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 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
- Fallot type - associated with tetralogy of Fallot
- non-Fallot type
- tetralogy of Fallot
- other congenital cardiac syndromes including
Can show aneurysmal dilatation of pulmonary artery with some authors suggest 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 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.
- 1 .Grewal DS, Chamoli SC, Saxena S. Absent pulmonary valve syndrome - Antenatal diagnosis. (2014) Medical journal, Armed Forces India. 70 (2): 198-200. doi:10.1016/j.mjafi.2013.07.002 - Pubmed
- 2. Yong M et.al, Long-term outcomes of patients with absent pulmonary valve syndrome: 38 years of experience. (2014) The Annals of thoracic surgery. doi:10.1016/j.athoracsur.2014.01.035 - Pubmed
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- 4. Nørgaard, Martin A., Alphonso, Nelson, Newcomb, Andrew E., Brizard, Christian P., Cochrane, Andrew D.. Absent pulmonary valve syndromeSurgical and clinical outcome with long-term follow-up. (2006) European Journal of Cardio-Thoracic Surgery. 29 (5): 682. doi:10.1016/j.ejcts.2006.01.050
- 5. Farhood Saremi, Atul Gera, S. Yen Ho, Ziyad M. Hijazi, Damián Sánchez-Quintana. CT and MR Imaging of the Pulmonary Valve. (2014) RadioGraphics. doi:10.1148/rg.341135026
- 6. Joshi A, et.al Prenatal diagnosis of absent pulmonary valve syndrome: report of 2 cases, most common and most rare presentations. (2010) Journal of ultrasound in medicine : official journal of the American Institute of Ultrasound in Medicine. doi:10.7863/jum.2010.29.5.823 - Pubmed
- 7. Zhang W et.al. Prenatal ultrasonic diagnosis of absent pulmonary valve syndrome: A case report. (2017) Medicine. doi:10.1097/MD.0000000000007747 - Pubmed