2024 年第 11 期 第 19 卷

完全型肺静脉异位引流外科矫治术后垂直静脉-左心房残余分流介入治疗2例并文献复习

Two cases of vertical venous left atrial residual shunt intervention therapy following surgical correction of total anomalous pulmonary venous drainage and literature review

作者：栗政伟胡海波关璐茜吕建华张戈军潘湘斌

英文作者：Li Zhengwei Hu Haibo Guan Luxi Lyu Jianhua Zhang Gejun Pan Xiangbin

单位：国家心血管病中心中国医学科学院北京协和医学院阜外医院结构性心脏病中心，北京100037

英文单位：Center of Structural Heart Disease Fuwai Hosapital National Center for Cardiovascular Diseases Chinese Academy of Medical Sciences and Peking Union Medical College Beijing 100037 China

关键词：完全型肺静脉异位引流；垂直静脉；残余分流；介入封堵

英文关键词：Totalanomalouspulmonaryvenousdrainage;Verticalvenous;Residualshunt;Interventionalocclusion

• 摘要：

• 完全型肺静脉异位引流（TAPVC)是指肺静脉未能直接与左心房连接而均与右心房或体静脉系统连接的先天性心血管畸形。一旦发现，即有手术指征，通常需要行外科矫治术以重建其血管连接，恢复正常的血液循环。在TAPVC中，由于左心系统缺少正常的血液来源，因此左心房室发育不良（心腔容积明显缩小、顺应性减低），若又合并肺静脉梗阻，结扎垂直静脉（肺静脉常经此血管与体静脉系统连接）后易引起肺动脉高压危象和急性心力衰竭。因此，术者有时会保持垂直静脉开放，使其在术后循环中充当“减压阀”，以减少围手术期肺动脉高压危象。在大多数情况下，随着人体对重建的血液循环的适应，未结扎的垂直静脉会自发关闭；然而在少数情况下，它们会保持开放状态，导致大量左向右分流和右心扩张，需要随后对其进行手术结扎或介入封堵。这种病例非常少见，仅限于病例报道。在此，我们报道了2例效果良好的TAPVC矫治术后垂直静脉残余分流的介入封堵并对相关文献进行了系统复习。

• Total anomalous pulmonary venous connection (TAPVC) represents a congenital cardiac anomaly characterized by abnormal drainage of all four pulmonary veins into either systemic veins or directly into one of its tributaries rather than connecting normally with the left atrium. Upon diagnosis, prompt surgical intervention is warranted for restoration of physiological vascular connections. In TAPVC cases where inadequate blood supply affects cardiac development on the left side resulting in diminished chamber dimensions and compliance alongside potential complications from concomitant obstructive lesions within these vessels; preservation without ligating any persisting vertical conduit-often serving as an alternative pathway for aberrant drainage-may be considered by surgeons as a means for mitigating perioperative hypertensive crises post-reconstruction surgery. While spontaneous closure typically occurs following adaptation after surgery, persistent patency leading to significant intracardiac shunting necessitating further interventional management remains exceedingly uncommon based on limited reported instances. Here, we report two cases of good effect of postoperative residual shunt intervention to block vertical vein TAPVC correction and makes a systematic review of the literature.