Total Anomalous Pulmonary Venous Connection Morphology and Outcome From an International Population-Based Study
Total Anomalous Pulmonary Venous Connection Morphology and Outcome From an International Population-Based Study
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DOI：
10.1161/circulationaha.110.940825
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发表时间：
2010-12-21
期刊：
影响因子：
37.8
通讯作者：
Daubeney, Piers E. F.
中科院分区：
文献类型：
--
作者：
Seale, Anna N.;Uemura, Hideki;Daubeney, Piers E. F.
背景：全异常肺静脉连接修复后的晚期死亡率通常与肺静脉阻塞（PVO）有关。我们的目的是描述完全异常肺静脉连接的形态学谱，并确定死亡和术后PVO的危险因素。方法和结果：我们进行了一项回顾性、国际性、合作性、基于人群的研究，涉及英国、爱尔兰和瑞典的所有19个儿科心脏中心。所有在1998年至2004年间出生的肺静脉连接完全异常的婴儿均被确定。排除单室循环功能或心房异构的病例。回顾了所有可用的数据和影像。在422例活产病例中，205例（48.6%）为心上连接，110例（26.1%）为心下连接，67例（15.9%）为心脏连接，37例（8.8%）为混合连接。常见肺静脉闭锁2例（0.5%）。有些患者静脉发育严重不足，或个别静脉出现离散狭窄。60例（14.2%）伴有心脏异常。16人在干预前死亡。手术治疗患者的三年生存率为85.2%（95%置信区间为81.3%至88.4%）。在多变量分析中，死亡的危险因素包括手术年龄较早、肺静脉发育不良/狭窄、相关的复杂心脏病变、术后肺动脉高压和术后PVO。406例接受完全异常肺静脉连接修复的患者中，60例（14.8%）术后PVO需要再干预。术后PVO患者初始手术后三年生存率为58.7%（95%可信区间为46.2%至69.2%）。术后PVO的危险因素包括术前肺静脉发育不良/狭窄和缺少共同汇合处。结论：术前临床和形态学特征是影响PVO术后生存的重要危险因素。(循环。2010;122:2718 - 2726)。
Background-Late mortality after repair of total anomalous pulmonary venous connection is frequently associated with pulmonary venous obstruction (PVO). We aimed to describe the morphological spectrum of total anomalous pulmonary venous connection and identify risk factors for death and postoperative PVO.Methods and Results-We conducted a retrospective, international, collaborative, population-based study involving all 19 pediatric cardiac centers in the United Kingdom, Ireland, and Sweden. All infants with total anomalous pulmonary venous connection born between 1998 and 2004 were identified. Cases with functionally univentricular circulations or atrial isomerism were excluded. All available data and imaging were reviewed. Of 422 live-born cases, 205 (48.6%) had supracardiac, 110 (26.1%) had infracardiac, 67 (15.9%) had cardiac, and 37 (8.8%) had mixed connections. There were 2 cases (0.5%) of common pulmonary vein atresia. Some patients had extremely hypoplastic veins or, rarely, discrete stenosis of the individual veins. Sixty (14.2%) had associated cardiac anomalies. Sixteen died before intervention. Three-year survival for surgically treated patients was 85.2% (95% confidence interval 81.3% to 88.4%). Risk factors for death in multivariable analysis comprised earlier age at surgery, hypoplastic/stenotic pulmonary veins, associated complex cardiac lesions, postoperative pulmonary hypertension, and postoperative PVO. Sixty (14.8%) of the 406 patients undergoing total anomalous pulmonary venous connection repair had postoperative PVO that required reintervention. Three-year survival after initial surgery for patients with postoperative PVO was 58.7% (95% confidence interval 46.2% to 69.2%). Risk factors for postoperative PVO comprised preoperative hypoplastic/stenotic pulmonary veins and absence of a common confluence.Conclusions-Preoperative clinical and morphological features are important risk factors for postoperative PVO and survival. (Circulation. 2010;122:2718-2726.)