Pulmonary Venous Anomalies and Interventions

Pulmonary venous anomalies encompass a spectrum of congenital and acquired alterations in the anatomy or calibre of the veins conveying oxygenated blood from the lungs to the left atrium. These anomalies range from obstructive lesions, such as pulmonary vein stenosis, to malpositioned connections exemplified by total anomalous pulmonary venous connection (TAPVC) and its variant Scimitar syndrome. Clinically, they present with pulmonary hypertension, right ventricular strain and impaired oxygenation, often in early life. Management strategies are multidisciplinary, combining advances in imaging, surgical correction, catheter‐based therapies and emerging pharmacological interventions. Sutureless techniques and stent placement aim to minimise restenosis, while animal models and translational studies are refining our understanding of pathophysiology and therapeutic targets. The global burden of these conditions has driven innovation in resource-tailored diagnostic pathways and postoperative care, improving survival and functional outcomes. Continued research is bridging the gap between molecular mechanisms and clinical practice, offering hope for personalised interventions in high-risk paediatric and adult populations.

Research from Nature Portfolio

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Pulmonary Venous Anomalies and Interventions publication trend

The graph below shows the total number of articles in pulmonary venous anomalies and interventions across all publications each year (not limited to Nature Index journals).

Technical terms

Pulmonary vein stenosis (PVS): Pathological narrowing of one or more pulmonary veins, leading to impaired venous return and secondary pulmonary hypertension.

Total anomalous pulmonary venous connection (TAPVC): A congenital defect in which all pulmonary veins drain into the systemic venous circulation instead of the left atrium.

Scimitar syndrome: A form of partial anomalous pulmonary venous connection characterised by a curvilinear “Scimitar” vein draining the right lung and associated cardiopulmonary anomalies.

Bronchopulmonary dysplasia (BPD): A chronic lung disease in premature infants marked by arrested alveolar development and inflammation, contributing to vascular remodelling.

Sutureless repair: A surgical technique that reconstructs the pulmonary venous pathway without direct suturing of vein tissue, reducing postoperative restenosis.

References

1. A neonatal rat model of pulmonary vein stenosis. Cell & Bioscience (2023).
2. Prematurity and Pulmonary Vein Stenosis: The Role of Parenchymal Lung Disease and Pulmonary Vascular Disease. Children (2022).
3. Treatment and prognosis of Scimitar syndrome: A retrospective analysis in a single center of East China. Frontiers in Cardiovascular Medicine (2022).
4. Comparison of conventional and primary sutureless surgery for repairing supracardiac total anomalous pulmonary venous drainage. Journal of Cardiothoracic Surgery (2019).