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[Steinert's disease and conduction disorders. Apropos of a familial study]
F Maloisel1, F Wolff, M Chauvin
1Service de Cardiologie, Centre Médico-Chirurgical et Obstétrical de la Sécurité Sociale, Strasbourg.
Insights
This study followed a family with Steinert disease for 12 years, revealing distinct muscular and myocardial injury patterns. It highlights the importance of electrophysiological studies for managing conduction disturbances and syncope in these patients.
Area of Science:
- Neurology
- Cardiology
- Genetics
Background:
- Steinert disease, also known as myotonic dystrophy, is a multisystem disorder.
- Cardiac involvement is common and can lead to significant morbidity and mortality.
- Longitudinal family studies are crucial for understanding disease progression.
Observation:
- A family cohort with Steinert disease was followed for twelve years.
- Electrophysiological studies of the bundle of His revealed intra- and infra-Hisian blocks.
- A discrepancy was noted between the evolution of muscular and myocardial injury.
Findings:
- Conduction disturbances were identified in two family members.
- One patient required pacemaker implantation due to conduction abnormalities.
- Electrophysiological studies are indicated for assessing syncope risk.
Implications:
- Understanding the differing evolutions of muscular and myocardial injury is vital for patient management.
- Early electrophysiological assessment can guide interventions like pacemaker implantation.
- This family case provides valuable insights into the long-term cardiac manifestations of Steinert disease.
Abstract:
The authors reported a new family case of Steinert with twelve years follow-up. The study of potentials in the bundle of his was performed in all patients, showed an intra- and infra-hisian blocks. This study showed a discrepancy between muscular and myocardial evolutive injury, a very important knowledge for follow-up. Conduction disturbances were found in two cases and pacemaker insertion was performed in one patient. The authors specified the indication of electrophysiological studies during the onset and pacemaker implantation in all patient with a typical or suggestive history of syncope.