Noninvasive assessment of left ventricular function in myotonic muscular dystrophy.
Noninvasive assessment of left ventricular function in myotonic muscular dystrophy.
复制标题
强直性肌营养不良症左心室功能的无创评估。
作者：
A. Venco;M. Saviotti;D. Besana;G. Finardi;G. Lanzi
为了评估左心室功能，通过超声心动图对 7 名强直性肌营养不良患者及其其中一名临床正常但肌肉活检异常的 3 个孩子进行了左心室内部尺寸及其变化率的测量。还记录了所有人的心电图和收缩时间间隔。只有一名患者有明显的心脏病迹象和心电图异常（B 型 WPW）。所有 7 名患者的收缩时间间隔均正常。五名受试者有超声心动图异常，除了患有明显心脏病的患者外，超声心动图异常程度较轻，该患者左心室收缩功能和舒张功能均严重受损。另一名患者的收缩和舒张功能均异常；一名患者仅出现收缩异常，一名亲属仅出现舒张异常。结论是，强直性肌营养不良且无心脏病临床症状的患者可能有超声心动图显示的轻微左心室功能异常。超声心动图在检测这些异常方面比收缩时间间隔更敏感；收缩功能和舒张功能异常都可能单独或同时发生。骨骼肌和心肌的受累之间似乎没有关系。
In order to assess left ventricular function, measurements of left ventricular internal dimension and its rate of change have been made by echocardiography in 7 patients with myotonic dystrophy and the three children of one of them, who were clinically normal but had abnormal muscle biopsies. Electrocardiograms and systolic time intervals were also recorded in all. Only one patient had signs of overt heart disease and an abnormal electrocardiogram (type B WPW). Systolic time intervals were normal in all 7 patients. Five subjects had echocardiographic abnormalities, which were of minor degree except in the patient with overt heart disease who had considerable impairment of both systolic and diastolic left ventricular function. Another patient had abnormalities of both systolic and diastolic function; systolic abnormalities occurred alone in one patient and diastolic abnormalities alone in one relative. It is concluded that patients with myotonic dystrophy and no clinical signs of heart disease may have minor abnormalities of left ventricular function as shown by echocardiography. Echocardiography is more sensitive than systolic time intervals in detecting these abnormalities; both systolic and diastolic function abnormalities, alone or together, can occur. There seems to be no relation between involvement of skeletal and cardiac muscle.