Congenital and Acquired Pulmonary Vein Stenosis

Pulmonary vein stenosis is a fascinating yet frustrating and difficult to manage condition with an exceptionally high mortality rate. Until recently, the disease was seen almost exclusively in young children with or without various forms of congenital heart disease. Pulmonary vein stenosis is a relatively rare condition. In most published series from large centers, there has been an average of ≈2 or 3 cases per year that require treatment. Pulmonary vein stenosis in the adult population is even more rare, and the small number of reported cases has often been associated with mediastinal processes such as neoplasms or fibrosing mediastinitis. However, with the advent of aggressive treatment strategies for atrial fibrillation, we have seen a new group of pulmonary vein stenosis patients. The stenosis appears as a complication of radiofrequency ablation procedures around the pulmonary veins. Small series of new surgical and interventional catheterization procedures for treatment of both the pediatric and adult forms of pulmonary vein stenosis suggest an improving prognosis in centers with specialized expertise. However, the prognosis of patients affected with pulmonary vein stenosis remains guarded and requires diligent follow-up and often repeated procedures. The purpose of this article is to review concepts of causation and possible treatments for this rare but serious condition as they evolve.The left atrium and pulmonary veins initially develop separately in the 3- to 5-mm embryo (25 to 27 days gestation).1 The primordial pulmonary venous system is part of the splanchnic plexus, which initially connects to the cardinal and umbilicovitelline veins. At 27 to 29 days gestation, a small endothelial outgrowth from the posterior superior wall of the primordial left atrium develops just to the left of the developing septum primum. At 28 to 30 days gestation, this common pulmonary venous out-pouching engages the pulmonary venous portion of the splanchnic …