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Patient
Young man, 27 years old, with myotonic dystrophy (Steinert disease);
Trace
Sinus rhythm with increased PR-interval (first degree AV-block), complete left bundle branch block (QRS-width > 120 ms, qS and rS morphology in V1-V2, positive complexes in V6 with delayed intrinsicoïd deflection, repolarisation abnormalities;
Exergue
In Steinert disease, the most frequently observed ECG abnormality is the existence of conduction abnormalities both located above and below the His-bundle.
Myotonic dystrophy, also known as Steinert disease (autosomal dominant inheritance) is the most common muscle dystrophy in the adults and is characterized by the existence of muscular weakness and progression into various organs with possible involvement of the occular, respiratory, endocrinology