Peer-review started: June 2, 2015
First decision: August 22, 2015
Revised: September 5, 2015
Accepted: November 23, 2015
Article in press: November 25, 2015
Published online: January 26, 2016
Pulmonary vein stenosis (PVS) is rare condition characterized by a challenging diagnosis and unfavorable prognosis at advance stages. At present, injury from radiofrequency ablation for atrial fibrillation has become the main cause of the disease. PVS is characterized by a progressive lumen size reduction of one or more pulmonary veins that, when hemodynamically significant, may raise lobar capillary pressure leading to signs and symptoms such as shortness of breath, cough, and hemoptysis. Image techniques (transesophageal echocardiography, computed tomography, magnetic resonance and perfusion imaging) are essential to reach a final diagnosis and decide an appropriate therapy. In this regard, series from referral centers have shown that surgical and transcatheter interventions may improve prognosis. The purpose of this article is to review the etiology, assessment and management of PVS.
Core tip: Several papers in literature focus either on the causes, diagnosis or treatment of pulmonary vein stenosis. However this is simple yet complete and updated review of all these matters that may guide physician’s decision making when facing a suspected or confirmed case of this unusual disease.