[Total A-V block due to tuberous sclerosis. A case report]

Tuberous sclerosis is a neurologic disease affecting various organs with a triade: sebaceum adenoma, mental retardation and seizures. This report presents a case of a patient with tuberous sclerosis and third degree A-V block with complete invasive and non-invasive evaluation. The patient had sincope and complete A-V block with QRS complexes showing right bundle branch block morphology. The echocardiogram showed dilated cardiomyopathy with diffuse left ventricular dysfunction and had normal coronary arteriography. The eletrophysiologic evaluation showed complete infra-hisian A-V block and QRS with left bundle branch block pattern with normal sinus nodal and A-V nodal function. It was not possible to induce ventricular tachtyarrhythmias up to two extrastimuli. Histologic study showed normal myocardium under light and electronic microscopy. After permanent VVI pacemaker implant, the patient in follow-up for 16 years. This case seems to be the first in the international medical literature of tuberous sclerosis with complete heart block.