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Updated: Mar 21, 2026

Author Spotlight: Enhancing Coronary Artery Revascularization
Published on: September 15, 2023
Coronary artery bypass graft in a patient with Fabry's disease
Hiroaki Osada1, Naoki Kanemitsu1, Masahisa Kyogoku2
1Department of Cardiovascular Surgery, Mitsubishi Kyoto Hospital, 1 Katsuragoshomachi, Nishikyo-ku, Kyoto, 615-8087, Japan.
Insights
Fabry disease, a rare genetic disorder, can affect the heart. This case highlights the unusual occurrence of coronary artery disease in an elderly patient with Fabry disease.
Area of Science:
- Genetics
- Metabolic Disorders
- Cardiology
Background:
- Fabry disease is a lysosomal storage disorder caused by alpha-galactosidase A deficiency.
- This deficiency leads to the buildup of ceramide trihexoside within cells.
- Cardiac involvement is common, but coronary artery disease is a rare manifestation.
Observation:
- A 72-year-old male patient with Fabry disease presented with left main coronary artery disease.
- The case involved a detailed examination of the arterial wall pathology in this rare clinical scenario.
Findings:
- The study focuses on the arterial wall pathology in a patient with Fabry disease and left main coronary artery disease.
- This highlights the complex interplay between genetic metabolic disorders and cardiovascular manifestations.
Implications:
- Understanding arterial wall pathology in Fabry disease is crucial for managing coronary artery disease in affected individuals.
- This case contributes to the limited literature on cardiovascular interventions in Fabry disease patients.
- Further research is needed to elucidate the specific mechanisms driving coronary artery disease in Fabry disease.
Abstract:
Fabry's disease is a lysosomal storage disease characterized by intracellular accumulation of ceramide trihexoside resulting from alpha-galactosidase A deficiency. While the heart is often involved, coronary artery disease and its management in Fabry's disease patients are extremely rare clinical entities. We report a case of a 72-year-old man with left main disease in Fabry's disease with special consideration of the arterial wall pathology.
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