Bataille J, Guillon F, Urtizberea A, Estournet B, Richard S, Barois A
Service de Réanimation Infantile, Hôpital Raymond Poincaré, Garches.
Ann Med Interne (Paris). 1991;142(1):5-8.
In progressive muscular dystrophy, the heart is always affected and presents characteristic histological lesions: irregular, diffuse and intense rearrangements predominantly in the left ventricle, the septum and conductive tissue, consisting of wide, poorly vascularized fibrous bands, that are destructive but without an inflammatory aspect. The remaining myocardium is dystrophic with degeneration of the fibers (hyalin, atrophic or hypertrophic) with irregular nuclei. Plaques of adipose tissue are found under the epicardium within the heart wall. Sometimes, a fibrous thickening of the intracoronary arteries is observed without modification of the intima, but vascular lesions are not systematically seen. In congenital muscular dystrophy, cardiomyopathy certainly exists, but there is no histological description. Half of the patients suffering from myopathy with intracytoplasmic inclusions also have dystrophic and fibrotic cardiac involvement. Congenital myopathies may have their own specific cardiomyopathy, as in central core myopathy, nemaline (rod) myopathy and especially myotubular myopathy, where involvement is common. Werdnig-Hoffmann disease types I and II do not affect the heart. In contrast, several cases of fibrotic lesions have been described in KugelbergWelander disease.
在进行性肌营养不良症中，心脏总会受到影响，并呈现出特征性的组织学病变：主要在左心室、室间隔和传导组织中出现不规则、弥漫性且强烈的重排，由宽阔、血管化不良的纤维带组成，这些纤维带具有破坏性但无炎症表现。其余心肌营养不良，纤维（透明、萎缩或肥大）发生变性，细胞核不规则。在心壁的心外膜下可发现脂肪组织斑块。有时，可观察到冠状动脉内纤维性增厚，但内膜无改变，但并非总能看到血管病变。在先天性肌营养不良症中，心肌病肯定存在，但尚无组织学描述。一半患有胞浆内包涵体肌病的患者也有心肌营养不良和纤维化累及。先天性肌病可能有其自身特定的心肌病，如中央轴空病、线状体（杆状体）肌病，尤其是肌管性肌病，其累及较为常见。I型和II型韦-霍二氏病不影响心脏。相比之下，在库-韦二氏病中已描述了几例纤维化病变。
