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在Fontan完成后,解剖亚型对低可塑性左心综合征的长期影响

Jiyong Moon¹, Timothy Lancaster¹, Vikram Sood¹

¹Section of Section of Pediatric Cardiac Surgery, Department of Cardiac Surgery, University of Michigan Health System, C.S. Mott Children's Hospital, Ann Arbor, Mich.

The Journal of thoracic and cardiovascular surgery

|November 11, 2023

概括

大动脉缩/心心狭窄 (AA/MS) 与因左心缩综合征 (HLHS) 的Fontan手术后的长期存活率较差有关. 这是由于单心室衰竭的增加,而不是门问题.

科学领域:

儿童心脏病学儿童心脏病学
遗传性心脏病是一种先天性心脏病.
心脏外科手术心脏外科手术

背景情况:

大动脉缩 (AA) 和心肌狭窄 (MS) 是诺伍德手术后生存的已知风险因素.
针对AA/MS的特定解剖学亚型对手术缓解后的结果的长期影响尚不清楚.

研究的目的:

为了研究患者的长期临床结果和功能状况低可塑性左心综合征 (HLHS) 谁经历了Fontan完成.
确定不同HLHS解剖学亚型,特别是涉及AA/MS的亚型对存活率和心室功能的影响.

主要方法:

对418名接受Fontan手术的HLHS患者进行了回顾性单中心研究 (1989-2017年).
对临床结果,心室功能和心房门 (AVV) 功能进行分析.
多变量统计分析,以评估HLHS亚型的影响.

主要成果:

在20年后,总体无移植生存率为70.1%,AVV衰竭为35.9%,心室衰竭为17.9%.
在AA/MS亚型的患者中,17年后的存活率 (56.1%) 显著较低,而在AA/心肌缩 (74.6%) 和大动脉狭窄/MS (79.1%) 则相比较低.
与AA/MS患者相比,AA/MS患者心室衰竭的发病率较高 (26.2%对11.2%),但AVV衰竭的发病率并不高.

结论:

在Fontan完成后,AA/MS解剖学亚型与持续的长期生存风险有关.
增加的心室衰竭,而不是AVV衰竭,似乎是AA/MS中较差结果的主要驱动因素.
由于AA/MS导致的低塑性左心室的异常压力过载会对单右心室功能产生不利影响.

关键词:

这里是Fontan Fontan.解剖学子类型解剖学子类型大动脉缩/心心管狭窄症低可塑性左心综合征是什么

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