Pathogenesis of Pulmonary Arterial Hypertension: Clues From Patient and Animal Models of Hereditary Hemorrhagic Telangiectasia

Advances in Pulmonary Hypertension

关键词: Cardiology;    WHO Group 1- Pulmonary Arterial Hypertension;    Anatomy;    Physiology/Pathophysiology;    nutrition;    Genetics;    BMPR2;    Endoglin;    HHT (hereditary hemorrhagic telangiectasia);    hematologic disorder;    pharmacokinetics;    nitric oxide;    Inotropes;    Non-invasive estimate of PVR;    Pulmonary vascular resistance;    vasculogenesis;    vascular remodeling;    endothelial proliferation;    endothelial dysfunction;    Endothelial progenitor cells;    hypoxia;    biomarkers;    Animal Models of PH;    Angiogram;    Epidemiology;   

DOI  :  

学科分类：医学（综合）

来源: Pulmonary Hypertension Association

【 摘 要 】

Hereditary hemorrhagic telangiectasia (HHT) is a vascular disease characterized by multiple focal telangiectases and arteriovenous malformations (AVMs) in the pulmonary, hepatic, and cerebral microcirculations. These fragile structures are low-pressure conduits that can affect local tissue blood flow, and their potential rupture in vital organs can lead to internal hemorrhage, anemia, and death. Patients with HHT1 and HHT2 display very similar vascular lesions, but diverge with respect to organ involvement, where a higher prevalence of pulmonary AVMs (PAVMs) is seen in HHT1.1.

【 授权许可】

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