遗传性出血性远端血管扩张症的心脏和血液动力学表现。

IF 0.5 Q4 PERIPHERAL VASCULAR DISEASE International Journal of Angiology Pub Date : 2022-07-09 eCollection Date: 2022-06-01 DOI:10.1055/s-0042-1745842
Ahmed Farhan, Muhammad A Latif, Anum Minhas, Clifford R Weiss
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引用次数: 0

摘要

遗传性出血性毛细血管扩张症(HHT)是一种常染色体显性遗传病,会引起多器官血管发育不良病变,从而激活多种生理级联,导致一系列心血管疾病。高达 78% 的 HHT 患者会出现肝动静脉畸形 (AVM),继发于肝/门分流,导致高动力循环状态。这种情况最终会发展为高输出性心力衰竭(HOCF),并伴有持续的外周组织低氧血症。HOCF 的治疗通常仅限于支持性措施(利尿剂和贫血治疗);不过,最近使用贝伐珠单抗进行的系统性研究显示,该疗法可大幅降低心脏指数,前景看好。在肝脏动静脉畸形和高心输出量的情况下,肺血管也会出现高流量。如果肺血管得不到充分扩张,就会出现毛细血管后肺动脉高压。在 HHT 中观察到的另一种肺动脉高压是由 HHT 相关的 ENG 和 ACVRL1 基因突变引起的充血性动脉病变。毛细血管后发病机制通过降低高输出状态来解决,而毛细血管前发病机制则通过支持机制(利尿剂、氧气)和针对肺血管活性的药物(内皮素-1 受体拮抗剂和磷酸二酯酶-5 抑制剂)来治疗。如果任一种肺动脉高压未得到治疗或证明难治并发展,常见的血流动力学并发症就是右心衰竭。右心靶向治疗涉及与肺动脉高压类似的策略,有几种实验方法正在研究中。在本综述中,我们将详细介绍 HHT 患者可能出现的血流动力学并发症和相关心血管疾病的发病机制、诊断和治疗方法。
本文章由计算机程序翻译,如有差异,请以英文原文为准。

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Cardiac and Hemodynamic Manifestations of Hereditary Hemorrhagic Telangiectasia.

The autosomal dominant trait hereditary hemorrhagic telangiectasia (HHT) causes multiorgan dysplastic lesions of the vasculature that can activate multiple physiological cascades leading to a broad array of cardiovascular diseases. Up to 78% of patients with HHT develop hepatic arteriovenous malformations (AVMs), which cause a hyperdynamic circulatory state secondary to hepatic/portal shunting. This condition can eventually progress to high-output cardiac failure (HOCF) with continued peripheral tissue hypoxemia. Treatment for HOCF is often limited to supportive measures (diuretics and treatment of anemia); however, recent studies using systemic bevacizumab have shown promise by substantially reducing the cardiac index. In the context of liver AVMs and high cardiac output, the pulmonary vasculature can also experience high flow. Without adequate dilation of pulmonary vessels, post-capillary pulmonary hypertension can develop. Another form of pulmonary hypertension observed in HHT, pulmonary arterial hypertension, is caused by HHT-related mutations in ENG and ACVRL1 causing congestive arteriopathy. Post-capillary pathogenesis is addressed by reducing the high-output state, whereas the pre-capillary state is treated with supportive mechanisms (diuretics, oxygen) and agents targeting pulmonary vasoreactivity: endothelin-1 receptor antagonists and phosphodiesterase-5 inhibitors. If either form of pulmonary hypertension is left untreated or proves refractory and progresses, the common hemodynamic complication is right heart failure. Targeted right heart therapies involve similar strategies to those of pulmonary arterial hypertension, with several experimental approaches under study. In this review, we describe in detail the mechanisms of pathogenesis, diagnosis, and treatment of the hemodynamic complications and associated cardiovascular diseases that may arise in patients with HHT.

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来源期刊
International Journal of Angiology
International Journal of Angiology PERIPHERAL VASCULAR DISEASE-
CiteScore
1.30
自引率
16.70%
发文量
57
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