Relationships Among Electrophysiological Findings and Clinical Status, Heart Function, and Extent of DNA Mutation in Myotonic Dystrophy

Abstract

Prolongation of the HV interval is the most common conduction abnormality in MD and can be reliably recognized only by invasive EP testing. It raises the issue of prophylactic pacing to limit the incidence of sudden death in MD. Atrial and ventricular arrhythmias are often inducible, although their predictive value remains to be determined. Young age emerged as the most powerful predictor of inducible ventricular tachyarrhythmias. Conversely, we found no relationship between ECG or EP abnormalities recorded during invasive testing and the DNA mutation size or severity of peripheral muscle involvement.

Usefulness of clinical and electrocardiographic data for predicting adverse cardiac events in patients with myotonic dystrophy

“…Of note is the study by Lazarus et al (17); using precise invasive electrophysiological measurements, the study was unable to show such a relationship between conduction delays and the extent of CTG repeats. These differences among studies likely indicate that the conduction system is influenced by many other factors, some possibly still unidentified, that interact and create an unpredictable evolution of the conduction anomalies.…”

“…Although such a correlation between the extent of CTG repeats and the degree of cardiac involvement has been described in some series, others have shown conflicting results (9,17,18,25,(34)(35)(36)(37)(38)(39). Of note is the study by Lazarus et al (17); using precise invasive electrophysiological measurements, the study was unable to show such a relationship between conduction delays and the extent of CTG repeats.…”

“…Many studies have been performed to find variables that could help predict which patients are at increased risk for an adverse cardiac event (15)(16)(17)(18)(19)(20)(21)(22)(23). These studies, using different approaches, usually showed that advancing age and severity of neurological impairment, and a higher degree of conduction disturbance, assessed either by surface electrocardiogram (ECG) or by electrophysiological study, correlate with a worse outcome.…”

Re-entry Circuit in Ventricular Tachycardia Due to Focal Fatty-fibrosis in a Patient with Myotonic Dystrophy

“…Regarding cardiac involvement, Tokgozoglu et al reported that myocardial wall motion and abnormalities in the electrocardiogram correlate with CTG-repeat length (20), whereas Lazarus et al reported that impediment of the atrioventricular conducting system does not correlate with CTG-repeat length (21). It remains uncertain whether this patient's cardiac involvement is severe or not; that is the patient has a potentially lethal arrhythmia, and the fatty infiltration was detected by biopsy of the cardiac muscle, but not detected by MRI.…”

Exploration du sentiment de compétence et d'autonomie professionnelle des infirmières cliniciennes avant et après l'implantation d'un outil de gestion intégrée de la santé chez les personnes atteintes de dystrophie myotonique de type I /

“…Plusieurs anomalies du système cardiovasculaire ont été observées (Phillips & Harper, 1997) et leur niveau de progression est généralement lent, mais demeure imprévisible (Antonini et al, 2000;Pelargonio, Dello Russo, Sanna, De Martino, & Bellocci, 2002 (Lazarus et al, 1999;Melacini et al, 1988).…”