Broad QRS complex tachycardia in a patient with myotonic dystrophy (Steinert disease) (RCD code: V‑4O)

Jacek Majewski, Jacek Lelakowski

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Myotonic dystrophy is a rare inherited condition affecting primarily skeletal muscles and commonly associated with cardiovascular pathology. We report a case of a 50- year old female with myotonic dystrophy type 1 who presented with wide‑QRS complex tachycardia and hemodynamic compromise which was treated with direct current cardioversion. Echocardiogram did not reveal any structural heart disease and coronary angiogram was normal. Electrocardiogram in sinus rhythm showed non‑specific intraventricular conduction delay and borderline PR interval. She refused cardioverter‑defibrillator (ICD) implantation and was discharged on oral amiodarone. The case report shows that life‑threatening arrhythmia could be the first cardiac manifestation in patients with myotonic dystrophy. JRCD 2017; 3 (3): 89–91.


rare disease; ventricular tachycardia; myotonic dystrophy; implantable cardioverter‑defibrillator; cardioversion


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