Pulmonary Vein Stenosis: A Review

Pulmonary vein stenosis (PVS) is an extremely rare and lethal disease caused by multiple etiologies. PVS has a bimodal distribution in the population, affecting children and adults. Congenital PVS is the usual PVS type in children, which sometimes develops after cardiothoracic surgery. Acquired PVS, in turn, is the most common PVS type in adults. A review of the relevant literature has shown that PVS after radiofrequency ablation of atrial fibrillation is the most common, as well as that caused by compression of proliferative fibrous tissues or tumor in the mediastinum (eg, PVS caused by fibrosing mediastinitis, lung tumors, metastases, etc). This article provides a comprehensive review of PVS in terms of embryology and anatomy, etiology and triggers, classification, clinical symptoms and signs, treatment, and prognosis, intending to promote the understanding and treatment of this disease.