在RNF213相关的血管病变中对周围肺动脉狭窄的内血管治疗
Woochan Kwon1, Sung-A Chang2, Taek Kyu Park2
1Division of Cardiology, Department of Medicine, Heart Vascular Stroke Institute, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Republic of Korea; Division of Cardiology, Department of Medicine, Kangbuk Samsung Hospital, Seoul, Republic of Korea.
Journal of vascular and interventional radiology : JVIR
|July 29, 2024
概括
皮肤透光肺动脉增生术 (PTPA) 改善了RNF213基因相关的肺动脉狭窄症患者的症状和肺动脉压. 由于船只反弹,经常需要进行支架.
科学领域:
- 心脏病学 心脏病学
- 遗传学 遗传学 是一个
- 干预性放射学 干预性放射学
背景情况:
- 周围肺动脉狭窄症 (PPAS) 可能与环指蛋白213 (RNF213) 基因的p.Arg4810Lys变体有关.
- 这种遗传变异可能导致显著的狭窄,影响患者的预后和生活质量.
研究的目的:
- 审查与RNF213基因相关的PPAS患者的临床,程序和成像数据,这些患者接受了皮肤透光肺血管增生术 (PTPA).
- 评估PTPA在这个特定患者队列中的结果.
主要方法:
- 对7名患有p.Arg4810Lys RNF213变种的患者的临床,程序和成像数据的回顾性审查.
- 对人口统计,临床表现,血管造形术指示,手术细节和临床结果的分析.
- 诊断后的中位数随访时间为64.4个月.
主要成果:
- 在7名患者的38个会议中,PTPA在62个细分肺动脉上进行.
- 在77%的手术中,由于气球扩张阻力和弹性反弹,需要安装血管支架.
- 在6名患者 (气喘减轻) 和5名患者 (肺动脉平均压力从55.5mmHg降至42.7mmHg,步行距离增加6分钟) 观察到显著改善.
- 在57%的患者 (16%的疗程) 发生了再输血.
结论:
- 对于与RNF213基因相关的PPAS,PTPA是一种可行的治疗选择,具有临床益处.
- 对于这种情况,在PTPA期间经常需要安装支架.
- 虽然PTPA是有效的,但与显著的反胀发病率有关.
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