Miscellaneous TopicCardiac involvement in myotonic muscular dystrophy (Steinert's disease): A prospective study of 25 patients*
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Cited by (116)
An overview of heart rhythm disorders and management in myotonic dystrophy type 1
2022, Heart RhythmCitation Excerpt :The observation that patients with DM developed conduction abnormalities was made early in the 20th century, with researchers highlighting advanced-degree AV block as the principal feature in DM1-related cardiac involvement.33 Electrophysiological testing used early in the investigation of DM1 patients demonstrated a high prevalence of His-Purkinje abnormalities, as reflected by HV interval prolongation.34 The progressive nature of conduction defects was confirmed in small cohorts, utilizing longitudinal monitoring of both ECG- and EPS-derived indices.35–38
Cardiovascular manifestations of neurologic disease
2014, Handbook of Clinical NeurologyCitation Excerpt :Cardiac involvement is very common in MMD and includes conduction abnormalities and structural heart disease as a result of interstitial myocardial fibrosis and myofibrillar degeneration (Motta et al., 1979). Electrocardiographic abnormalities are reported in 65–80% of patients, who most commonly experience PVCs, QRS prolongation, PR interval prolongation with atrioventricular block, and left fascicular block (Perloff et al., 1984; Groh et al., 2002). Atrial fibrillation and flutter are also commonly reported (Finsterer and Stollberger, 2008a).
2012 ACCF/AHA/HRS focused update incorporated into the ACCF/AHA/HRS 2008 guidelines for device-based therapy of cardiac rhythm abnormalities: A report of the American college of cardiology foundation/American heart association task force on practice guidelines and the heart rhythm society
2013, Journal of the American College of CardiologyCitation Excerpt :Permanent pacemaker implantation may be considered in the setting of neuromuscular diseases such as myotonic muscular dystrophy, Erb dystrophy (limb-girdle muscular dystrophy), and peroneal muscular atrophy with bifascicular block or any fascicular block, with or without symptoms. ( Level of Evidence: C) (91–97) Permanent pacemaker implantation is not indicated for fascicular block without AV block or symptoms. (
Cardiac Management in Neuromuscular Diseases
2012, Physical Medicine and Rehabilitation Clinics of North AmericaCitation Excerpt :These findings typically worsen over time in approximately 75% of patients. Rarely, dilated cardiomyopathy will be present in adults with DM1 or DM2.46–50 Recent MRI studies have shown fatty infiltration of the right ventricle in patients with ventricular tachydysrhythmias.51
A novel KCNQ1 variant (L203P) associated with torsades de pointes-related syncope in a steinert syndrome patient
2011, Canadian Journal of CardiologyCardiological aspects of systemic disease
2010, Paediatric Cardiology
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This study was supported by Grant E800421 from the Muscular Dystrophy Association, New York, New York, Clinical Research Center Grant R 1865 from the National Institutes of Health, Bethesda, Maryland, and by the Streisand Professorship/American Heart Association, Greater Los Angeles Affiliate, Los Angeles, California.