Journal of Rare Cardiovascular Diseases

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

Sovari AA, Bodine CK, Farokhi F. Cardiovascular manifestations of myotonic dystrophy‑1. Cardiol Rev 2007;15:191–194

Elliott P. Myotonic dystrophy: time for evidence‑based therapy. Eur Heart J 2014; 35: 2135–2137.

Nakamori M, Sobczak K, Puwanant A, et al. Splicing biomarkers of disease severity in myotonic dystrophy. Ann Neurol 2013;74: 862–872.

Lund M, Diaz LJ, Ranthe MF, et al. Cardiac involvement in myotonic dystrophy: a nationwide cohort study. Eur Heart J 2014; 35: 2158–2164.

Priori SG, Blomström‑Lundquist C, Mazzanti A, et al. 2015 ESC guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death. Eur Heart J 2015; 36: 2793–2867.

Brembilla‑Perrot B, Schwartz J, Huttin O, et al. Atrial flutter or fibrillation is the most frequent and life‑threatening arrhythmia in myotonic dystrophy. Pacing Clin Electrophysiol 2014; 37: 329–335.

Tamura K, Tsuji H, Masui A, et al. Sustained ventricular tachycardia associated with myotonic dystrophy. Clin Cardiol 1996;19: 674–677.

Rakocevic‑Stojanovic V, Pavlovic S, Seferovic P, et al. Pathohistological changes in endomyocardial biopsy specimens in patients with myotonic dystrophy. Panminerva Med 1999; 41: 27–30.

Bhakta D, Shen C, Kron J, et al. Pacemaker and implantable cardioverter‑defibrillator use in a US myotonic dystrophy type 1 population. J Cardiovasc Electrophysiol 2011; 22: 1369–1375.

Benhayon D, Lugo R, Patel R, et al. Long‑term arrhythmia follow‑up of patients with myotonic dystrophy. J Cardiovasc Electrophysiol 2015; 26: 305–310.

Cakmak N, Osmonov D, Ozcan KS, et al. Ventricular tachycardia: first manifestation of myotonic dystrophy. Acta Cardiol 2011; 66: 267–269.

Takeda K, Takemoto M, Mukai Y, et al. Bundle branch reentry ventricular tachycardia in a patient with myotonic dystrophy. J Cardiol 2009; 53: 463–466.

Merino JL, Carmona JR, Fernandez‑Lozano I, et al. Mechanisms of sustained ventricular tachycardia in myotonic dystrophy: implications for catheter ablation. Circulation 1998; 98: 541–546.

Shiraishi H, Shirayama T, Inoue K, et al. Successful catheter ablation against ventricular tachycardia associated with myotonic dystrophy. Intern Med 2000; 39: 39–44.

Blomström‑Lundqvist C, Scheinmann MM, Aliot EM, et al. ACC/AHA/ESC guidelines for the management of patients with supraventricular arrhythmias‑executive summary. A report of the American College of Cardiology / American Association Task Force on practice guidelines and the European Society of Cardiology committee for practice guidelines (writing committee to develop guidelines for the management of patients with supraventricular arrhythmias) developed in collaboration with NASPE‑Heart Rhythm Society. J Am Coll Cardiol 2003;42:1493–1531.

Brugada P, Brugada J, Mont L, et al. A new approach to the differential diagnosis of a regular tachycardia with a wide QRS complex. Circulation 1991; 83: 1649–1659.

Jastrzębski M, Kukla P, Czarnecka D, et al. Comparison of five electrocardiographic methods for differentiation of wide QRS‑complex tachycardias. Europace 2012; 14: 1165–1171.