Cooper L T, Berry G J, Rizeq M, Schroeder J S
Department of Medicine, University of California, San Diego 92103-8411, USA.
J Heart Lung Transplant. 1995 Mar-Apr;14(2):394-401.
Giant cell myocarditis is a rare and frequently fatal disorder of unknown origin that is defined histopathologically as diffuse myocardial necrosis with multinucleated giant cells in the absence of sarcoidlike granulomata. The clinical and pathologic features of lymphocytic myocarditis have been described in several recent publications, but the features of idiopathic giant cell myocarditis have not been adequately addressed.
We describe five patients with idiopathic giant cell myocarditis who were seen at Stanford University over the past 10 years. In each case the onset was subacute congestive heart failure. After diagnosis each patient received immunosuppressive therapy and was evaluated for heart transplantation. Progressive heart failure and ventricular arrhythmias developed in all. Three died rapidly, two of progressive heart failure and one of sudden cause. Two patients underwent orthotopic heart transplantation and are currently alive, one with disease recurrence. Pathologic studies, including endomyocardial biopsy and evaluation of postmortem or explanted material at transplantation were reviewed. The pathologic studies provided additional support that the giant cells derive from a monocytic/histiocytic lineage. Segmental wall motion abnormalities suggest giant cell myocarditis can be a focal, as well as diffuse process at certain stages of its course. This experience is compared with published cases and implications for diagnosis and treatment are discussed.
In view of the uniformly fatal nature of the disease, heart transplantation should be a serious consideration, and the patients evaluated once the diagnosis is established. Triple-drug immunosuppressive therapy should be considered at the time of diagnosis.
巨细胞性心肌炎是一种罕见且常致命的病因不明的疾病，其组织病理学定义为弥漫性心肌坏死伴多核巨细胞，且无结节病样肉芽肿。近期有几篇文献描述了淋巴细胞性心肌炎的临床和病理特征，但特发性巨细胞性心肌炎的特征尚未得到充分探讨。
我们描述了过去10年在斯坦福大学就诊的5例特发性巨细胞性心肌炎患者。每例患者均以亚急性充血性心力衰竭起病。诊断后，每位患者均接受免疫抑制治疗并接受心脏移植评估。所有患者均出现进行性心力衰竭和室性心律失常。3例患者迅速死亡，2例死于进行性心力衰竭，1例死因不明。2例患者接受了原位心脏移植，目前仍存活，其中1例疾病复发。回顾了病理研究，包括心内膜心肌活检以及对移植时的尸检或切除材料的评估。病理研究进一步支持巨细胞来源于单核细胞/组织细胞谱系。节段性室壁运动异常提示巨细胞性心肌炎在病程的某些阶段可以是局灶性的，也可以是弥漫性的。将该经验与已发表的病例进行比较，并讨论其对诊断和治疗的意义。
鉴于该疾病的一致致命性，一旦确诊应认真考虑心脏移植，并对患者进行评估。诊断时应考虑三联免疫抑制治疗。
