Interventions for Pulmonary Vein Stenosis.

ABSTRACT

Pulmonary vein stenosis (PVS) is a rare and unique disease of infants and young children. PVS is attended by high morbidity and mortality, and for many decades, effective therapy eluded the practitioner. However, in the most recent era, interventional techniques when employed in combination with systemic (primary) therapy have had a remarkable impact on outcomes in these at-risk children. Despite apparent complete relief of PVS in a discrete region of a pulmonary vein, stenosis reliably recurs and progresses. In this review, we discuss the current state-of-the-art interventional techniques, through the lens of our collective experiences and practices.