Related Experiment Videos
[Familial complete atrioventricular block in patients with hypertrophic cardiomyopathy]
F M Albanesi Filho1, P Ginefra, M B Castier
1Hospital Universitário Pedro Ernesto, UERJ.
Insights
This study details a rare familial link between hypertrophic cardiomyopathy and spontaneous complete heart block. Pacemaker implantation successfully managed symptoms in three affected family members.
Area of Science:
- Cardiology
- Genetics
- Electrophysiology
Background:
- Hypertrophic cardiomyopathy (HCM) is a genetic heart muscle disease.
- Spontaneous complete heart block is an uncommon cardiac conduction abnormality.
- The co-occurrence of HCM and heart block within families is exceptionally rare.
Observation:
- Three male patients from the same family (brothers and nephew), aged 19-41, presented with confirmed hypertrophic cardiomyopathy.
- Diagnostic confirmation involved hemodynamic and angiographic assessments.
- All affected individuals developed spontaneous complete heart block.
Findings:
- All three patients received permanent cardiac pacemaker implantation for heart block.
- Following pacemaker implantation, patients became asymptomatic.
- Long-term follow-up (157-176 months) showed sustained asymptomatic status in patients aged 33-55.
Implications:
- This case series highlights a potential genetic link between HCM and conduction system disease.
- Permanent pacemaker implantation is an effective treatment for heart block in HCM patients.
- Further research into the genetic underpinnings of this association is warranted.
Abstract:
The association of spontaneous complete heart block and hypertrophic cardiomyopathy is rare. We have studied three patients of the same family, two brothers and one nephew, ages 19-41 years, with hypertrophic cardiomyopathy confirmed by hemodynamic and angiographic studies. All patients were treated with permanent cardiac pacemaker implant. They are asymptomatic, aging 33 to 55 years, with follow-up of 157 to 176 months after the onset of the heart block.