Background

Pulmonary vein stenosis (PVS) is a rare and complex progressive condition in children with high mortality. Pulmonary vein obstruction can be primary or secondary, and various etiologies for the secondary form include surgical scar, cor-triatriatum or other atrial membranes, baffle obstructions or pulmonary vein compression by the descending aorta or other dilated cardiac structures. Many of these types of PVS are amenable to surgery and/or catheter-based interventions and subsequently remain stable. The more worrisome disease is recurring and progressive and is often relentless and fatal, despite aggressive forms of treatment including surgical treatment, various types of stents, cutting balloon angioplasty, and lung transplantation. Our objectives are to review the current literature on obstructive pulmonary vein disease, provide descriptors of progressive PVS and summarize the outcomes associated with treatment of PVS.