手术矫治孤立型部分性肺静脉异位引流的安全性及中长期疗效分析

Safety and mid-to long-term efficacy analysis of surgical correction of isolated partial anomalous pulmonary venous connection

目的评价孤立型部分性肺静脉异位引流(isolated partial anomalous pulmonary venous connection,IPAPVC)行外科手术矫治的安全性及中长期疗效。方法回顾性连续纳入2009年6月—2019年5月于中国医学科学院阜外医院及云南省阜外心血管病医院被诊断为IPAPVC并行外科手术矫治患者的临床资料,分析患者术前、术中基本情况,统计术后及中长期随访结果。结果共纳入54例患者,其中男29例、女25例,年龄1个月~62岁(16.20±2.40)岁;28例(51.9%)患者出现不同程度的心律失常,22例(40.7%)患者存在心功能不全,39例(72.2%)患者出现肺动脉高压。根据Bordy分型,A型14例(25.9%)、B型23例(42.6%)、C型4例(7.4%)、D型5例(9.3%)、混合型8例(14.8%)。全组患者行经胸超声心动图检查且分型诊断准确率为66.7%(36/54),37例患者行CT血管造影检查且分型诊断准确率为94.6%(35/37)。所有手术均使用体外循环辅助,主动脉阻断时间0~219(67.02±5.23)min,体外循环时间40~261(105.09±5.23)min,术中无严重并发症;术后气管插管时间0~230(13.33±4.20)h,住ICU时间0~13(1.89±0.28)d,术后住院时间5~18(7.20±0.38)d。随访时间16~140(62.58±5.12)个月。术后全因死亡2例(3.7%),其中院内死亡1例,随访期间死亡1例,无术中死亡。生存患者中,手术相关并发症3例:1例出现房间隔缺损并行二次手术治疗,上腔静脉早期梗阻及心律失常各1例。另有2例患者为术前IPAPVC导致的并发症(心房颤动、侧枝循环),在外科手术后再次进行手术治疗,且预后不佳;1例术前并发心功能不全、心房颤动,随访期间心功能不全、心房颤动持续存在。结论IPAPVC在部分性肺静脉异位引流中占比较低,经胸超声心动图联合CT血管造影检查可提高诊断准确率,确诊后应择期手术治疗,手术方式应结合疾病分型、引流支数及引流位置等影像学特征制定个体化方案,手术治疗安全有效,术后应定期随访。

Objective To evaluate the safety and mid-to long-term efficacy of surgical correction of isolated partial anomalous pulmonary venous connection(IPAPVC).Methods We retrospectively collected consecutive patients who were diagnosed with IPAPVC and underwent surgical correction at Fuwai Hospital of Chinese Academy of Medical Sciences and Fuwai Yunnan Cardiovascular Hospital from June 2009 to May 2019,summarized the basic preoperative and intraoperative data of patients,analyzed the postoperative and mid-to long-term follow-up results.Results A total of 54 patients were enrolled,including 29 males and 25 females,with an average age of 16.20±2.40 years,ranging from 1 month to 62 years.There were 28(51.9%)patients with varying degrees of arrhythmia,22(40.7%)patients with cardiac insufficiency,and 39(72.2%)patients with pulmonary hypertension.According to Bordy's typing,14(25.9%)patients were classified as type A,23(42.6%)type B,4(7.4%)type C,5(9.3%)type D and 8(14.8%)mixed type.Transthoracic echocardiography was performed in the whole group of patients and the accuracy of staging diagnosis was 66.7%(36/54),and cardiac CT angiography(CTA)was performed in 37 patients and the accuracy of staging diagnosis was 94.6%(35/37).All surgical procedures were assisted with cardiopulmonary bypass,aortic cross-clamping time was 0-219(67.02±5.23)min,cardiopulmonary bypass time was 40-261(105.09±5.23)min,and there was no serious intraoperative complication.Postoperative tracheal intubation time was 0-230(13.33±4.20)h,intensive care unit stay was 0-13(1.89±0.28)days,postoperative hospital stay was 5-18(7.20±0.38)days,and follow-up time was 16-140(62.58±5.12)months.There were 2(3.7%)all-cause postoperative deaths,including 1 in-hospital death and 1 death during the follow-up,and there was no intraoperative death.Among the survivors,there were 3 patients with surgery-related complications:1 patient had atrial septal defect with the second surgical treatment,1 early obstruction of the superior vena cava and 1 arrhythmia.Two patients had complications of IPAPVC(atrial fibrillation,collateral circulation)prior to surgery and underwent the second surgery with a poor prognosis,and 1 patient had preoperative cardiac insufficiency and atrial fibrillation,whose symptoms persisted for a long time during the follow-up.Conclusion IPAPVC accounts for a lower percentage of partial anomalous pulmonary venous connection,transthoracic echocardiography combined with CTA improves diagnostic accuracy,and IPAPVC should be treated with elective surgery after diagnosis.The surgical approach should be individualized with imaging features such as disease staging,number of drains and drainage location.Surgical treatment of IPAPVC is safe and effective,and regular follow-up is warranted.