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.
Learning the basics about pulmonary vein stenosis—what it involves, how it develops and how it can be treated—can be a helpful first step in better understanding what to expect in the weeks and months to come.
- Pulmonary vein stenosis is a rare condition in which the veins that carry oxygen-rich blood from the lungs back to the heart are narrowed. This is a different condition than pulmonary (valve) stenosis (PVS, PS) and peripheral pulmonary stenosis.
- Pulmonary vein stenosis can occur in several forms. Many children have what is called intraluminal pulmonary vein stenosis, meaning that abnormal growth in connective tissue cells causes the walls in the pulmonary veins to become too thick.
- It is usually a progressive condition, meaning its severity and symptoms increase over time.
- Sometimes, pulmonary vein stenosis can occur as a complication of another heart or lung problem.
- The exact cause of pulmonary vein stenosis is not known.
- Some children will need many interventions—including interventional catheterization or surgery—to restore blood flow to the heart, as pulmonary vein stenosis tends to recur and even worsen over time.
- While a lung transplant does become a necessary treatment option for some children, not every child with the condition will need one.