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(Circulation. 2007;115:103-108.)
© 2007 American Heart Association, Inc.

Contemporary Reviews in Cardiovascular Medicine

Congenital and Acquired Pulmonary Vein Stenosis

Larry A. Latson, MD; Lourdes R. Prieto, MD

From the Department of Pediatric Cardiology and Congenital Heart Diseases, Cleveland Clinic Foundation, Cleveland, Ohio.

Correspondence to Dr Larry A. Latson, Cleveland Clinic Foundation, Department of Pediatric Cardiology and Congenital Heart Diseases, 9500 Euclid Ave, M41, Cleveland, OH 44195. E-mail latsonl@ccf.org

Key Words: veins, anomalous pulmonary • heart diseases, congenital • pulmonary heart disease • catheter ablation • pulmonary veins, surgery • pulmonary veins, pathology

An extract of the first 250 words of the full text is provided, because this article has no abstract.

	Introduction

 
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.

	Embryology and Anatomy of the Pulmonary Veins

 
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. . . . [Full Text of this Article]

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