Shih Jeffrey A, Shih Jennifer A
University of Massachusetts, 55 Lake Avenue North, Worcester, MA, 01655, USA,
Curr Heart Fail Rep. 2015 Jun;12(3):263-8. doi: 10.1007/s11897-015-0260-x.
Idiopathic giant cell myocarditis (IGCM) is a rare disease causing progressive myocarditis characterized by myocardial necrosis and giant cells. Patients often present with rapidly progressive heart failure, ventricular arrhythmias, and heart block. Without treatment, the disease often results in progressive pump failure requiring urgent cardiac transplantation or the need for mechanical circulatory support. The underlying pathophysiologic mechanisms are not yet defined but appear to involve genetics, autoimmune disorders, and possibly environmental factors such as viruses. Combined immunosuppressive regimens appear to prolong survival from death or cardiac transplant. Nevertheless, cardiac transplant is an effective treatment. The disease can recur in the transplanted heart resulting in death or the need for retransplant.
特发性巨细胞性心肌炎（IGCM）是一种罕见疾病，可导致以心肌坏死和巨细胞为特征的进行性心肌炎。患者常表现为快速进展的心力衰竭、室性心律失常和心脏传导阻滞。未经治疗，该疾病常导致进行性泵衰竭，需要紧急心脏移植或机械循环支持。其潜在的病理生理机制尚未明确，但似乎涉及遗传学、自身免疫性疾病以及可能的环境因素，如病毒。联合免疫抑制方案似乎可延长生存期，避免死亡或心脏移植。尽管如此，心脏移植仍是一种有效的治疗方法。该疾病可在移植心脏中复发，导致死亡或需要再次移植。
