Key result
EPS-guided pacemaker implantation was associated with a higher rate of 9-year survival than a non-invasive strategy in patients with Myotonic Dystrophy type 1.
This letter highlights a gap in current guidelines regarding the optimal timing for electrophysiological studies in patients with Myotonic Dystrophy type 1.
We read with great interest the 2021 ESC Guidelines on cardiac pacing and cardiac resynchronization therapy.1 Regarding Myotonic Dystrophy type 1 (DM1) patients, it was stated that the cardiac pacing is indicated in patients with any second- and third-degree atrioventricular block or His-ventricle (HV) interval >70 ms, regardless the symptoms; and it may be considered in those with QRS >120 ms and PR >240 ms. As in the 2013 version,2 the appropriate timing of electrophysiological study (EPS) was not addressed, making it difficult to determine when EPS for assessing HV interval is necessary in the clinical practice management of DM1 patients. In a large retrospective registry including 486 genetically confirmed DM1 patients with PR interval >200 ms and/or QRS duration >100 ms, the EPS-guided (HV interval >70 ms) pacemaker implantation was associated with a higher rate of 9-year survival than a non-invasive strategy.3 Moreover, in a recent...
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Russo et al. (2021) conducted a letter in Myotonic Dystrophy type 1 (DM1) (n=486). Electrophysiological study (EPS)-guided pacemaker implantation vs. Non-invasive strategy was evaluated on 9-year survival. EPS-guided pacemaker implantation was associated with a higher rate of 9-year survival than a non-invasive strategy in patients with Myotonic Dystrophy type 1.
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