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原发性肺动脉高压症是一种罕见的原因不明的进行性疾病。系指原发于肺动脉的增生性病变,伴有丛状病变形成,以致肺动脉广泛阻塞,肺动脉压持续增高,继而右心肥大。本病常易误诊,预后不佳。本文报道4例典型原发性肺动脉高压症尸检病例,其病理组织学所见是:肌型肺动脉中膜肥厚;细动脉肌化;肌型肺动脉内膜纤维化及弹力纤维增生,分裂呈典型“洋葱皮样”结构;以及特征性丛状病变形成等。其丛状病变形成,可能是肺动脉高压形成后的继发性晚期改变(即反应性形态改变),而不是先天性畸形或血栓栓塞的后果。
Primary pulmonary hypertension is a rare progressive disease of unknown cause. Refers to the primary pulmonary artery proliferative lesions, accompanied by the formation of plexiform lesions, resulting in extensive obstruction of the pulmonary artery, pulmonary hypertension continued to rise, then right heart hypertrophy. The disease is often misdiagnosed, the prognosis is poor. This paper reports 4 cases of autopsy cases of typical primary pulmonary hypertension, the histopathological findings are: muscular pulmonary arterial hypertrophy; arteriolar myofascial; pulmonary muscular pulmonary fibrosis and fibroepithelial fibrosis, split typical “ Onion skin-like ”structure; and the formation of characteristic plexiform lesions. The formation of its plexiform lesions may be secondary to advanced changes in pulmonary hypertension (ie, reactive morphological changes), rather than the consequences of congenital malformations or thromboembolism.