Absent pulmonary valve syndrome

Last revised by David Luong on 12 Jul 2021

Absent pulmonary valve syndrome (APVS) also known as congenital absence of pulmonary valve or pulmonary valve agenesis is a rare cardiac outflow tract anomaly. 

It is characterized by a completely absent or rudimentary pulmonary valve. It can be associated with aneurysmal dilatation of the pulmonary arteries and compression of the tracheobronchial tree and in turn lead to significant respiratory compromise.

Two types have been described

Can show aneurysmal dilatation of pulmonary artery with some authors suggesting a bow-tie or balloon-like configuration of pulmonary arteries as an indicative feature 1. May also show massive regurgitation of the pulmonary valve +/- presence of a VSD and/or an overriding aorta. 

May allow direct visualization of a rudimentary or absent pulmonary valve, dilated main pulmonary artery with or without dilatation of its branches.

The prognosis is generally considered poor. Several surgical methods have been adopted which include 2:

It is thought to have been first described by Cheevers in 1847 1.

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Cases and figures

  • Case 1: with tetralogy of Fallot
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