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Review
. 2002 Dec;88(6):665-70.
doi: 10.1136/heart.88.6.665.

Myotonic dystrophy and the heart

G Pelargonio  1 , A Dello Russo, T Sanna, G De Martino, F Bellocci
Affiliations
Review

Myotonic dystrophy and the heart

G Pelargonio et al. Heart. 2002 Dec.
No abstract available

PubMed Disclaimer

Figures

Figure 1
Figure 1
The grip test is a quick and easy way to determine the presence of active myotonia. After contraction of the fist (A) the patient is unable to relax the muscles of the hand (B). Photographs courtesy of Dr Gabriella Silvestri, Unione Italiana Lotta alla Distrofia Muscolare, Sezione Lazio, Italy.
Figure 2
Figure 2
(A) Intracardiac electrograms (EGM) of a spontaneous episode of sustained ventricular tachycardia as recorded from the cardioverter-defibrillator (ICD) implanted in a 32 year old male patient affected by DM1. From top to bottom the tracings show the atrial EGM, the ventricular EGM, and a pseudo-surface lead II derived from signals recorded between the shock coils and the ICD. Atrioventricular dissociation, enabling a diagnosis of ventricular tachycardia, is evident. (B) Resumption of sinus rhythm after a 17 J biphasic DC shock.
Figure 3
Figure 3
Bundle branch re-entry. (A) A ventricular premature impulse is blocked retrogradely in the right bundle branch (RB); conduction proceeds retrogradely through the left bundle branch (LB) up to the His bundle (His); from the His bundle, the impulse travels anterogradely over the right bundle branch which is still refractory, thus blocking propagation. (B) Conversely, if the right bundle branch has enough time to recover, the impulse can be conducted anterogradely to the ventricles, then retrogradely through the left bundle branch up to the His bundle, thus maintaining a macro-reentry. AVN, atrioventricular node.
See this image and copyright information in PMC

References

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    1. Melacini P, Villanova C, Menegazzo E, et al. Correlation between cardiac involvement and CTG trinucleotide repeat length in myotonic dystrophy. J Am Coll Cardiol 1995;25:239–45. ▸ This excellent work points out the possible predictive value of CTG repeats size on cardiac conduction abnormalities and their severity, adding also important information on the role of late potentials in predicting VT. - PubMed
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