Cassandra Campbell, Kawaljeet Singh, Mark Weems, Panjit Philip
University of Tennessee Health Science Center.
United States
British Medical Journals Case Reports
BMJ Case Rep 2025; 18:
DOI: 10.1136/bcr-2024-263731
Abstract
A term, healthy infant presented with respiratory distress and severe pulmonary hypertension (PH). With an unclear aetiology and the intent to decrease right ventricular afterload, pulmonary vasodilators were initiated. Follow-up imaging revealed a supravalvular mitral ring as the cause of the PH which resolved after surgical resection of the membrane. Supravalvular mitral ring is a rare left-side congenital heart disease lesion that can lead to PH usually between infancy and toddler years, but can be easily missed in the setting of a concurrent large atrial septal defect that masks the gradient across the left ventricular inflow. While bronchopulmonary dysplasia is the most common cause of PH in infancy, a thorough evaluation is critical for the diagnosis of this reversible cause of PH as a missed diagnosis may redirect care with pulmonary vasodilators in the acute setting and potentially worsen the clinical status.
Category
Class II. Pulmonary Hypertension Associated with Valvular Disease of the Left Side of the Heart
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