Arch Dis Child 1999;80:267-270 ( March )
Left ventricular diastolic function in congenital myotonic
dystrophy
F A Bu'Lock,
M Sood,
J V De Giovanni,
S H Green
Departments of
Cardiology and Neurology, Birmingham Children's Hospital, Birmingham,
UK
Correspondence to: Dr F A Bu'Lock, Department of Paediatric Cardiology, Alder Hey
Hospital, Eaton Road, Liverpool L12 2AP, UK.
Accepted 25 August
1998
OBJECTIVE
Examination
of left ventricular function and conduction abnormalities in myotonic dystrophy.
DESIGNTwelve 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.
RESULTSFractional
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.
CONCLUSIONSYoung
patients with myotonic dystrophy have myocardial diastolic
dysfunction as well as abnormal electrophysiology. The prognostic implications of such abnormalities require further study.
Keywords:
diastolic filling;
myotonic dystrophy;
Doppler
echocardiography
© 1999 by Archives of Disease in Childhood
