论文部分内容阅读
为分析马凡综合征心血管病变的临床表现及其与病理解剖形态学改变的关系,复习经临床检查并经手术或尸检证实的25例成年马凡综合征临床资料和4例尸检资料。心血管病变包括:25例主动脉瓣返流,9例并夹层动脉瘤,16例升主动脉瘤,3例二尖瓣脱垂并关闭不全;此外,1例并发急性心肌梗死。根据病理组织学检查,升主动脉中层囊性坏死和结缔组织退行性变是心血管病变的主要病理基础。25例中18例行Bentall手术,6例住院中死亡,1例保守治疗。死亡主要原因为充血性心衰和主动脉瘤破裂猝死。根据18例手术后随访结果,认为早期诊断和手术治疗是延长本病寿命的唯一方法。
To analyze the clinical manifestations of cardiovascular complications of Marfan syndrome and its relationship with pathological anatomical changes, clinical data of 25 adult Marfan syndromes confirmed by clinical examination and confirmed by surgery or autopsy and 4 autopsy data were reviewed. Cardiovascular disease included 25 aortic regurgitation, 9 aneurysm dissection, 16 ascending aortic aneurysms, 3 mitral valve prolapse and insufficiency; in addition, 1 patient had acute myocardial infarction. According to histopathological examination, ascending aortic middle layer of cystic necrosis and connective tissue degeneration is the main pathological basis of cardiovascular disease. Of the 25 patients, 18 underwent Bentall’s surgery, 6 were hospitalized and 1 were conservatively treated. The main cause of death is congestive heart failure and sudden rupture of the aortic aneurysm. According to 18 cases of postoperative follow-up results, that early diagnosis and surgical treatment is the only way to extend the life of the disease.