Division of Cardiology, Cardiac and Vascular Center, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.
Korean Circ J. 2011 Apr;41(4):209-12. doi: 10.4070/kcj.2011.41.4.209. Epub 2011 Apr 30.
A 77-year-old man visited our hospital complaining of aggravated exertional chest pain. He was diagnosed with syndrome X 7 years ago and underwent medical treatment in a regional hospital. Coronary angiography and echocardiography did not show any significant abnormalities. On the seventh in-hospital day, cardiogenic shock developed and echocardiography showed a dilated left ventricular (LV) cavity and severe LV systolic dysfunction. We thus inserted an intra-aortic balloon pump for hemodynamic support and were forced to maintain it because of weaning failure several times. Finally, heart transplantation was the decided necessary procedure. After successful heart transplantation, the biopsy specimen revealed a wild-type transthyretin deposition indicating senile systemic amyloidosis in the intramuscular coronary vessels and interstitium. Cardiac biopsy at the 4-year follow-up showed no recurrence of amyloid deposition.
一位 77 岁男性因劳累性胸痛加重就诊于我院。7 年前被诊断为 X 综合征,曾于外院接受药物治疗。冠状动脉造影及超声心动图均未见明显异常。入院第 7 天行床旁超声心动图检查提示心源性休克,左心室腔扩大,左心室收缩功能严重受损。遂置入主动脉内球囊反搏辅助循环,但因反复撤机失败,一直保留至今。最终决定行心脏移植术。心脏移植术后,活检标本显示野生型转甲状腺素蛋白沉积,提示肌内冠状动脉和间质存在老年性系统性淀粉样变性。心脏移植术后 4 年的随访活检显示淀粉样物质无复发沉积。