合并室间隔缺损及粗大体肺侧支的肺动脉闭锁的选择性侧支融合术及粗大体肺侧支的再认识

目的探讨选择性侧支融合术或单源化治疗(unifocalization,UF)在室间隔缺损(VSD)及粗大体肺侧支(MAPCAs)的肺动脉闭锁(PA)患儿中的应用,并从组织病理学角度深入剖析不同解剖形态的MAPCAs。方法2017年6月至2018年12月,6例合并粗大体肺侧支及室间隔缺损的肺动脉闭锁(MAPCAs/VSD/PA)患儿实施选择性UF术加一期根治术,男4例,女2例;年龄6~96个月;体质量5.0~23.0 kg。术前均行心导管、选择性体肺侧支造影及心脏CTA检查,明确体肺侧支种类及解剖分布。对造影提示参与肺"气血交换"的粗大体肺侧支实行选择性UF,对不参与肺"血气交换"的功能性体肺侧支术中结扎或不处理。术中留取不同种类粗大体肺侧支的组织行病理检查,根据组织病理学结果明确侧支类型。结果围术期及随访期间无死亡。术前心导管、选择性侧支检查及术中组织病理学检查证实,参与肺"血气交换"的功能性MAPCAs具有肺动脉壁结构,属于弹性动脉;不参与肺"血气交换"的非功能性MAPCAs为小动脉壁结构,属于肌型动脉。结论术前可通过选择性MAPCAs造影检查明确参与肺"血气交换"功能性侧支,功能性侧支属于弹性动脉,具有肺动脉壁结构,术中对功能性侧支进行UF术可取得良好的手术效果。

The research of selective unifocalization in patients with pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals and recognition of major aortopulmonary collaterals from the perspective of histopathology

Objective To investigate the clinical outcomes of selective major aortopulmonary collaterals(MAPCAs)unifocalization and report histopathological findings in patients with pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals(PA/VSD/MAPCAs).Methods The study enrolled 6 MAPCAs/VSD/PA patients with age ranged from 6 to 96 months and body weight ranged from 5.0 to 23.0 kg.These patients underwent selective MAPCAs unifocalization and primary repairs.Preoperative cardiac catheter,selective arteriography,cardiac CTA and intraoperative pathology were performed to identify different function,anatomic distribution and histopathology of MAPCAs.Results 6 MAPCAs/VSD/PA patients underwent selective MAPCAs unifocalization and primary repair.No death occurred after operation and at follow-up which lasted for 1 to 20 months.Preoperative cardiac catheter,selective arteriography and intraoperative histopathology demonstrated distribution of functional MAPCAs similar to native pulmonary artery arborization and participating in arterial gas exchange.Functional MAPCAs were classified into elastic arteries according to histopathology.Conclusion There are two histological type of MAPCAs which play different roles.Selective unifocalization to functional MAPCAs which are classified into elastic arteries like native pulmonary artery is a safe and effective treatment approach for PA/VSD/MAPCAs.