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Absent aortic valve: a complex anomaly.

A E Lin1, A J Chin

  • 1Department of Pediatrics, Children's Hospital of Philadelphia, Pennsylvania.

Pediatric Cardiology
|October 1, 1990
PubMed
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Absent aortic valve cusps, a rare congenital heart defect, led to early death in all seven male infants studied. Associated malformations were common, highlighting the need for early recognition and potential palliative strategies.

Area of Science:

  • Pediatric Cardiology
  • Congenital Heart Disease
  • Neonatal Cardiology

Background:

  • Absent aortic valve cusps (AAV) is a rare congenital heart defect.
  • This study reviews seven cases, including three new ones, to better understand AAV.

Observation:

  • All seven patients were male, with most born full-term and one with nonimmunologic hydrops.
  • None survived past the first week of life due to low cardiac output and hypoxemia.
  • The aortic valve remnant was often a fibrous ridge, sometimes with rudimentary leaflets.

Findings:

  • AAV was consistently associated with other severe cardiac malformations.
  • These included atrioventricular valve defects, double outlet right ventricle, and abnormal pulmonary venous connections.
  • No patients underwent surgical intervention.

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Implications:

  • Early recognition of AAV is crucial due to its association with complex defects.
  • Hypoxemia necessitates prompt positive pressure ventilation.
  • Potential palliative strategies, such as using the right ventricle as the systemic ventricle, warrant further investigation.