Price W H, Wilson J
J Med Genet. 1983 Feb;20(1):61-3. doi: 10.1136/jmg.20.1.61.
Three deaths from dissection of the aorta in a series of 157 adult women with Turner's syndrome are reported. These are greatly in excess of the numbers expected. None of the three patients had a coarctation of the aorta. One had aortic regurgitation but there was no reason to believe that the aorta in the other two patients had been subjected to unusual haemodynamic stresses. Cystic medial necrosis of the aorta was described in two patients on whom necropsies were carried out. It is concluded that there is probably a greatly increased risk of dissection of the aorta in Turner's syndrome even in the absence of any other abnormality of the aorta and aortic valve. Previously reported cases of aortic dissection in Turner's syndrome are discussed.
据报道，在157例成年特纳综合征女性患者中，有3例死于主动脉夹层。这些死亡人数大大超过预期。这3例患者均无主动脉缩窄。1例有主动脉反流，但没有理由认为另外2例患者的主动脉承受了异常的血流动力学压力。对2例进行尸检的患者描述了主动脉中层囊性坏死。结论是，即使在没有主动脉和主动脉瓣任何其他异常的情况下，特纳综合征患者发生主动脉夹层的风险可能也会大大增加。文中还讨论了先前报道的特纳综合征主动脉夹层病例。
