Treatment of Congenital and Acquired Pulmonary Vein Stenosis

Pulmonary vein stenosis (PVS) is a rare entity that until the last 2 decades was seen primarily in infants and children. Percutaneous and surgical interventions have limited success due to relentless restenosis, and mortality remains high. In adults, acquired PVS following ablation for atrial fibrillation has emerged as a new syndrome. This work will review these two entities with emphasis on current treatment.

Greater emphasis on understanding and addressing the mechanism of restenosis for congenital PVS has led to the use of drug-eluting stents (DES) and systemic drug therapy to target neo-intimal growth. Frequent reinterventions are positively affecting outcomes. Longer-term outcomes of percutaneous treatment for acquired PVS are emerging. Treatment of congenital PVS continues to be plagued by restenosis. DES show promise, but frequent reinterventions are required. Larger upstream vein diameter predicts success for congenital and acquired PVS interventions. Efforts to induce/maintain vessel growth are important for future treatment strategies.