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CASE REPORT
Prader-Willi syndrome: a nest for premature coronary artery disease?
  1. Diogo Rodrigues Brás1,
  2. Pedro Semedo1,
  3. Bruno Cordeiro Piçarra1,
  4. Renato Fernandes1,2
  1. 1Department of Cardiology, Hospital do Espirito Santo EPE, Evora, Portugal
  2. 2Digital Angiography and Interventional Cardiology Unit, Hospital do Espirito Santo EPE, Evora, Portugal
  1. Correspondence to Dr Diogo Rodrigues Brás, diogobras8{at}gmail.com

Summary

Individuals affected by Prader-Willi syndrome (PWS) may show increased risk for coronary artery disease (CAD), which probably relates, at least, with high burden of cardiovascular risk factors.

A 27-year-old man with PWS, obesity, hypertension, diabetes mellitus and dyslipidaemia attended the emergency department with complaints of flu-like condition and chest pain. The ECG revealed a mild ST-segment elevation in inferior leads, followed by positive myocardial necrosis biomarkers. Attending to the high cardiovascular risk profile, ST-segment elevation in inferior territory and wall motion abnormalities, a coronary angiogram was performed. The latter showed a three-vessel CAD, 60% stenosis in midanterior descending artery, total occlusion (100%) of the obtuse marginal artery and 99% stenosis with high thrombi burden in the proximal right coronary artery.

The present case report emphasises the plausibility of premature CAD in patients with PWS, a possible underdiagnosed feature of this condition.

  • ischaemic heart disease
  • genetics
  • endocrinology
  • interventional cardiology
  • metabolic disorders

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Footnotes

  • Competing interests None declared.

  • Patient consent Guardian consent obtained.

  • Provenance and peer review Not commissioned; externally peer reviewed.