Wagenvoort C A, Mulder P G
Department of Pathology, Erasmus University, Rotterdam, the Netherlands.
Chest. 1993 Mar;103(3):844-9. doi: 10.1378/chest.103.3.844.
In 78 patients with primary plexogenic arteriopathy (PPA), numbers of organized and recanalized thrombi were established in histologic slides of lung tissue and expressed per square centimeter of section. Three control groups of ten individuals each were used: normal, plexogenic arteriopathy secondary to ventricular septal defect, and hypoxic pulmonary hypertension. Thrombotic lesions were scarce in normal individuals but numerous in all three groups with pulmonary hypertension. There is also a positive correlation with age. Thrombotic lesions are absent or scarce in children but more common in adults, even in normal control subjects and particularly in pulmonary hypertension by whatever cause. In PPA there is likely to be a relation with the duration of illness but not with the stage of the disease. The complete pattern of plexogenic arteriopathy may develop in the absence of thrombotic lesions, which clearly are not essential for its pathogenesis. Rather than being part specifically of PPA, as sometimes suggested, thrombotic lesions complicate various types of hypertensive pulmonary vascular disease. Apparently the combination of sustained pulmonary hypertension and age, possibly through endothelial injury, may elicit thrombosis and its sequelae, which in turn may aggravate the pulmonary arterial pressure.
在78例原发性丛状动脉病（PPA）患者中，对肺组织组织学切片中机化和再通血栓的数量进行了测定，并以每平方厘米切片面积表示。使用了三个对照组，每组10人：正常组、继发于室间隔缺损的丛状动脉病组和低氧性肺动脉高压组。正常个体的血栓性病变很少，但在所有三组肺动脉高压患者中都很多。血栓性病变与年龄也呈正相关。儿童不存在或很少有血栓性病变，但在成年人中更常见，即使在正常对照者中也是如此，尤其是在任何原因引起的肺动脉高压患者中。在PPA中，血栓性病变可能与病程有关，但与疾病阶段无关。丛状动脉病的完整模式可能在没有血栓性病变的情况下发展，显然血栓性病变对其发病机制并非必不可少。血栓性病变并非如有时所认为的那样是PPA特有的一部分，而是使各种类型的高血压性肺血管疾病复杂化。显然，持续的肺动脉高压和年龄的结合，可能通过内皮损伤，引发血栓形成及其后遗症，进而可能加重肺动脉压力。
