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Prizadetost srca pri mišičnih in živčno-mišičnih obolenjih = Cardiac involvement in muscle and neuromuscular disorders
Zajc, Milita, dr. med. ; Zupan, Anton, 1954- ; Bunc, Matjaž
Neuromuscular disorders are a group of hereditary, chronic, degenerative and progressive diseases that affect museles, whether direct\y or indirect\y. The heart is a musele that responds to the ...nervous system and contains special nervelike tissue, so it isn't surprising that it is affected in many neuromuscular disorders. The principallesion is a diffuse myocardial fibrosis with minor degenerative changes in myocardial fibres. Di\ated cardiomyopathy is the kind of abnormality most often seen in Duchenne and Becker muscular dystrophy, whi\e conduction system disorders are the enes more commonly seen in myotonic and Emery-Dreifuss muscular dystrophy. Reasons for cardiac involvement in muscular dystrophies are lack of functional cytoskeleton proteins and abnormal respiratory thorax movement, which leads to restrictive pulmonary disease, hypoxemia and pulmonary hypertension, thus causing secundary cardiomyopathy. Patients as well as asympthomatic carriers need to have electrocardiography, echocardiography, magnetic resonance imaging and electrophysiology investigations done at time of diagnosis and regularly afterwards to assess cardiac function. Cardiomyopathy shuld be treated with inhibitors of angiotensin-converting enzyme and beta-adrenoreceptor blocking drugs, which promote favourable remodelling of heart musele. In case of endangering cardiac arrhythmia pacemaker or cardioverter-defibril\ator shuld be implanted.
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