Ronald W. Day, Parker W. Clement, Aimee O. Hersh, Susan M. Connors, Kelli L. Sumner, D. Hunter Best, Mouied Alashari
University of Utah and Primary Children’s Hospital
United States
Respiratory Medicine Case Reports
Respir Med Case Rep 2017; 20: 82-86
DOI: 10.1016/j.rmcr.2016.12.007
Abstract
Pulmonary veno-occlusive disease and pulmonary capillary hemangiomatosis are rare forms of pulmonary vascular disease. We report two cases of affected children who had evidence of pulmonary hypertension 3-5 years before developing radiographic findings of pulmonary veno-occlusive disease or pulmonary capillary hemangiomatosis. Both patients experienced a moderate decrease in pulmonary arterial pressure during acute vasodilator testing. Both patients experienced an improvement in six-minute walk performance without an increase in pulmonary edema when treated with targeted therapy for pulmonary hypertension. In some patients, pulmonary veno-occlusive disease and pulmonary capillary hemangiomatosis may progress slowly over a period of months to years. A favorable acute vasodilator response may identify patients who will tolerate, and demonstrate transient clinical improvement with, medical therapy.
Category
Class 1. Pulmonary Veno-occlusive Disease and Pulmonary Capillary Hemangiomatosis
Class 1. Pulmonary Hypertension Associated with Connective Tissue 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: Yes