Premature coronary artery disease in a patient with glycogen storage disease III

Matthew LaBarbera1, Gary Milechman, Fiona Dulbecco

  • 1Department of Cardiology, California Pacific Medical Center, San Francisco, California 94115, USA.

Insights

Glycogen storage disease type III (GSD III) may increase the risk of early heart problems. This case report highlights a young patient with GSD III experiencing a heart attack and cardiac arrest, suggesting a need for cardiac screening.

Area of Science:

  • Metabolic disorders
  • Cardiovascular medicine
  • Genetics

Background:

  • Glycogen storage disease type III (GSD III) is a rare inherited metabolic disorder impacting intracellular glycogen metabolism.
  • Existing research links GSD III to dyslipidemia and endothelial dysfunction.
  • Premature atherosclerotic heart disease in GSD III patients has not been previously documented.

Observation:

  • A 24-year-old patient diagnosed with GSD III presented with ventricular fibrillation cardiac arrest.
  • The cardiac arrest occurred in the context of an anterior wall myocardial infarction.

Findings:

  • This case represents the first reported instance of premature atherosclerotic heart disease in a patient with GSD III.
  • The patient's presentation underscores a potential link between GSD III and early-onset cardiovascular events.

Implications:

  • Further research is crucial to determine the prevalence of atherosclerotic heart disease in the GSD III population.
  • Developing screening protocols and preventative strategies for at-risk GSD III patients is essential.
  • This case highlights the importance of considering cardiovascular complications in patients with rare metabolic disorders.

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