Tetralogy of Fallot with congenital aortic valvar stenosis: The tetralogy-truncus interrelationship

G. M. Aru, A. Juraszek, I. Moskowitz, R. Van Praagh

Research output: Contribution to journalArticlepeer-review

13 Scopus citations


Two rare patients are reported with tetralogy of Fallot and congenital aortic valvar stenosis. The anatomic and developmental interrelationship between tetralogy of Fallot and truncus arteriosus is summarized. A study of 100 randomly selected postmortem cases of tetralogy revealed aortic valve pathology in 8%, myxomatous aortic valve leaflets without stenosis in 4%, bicuspid aortic valves without stenosis in 3%, and congenital aortic valvar stenosis in 1%. The frequency of systemic semilunar valve pathology in truncus was much higher (66%): moderate to marked myxomatous change in 44%, mild myxomatous change in 22%, truncal valvar stenosis in 11%, and truncal valvar regurgitation in 15%. Being aware of the tetralogy-truncus interrelationship and knowing that myxomatous aortic valves are prone to premature calcific aortic stenosis and/or regurgitation, physicians should follow the aortic valves of surgically repaired patients with tetralogy of Fallot and truncus arteriosus long term with great care. Timely aortic valvuloplasty or replacement may well prove life-saving in such patients.

Original languageEnglish (US)
Pages (from-to)354-359
Number of pages6
JournalPediatric Cardiology
Issue number3
StatePublished - Jun 2006


  • Congenital aortic valvar stenosis
  • Long-term follow-up
  • Myxomatous aortic valves
  • Tetralogy of Fallot
  • Tetralogy-truncus interrelationship
  • Truncus arteriosus

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Cardiology and Cardiovascular Medicine


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