The heart and tuberous sclerosis. An echocardiographic and electrocardiographic study

Cross sectional echocardiography, 12 lead electrocardiography, and 24 hour ambulatory electrocardiography were performed in eleven patients with tuberous sclerosis. Echocardiography showed single or multiple intramyocardial masses, most commonly in the ventricular septum, suggestive of rhabdomyomata in seven of the eleven cases. One patient with a normal echocardiogram showed ventricular pre-excitation on electrocardiography, with tachycardias of 180 beats per minute on ambulatory monitoring. Sinus bradycardia, sinus tachycardia, and supraventricular tachycardia were also seen, but their importance is uncertain. Echocardiography and electrocardiography should be routinely performed in the assessment of patients with tuberous sclerosis and ambulatory electrocardiography should be considered in those with seizures that respond poorly to anticonvulsants.