Progress in Pulmonary Vein Stenosis: Lessons from Success in Treating Pulmonary Arterial Hypertension.

Pulmonary vein stenosis (PVS) is a rare and poorly understood condition that can be classified as primary, acquired, status-post surgical repair of PVS, and/or associated with developmental lung disease. Immunohistochemical studies demonstrate that obstruction of the large (extrapulmonary) pulmonary veins is associated with the neointimal proliferation of myofibroblasts. This rare disorder is likely multifactorial with a spectrum of pathobiology. Treatments have been historically surgical, with an increasing repetitive interventional approach. Understanding the biology of these disorders is in its infancy; thus, medical management has lagged behind. Throughout medical history, an increased understanding of the underlying biology of a disorder has led to significant improvements in care and outcomes. One example is the treatment of pulmonary arterial hypertension (PAH). PAH shares several common themes with PVS. These include the spectrum of disease and biological alterations, such as vascular remodeling and vasoconstriction. Over the past two decades, an exponential increase in the understanding of the pathobiology of PAH has led to a dramatic increase in medical therapies that have changed the landscape of the disease. We believe that a similar approach to PVS can generate novel medical therapeutic targets that will markedly improve the outcome of these vulnerable patients.