Cooper L T, Berry G J, Shabetai R
Department of Medicine, University of California at San Diego Medical Center, USA.
N Engl J Med. 1997 Jun 26;336(26):1860-6. doi: 10.1056/NEJM199706263362603.
Idiopathic giant-cell myocarditis is a rare and frequently fatal disorder. We used a multicenter data base to define the natural history of giant-cell myocarditis and the effect of treatment.
We identified 63 patients with idiopathic giant-cell myocarditis through journal announcements and direct mailings to cardiovascular centers worldwide.
The patients consisted of 33 men and 30 women with an average age of 42.6 years; 88 percent were white, 5 percent were black, 5 percent were Southeast Asian or Indian, and 2 percent were Middle Eastern. Most presented with congestive heart failure (47 patients, or 75 percent), ventricular arrhythmia (9 patients, or 14 percent), or heart block (3 patients, or 5 percent), although in some cases the initial symptoms resembled those of acute myocardial infarction (4 patients). Nineteen percent had associated autoimmune disorders. The rate of survival was worse than among 111 patients with lymphocytic myocarditis in the Myocarditis Treatment Trial (P<0.001); among our patients, the rate of death or cardiac transplantation was 89 percent, and median survival was only 5.5 months from the onset of symptoms. The 22 patients treated with corticosteroids and cyclosporine, azathioprine, or both therapies survived for an average of 12.3 months, as compared with an average of 3.0 months for the 30 patients who received no immunosuppressive therapy (P=0.001). Of the 34 patients who underwent heart transplantation, 9 (26 percent) had a giant-cell infiltrate in the transplanted heart and 1 died of recurrent giant-cell myocarditis.
Giant-cell myocarditis is a disease of relatively young, predominantly healthy adults. Patients usually die of heart failure and ventricular arrhythmia unless cardiac transplantation is performed. Despite the possibility of fatal disease recurrence, transplantation is the treatment of choice for most patients.
特发性巨细胞性心肌炎是一种罕见且常致命的疾病。我们使用一个多中心数据库来确定巨细胞性心肌炎的自然病程及治疗效果。
我们通过期刊公告及直接邮寄给全球心血管中心的方式，确定了63例特发性巨细胞性心肌炎患者。
患者包括33名男性和30名女性，平均年龄42.6岁；88%为白人，5%为黑人，5%为东南亚人或印度人，2%为中东人。多数患者表现为充血性心力衰竭（47例，占75%）、室性心律失常（9例，占14%）或心脏传导阻滞（3例，占5%），尽管在某些病例中初始症状类似急性心肌梗死（4例）。19%的患者伴有自身免疫性疾病。生存率低于心肌炎治疗试验中111例淋巴细胞性心肌炎患者（P<0.001）；在我们的患者中，死亡或心脏移植率为89%，从症状出现起的中位生存期仅为5.5个月。接受皮质类固醇和环孢素、硫唑嘌呤或两种疗法治疗的22例患者平均存活12.3个月，而未接受免疫抑制治疗的30例患者平均存活3.0个月（P=0.001）。在接受心脏移植的34例患者中，9例（26%）移植心脏中有巨细胞浸润，1例死于复发性巨细胞性心肌炎。
巨细胞性心肌炎是一种主要发生在相对年轻、基本健康成年人中的疾病。患者通常死于心力衰竭和室性心律失常，除非进行心脏移植。尽管存在致命疾病复发的可能性，但移植仍是大多数患者的首选治疗方法。
