Sudden death in supravalvular aortic stenosis: Fusion of a coronary leaflet to the sinus ridge, dysplasia and stenosis of aortic and pulmonic valves

Chen Chih J. Sun, Jorge Jacot, Joel I. Brenner

Research output: Contribution to journalArticle


Supravalvular aortic stenosis (SVAS) is an uncommon congenital cardiac anomaly. We report sudden death, occurring during exercise, of a child who had SVAS with fusion of the right coronary aortic leaflet to the supravalvular aortic ridge, resulting in a closed sinus of Valsalva except for a few pinpoint fenestrations in the dysplastic leaflet. In addition, both aortic and pulmonic valves were dysplastic and stenotic. We postulate that near total isolation of the right coronary artery ostium from the aortic lumen compromised the blood supply to the hypertrophied ventricles. We emphasize the importance of other cardiac anomalies associated with SVAS as well as the development of coronary insufficiency..

Original languageEnglish (US)
Pages (from-to)751-759
Number of pages9
JournalFetal and Pediatric Pathology
Issue number5
StatePublished - Jan 1 1992



  • Aortic valve
  • Coronary ostium
  • Pulmonic valve
  • Stenosis
  • Supravalvular stenosis
  • Valvular dysplasia

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Pathology and Forensic Medicine

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