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Anomalous aortic origin of a coronary artery with an interarterial course: should family screening be routine?

Julie A Brothers1, Paul Stephens, J William Gaynor

  • 1Division of Cardiology, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania 19104, USA. brothersj@email.chop.edu

Journal of the American College of Cardiology
|May 24, 2008
PubMed

Insights

Familial occurrence of anomalous aortic origin of a coronary artery with an interarterial course (AAOCA) suggests a genetic link. Screening first-degree relatives with echocardiography is recommended to prevent sudden death.

Area of Science:

  • Cardiology
  • Pediatric Cardiology
  • Medical Genetics

Background:

  • Anomalous aortic origin of a coronary artery with an interarterial course (AAOCA) is a rare congenital heart anomaly.
  • AAOCA is associated with an increased risk of sudden death in pediatric patients.
  • No previous reports documented familial AAOCA in the pediatric population.

Purpose of the Study:

  • To present cases of familial AAOCA.
  • To investigate the potential genetic link of AAOCA.
  • To evaluate current screening and management recommendations for AAOCA.

Main Methods:

  • A questionnaire was distributed to pediatric institutions in North and South America.
  • Families with multiple members diagnosed with AAOCA were identified.
  • Patient charts were retrospectively reviewed.

Main Results:

  • Five families with familial AAOCA were identified.
  • Screening identified asymptomatic family members with AAOCA.
  • The occurrence of familial AAOCA was statistically significant, suggesting a genetic predisposition.

Conclusions:

  • A genetic link for AAOCA is plausible.
  • Screening first-degree relatives of AAOCA patients using transthoracic echocardiography is recommended.
  • Early detection through screening may prevent sudden cardiac events.
Abstract

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