Absent pulmonary valve syndrome

Absent pulmonary valve syndrome is a congenital heart defect that occurs when the flaps of the pulmonary valve do not develop or are severely underdeveloped (hypoplasia) resulting in aneurysms (dilation) of the pulmonary arteries and softening of the trachea and bronchi (tracheobronchomalacia). Usually, APVS occurs together with other congenital heart defects, most commonly ventricular septal defect and right ventricular outflow tract obstruction. It is sometimes considered a variant of Tetralogy of Fallot.[1]

Absent pulmonary valve syndrome
Other namesCongenital absence of the pulmonary valve


  1. Nölke, Lars; Azakie, Anthony; Anagnostopoulos, Petrous V.; Alphonso, Nelson; Karl, Tom R. (May 2006). "The Lecompte Maneuver for Relief of Airway Compression in Absent Pulmonary Valve Syndrome". The Annals of Thoracic Surgery. 81 (5): 1802–1807. doi:10.1016/j.athoracsur.2005.12.001. ISSN 0003-4975. PMID 16631676.
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