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Prader-Willi syndrome: a nest for premature coronary artery disease?

Diogo Rodrigues Brás1, Pedro Semedo1, Bruno Cordeiro Piçarra1

  • 1Department of Cardiology, Hospital do Espirito Santo EPE, Evora, Portugal.

BMJ Case Reports
|February 14, 2018
PubMed

Insights

Prader-Willi syndrome (PWS) patients face a higher risk of premature coronary artery disease (CAD). This case highlights severe, multi-vessel CAD in a young PWS patient with multiple cardiovascular risk factors.

Area of Science:

  • Cardiology
  • Genetics
  • Internal Medicine

Background:

  • Prader-Willi syndrome (PWS) is associated with an increased risk of cardiovascular disease.
  • Cardiovascular risk factors are common in individuals with PWS, potentially contributing to disease burden.

Observation:

  • A 27-year-old male with PWS, obesity, hypertension, diabetes mellitus, and dyslipidemia presented with chest pain.
  • Electrocardiogram showed ST-segment elevation and elevated myocardial necrosis biomarkers, suggesting acute coronary syndrome.
  • Coronary angiogram revealed extensive three-vessel coronary artery disease (CAD), including total occlusion and significant stenosis with thrombus.

Findings:

  • The case demonstrates severe, premature CAD in a young individual with PWS.
  • The extent of CAD suggests a significant predisposition beyond typical risk factors.

Implications:

  • Premature CAD may be an underdiagnosed complication in Prader-Willi syndrome patients.
  • Increased vigilance and proactive cardiovascular risk management are crucial for this population.

Keywords:
endocrinologygeneticsinterventional cardiologyischaemic heart diseasemetabolic disorders

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