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Pathogenesis, Evaluation, and Management of Pulmon ...
Article: Pathogenesis, Evaluation, and Management ...
Article: Pathogenesis, Evaluation, and Management of Pulmonary Vein Stenosis: JACC Review Topic of the Week
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Pulmonary vein stenosis (PVS) can result from congenital, acquired, or iatrogenic causes and often presents with symptoms like dyspnea, hemoptysis, chest discomfort, and cough. PVS typically goes unrecognized for long periods due to subtle onset and overlap with other conditions, necessitating high clinical suspicion and dedicated diagnostic strategies for timely intervention and optimal management.<br /><br />PVS can result from external compression or internal narrowing, often classified by severity as mild, moderate, or severe. Symptoms correlate with the degree of stenosis and the number of affected veins. Severe stenoses can lead to exercise-induced pulmonary hypertension and reduced pulmonary perfusion, potentially resulting in pulmonary infarction.<br /><br />Noninvasive diagnostic methods include computed tomography angiography (CTA) and magnetic resonance angiography (MRA), which offer detailed anatomical visualization. Ventilation/perfusion (V/Q) scanning and echocardiography (including transesophageal echocardiography, TEE) help assess functional impairments. Invasive evaluations, such as right heart catheterization and selective pulmonary vein assessment, provide critical hemodynamic data and guide interventional decisions.<br /><br />Management of PVS involves addressing underlying reversible pathologies, followed by interventional techniques like balloon angioplasty and stent placement, especially for severe, persistent stenoses. Stents, particularly those used for peripheral/biliary applications, have shown improved patency and reduced restenosis compared to balloon angioplasty alone.<br /><br />Post-intervention, patients typically require follow-up with CTA at regular intervals to monitor for in-stent restenosis (ISR) or thrombus formation. Medical management includes dual antiplatelet therapy shortly after the intervention, followed by monotherapy or anticoagulation as indicated by clinical guidelines. Long-term outcomes are enhanced by evolving interventional techniques and medical therapies.<br /><br />Further study is necessary to optimize management strategies, including the role of newer diagnostic technologies like photon-counting CT systems and pharmacotherapies like drug-coated balloons and systemic sirolimus. The review underscores the importance of comprehensive evaluation and individualized treatment to improve outcomes for patients with PVS.
Keywords
Pulmonary vein stenosis
PVS symptoms
diagnostic methods
computed tomography angiography
magnetic resonance angiography
balloon angioplasty
stent placement
in-stent restenosis
dual antiplatelet therapy
photon-counting CT
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