Taussig-Bing畸形患儿动脉调转术后中远期主动脉根部结局的单中心回顾性研究

Mid-to-long term fate of neo-aortic root after arterial switch operation for Taussig-Bing anomaly:A retrospective study in a single center

目的探究接受动脉调转术后的Taussig-Bing畸形患儿中远期主动脉根部生长情况以及新主动脉瓣反流情况。方法回顾性纳入2002—2017年于上海儿童医学中心接受动脉调转术治疗早期存活且随访时间>3年的患儿,收集随访超声数据并进行分析。结果共纳入127例患者,其中男98例(77.2%),中位手术年龄73.0 d,平均体重4.7 kg,术前45例(35.4%)患儿合并轻度至轻中度肺动脉瓣反流。平均随访时间7.0年。随访中14例(11.0%)患儿出现中度及以上新主动脉瓣反流,3例接受新主动脉瓣机械瓣置换。新主动脉瓣环及窦部内径在随访过程中始终大于同龄患儿正常水平,随访5年平均瓣环内径18.0 mm,随访10年20.5 mm。随访5年窦部内径25.9 mm,随访10年31.1 mm。新主动脉瓣反流随年龄增长逐渐发展。随访过程中出现新主动脉瓣中度及以上反流的患儿其瓣环直径始终大于未出现中度及以上反流的患儿(χ2=18.3,P<0.001)。术前肺动脉瓣轻度及轻中度反流增加中远期新主动脉瓣中度及以上反流的风险(c-HR=3.46,P=0.03)。结论接受动脉调转术的Taussig-Bing患儿其新主动脉瓣环及窦部内径持续增长并始终高于正常水平,随访过程中瓣环扩张与新主动脉瓣反流的进展有显著相关性。动脉调转术前肺动脉瓣反流增加中远期新主动脉瓣反流风险。

Objective To explore growth pattern of neo-aortic root as well as development of neo-aortic regurgitation after arterial switch operation(ASO)for Taussig-Bing anomaly.Methods From 2002 to 2017,the patients who received ASO,and were discharged alive from Shanghai Children’s Medical Center and followed up for more than 3 years were retrospectively involved in this study.Results A total of 127 patients were enrolled.There were 98(77.2%)males,the median age at ASO was 73.0 d and the average weight was 4.7 kg.Forty-five(35.4%)children were complicated with mild or mild-to-moderate pulmonary insufficiency(PI)before ASO.The average follow-up time was 7.0 years.During the follow-up,14(11.0%)children presented moderate or greater neo-aortic regurgitation(neo-AR).The diameter of neo-aortic annulus and sinus of Valsalva was beyond normal range during the entire follow-up.The average diameter of neo-aortic annulus was 18.0 mm at 5 years and 20.5 mm at 10 years.The average diameter of sinus of Valsalva was 25.9 mm at 5 years and 31.1 mm at 10 years.Neo-AR continued to develop over time.The diameter of children who developed moderate or greater neo-AR was constantly larger than that of children who did not(χ2=18.3,P<0.001).Preoperative mild or mild-to-moderate PI was an independent risk factor for the development of moderate or greater neo-AR during mid-tolong term follow-up(c-HR=3.46,P=0.03).Conclusion The diameters of neo-aortic annulus and sinus of Valsalva of Taussig-Bing children who receive ASO repair continue to expand without normalization.The dilation of annulus correlates with the development of neo-AR.PI before ASO repair increases the risk of neo-AR development.