Tilman Humpl, Jeffrey Fineman, Athar M. Qureshi
University Children’s Hospital Berne. University of California, San Francisco. Texas Children’s Hospital and Baylor College of Medicine.
Switzerland and United States
Pediatric Pulmonology
Pediatr Pulmonol 2021; 56: 649-655
DOI: 10.1002/ppul.24848
Abstract
Pulmonary vein stenosis is a rare and poorly understood condition causing obstruction of the large pulmonary veins and of blood flow from the lungs to the left atrium. This results in elevated pulmonary venous pressure and pulmonary edema, pulmonary hypertension, potentially cardiac failure, and death. Clinical signs of the disease include failure to thrive, increasingly severe dyspnea, hemoptysis, respiratory difficulty, recurrent respiratory tract infections/pneumonia, cyanosis, and subcostal retractions. On chest radiograph, the most frequent finding is increased interstitial, ground-glass and/or reticular opacity. Transthoracic echocardiography with pulsed Doppler delineates the stenosis, magnetic resonance imaging and multislice computerized tomography are used for further evaluation. Interventional cardiac catherization, surgical techniques, and medical therapies have been used with varying success as treatment options.
Category
Review Articles Concerning Pulmonary Vascular Disease
Class II. Pulmonary Hypertension Associated with Pulmonary Vein Stenosis
Diagnostic Testing for Pulmonary Vascular Disease. Non-invasive Testing
Surgical and Catheter-mediated Interventions for Pulmonary Vascular Disease
Age Focus: Pediatric Pulmonary Vascular Disease
Fresh or Filed Publication: Filed (PHiled). Greater than 1-2 years since publication
Article Access
Free PDF File or Full Text Article Available Through PubMed or DOI: No