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Abrupt aggravation of atrioventricular block and syncope in hypertrophic cardiomyopathy
Archives of Disease in Childhood
|December 1, 1995
Insights
A rare complication of hypertrophic cardiomyopathy (HCM) is high-grade atrioventricular (A-V) block, which can cause syncope. Prompt cardiac pacing is crucial for progressive cases to prevent sudden death.
Area of Science:
- Cardiology
- Pediatric Cardiology
- Electrophysiology
Background:
- Hypertrophic cardiomyopathy (HCM) is a genetic heart muscle disease often diagnosed in childhood.
- Atrioventricular (A-V) block is a conduction abnormality affecting the heart's electrical signals.
- Sudden cardiac death and syncope are significant risks associated with HCM.
Observation:
- A 10-year-old girl with known HCM presented with unexpected high-grade A-V block.
- The A-V block immediately precipitated a syncopal episode in the patient.
- This complication necessitated the implantation of a permanent pacemaker.
Findings:
- High-grade A-V block is an uncommon but serious complication of HCM.
- The development of A-V block in HCM is closely linked to syncope and sudden cardiac death.
- Progressive A-V block in HCM warrants urgent consideration for cardiac pacing.
Implications:
- Early recognition and management of A-V block are vital in pediatric HCM patients.
- Cardiac pacing can be a life-saving intervention for HCM patients with conduction abnormalities.
- This case highlights the importance of vigilant monitoring for cardiac conduction disturbances in young HCM patients.
Abstract:
A 10 year old girl with hypertrophic cardiomyopathy (HCM) developed high grade atrioventricular (A-V) block unexpectedly, which instantly led to syncope; she required a permanent pacemaker. High grade A-V block, a rare complication of HCM, relates closely to syncope or sudden death in this disease and if progressive the use of cardiac pacing should be considered without delay.