Congenital and acquired pulmonary vein stenosis

Pulmonary vein stenosis is a rare condition with a bimodal age distribution. In pediatric patients the primary (ie, not associated with any preceding surgery) form of the disease may be related to inadequate embryological connections between the intrapulmonary venous system, the common pulmonary vein, and the left atrium. However, the stenosis is usually not static, and postnatal worsening of stenosis may be caused by abnormal proliferation of unusual myofibroblastic cells. It is not clear whether the same cell type may be involved in secondary pulmonary vein stenosis after surgical procedures that involve the pulmonary veins, such as in patients with anomalous pulmonary venous return. Recurrence of stenosis in both primary and secondary forms of pediatric pulmonary vein stenosis occurs in the majority of patients. Our current approach to most of these patients is to attempt surgical repair with the sutureless marsupialization procedure. Patients must then be followed carefully, and noninvasive imaging is usually adequate for screening. Catheter intervention is performed in veins with evidence of increasing stenosis. We generally perform high-pressure balloon dilation or cutting balloon dilation initially. Repeat procedures are frequently needed, but we have found that these repeat procedures may eventually slow the progress of restenosis. Placement of stents in the pulmonary veins of pediatric patients is usually considered only as a final mode of therapy before lung transplantation. Pulmonary vein stenosis in adult patients is now most commonly associated with prior radiofrequency ablation procedures for atrial fibrillation. Balloon angioplasty and stenting are reasonably successful in treating these patients. Repeat procedures are commonly needed, but aggressive intervention to prevent complete occlusion has resulted in good long-term clinical results. © 2007 American Heart Association, Inc.