The pathophysiology of chronic thromboembolic pulmonary hypertension.
The pathophysiology of chronic thromboembolic pulmonary hypertension.
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DOI：
10.1183/16000617.0112-2016
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发表时间：
2017-03-31
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影响因子：
--
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慢性血栓栓塞性肺动脉高压（CTEPH）是一种罕见的进行性肺血管疾病，通常是既往急性肺栓塞的结果。CTEPH通常始于机化血栓导致的大和/或中等大小肺动脉的持续性阻塞。血栓未能溶解可能与异常纤维蛋白溶解或潜在的血液学或自身免疫性疾病有关。目前已知小血管异常也会导致CTEPH的血流动力学受损、功能损害和疾病进展。小血管疾病可能发生在阻塞区域，可能由未溶解的血栓物质触发，以及闭塞的下游，可能是因为来自高压支气管和全身动脉的侧支供血过多。小血管疾病的分子基础过程尚未完全了解，需要在这一领域进行进一步研究。小血管病变的程度对CTEPH的严重程度和术后结局有重大影响。CTEPH的介入和药物治疗应旨在恢复肺血管内的正常血流分布、卸载右心室并预防或治疗小血管疾病。它需要早期、可靠地识别CTEPH患者，并在专家中心使用最佳治疗方式。CTEPH具有复杂的病理生理学，包括持续性机化血栓和广泛的小血管疾病http://ow.ly/IgMH309hwmq
Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare, progressive pulmonary vascular disease that is usually a consequence of prior acute pulmonary embolism. CTEPH usually begins with persistent obstruction of large and/or middle-sized pulmonary arteries by organised thrombi. Failure of thrombi to resolve may be related to abnormal fibrinolysis or underlying haematological or autoimmune disorders. It is now known that small-vessel abnormalities also contribute to haemodynamic compromise, functional impairment and disease progression in CTEPH. Small-vessel disease can occur in obstructed areas, possibly triggered by unresolved thrombotic material, and downstream from occlusions, possibly because of excessive collateral blood supply from high-pressure bronchial and systemic arteries. The molecular processes underlying small-vessel disease are not completely understood and further research is needed in this area. The degree of small-vessel disease has a substantial impact on the severity of CTEPH and postsurgical outcomes. Interventional and medical treatment of CTEPH should aim to restore normal flow distribution within the pulmonary vasculature, unload the right ventricle and prevent or treat small-vessel disease. It requires early, reliable identification of patients with CTEPH and use of optimal treatment modalities in expert centres. CTEPH has complex pathophysiology including persistent organised thrombus and extensive small-vessel disease http://ow.ly/IgMH309hwmq
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DOI：
10.1161/atvbaha.113.302991
发表时间：
2014-04-01
影响因子：
8.7
作者：
Alias, Sherin;Redwan, Bassam;Lang, Irene M.
通讯作者：
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影响因子：
24.3
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影响因子：
5
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影响因子：
24.3
作者：
Dorfmueller, Peter;Guenther, Sven;Mercier, Olaf
通讯作者：
Mercier, Olaf
影响因子：
5.4
作者：
Frey MK;Alias S;Winter MP;Redwan B;Stübiger G;Panzenboeck A;Alimohammadi A;Bonderman D;Jakowitsch J;Bergmeister H;Bochkov V;Preissner KT;Lang IM
通讯作者：
Lang IM