Yamaki S, Wagenvoort C A
Am J Pathol. 1981 Oct;105(1):70-5.
Plexogenic pulmonary arteriopathy runs a more progressive course in transposition of the great arteries with pulmonary hypertension (TGA) and in primary pulmonary hypertension (PPH) than, for instance, in isolated ventricular septal defect (VSD). Fibrinoid necrosis and plexiform lesions occur more often and at a younger age in TGA and PPH than in VSD. A morphometric study in 18 patients with VSD, 14 patients with TGA, 38 patients with PPH, and 21 controls showed that the media of muscular pulmonary arteries is significantly thicker in VSD than in TGA or PPH, with the sole exception of very young children with PPH. This difference in medial thickness is apparent at any level of pulmonary arterial pressure. Conversely, the more advanced pulmonary vascular lesions were particularly prominent in TGA and PPH. It is likely that medial hypertrophy, at least to a certain extent, protects the pulmonary arteries from the development of more ominous vascular alterations.
与例如单纯室间隔缺损（VSD）相比，丛状肺动脉病在伴有肺动脉高压的大动脉转位（TGA）和原发性肺动脉高压（PPH）中进展更为迅速。与VSD相比，纤维蛋白样坏死和丛状病变在TGA和PPH中更常出现且发病年龄更小。一项对18例VSD患者、14例TGA患者、38例PPH患者及21例对照者的形态学研究显示，肌型肺动脉中层在VSD中比在TGA或PPH中显著更厚，但PPH的幼儿是唯一例外。这种中层厚度的差异在任何肺动脉压水平都很明显。相反，更严重的肺血管病变在TGA和PPH中尤为突出。中层肥厚很可能至少在一定程度上保护肺动脉免于发生更严重的血管改变。
