Pathogenesis, Evaluation, and Management of Pulmonary Vein Stenosis

Pulmonary vein stenosis (PVS) can arise from several etiologies, including congenital, acquired, and iatrogenic sources. PVS presents insidiously, leading to significant delays in diagnosis. A high index of suspicion and dedicated noninvasive evaluation are key to diagnosis. Once diagnosed, both noninvasive and invasive evaluation may afford further insights into the relative contribution of PVS to symptoms. Treatment of underlying reversible pathologies coupled with trans-catheter balloon angioplasty and stenting for persistent severe stenoses are established approaches. Ongoing refine-ments in diagnostic modalities, interventional approaches, postintervention monitoring, and medical therapies hold promise to further improve patient outcomes. (J Am Coll Cardiol 2023;81:2361-2373) & COPY; 2023 by the American College of Cardiology Foundation.