Walley V M, Virmani R, Silver M D
University of Ottawa Heart Institute, Ottawa Civic Hospital, Canada.
Pathology. 1990 Jan;22(1):1-4. doi: 10.3109/00313029009061416.
Four unusual cases of sudden death due to pulmonary arterial hypertension complicated by dissection and/or rupture of the main pulmonary artery are reported. The patients, 3 males and 1 female, ranged from 17 to 77 years old. Each had chronic pulmonary arterial hypertension, marked pulmonary arterial dilation and degenerative medial changes of the large elastic pulmonary arteries. The first patient had a partial thickness tear of the main pulmonary artery with local dissection without external rupture and died of shock. The other three patients died after external rupture of the main pulmonary artery, based on a full thickness tear in one case, a small dissection in another and extensive dissection in the third. In the setting of pulmonary arterial hypertension, dissection, rupture, or dissection and rupture of the pulmonary artery should be considered in the differential diagnosis when patients present in cardiogenic shock or with sudden death.
报告了4例因肺动脉高压并发主肺动脉夹层和/或破裂导致猝死的罕见病例。患者为3名男性和1名女性，年龄在17至77岁之间。每位患者均患有慢性肺动脉高压、明显的肺动脉扩张以及大弹性肺动脉的中层退行性改变。首例患者主肺动脉有部分厚度撕裂并伴有局部夹层，无外部破裂，死于休克。另外3例患者主肺动脉外部破裂后死亡，其中1例为全层撕裂，1例为小夹层，第3例为广泛夹层。在肺动脉高压的情况下，当患者出现心源性休克或猝死时，在鉴别诊断中应考虑肺动脉夹层、破裂或夹层并破裂。
