Management and Outcomes of Absent Pulmonary Valve Syndrome

Absent pulmonary valve syndrome is a rare congenital cardiac anomaly characterised by failure of pulmonary valve development, resulting in severe regurgitation and aneurysmal dilatation of the pulmonary arteries. It is most commonly seen in combination with tetralogy of Fallot, leading to right ventricular volume overload, tracheobronchial compression and respiratory compromise. Management begins with prenatal detection and multidisciplinary perinatal planning, followed by stabilisation of respiratory function and timely surgical reconstruction of the right ventricular outflow tract. Surgical strategies focus on reducing pulmonary artery size, relieving bronchial compression and restoring valve competence, often using valved conduits or pulmonary arterioplasty. Early survival rates have improved markedly, yet reinterventions for conduit stenosis or residual regurgitation are frequent. Longitudinal follow-up emphasises assessment of right ventricular function, exercise capacity and airway integrity. Collaborative registries and evolving imaging modalities continue to refine risk stratification and optimise timing of intervention.

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Management and Outcomes of Absent Pulmonary Valve Syndrome publication trend

The graph below shows the total number of articles in management and outcomes of absent pulmonary valve syndrome across all publications each year (not limited to Nature Index journals).

Technical terms

Absent pulmonary valve syndrome (APVS): Congenital defect in which the pulmonary valve leaflets are missing or underdeveloped, causing severe regurgitation and dilated pulmonary arteries.

Tetralogy of Fallot (TOF): A combination of four heart defects—ventricular septal defect, pulmonary stenosis, overriding aorta and right ventricular hypertrophy—often associated with APVS.

Pulmonary arterioplasty: Surgical reduction or reshaping of dilated pulmonary arteries to alleviate airway compression and improve blood flow.

Intact ventricular septum (IVS): A variant of APVS in which the interventricular septum remains closed, altering surgical strategy and prognosis.

Bronchomalacia: Weakening of the bronchial walls leading to airway collapse, frequently secondary to external compression by enlarged pulmonary arteries.

References

1. Management and outcome of prenatal absent pulmonary valve syndrome. Archives of Gynecology and Obstetrics (2022).
2. A rare case of a neonate with fallot-type absent pulmonary valve and occlusion of the left main bronchus. Journal of Cardiothoracic Surgery (2024).
3. Outcomes following surgical repair of absent pulmonary valve syndrome: 30 years of experience from a Swedish tertiary referral centre. Interdisciplinary CardioVascular and Thoracic Surgery (2022).