CARDIOMYOPATHY OF PROGRESSIVE MUSCULAR DYSTROPHY
CARDIOMYOPATHY OF PROGRESSIVE MUSCULAR DYSTROPHY
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DOI：
10.1161/01.cir.33.4.625
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发表时间：
1966-01-01
期刊：
影响因子：
37.8
通讯作者：
ODOHERTY, D
中科院分区：
文献类型：
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作者：
PERLOFF, JK;DELEON, AC;ODOHERTY, D
我们进行了一项前瞻性研究，目的是检查 55 名仅根据一种主要类型的进行性肌营养不良症患者选择的患者的病史、体征、心音图、心电图、心向量图、X 射线、血清酶、肺通气检查结果和血流动力学（表 9）。分类包括：（1）经典杜氏型，（2）肢带型，（3）面肩肱型，（4）一组临床上无法区分良性杜氏营养不良和假性肥大的肢带营养不良的患者。在选择之前没有患者认识到患有心脏病。随访期从几个月到五年不等。广泛回顾了相关文献。在所有组中，心脏病的临床证据都必须在躯体肌肉骨骼缺陷对“心脏”症状、心血管体征和心脏放射学外观的影响的背景下进行解释。另一方面，在进行性肌营养不良症的每个主要类别中都发现了内在心脏病的令人信服的证据（从微妙到明显）。在经典 Duchenne 组中，心肌病的检测是最简单和最频繁的，因为标量心电图的高度特征性特征可能会在临床识别之前引起人们对全身性肌病的关注。然而，除了典型的杜氏营养不良之外，其他形式的营养不良患者中心脏病的存在值得特别强调。后一种类型的心肌病的证据各不相同，从肢带和面肩肱组的微妙四律奔马节律到良性杜兴氏和肢带假性肥大组的心肌病死亡。目前关于肌营养不良症心脏的信息通常依赖于回顾性研究，以及对已发表报告的回顾。 根据对个体心血管特征的讨论或对个体营养不良类型的观察，对不完全分类的病例进行分类。本前瞻性研究是为了消除其中一些缺点而进行的。对大量且仔细分类的营养不良受试者进行的综合评估提供了有关营养不良性心肌病临床谱的更多信息。
A prospective study was undertaken in order to examine the histories, physical signs, phonocardiograms, electrocardiograms, vectorcardiograms, X-rays, serum enzymes, pulmonary ventilatory findings, and hemodynamics in a group of 55 patients selected solely on the basis of one of the major types of progressive muscular dystrophy (table 9). The classification included: (1) the classic Duchenne type, (2) the limb girdle type, (3) the facioscapulohumeral type, and (4) a group of patients in whom clinical distinction between benign Duchenne dystrophy and limb girdle dystrophy with pseudohypertrophy could not be made. No patient had recognized heart disease prior to selection. Follow-up periods ranged from a few months to 5 years. The pertinent literature was extensively reviewed.In all groups clinical evidence of heart disease had to be interpreted in the context of the influence of the somatic musculoskeletal defects on "cardiac" symptoms, cardiovascular physical signs, and radiological appearance of the heart. On the other hand, convincing evidence of intrinsic heart disease-subtle to overt-was found in each of the major categories of progressive muscular dystrophy. Detection of cardiomyopathy was simplest and most frequent in the classic Duchenne group because of the highly characteristic features of the scalar electrocardiogram which might direct attention to the systemic myopathy prior to its clinical recognition. However, the presence of heart disease in patients with forms of dystrophy other than the classic Duchenne deserves particular emphasis. Evidence of cardiomyopathy in these latter forms varied from subtle quadruple gallop rhythms in the limb girdle and facioscapulohumeral groups to cardiomyopathic death in the benign Duchenne and limb girdle-pseudohypertrophic group.Currently available information on the heart in muscular dystrophy has as a rule depended upon retrospective studies, upon reviews of published reports of incompletely classified cases, upon discussions of individual cardiovascular features, or upon observations of individual types of dystrophy. The present prospective study was undertaken to obviate some of these shortcomings. Comprehensive assessment of a large and carefully classified group of dystrophic subjects has provided additional information regarding the clinical spectrum of dystrophic cardiomyopathy.