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
- tetralogy of Fallot
- other congenital cardiac syndromes including
- interrupted aortic arch - type B
- transposition of the great arteries
- pulmonary branching abnormalities
- atrioventricular septal defect (ASD)
- persistent ductus arteriosus
- agenesis of ductus arteriosus
- syndromes
Radiographic features
Antenatal 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
- pulmonary artery reduction
- Lecompte maneuver
History and etymology
It is thought to have been first described by Cheevers in 1847 1.