Ogawa Satoshi, Matsumura Shin-ichiro, Yoshikawa Tsutomu, Satoh Toru, Kumagai Hiroo, Mitamura Hideo, Iwanaga Shiro, Umezawa Akihiro
Departments of Internal Medicine, The Suntory Fund for Advanced Cardiac Therapeutics, School of Medicine, Keio University, Tokyo, Japan.
Keio J Med. 2002 Sep;51(3):165-77.
A 55-year-old Asian man first visited to our hospital with complaining of exertional dyspnea eight years ago, and was diagnosed as having idiopathic dilated cardiomyopathy. One of his siblings also suffered from idiopathic dilated cardiomyopathy. His symptoms became worse gradually, and he was hospitalized again because of disturbance of consciousness on February 21, 2001. Hemodynamic monitoring with a Swan-Ganz catheter was started, which revealed that the cardiac index was 1.1 L/ min/BSA, cardiac output 1.8 L/min, and pulmonary artery pressure 43/33 mmHg. The echocardiographic observation showed that the left ventricular ejection fraction was 32%, and serum BNP was elevated to 5,411 pg/mL. Multi-organ failure including renal and hepatic dysfunction developed because of the low cardiac output status. Continuous hemodiafiltration (CHDF) was introduced to reduce the volume overload, improve renal failure, and eliminate adverse cytokines. Although his hemodynamic status was temporarily improved after starting CHDF, weaning from CHDF was difficult and he finally died from cardiogenic shock after two month of intensive therapy. The autopsy showed thinning of the left ventricular wall, and histological examination revealed diffuse fibrous hyperplasia and myocardial fiber deficit in the ventricular myocardium. CHDF was effective in reducing the volume overload and improving renal function; however, heart transplantation is inevitable for the patients with severe heart failure due to dilated cardiomyopathy.
一名55岁的亚洲男性8年前因劳力性呼吸困难首次就诊于我院，被诊断为特发性扩张型心肌病。他的一名兄弟姐妹也患有特发性扩张型心肌病。他的症状逐渐加重，2001年2月21日因意识障碍再次住院。开始使用Swan-Ganz导管进行血流动力学监测，结果显示心脏指数为1.1L/min/BSA，心输出量为1.8L/min，肺动脉压为43/33mmHg。超声心动图观察显示左心室射血分数为32%，血清脑钠肽升高至5411pg/mL。由于心输出量低，出现了包括肾功能和肝功能障碍在内的多器官功能衰竭。采用持续血液透析滤过（CHDF）来减轻容量负荷，改善肾衰竭，并清除不良细胞因子。虽然开始CHDF后他的血流动力学状态暂时得到改善，但撤机困难，经过两个月的强化治疗后最终死于心源性休克。尸检显示左心室壁变薄，组织学检查显示心室心肌弥漫性纤维增生和心肌纤维缺失。CHDF在减轻容量负荷和改善肾功能方面是有效的；然而，对于因扩张型心肌病导致严重心力衰竭的患者，心脏移植是不可避免的。
