Left ventricular diastolic function in congenital myotonic dystrophy

OBJECTIVE Examination of left ventricular function and conduction abnormalities in myotonic dystrophy. DESIGN Twelve patients (median age, 13.7 years) with myotonic dystrophy had detailed electrocardiography and echocardiography performed. Echocardiographic parameters were compared with body surface...

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Bibliographic Details
Published in:Archives of disease in childhood 1999-03, Vol.80 (3), p.267-270
Main Authors: Bu’Lock, F A, Sood, M, De Giovanni, J V, Green, S H
Format: Article
Language:English
Subjects:
Adolescent
Adult
Biological and medical sciences
Case-Control Studies
Child
Child, Preschool
Congenital diseases
Control Groups
Correlation
Diastole
diastolic filling
Diseases of striated muscles. Neuromuscular diseases
Doppler echocardiography
Doppler effect
Dystrophy
Echocardiography, Doppler
Electrocardiography
Electrocardiography, Ambulatory
Female
Heart
Heart Disorders
Heart rate
Humans
Male
Medical sciences
Motor Development
Musculoskeletal system
Myotonia Congenita - diagnostic imaging
Myotonia Congenita - physiopathology
myotonic dystrophy
Neurology
Original
Patients
Pregnancy
Regression Analysis
Scientific Concepts
Statistical Analysis
Statistics, Nonparametric
Ventricular Dysfunction, Left - physiopathology
Young adults
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Summary:OBJECTIVE Examination of left ventricular function and conduction abnormalities in myotonic dystrophy. DESIGN Twelve patients (median age, 13.7 years) with myotonic dystrophy had detailed electrocardiography and echocardiography performed. Echocardiographic parameters were compared with body surface area (BSA) matched median normal values. RESULTS Fractional shortening was slightly reduced (by 28–29%) in three patients and three patients had mild mitral valve prolapse. Diastolic function was abnormal; isovolumic relaxation time (IVRT) and duration of early filling were prolonged compared with control values (median IVRT, 74v 61 ms). Peak E velocity was increased (median, 0.82 v 0.78 m/s) but atrial phase filling was normal. Heart rate was reduced (median, 68v 81 beats/min). Conduction abnormalities were common but showed no clear relations with diastolic abnormalities. CONCLUSIONS Young patients with myotonic dystrophy have myocardial diastolic dysfunction as well as abnormal electrophysiology. The prognostic implications of such abnormalities require further study.
ISSN:0003-9888
1468-2044
DOI:10.1136/adc.80.3.267