Abstract

Soukaina Kadiri*, Ryme Khalloq, Najlaa Salmi, Ibtissam Fellat and Mohamed Cherti

ABSTRACT

Introduction: The most prevalent muscular dystrophy in adults, Steinert's myotonic dystrophy, is a multisystem illness with autosomal dominant transmission. The cause is the growth of CTG triplets in the gene that codes for the enzyme known as "dystrophia myotonica protein kinase". It is distinguished by the existence of rhythm and conduction abnormalities, which may result in unexpected death. Methods: A total of 21 cases were gathered for this 13-year retrospective investigation. Results: The purpose of this study is to identify the conductive problems that are present during DM1 and to emphasize the value of doing thorough cardiac exams and electrophysiological investigation. Systematic cardiac exploration was beneficial for 21 DM1 patients. Cardiovascular symptoms affect 68% of the patients. In 28% of the patients, an intraventricular conduction problem predominated the ECG abnormalities, and in 33% of the cases, an entire AV block was seen. Hyperexcitability is evident in the atrial and/or ventricular levels of the Holter ECG. 95 % of patients have normal ETTs. Three symptomatic patients had electrophysiological explorations, which objectified a prolongation of the HV interval and resulted in the implantation of a dual-chamber pacemaker. Conclusion: To identify patients at risk for severe disease, rigorous clinical and diagnostic examination is crucial. This is demonstrated by conduction system anomalies, such as atrial or ventricular arrhythmias.