Is it possible to identify infrahissian cardiac conduction abnormalities in myotonic dystrophy by non-invasive methods?
D Babutya, L Fauchiera, D Tena-Carbia, P Poreta, J Lecheb, M Raynaudc, J P Fauchiera, P Cosnaya
a Department of
Cardiology B, Faculté de Médecine Tours, Hôpital Trousseau, 37044 Tours Cedex, France, b Department of Neurology, Faculté de Médecine
Tours, c Department of Genetics,
Faculté de Médecine Tours
Correspondence to: Dr Babuty.
Accepted for publication 4 June 1999
OBJECTIVE
To identify
intracardiac conduction abnormalities in patients with myotonic
dystrophy from their clinical, ECG, and genetic features.
METHODS39 consecutive
patients (mean (SD) age 42.9 (12.1) years; 16 female, 23 male)
underwent clinical examination, genetic studies, resting and 24 hour
ambulatory ECG, signal averaged ECG, and electrophysiological studies.
RESULTS23 patients
suffered from cardiac symptoms, 23 had one or more cardiac conduction
abnormality on resting ECG, one had sinus deficiency, and 21 (53.8%)
had prolonged HV intervals. No correlation was found between the
severity of the neurological symptoms, onset of disease, cardiac
conduction abnormalities on ECG, and the intracardiac conduction
abnormalities on electrophysiological study. The size of the DNA
mutation was longer in the abnormal HV interval group than in the
normal HV interval group (3.5 (1.8) v 2.2 (1.0) kb, p < 0.02). Signal averaged ECG parameters (total QRS
duration (QRSD) and duration of low amplitude signals 
40 µV (LAS
40)) were greater in patients with an abnormal HV interval than in those with a normal HV interval (123.4 (24.6)
v 102.8 (12.3) ms and 47.5 (12.8)
v 35.3 (8.8) ms, respectively;
p < 0.005). Only the association of QRSD 
100 ms with LAS 40 36 ms identified patients with an abnormal HV interval with good
sensitivity (80%) and specificity (83.3%).
CONCLUSIONSInfrahissian
conduction abnormalities are common in myotonic dystrophy and can be
identified using signal averaged electrocardiography.
Keywords: myotonic dystrophy; atrioventricular block; genetic factors; signal averaged ECG
© 1999 by Heart
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