Right Ventricular MR Abnormalities in Myotonic Dystrophy and Relationship with Intracardiac Electrophysiologic Test Findings: Initial Results

Abstract

The relationship between MR morphologic and functional RV abnormalities and EP testing suggests potential for the use of MR imaging as a noninvasive method to estimate the individual risk of arrhythmia in patients with myotonic dystrophy.

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

“…Several authors have reported that the mechanism of VT in DM1 might be due to His-Purkinje conduction delay caused by massive fatty fibrosis (11,13). Additionally, it was recently reported that the focal fatty degeneration of the right ventricle could be demonstrated by MRI in patients with DM1 (17). This suggests that the re-entry circuit could be formed in this site and may become a mechanism of VT.…”

Myotonic dystrophy and the heart: A systematic review of evaluation and management

“…In a prospective study of patients with MD undergoing invasive EPS, there was a strong correlation between inducibility of ventricular arrhythmias at invasive EPS and increased fatty infiltration, myocardial thinning and hypokinesis or dyskinesis of the RV on CMR [69]. No morphological or contraction abnormalities were detected in patients without inducible ventricular tachyarrhythmias [69].…”

“…The inducibility of atrial arrhythmias at EP correlates positively with prolonged AH intervals, but this invasive measurement does not correlate with P wave duration or clinical factors such as muscle weakness [66]. The only factor that has been found to correlate with the inducibility of ventricular arrhythmia during EP study is young age [66,69]. The prognostic value of inducible VT in patients without documented spontaneous VT is uncertain [1].…”

Early detection of biventricular involvement in myotonic dystrophy by tissue Doppler

“…Cardiac abnormalities in MD are not limited to the conduction tissue but may extend diffusely into the right myocardium. Morphological and functional abnormalities of RV myocardium have been previously described in MD by using cardiac magnetic resonance, able to identify RV hypertrophy, fibrosis and fatty infiltration [30,31]. Of note, these RV abnormalities have been found to be associated with arrhythmias inducibility during electrophysiological testing in patients with MD [31].…”