Allegra J. VanderWilde, Kimberley G. Miles, Kristian C. Becker, Paul J. Critser, Michelle Cash, Melissa Magness, Wayne A. Mays, Russel Hirsch, Adam W. Powell
University of Cincinnati and Cincinnati Children’s Hospital Medical Center. Nemours Children’s Health.
United States
Case Reports in Pediatrics
Case Rep Pediatr 2026;
DOI: 10.1155/crpe/6697634
Abstract
Two patients with repaired congenital heart disease (CHD) and CHD-associated pulmonary arterial hypertension (PAH) are presented to illustrate the utility of cardiopulmonary exercise testing in this population. Novel markers used include the oxygen pulse curve (O2pulse) as an initial marker of PAH-CHD disease progression. Both patients had an abrupt decrease in their peak oxygen consumption on serial exercise testing. In addition to the decrease in the peak oxygen consumption, Patient 1 also had a fall in the O2pulse curve during progressive exercise and an episode of concerning symptoms, prompting an alteration medication management. Patient 2 had a similar drop in peak oxygen consumption without a fall in the O2pulse curve, and this patient remained stable on their current medical therapies. The overall aim of this case study is to highlight the utility of cardiopulmonary exercise testing in patients with CHD-associated PAH and showcase the utility of exercise testing in optimizing clinical management.
Category
Class I. Pulmonary Hypertension Associated with Congenital Cardiovascular Disease
Diagnostic Testing for Pulmonary Vascular Disease. Non-invasive Testing
Diagnostic Testing for Pulmonary Vascular Disease. Risk Stratification
Age Focus: Pediatric Pulmonary Vascular Disease
Fresh or Filed Publication: Fresh (PHresh). Less than 1-2 years since publication
Article Access
Free PDF File or Full Text Article Available Through PubMed or DOI: Yes