Fragola P V, Autore C, Magni G, Antonini G, Picelli A, Cannata D
Department of Internal Medicine, School of Cardiovascular Diseases, University of Rome, Italy.
Cardiology. 1991;79(2):93-8. doi: 10.1159/000174865.
In this study we noninvasively followed for a mean period of 46 months 21 patients with different grades of myotonic dystrophy to evaluate the course of the involvement of the cardiac conducting system. Six patients (28.6%), 1 affected by a mild and 5 by a severe form of the neuromuscular disorder, showed appearance or deterioration of conduction disturbances suggesting a serious derangement of the specialized tissue; in 3 of them a permanent demand pacemaker was implanted. Conduction defects are the most frequent cardiac manifestations in myotonic dystrophy and become worse with time, mainly in patients with higher degrees of the disease. Furthermore, first-degree atrioventricular block may represent an early sign of developing of more advanced conduction defects.
在本研究中，我们对21例不同程度的强直性肌营养不良患者进行了平均46个月的无创随访，以评估心脏传导系统受累的病程。6例患者（28.6%），其中1例为轻度、5例为重度神经肌肉疾病患者，出现传导障碍或传导障碍恶化，提示特殊组织严重紊乱；其中3例植入了永久性按需起搏器。传导缺陷是强直性肌营养不良最常见的心脏表现，且会随时间恶化，主要见于疾病程度较高的患者。此外，一度房室传导阻滞可能是更严重传导缺陷发展的早期迹象。
