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Cardiac Resynchronization Therapy in a Case of Myotonic Dystrophy (Steinert's Disease) and Dilated Cardiomyopathy
Author(s) -
KILIC TEOMAN,
VURAL AHMET,
URAL DILEK,
SAHIN TAYFUN,
AGACDIKEN AYSEN,
ERTAS GÖKHAN,
YILDIZ YELDA,
KOMSUOGLU BAKI
Publication year - 2007
Publication title -
pacing and clinical electrophysiology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.686
H-Index - 101
eISSN - 1540-8159
pISSN - 0147-8389
DOI - 10.1111/j.1540-8159.2007.00782.x
Subject(s) - medicine , cardiology , myotonic dystrophy , heart failure , cardiac resynchronization therapy , left bundle branch block , cardiomyopathy , dilated cardiomyopathy , myotonia , heart disease , subclinical infection , ejection fraction
Steinert's disease (Dystrophia myotonica type 1) is an autosomal dominant neuromuscular disease characterized by myotonia, muscle weakness, frontal balding, cataracts, cardiac conduction abnormalities, especially long PR interval and wide QRS complex. Although subclinical mild myocardial dysfunction may be detected in this syndrome, overt myocardial dysfunction with heart failure called as “myotonic heart disease” is not frequent. Cardiac resynchronization therapy is an effective treatment modality to improve morbidity and mortality in patients with intraventricular conduction delay and congestive heart failure. We report improvement of cardiac dyssynchrony and symptoms of heart failure with biventricular pacing in a 37‐year‐old male patient with overt myotonic heart disease, PR segment prolongation, and complete left bundle branch block.