Left ventricular diastolic function in congenital myotonic dystrophy. Arch Dis Child 1999 Mar;80(3):267-70
Date
05/18/1999Pubmed ID
10325709Pubmed Central ID
PMC1717875DOI
10.1136/adc.80.3.267Scopus ID
2-s2.0-0033018308 (requires institutional sign-in at Scopus site) 20 CitationsAbstract
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, 74 v 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, 68 v 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.
Author List
Bu'Lock FA, Sood M, De Giovanni JV, Green SHMESH terms used to index this publication - Major topics in bold
AdolescentAdult
Case-Control Studies
Child
Child, Preschool
Diastole
Echocardiography, Doppler
Electrocardiography, Ambulatory
Female
Humans
Male
Myotonia Congenita
Regression Analysis
Statistics, Nonparametric
Ventricular Dysfunction, Left
