Ventricular dysrhythmias in children with Marfan's syndrome
S. Chen, L. F. Fagan, S. Nouri and J. L. Donahoe
A teenager with Marfan's syndrome required resuscitation and was found to
have multiform premature ventricular contractions and ventricular
tachycardia. Of 24 children with Marfan's syndrome, eight (33.3%) were
found to have ventricular dysrhythmias, including three with ventricular
tachycardia. Six of these eight patients had mitral valve prolapse, and
five had prolonged QT or QTU intervals corrected for heart rate. However,
only two patients had severe mitral regurgitation, five had only mild heart
disease, and one had no detectable heart lesion. The role of mitral valve
prolapse and/or delayed repolarization in the development of ventricular
dysrhythmia was explored. Delayed repolarization, especially when combined
with mitral valve prolapse, is associated with occurrence of ventricular
dysrhythmia. Serious ventricular dysrhythmia can occur in children with
Marfan's syndrome with or without substantial valve disease, and the
dysrhythmia appears to progress with age.
