Pulmonary vein stenosis: future optimism

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.

Intervention for multivessel pediatric PVS may be surgical or transcatheter, both with high restenosis rates. At a threshold upstream diameter of 7 mm, 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.

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.