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
Stenosis or Obstruction of Normal Pulmonary Venous Connections
Stenosis or Obstruction of Pulmonary Veins Following Surgical Repair of Anomalous Pulmonary Venous Connections
Symptoms and Quality of Life Associated with Pulmonary Venous Obstruction
Length of Life Associated with Pulmonary Venous Obstruction
Diagnostic Testing. Noninvasive
Medical Therapy to Prevent or Reverse the Onset of Disease. Efficacy or Lack of Efficacy
Medical Therapy to Prevent Progression of Disease. Efficacy or Lack of Efficacy
Medical Therapy to Prevent Recurrence of Disease after an Intervention. Efficacy or Lack of Efficacy
Catheter-mediated Interventions: Efficacy or Lack of Efficacy
Surgical Interventions for Pulmonary Venous Obstruction After the Onset of Disease
Year of Publication: 2021
Age Focus: Pediatric
Article Type: Review
Article Access: Free PDF File or Full Text Article Available Through PubMed or DOI: No