Forsberg H, Olofsson B O, Eriksson A, Andersson S
Department of Internal Medicine, Central Hospital, Boden, Sweden.
Br Heart J. 1990 Feb;63(2):119-21. doi: 10.1136/hrt.63.2.119.
Seven young patients (mean age 19 years 8 months) with congenital myotonic dystrophy and with defined symptoms at birth were investigated by electrocardiography and echocardiography. None had cardiovascular symptoms. Electrocardiograms or echocardiograms or both were abnormal in all patients. Atrioventricular and intraventricular conduction defects were the most common electrocardiographic abnormalities and were seen in five patients. The echocardiographic examinations showed impaired left ventricular systolic function in one patient. Other echocardiographic findings were a small left ventricle and atrium, minor valve defects, and mitral valve prolapse. This study shows that the heart is often affected in young patients with congenital myotonic dystrophy. The specialised conduction system is often affected and so too is the myocardium, causing impaired systolic function.
对7例先天性肌强直性营养不良且出生时即有明确症状的年轻患者（平均年龄19岁8个月）进行了心电图和超声心动图检查。所有患者均无心血管症状。所有患者的心电图或超声心动图或两者均异常。房室和室内传导缺陷是最常见的心电图异常，5例患者出现此类情况。超声心动图检查显示1例患者左心室收缩功能受损。其他超声心动图表现为左心室和左心房较小、轻度瓣膜缺陷以及二尖瓣脱垂。本研究表明，先天性肌强直性营养不良的年轻患者心脏常受累。特殊传导系统常受影响，心肌也如此，导致收缩功能受损。
