源自双边动脉管道的先天性非流动性肺动脉:一种罕见的异常
Koji Miwa1, Shigemitsu Iwai2, Tomomitsu Kanaya2
1Department of Cardiovascular Surgery, Osaka Women's and Children's Hospital, 840 Murodocho, Izumi, Osaka, 594-1101, Japan. komiwa0712@gmail.com.
Pediatric cardiology
|July 15, 2023
概括
来自双侧动脉管 (AD) 的非流动性肺动脉不会阻止成功的Fontan循环. 这种情况可以通过平衡肺血流来实现良好的结果,尽管需要进一步的研究.
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.
- 外科手术的结果
背景情况:
- 丰坦循环是针对单心室缺陷的复杂外科手术.
- 来自双侧动脉管 (AD) 的非流动性肺动脉在Fontan息治疗中提出了独特的挑战.
- 了解这种特定解剖学对Fontan结果的影响对于手术规划至关重要.
研究的目的:
- 评估来自双边AD的非流动性肺动脉对Fontan循环结果的影响.
- 评估Fontan完成在患有这种解剖变异的患者中的可行性和成功.
- 在这个队列中分析Fontan程序后的中期和长期结果.
主要方法:
- 九名患有双边AD和非流动性肺动脉的患者进行了单心室息 (1993-2023年) 的回顾性审查.
- 对手术策略的分析,包括全身肺部分流,血管整形和AD支架.
- 评估Fontan前肺动脉指数 (PAI) 和PAI比率,间期死亡率和Fontan后的结果,包括住院和干预措施.
主要成果:
- 在研究队列中没有观察到间期死亡率.
- 肺动脉表现出增长,并实现了平衡的流量 (Fontan 之前的 PAI: 164 ± 27 mm2/m2; R/L PAI 比率: 1.06 ± 0.23).
- 所有患者成功完成了Fontan手术,平均随访时间为7.8年; 一名患者心力衰竭,三名患者需要为肺狭窄术后进行干预.
结论:
- 来自双边AD的非流动性肺动脉并不排除成功完成Fontan和有利的结果.
- 恢复平衡的肺血流是这些患者获得良好结果的关键.
- 需要使用更大的样本大小进行进一步的研究,以优化这种特定疾病的外科手术方法.
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