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Fabry's disease with complete atrioventricular block: histological evidence of involvement of the conduction system
Y Ikari1, K Kuwako, T Yamaguchi
1Division of Cardiology, Mitsui Memorial Hospital, Tokyo, Japan.
British Heart Journal
|September 1, 1992
Insights
Fabry
Area of Science:
- Cardiology
- Genetics
- Pathology
Background:
- Fabry's disease is a rare genetic disorder.
- Electrocardiographic findings like short PR interval are common.
- Complete atrioventricular block is an exceptionally rare complication.
Observation:
- A 63-year-old male patient presented with complete atrioventricular block.
- The patient was diagnosed with Fabry's disease.
Findings:
- Necropsy revealed lipid accumulation in the atrioventricular conduction system.
- This lipid accumulation was identified as the likely cause of the patient's atrioventricular block.
Implications:
- This case highlights a rare but severe cardiac manifestation of Fabry's disease.
- Understanding the pathological basis of cardiac conduction abnormalities in Fabry's disease is crucial for patient management.
- Further research into the mechanisms of lipid deposition in the cardiac conduction system is warranted.
Abstract:
A 63 year old man with complete atrioventricular block was diagnosed as having Fabry's disease. A short PR interval is a common electrocardiographic finding in Fabry's disease, but complete atrioventricular block is a very rare complication. Necropsy indicated that lipid accumulation in the atrioventricular conduction system was the probable cause of this patient's atrioventricular block.