Arrhythmia in Children and Adults

The aim of this project is to study the types and frequency of extra or missing heart beats, which can cause sudden death in Marfan syndrome.

A recent study of Marfan syndrome patients using ambulatory electrocardiograms has shown a higher prevalence of arrhythmias than in the normal population, with premature ventricular and atrial contractions, QT segment lengthening and ST depression found more often than in controls. Arrhythmia is not an infrequent cause of sudden death. Occasionally, patients have primary conduction delay or aberrant pathways. Both atrial and ventricular dysrhythmias are often associated with mitral valve prolapse causing regurgitation.

Cardiomyopathy, out of proportion to valvular problems, appears occasionally, and some have speculated that there is a primary defect in cardiac muscle.

A cardiologist will analyse 24 hour tape recordings from 100 mutation - proven Marfan syndrome patients aged 18-75, who attend our tertiary referral clinics. Type and frequency of arrhythmia will be compared with those found in 100 age and sex matched control ECGs already available, and medication will be taken into
account. The project will run for 2 years.

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