心内型部分性肺静脉异位引流病理类型细化分型对术式选择的指导意义

Value of Subtype Classification of Intracardiac Partial Anomalous Pulmonary Venous Connection on Guiding the Surgical Options

目的:探讨心内型部分性肺静脉异位引流(PAPVC)病理类型的细化分型对手术方式选择的指导意义。方法:回顾分析2009年1月至2019年12月于首都医科大学附属北京儿童医院行经胸超声心动图检查诊断为心内型PAPVC并接受手术治疗的患儿46例。根据PAPVC肺静脉回流部位与房间隔缺损病理解剖特点,并结合外科治疗选择的不同术式进行细化分型。结果:(1)Ⅰ型腔静脉型33例(71.7%):回流至腔静脉-右心房结合部,此型中根据有无合并腔静脉窦型房间隔缺损再分Ⅰa骑跨型26例(56.5%),手术方式多采用房间隔修补;Ⅰb非骑跨型7例(15.2%),手术需做心内板障。(2)Ⅱ型右心房内型10例(21.7%):直接回流至右心房后壁,手术也需做心内板障。(3)Ⅲ型冠状静脉窦型3例(6.5%):回流至冠状静脉窦,手术需切开冠状静脉窦壁。结论:PAPVC细化后的病理亚型可以指导手术方式的精确选择。

Objectives:To investigate the clinical value of the subtype classification of intracardiac partial anomalous pulmonary venous connection(PAPVC)on the decision making on surgical procedures.Methods:This study retrospectively analyzed 46 children diagnosed as intracardiac PAPVC by transthoracic echocardiography at Beijing Children's Hospital from 2009 to 2019.Subtype classification was performed according to the pathological and anatomical characteristics of PAPVC,including pulmonary venous drainage site,atrial septal defect,and surgical methods.Results:There were 33 cases with pulmonary veins of typeⅠPAPVC with draining into the vena cava-right atrial junction.This type was further divided into overriding typeⅠPAPVC(n=26)and non-overriding typeⅠPAPVC(n=7)according to the presence or absence of vena cava sinus atrial septal defect.There were 10 cases of typeⅡPAPVC with draining into the right atrium posterior wall and 3 cases of typeⅢPAPVC with draining into the coronary sinus.Patch closure was performed for overriding type.Non-overriding type was treated with an open technique to establish the rim of the pulmonary veins.TypeⅡwas treated with a flap to cover above the orifices of the pulmonary veins.TypeⅢwas treated with incision of the coronary sinus wall.Conclusions:The subtype classification of PAPVC can guide the decision making on surgical procedures.