Absent pulmonary valve syndrome (APVS) also known as congenital absence of pulmonary valve or pulmonary valve agenesis is a rare cardiac outflow tract anomaly.
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Pathology
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.
Subtypes
Two types have been described
Fallot type - associated with tetralogy of Fallot
non-Fallot type
Associations
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other congenital cardiac syndromes including
interrupted aortic arch - type B
pulmonary branching abnormalities
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syndromes
Radiographic features
Ultrasound
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.
CT/MRI
May allow direct visualization of a rudimentary or absent pulmonary valve, dilated main pulmonary artery with or without dilatation of its branches.
Treatment and prognosis
The prognosis is generally considered poor. Several surgical methods have been adopted which include 2:
replacement with a valved conduit
replacement with a monocusp valve
valveless repair
History and etymology
It is thought to have been first described by Cheevers in 1847 1.
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