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Familial hypertrophic cardiomyopathy associated with spontaneous complete heart block
V Thongtang1, P Panchavinin, S Chaithiraphan
1Department of Medicine, Faculty of Medicine, Siriraj Hospital, Mahidol University, Bangkok, Thailand.
Insights
Spontaneous complete atrioventricular (A-V) block is rare in hypertrophic cardiomyopathy. This report details two siblings with hypertrophic cardiomyopathy who experienced this rare complication, requiring pacemaker implantation.
Area of Science:
- Cardiology
- Genetics
- Electrophysiology
Background:
- Hypertrophic cardiomyopathy (HCM) is a primary cardiac muscle disease.
- Atrioventricular (A-V) block is an uncommon complication in HCM patients.
- Genetic factors can influence HCM presentation and progression.
Observation:
- Two siblings with diagnosed hypertrophic cardiomyopathy presented with new-onset symptoms.
- Both siblings developed spontaneous complete A-V block.
- Cardiac symptoms deteriorated, leading to the need for intervention.
Findings:
- The occurrence of spontaneous complete A-V block in HCM is exceedingly rare.
- This case highlights a potential familial predisposition to A-V block in HCM.
- Progression of A-V block correlated with worsening cardiac status.
Implications:
- Complete A-V block necessitates careful monitoring in HCM patients, especially those with a family history.
- Early recognition and management of A-V block are crucial for preventing adverse cardiac events.
- Further research into the genetic underpinnings of A-V block in HCM may improve patient outcomes.
Abstract:
Spontaneous complete A-V block is very rare in hypertrophic cardiomyopathy. We report here two siblings with hypertrophic cardiomyopathy who developed spontaneous complete A-V block with deterioration of cardiac symptoms, necessitating implantation of permanent pacemaker.