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[活动性心肌炎的临床多形性表现及自然病程:60例经验]

[Clinical polymorphic presentation and natural history of active myocarditis: experience in 60 cases].

作者信息

Sinagra G, Maras P, D'Ambrosio A, Gregori D, Bussani R, Silvestri F, Morgera T, Pinamonti B, Salvi A, Alberti E, Di Lenarda A, Lardieri G, Klugmann S, Camerini F

机构信息

Divisione di Cardiologia, Ospedale Maggiore, Trieste.

出版信息

G Ital Cardiol. 1997 Aug;27(8):758-74.

PMID:9312504
Abstract

Eight-hundred thirty patients (pts) with suspected myocardial disease of undefined etiology were observed from 1978 to 1996. In 350 pts, the clinical diagnosis was of dilated cardiomyopathy (DCM) or myocarditis. An endomyocardial biopsy was performed on all patients and in 54 of them (15%), an active myocarditis was identified. In six cases, myocarditis was detected at autopsy. There were 37 male patients and 23 females, with an average age of 35.5 +/- 15 years (range 1.67). Mean time interval between clinical onset and diagnosis was 4 +/- 10 months. Clinical presentation was characterized in 4 cases by fulminant myocarditis (Group I), in 8 cases by chest pain (Group II), in 14 cases by arrhythmia (Group III: hypokinetic in 9 pts and hyperkinetic in 5) and, in the last 34 pts, by congestive heart failure (CHF) (Group IV). Improvement was defined at 9 +/- 3 months according to a clinical score based on left ventricular shortening fraction (increase > or = 5 units), New York Heart Association Class improvement by (at least one Class) and left ventricular end-diastolic diameter (decrease > or = 10%). The main clinical and instrumental parameters characterizing the groups were: a more severe dilatation and left ventricular dysfunction in the pts belonging to Group I or IV with respect to those in Group II and III; a significantly worse prognosis in terms of evolution in DCM or death/cardiac transplantation (CT) in the pts from the Group II and III. After a follow-up period of 48 +/- 46 months, the mortality in the four groups was: 100% (4/4), 0% (0/8), 21% (3/14), 38% (13/34). Fifty percent of deaths were concentrated in the first 2 years of follow-up. Left ventricular end-diastolic diameter (OR 1.09, p < 0.05), age (OR 0.95), presence of left ventricular bundle branch block (OR 2.32), right ventricular function (OR 2.43) at clinical onset and the status of improvement at 9 +/- 3 months of follow-up (OR 0.24, p < 0.05) are predictors of evolution in DCM or death/CT for the pts with onset from CHF (Group IV). Immunosuppressive treatment has been utilized for the 76% of the pts. No conclusion can be drawn on the efficacy of this therapy, but no adverse events significantly related to therapy have been observed in a 9 +/- 3 months follow-up period. In conclusion, myocarditis can show a clinical presentation polymorphism, which influences the prognosis and natural history of the disease. Evolution in DCM and adverse events (death/CT) are more common in Groups I and IV. Some simple parameters evaluated at clinical presentation and the proposed classification as "improved" or "not improved" after a short-term follow-up (9 +/- 3 months) show good predictive accuracy. The present study does not allow us to draw any conclusion about the efficacy of immunosuppressive treatment. A randomized, controlled, large-scale trial, with adequate follow-up and advanced histological diagnosis techniques will help define the role of immunosuppressive therapy and patient eligibility criteria for this treatment.

摘要

1978年至1996年期间,对830例病因不明的疑似心肌病患者进行了观察。在350例患者中,临床诊断为扩张型心肌病(DCM)或心肌炎。对所有患者均进行了心内膜心肌活检,其中54例(15%)被确诊为活动性心肌炎。有6例心肌炎是在尸检时发现的。患者中有37例男性和23例女性,平均年龄为35.5±15岁(范围为1.67岁)。临床发病与诊断之间的平均时间间隔为4±10个月。临床表现方面,4例为暴发性心肌炎(I组),8例为胸痛(II组),14例为心律失常(III组:9例为运动减弱型,5例为运动增强型),最后34例为充血性心力衰竭(CHF)(IV组)。根据基于左心室缩短分数(增加≥5个单位)、纽约心脏协会心功能分级改善(至少提高一级)和左心室舒张末期内径(减小≥10%)的临床评分,在9±3个月时定义为病情改善。各分组的主要临床和检查参数如下:与II组和III组患者相比,I组或IV组患者的左心室扩张和功能障碍更为严重;II组和III组患者发展为DCM或死亡/心脏移植(CT)的预后明显更差。经过48±46个月的随访,四组患者的死亡率分别为:100%(4/4)、0%(0/8)、21%(3/14)、38%(13/34)。50%的死亡病例集中在随访的前两年。临床发病时的左心室舒张末期内径(比值比1.09,p<0.05)、年龄(比值比0.95)、左束支传导阻滞的存在(比值比2.32)、右心室功能(比值比2.43)以及随访9±3个月时的改善状况(比值比0.24,p<0.05)是CHF发病患者(IV组)发展为DCM或死亡/CT的预测因素。76%的患者接受了免疫抑制治疗。关于该疗法的疗效无法得出结论,但在9±3个月的随访期内未观察到与治疗显著相关的不良事件。总之,心肌炎可呈现临床表型多态性,这会影响疾病的预后和自然病程。I组和IV组中DCM的进展和不良事件(死亡/CT)更为常见。在临床表现时评估的一些简单参数以及短期随访(9±3个月)后提出的“改善”或“未改善”分类显示出良好的预测准确性。本研究无法让我们得出关于免疫抑制治疗疗效的任何结论。一项随机、对照、大规模试验,具备充分的随访和先进的组织学诊断技术,将有助于明确免疫抑制治疗的作用以及该治疗的患者入选标准。

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