由于高输出心力衰竭而导致的肺高血压:遗传性出血性长膜外科病
Burçak Kılıçkıran Avcı1, Ali Uğur Soysal1, Emir Cerme2
1Department of Cardiology, İstanbul University-Cerrahpaşa, Cerrahpaşa Faculty of Medicine, İstanbul, Türkiye.
概括
肺高血压 (PH) 和高输出心力衰竭 (HF) 可能源于不可察觉的动脉静脉形 (AVM). 早期诊断遗传性出血性远程切除症 (HHT) 对于管理这些复杂的心血管疾病至关重要.
科学领域:
- 心脏病学 心脏病学
- 遗传学 是一个遗传学.
- 血管医学 血管医学
背景情况:
- 肺高血压 (PH) 需要一个多学科的方法.
- 左心脏病通常通过心脏成像诊断,但有些原因需要更深入的调查.
- 高输出心力衰竭 (HF) 从动脉静脉形 (AVMs) 可能是一个被忽视的原因的HF和PH.
研究的目的:
- 要突出遗传性出血性电脉切开症 (HHT) 与表现为肺高血压 (PH) 的高输出心力衰竭 (HF) 之间的关联.
- 强调评估肝脏AVM对HTT的重要性,即使在无症状患者中也是如此.
- 提出一个病例报告,一个患者最初被诊断为PH和HF,具有保存的喷射分数,后来发现与HHT相关的高输出HF.
主要方法:
- 病例报告详细介绍患者的诊断旅程.
- 对复杂心脏病的诊断程序的审查.
- 评估AVM及其系统影响.
主要成果:
- 这位患者最初被诊断为PH和HF,并保留了喷射分数.
- 进一步的调查显示,高输出HF是AVM的次要.
- 这种疾病的根本原因被确定为遗传性出血性脑膜切开症 (HHT,奥斯勒-韦伯-伦杜综合征).
结论:
- 遗传性出血性电脉切开症 (HHT) 可以表现为肺高血压 (PH) 由于高输出心力衰竭 (HF) 来自AVMs.
- 在肝脏AVM患者中,对HHT进行彻底评估至关重要,因为他们可能表现出多系统性症状.
- 这一案例强调了需要考虑PH和HF的不太常见原因,特别是与HHT相关的AVM.
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