Absent pulmonary valve syndrome

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Absent pulmonary valve syndrome
Other names: Congenital absence of the pulmonary valve
Prenatal ultrasonographic findings of absent pulmonary valve syndrome a) Marked dilatation of main and branch pulmonary arteries b-d) color and pulsed wave Doppler ultrasound shows to-and-fro pattern of pulmonary stenosis and regurgitation

Absent pulmonary valve syndrome is a congenital heart defect that occurs when the flaps of the pulmonary valve do not develop.[1] It presents at birth with breathing problems and a blue appearance.[1]

There is (hypoplasia) resulting in aneurysms (dilation) of the pulmonary arteries and softening of the trachea and bronchi (tracheobronchomalacia).

It is frequently associated with ventricular septal defect, with around 3% to 6% having Tetralogy of Fallot.[1][2]


  1. 1.0 1.1 1.2 "Absent pulmonary valve: MedlinePlus Medical Encyclopedia". medlineplus.gov. Archived from the original on 17 June 2022. Retrieved 27 October 2022.
  2. Ramaswamy, Prema (28 December 2020). "Tetralogy of Fallot With Absent Pulmonary Valve: Background, Pathophysiology, Etiology". Archived from the original on 31 August 2022. Retrieved 27 October 2022.