Victoria Sokoliuk, James A. DiNardo, Morgan L. Brown
Boston Children’s Hospital.
United States
A and A Practice
A A Pract 2019; 12: 205-207
DOI: 10.1213/XAA.0000000000000885
Abstract
Pulmonary vein stenosis (PVS) is a progressive disease with pulmonary hypertension (PH) as a major cause of morbidity and mortality. Traditional management of PH with inhaled nitric oxide (iNO) is typically avoided in PVS patients because, while iNO may reduce pulmonary vascular resistance, PH persists as pulmonary blood flow increases in the presence of a downstream resistive lesion. We report 3 cases with primary PVS and PH in which iNO was used to successfully decrease mean pulmonary artery pressures with clinical improvement. Based on this experience, we suggest that iNO can be used to treat PH in select patients with PVS.
Category
Class II. Pulmonary Hypertension Associated with Pulmonary Vein Stenosis
Medical Therapy. Efficacy or Lack of Efficacy
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