Related Experiment Videos
[Total A-V block due to tuberous sclerosis. A case report]
Arquivos Brasileiros De Cardiologia
|October 1, 1989
Summary
This report details a rare case of tuberous sclerosis (a neurologic disease) presenting with complete heart block. The patient received a pacemaker, highlighting a unique cardiac manifestation of this condition.
Area of Science:
- Cardiology
- Neurology
- Genetics
Background:
- Tuberous sclerosis is a genetic disorder impacting multiple organs, often presenting with neurological symptoms.
- Cardiac involvement in tuberous sclerosis is uncommon, making this case particularly noteworthy.
Observation:
- A patient with tuberous sclerosis experienced syncope due to complete atrioventricular (A-V) block.
- Cardiac evaluations revealed dilated cardiomyopathy with left ventricular dysfunction and infra-Hisian A-V block, despite normal coronary arteries and myocardium histology.
Findings:
- The patient exhibited a complete A-V block with right bundle branch block morphology on ECG, later showing a left bundle branch block pattern during electrophysiologic study.
- No inducible ventricular tachyarrhythmias were observed.
- This represents the first reported instance of complete heart block in a patient with tuberous sclerosis in medical literature.
Implications:
- This case expands the understanding of potential cardiac complications associated with tuberous sclerosis.
- Highlights the importance of comprehensive cardiac evaluation in patients with tuberous sclerosis presenting with syncope or bradycardia.
- Suggests that complete heart block can be a rare but significant manifestation of tuberous sclerosis, requiring long-term management with pacing.