7 ± 1.1. Sutureless repair was used in 21 patients (70%), endovenectomy was used in 5 patients, and patch venoplasty was used in 4 patients. Overall PV restenosis, reoperation, and mortality occurred in 50%, 40%, and 30% of patients respectively. Freedom from mortality, reoperation, and restenosis at 8 years of follow-up was 70% ± 8%, 62% ± 8%, and 47% ± 9%, respectively. Restenosis and mortality rates after sutureless repair versus nonsutureless repair were 57% (n = 12 of 21) versus 33% (n = 3 of 9) (p = 0.42) for restenosis and 38% (n = 8 of 21) versus 11% (n = 1 of 9) (p = 0.21) for mortality. Patients selected for a sutureless technique were younger and smaller and had more severe disease before operation. A postoperative high PVS score and pulmonary hypertension 1 month after the operation were independent risk factors for restenosis (hazard ratio [HR], 1.34; p = 0.002 and HR, 6.81; p = 0.02, respectively), reoperation (HR, 1.24; p = 0.01 and HR, 7.60; p = 0.02), and mortality (HR, 1.39; p = 0.01 and HR, 39.5; p = 0.008).
Primary PVS still has a guarded prognosis in the current era despite adoption of the sutureless technique. Postoperative pulmonary hypertension and severity of disease evaluated by a new severity score are independent prognostic factors regardless of surgical technique.
原发性肺静脉狭窄（PPVS）的预后仍然很差，预后因素仍存在争议。本研究的目的是确定当前时代PPVS修复后的结局和预后因素。
回顾性纳入2000年至2012年在10个欧洲/北美中心接受手术的30例PPVS且肺静脉（PV）连接正常的患者。基于对每条PV的评估制定了特定的PVS严重程度评分。研究的终点是死亡、PV再次手术和再狭窄。进行了单因素和多因素风险分析。
每位患者受影响的PV平均数量为2.7±1.1。21例患者（70%）采用了无缝合修复，5例患者采用了静脉内膜切除术，4例患者采用了补片静脉成形术。总体而言，分别有50%、40%和30%的患者发生PV再狭窄、再次手术和死亡。随访8年时无死亡、无再次手术和无再狭窄的生存率分别为70%±8%、62%±8%和47%±9%。无缝合修复与非无缝合修复后的再狭窄率分别为57%（21例中的12例）和33%（9例中的3例）（p=0.42），死亡率分别为38%（21例中的8例）和11%（9例中的1例）（p=0.21）。选择无缝合技术的患者更年轻、体型更小，术前疾病更严重。术后PVS评分高和术后1个月出现肺动脉高压是再狭窄（风险比[HR]，1.34；p=0.002和HR，6.81；p=0.02）、再次手术（HR，1.24；p=0.01和HR，7.60；p=0.02）和死亡（HR，1.39；p=0.01和HR，39.5；p=0.008）的独立危险因素。
尽管采用了无缝合技术，但在当前时代原发性PVS的预后仍然不佳。无论手术技术如何，术后肺动脉高压和通过新的严重程度评分评估的疾病严重程度都是独立的预后因素。
