Myotonic muscular dystrophy is a diffuse systemic disorder, which is inherited as an autosomal dominant trait, with appearance in both sexes, generally late onset of symptoms, and slowly progressive evolution. The clinical picture may include involvement of skeletal and cardiac muscle, cataracts, premature frontal baldness, bone changes, testicular atrophy, other mild endocrine abnormalities, and mental deterioration.Evidence of cardiac disease in patients with myotonic dystrophy is usually found in the electrocardiogram, the most common alterations being P and ST and T wave abnormalities, first degree atrioventricular block, left axis deviation, atrial flutter and fibrillation, bundle-branch block, and premature atrial and ventricular contractions
Left ventricular function was studied noninvasively in a group of 35 patients with noncomplicated and complicated old myocardial infarction (MI) by means of kinetocar-diography (KCG) and systolic time intervals (STI). Discriminant analysis with a group of 130 normal subjects documented that STI were significantly abnormal and that the abnormalities, i.e. prolongation of the preejection period, shortening of left ventricular ejection time and increase of PEP/LVET were more evident in patients with complicated old MI. KCG was more or less abnormal in all the patients studied, with the more striking abnormalities in those with complications. A good correlation was found between KCG and STI abnormalities. We consider that KCG is able to detect in a sensitive way abnormalities of left ventricular contraction in patients with old MI and that, most probably, alterations of STI in these patients are mainly due to incoordinate left ventricular contraction.
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