CHRONIC MAJOR-VESSEL THROMBOEMBOLIC PULMONARY-HYPERTENSION
CHRONIC MAJOR-VESSEL THROMBOEMBOLIC PULMONARY-HYPERTENSION
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DOI：
10.1161/01.cir.81.6.1735
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发表时间：
1990-06-01
期刊：
影响因子：
37.8
通讯作者：
FEDULLO, PF
中科院分区：
文献类型：
--
作者：
MOSER, KM;AUGER, WR;FEDULLO, PF
纵观医学史，不寻常的患者群体常常为疾病的发病机制提供重要线索。例如，一小群患有α1-蛋白酶抑制剂缺乏症的患者的发现为了解肺气肿的发病机制提供了重要的见解。血友病患者的研究开辟了令人兴奋的新研究和临床干预途径，这些途径仍在卓有成效的探索中。慢性大血管血栓栓塞性肺动脉高压（C TE PH）有望成为一个例子，其中相对较小的、不寻常的患者群体可以提供有关血栓栓塞和肺血管疾病的大量信息。C TE PH 是由静脉血栓形成部位引起的单次或复发性肺栓塞的结果。大多数栓塞的自然过程是完全消退，或消退后留下最少的残留，同时恢复正常的肺血流动力学。 1-5 原因尚不清楚，C TE PH 患者的栓子没有完全消退；相反，它们遵循异常的组织和再通路径，留下内皮化残留物，阻塞或显着缩小主要（主动脉、肺叶动脉或节段性）肺动脉。从其他人的报告6-1" 2141和我们自己对250多名C TE PH患者的经验来看，很明显，存在广泛的残余栓塞阻塞，因此肺血管阻力增加的程度。一些患有一根或多根节段或大叶动脉慢性阻塞的患者几乎没有症状。另一个极端是那些多发中央肺动脉严重阻塞并出现严重肺动脉高压和肺动脉高压的患者。 进行性右心室衰竭。这种疾病与所谓的“原发性”肺动脉高压截然不同，在“原发性”肺动脉高压中，阻塞性病变出现在远端小肺动脉中。而在原发性肺动脉高压中，这些小动脉可能存在血栓性病变。
T hroughout medical history, unusual patient groups have often provided vital clues to the pathogenesis of disease. For example, the discovery of a small group of patients with a1-proteinase inhibitor deficiency provided critical insights into the pathogenesis of emphysema. The study of hemophiliacs opened exciting new avenues of research and clinical intervention that are still being fruitfully explored. And chronic major-vessel thromboembolic pulmonary hypertension (C TE PH) promises to be an instance in which a relatively small, unusualgroup of patients may provide much information about both thromboembolism and pulmonary vascular disease.C TE PH is the result of single or recurrent pulmonary emboli arising from sites of venous throm-bosis. The natural history ofmost emboli is to undergo total resolution, or resolution leaving minimal residua, with restoration of normal pulmonary hemodynamics. 1-5 Forreasons still unclear, the emboli in C TE PH patients do not resolve com-pletely; rather, they follow an aberrant path of organization and recanalization, leaving endothelialized residua that obstruct or significantly narrow major (main, lobar, or segmental) pulmonary arteries. From the reports of others6-1" 2141 and our own experience with more than 250 C TE PH patients, it is clear that there is a wide spectrum of residual embolic obstruction and therefore the degree to which pulmonary vascular resistance is increased. Some patients with chronic obstruction of one or more segmental or lobar arteries are nearly asymptomatic. At the other extreme are those who have major obstructions in multiple central pulmonary arteries and develop severe pulmonary hypertension and progressive right ventricular failure. This disorder is quite distinct from so-called" primary" pulmonary hypertension in which the obstruct-ing lesions appear in the small, distal pulmonary arteries. While in primary pulmonary hypertension there may be thrombotic lesions in these small arter-