Cardiac Resynchronization Therapy in a Case of Myotonic Dystrophy (Steinert's Disease) and Dilated Cardiomyopathy

Abstract

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.

Heart Disease in Disorders of Muscle, Neuromuscular Transmission, and the Nerves

“… 121) In both types of myotonic dystrophy, the myocardium may be affected in the form of dCMP or LVHT. 122) 123) 124) Myocardial involvement may also progress to myocardial fibrosis, thereby manifesting as LGE on cMRI. 125) In a study of 129 MD1 patients, CI was found in 55%.…”

2022 HRS expert consensus statement on evaluation and management of arrhythmic risk in neuromuscular disorders

“…To date, very few studies have reported cardiac resynchronization in DMD and BMD 83,90,217,218 and DM1. [219][220][221] There are no data regarding His bundle or left bundle pacing in patients with NMDs, which are both promising strategies to minimize perioperative risks while potentially preserving the benefits of CRT. Early resynchronization in patients with LBBB or wide QRS complex inferring His-Purkinje disease may eliminate the detrimental effects of desynchronized ventricular conduction and further deterioration of LVEF.…”

An overview of heart rhythm disorders and management in myotonic dystrophy type 1

“…Nonetheless, the decision on cardiac synchronization needs to take into account the presence of comorbidities and their effect on patient prognosis, particularly with regards on pacing only versus defibrillator implantation [43]. Isolated case reports have demonstrated benefits from cardiac resynchronization suggesting this could be a viable option in DM1 patients fulfilling criteria on an individual basis [65][66][67].…”