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Coronary artery dissection in Prader-Willi syndrome: case report and literature review

Jason C Jacob1, Penelope Armada, Pavittarpaul Dhesi

  • 1Division of Internal Medicine, University of Connecticut, Farmington, CT, USA.

The Canadian Journal of Cardiology
|August 4, 2012
PubMed

Insights

Prader-Willi syndrome (PWS) is a rare genetic disorder. This case study highlights a 17-year-old PWS patient experiencing a myocardial infarction due to coronary artery dissection, a previously undocumented association.

Area of Science:

  • Cardiology
  • Genetics
  • Pediatrics

Background:

  • Prader-Willi syndrome (PWS) is a complex genetic disorder with characteristic infantile and childhood features.
  • PWS is typically associated with developmental delays, behavioral issues, and endocrine abnormalities.
  • Known complications include morbid obesity, hypoxemia, and right heart failure.

Observation:

  • A 17-year-old female patient with a confirmed diagnosis of Prader-Willi syndrome was admitted.
  • The patient presented with symptoms indicative of an acute myocardial infarction.
  • Diagnostic imaging revealed a dissection of the right coronary artery.

Findings:

  • The patient's myocardial infarction was directly attributed to spontaneous dissection of the right coronary artery.
  • This represents the first reported instance of coronary artery dissection in an individual with Prader-Willi syndrome.
  • The anatomical and physiological underpinnings of this association require further investigation.

Implications:

  • This finding expands the spectrum of cardiovascular complications associated with Prader-Willi syndrome.
  • It suggests a potential need for increased cardiovascular surveillance in PWS patients, particularly for coronary artery abnormalities.
  • Further research is warranted to elucidate the mechanisms linking PWS to coronary artery dissection.

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