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. 2018 Jun;13(2):152-154.
doi: 10.26574/maedica.2018.13.2.152.

Fast Progressing His-Purkinje Conduction Disturbances in a Myotonic Dystrophy Pacient

Radu Nicolae Ciudin  1 , Nic Claudiu Dragatoiu  1 , Sergiu Sipos  1 , Dan Nicolae Tesloianu  2 , Andreea Maria Ursaru  2 , Radu Brezeanu  3 , Ioan Mircea Coman  4
Affiliations

Fast Progressing His-Purkinje Conduction Disturbances in a Myotonic Dystrophy Pacient

Radu Nicolae Ciudin et al. Maedica (Bucur). 2018 Jun.

Abstract

Myotonic dystrophy leads to multiple systemic complications and the age of death is earlier in myotonic dystrophy patients than in the general population. These patients have a high frequency of sudden death related to respiratory failure, cardiac arrhythmias and in particular to cardiac conduction disturbances. Prophylactic pacemaker implantation should be considered in asymptomatic myotonic dystrophy patients, which in the early stages of disease present minor conduction disturbances in 12-leads ECG. Even if the rate of progression of conduction abnormalities is usually slow, fast progression has been often observed thus making the clinical course of individual patients rather unpredictable.

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Figures

FIGURE 1.
FIGURE 1.
(A) ECG: sinus rhythm, 75/minute, PR 185 ms, right bundle branch block (typical RsR’ pattern in V1, wide slurred S wave in lead I, aVL, typical pattern of T-wave inversion only in V1, unchanged cardiac axis); (B) EPS: at Wenckebach cycle length 560 ms intermittent second degree atrioventricular block and left anterior fascicular block; (C) EPS: prolonged HV interval – 81 ms.
TABLE 1.
TABLE 1.
Genetic study: correlation of phenotype and CTG repeat length in myotonic dystrophy type 1
See this image and copyright information in PMC

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