Hutchins G M, Ostrow P T
Am Heart J. 1976 Dec;92(6):797-803. doi: 10.1016/s0002-8703(76)80019-1.
Autopsy observations suggested that lesions of hypertensive pulmonary vascular disease (HPVD) due to elevated venous pressure differ from those with arterial hypertension only. Clinical and pathologic features were reviewed in patients from the Hopkins autopsy files with proved pulmonary hypertension; 50 had venous HPVD due to left-sided congestive heart failure, 50 had arterial HPVD due to congenital malformations, and 15 had idiopathic pulmonary hypertension (IPH). The two forms of HPVD have consistent distinctive histologic changes. In venous HPVD intimal fibroelastosis (IFE) develops in veins and arteries with retention of normal lumen diameters. Intensity of IFE correlates with severity and duration of venous hypertension. Arterial HPVD has IFE in conducting arteries, but the characteristic lesion is cellular intimal proliferation in regulatory arterioles, producing progresssive irreversible lumenal narrowing. Glomoid and angiomatoid lesions appear with prolonged severe arterial hypertension. They do not occur in venous HPVD. Hypertensive arteritis may develop with either form of HPVD. IPH has arterial-type HPVD. IFE of venous HPVD appears to be a response to increased mural tension. Arteriolar intimal cellular proliferations seen in arterial HPVD may be produced by blood flow boundary layer separations. IPH may be explainable as protracted inappropriate pulmonary arteriolar constriction.
尸检观察表明，由静脉压升高引起的高血压性肺血管疾病（HPVD）的病变与仅由动脉高血压引起的病变不同。对霍普金斯尸检档案中已证实患有肺动脉高压的患者的临床和病理特征进行了回顾；50例因左心充血性心力衰竭导致静脉性HPVD，50例因先天性畸形导致动脉性HPVD，15例患有特发性肺动脉高压（IPH）。两种形式的HPVD具有一致的独特组织学变化。在静脉性HPVD中，内膜纤维弹性组织增生（IFE）发生在静脉和动脉中，管腔直径保持正常。IFE的强度与静脉高压的严重程度和持续时间相关。动脉性HPVD在传导动脉中有IFE，但特征性病变是调节性小动脉中的细胞内膜增生，导致进行性不可逆的管腔狭窄。严重的动脉高血压持续时间延长会出现肾小球样和血管瘤样病变。它们不会出现在静脉性HPVD中。两种形式的HPVD都可能发生高血压性动脉炎。IPH具有动脉型HPVD。静脉性HPVD的IFE似乎是对壁张力增加的一种反应。动脉性HPVD中所见的小动脉内膜细胞增生可能是由血流边界层分离引起的。IPH可以解释为持续性不适当的肺小动脉收缩。
