Pulmonary Vein Stenosis

Pulmonary vein stenosis (PVSPulmonary Vein Stenosis (PVS)) is a rare disease associated with high mortality. Clinically, it can be broadly categorized into primary PVSPrimary PVS or post repair PVSPulmonary Vein Stenosis (PVS). While childrenChildren can present with variable involvement of pulmonary veinsPulmonary veins, childrenChildren with 2 or more stenotic veins or bilateral PVSPulmonary Vein Stenosis (PVS) typically have a poor prognosis. ChildrenChildren with progressive disease develop secondary pulmonary hypertensionPulmonary hypertension and subsequent right heart failureRight heart failure. PrematurityPrematurity is a non-modifiable risk factor associated with PVSPulmonary Vein Stenosis (PVS). Multidisciplinary approach to treat disease includes surgery and catheter-based interventions (balloon angioplasty, stents). Experimental medications (Imatinib, sirolimus, losartan) have been used at some centers as adjuncts. 1 year survivalSurvival is approximately 60% for childrenChildren with progressive multivessel disease).