Cardiopulmonary exercise testing, specifically the oxygen pulse curve, identified disease progression and guided medication management in 2 pediatric patients with CHD-associated PAH.
Case Report (n=2)
The O2 pulse curve during cardiopulmonary exercise testing may serve as a useful marker of disease progression in pediatric patients with CHD-associated PAH.
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 (O 2 pulse) 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 O 2 pulse 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 O 2 pulse 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.
VanderWilde et al. (2026) conducted a case report in Repaired congenital heart disease and CHD-associated pulmonary arterial hypertension (n=2). Cardiopulmonary exercise testing was evaluated on Peak oxygen consumption and oxygen pulse curve patterns. Cardiopulmonary exercise testing, specifically the oxygen pulse curve, identified disease progression and guided medication management in 2 pediatric patients with CHD-associated PAH.