Pulmonary vein stenosis: future optimism
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Issued Date
2025-01-01
Resource Type
ISSN
02684705
eISSN
15317080
Scopus ID
2-s2.0-105004277373
Journal Title
Current Opinion in Cardiology
Rights Holder(s)
SCOPUS
Bibliographic Citation
Current Opinion in Cardiology (2025)
Suggested Citation
Suntharos P., Satawiriya M., Prieto L.R. Pulmonary vein stenosis: future optimism. Current Opinion in Cardiology (2025). doi:10.1097/HCO.0000000000001217 Retrieved from: https://repository.li.mahidol.ac.th/handle/123456789/110079
Title
Pulmonary vein stenosis: future optimism
Author(s)
Author's Affiliation
Corresponding Author(s)
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Abstract
Purpose of review Pulmonary vein stenosis (PVS) is a rare disease with high morbidity and mortality. Prevention of restenosis remains challenging. This review will highlight recent advances in therapy that are beginning to show a survival benefit. Recent findings Intervention for multivessel pediatric PVS may be surgical or transcatheter, both with high restenosis rates. At a threshold upstream diameter of 7mm, the risk of restenosis decreases. Close vigilance and frequent reinterventions, typically transcatheter, are now accepted practice to maintain vein patency and achieve upstream growth. Suppressive agents targeting the exuberant myofibroblastic proliferation characteristic of PVS, specifically sirolimus, delivered locally on the surface of balloons and stents, and as adjunct systemic therapy, have been shown to increase survival and decrease reinterventions. Newer surgical techniques focused on shortening and straightening the vein to optimize flow dynamics, coupled with hybrid intraoperative stent placement in selected cases, also show a survival benefit. Adult-onset PVS, most commonly a complication of pulmonary vein isolation, now occurs rarely, and generally responds to transcatheter intervention. Further advances in ablation techniques aim to eliminate this complication. Summary An aggressive approach of frequent reinterventions is a necessary strategy rather than treatment failure. More granular understanding of the mechanisms underlying PVS leading to novel muti-pronged anatomic and suppressive therapy are yielding improved survival. Multispecialty PVS teams at the institutional level and multiinstitutional collaboration, now possible via the PVS registry, are crucial to optimal care and future progress.