Hong-Da Zhang, Yi Yan, Yang-Yang He, Qian-Qian Liu, Fu-Hua Peng, Xin-Xin Yan, Zhi-Yan Han
Fuwai Hospital and Chinese Academy of Medical Sciences & Peking Union Medical College. Shanghai Children’s Medical Center, National Children’s Medical Center and Shanghai Jiaotong University School of Medicine. Henan University.
China
International Journal of Cardiology
Int J Cardiol 2023; 375: 29-35
DOI: 10.1016/j.ijcard.2022.12.033
Abstract
Background: Exercise intolerance is a major manifestation of pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD). We aimed to investigate the characteristics of exercise intolerance in different subgroups of PAH-CHD.
Methods: We retrospectively enrolled 171 adult patients with PAH-CHD and 30 age and sex-matched healthy subjects and performed cardiopulmonary exercise testing. Gas exchange parameters, including peak oxygen uptake (peak V̇o2), anaerobic threshold, and the slope of ventilatory equivalent for carbon dioxide (V̇e/V̇co2 slope), were recorded.
Results: The median age of patients at enrollment was 27.8 years, and 131 (76.6%) were female. Peak V̇o2 was reduced in patients compared to healthy controls (median, 14.8 ml/kg/min versus 26.9 ml/kg/min, p < 0.001). Of all 171 patients, 60 (35.1%) had Eisenmenger syndrome, 35 (20.5%) had PAH associated with systemic-to-pulmonary shunts (PAH-SP), 39 (22.8%) had PAH with small defects (PAH-SD), and 37 (21.6%) had PAH after cardiac defect correction (PAH-CD). Patients with Eisenmenger syndrome had the lowest peak V̇o2 (p = 0.003) and the highest V̇e/V̇co2 slope (p = 0.012), compared with other patients, representing the worst exercise capacity and ventilatory efficiency. Patients with PAH-SP had the best exercise capacity among the four groups, indicated by the highest peak V̇o2 (p = 0.003) compared with other patients. Peak V̇o2 was negatively correlated with pulmonary vascular resistance (r = -0.411, p < 0.001).
Conclusions: Exercise capacity was severely reduced in patients with PAH-CHD. Among the four subgroups, patients with Eisenmenger syndrome had the worst exercise capacity and ventilatory efficiency.
Category
Class I. Pulmonary Hypertension Associated with Congenital Cardiovascular Disease
Pulmonary Hypertension Associated with Exercise
Diagnostic Testing for Pulmonary Vascular Disease. Non-invasive Testing
Symptoms and Findings Associated with Pulmonary Vascular Disease
Age Focus: Adult Pulmonary Vascular Disease
Fresh or Filed Publication: Filed (PHiled). Greater than 1-2 years since publication
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