Avtor/Urednik		Šabovič, M; Medica, I; Logar, N; Mandić, E; Zidar, J; Peterlin, B
Naslov		Relation of CTG expansion and clinical variables to electrocardiogram conduction abnormalities and sudden death in patients with myotonic dystrophy
Tip		članek
Vir		Neuromuscul Disord
Vol. in št.		Letnik 13, št. 10
Leto izdaje		2003
Obseg		str. 822-6
Jezik		eng
Abstrakt		We prospectively followed 63 patients with myotonic dystrophy (DM) after establishing diagnosis of DM for an average 8 years in an attempt to detect conduction disturbances (by electrocardiography and/or Holter monitoring) and sudden cardiac events (sudden death, cardiac syncope) and correlate them to potential predicting factors (CTG repeat expansion in the myotonin protein kinase gene and several clinical variables: clinical type and duration of DM, age and sex). Twenty-six patients developed conduction disturbances, five patients died suddenly, and two patients experienced cardiac syncope necessitating urgent implantation of pacemaker. Analysis showed no significant correlation between conduction disturbances and/or cardiac events and CTG expansion. Furthermore, no correlation was found with type of DM, whereas conduction disturbances and sudden cardiac events correlated with patients' age, duration of disease and male sex. Results on our cohort of DM patients show that CTG expansion has no role in predicting neither conduction abnormalities nor sudden death. It seems that risk of sudden death increases with duration of disease and age, and that risk is higher in male patients.
Deskriptorji		MYOTONIA ATROPHICA DEATH, SUDDEN, CARDIAC ARRHYTHMIA SYNCOPE HEART BLOCK ELECTROCARDIOGRAPHY, AMBULATORY ECHOCARDIOGRAPHY DNA MUTATIONAL ANALYSIS PROSPECTIVE STUDIES