The potential metabolic biomarkers of lactate, threonine and alanine were well correlated with NT-proBNP, while lactate and threonine were also well correlated with mPAP and PVR, suggesting that the metabolic disorders are parallel with the disease severity, and these potential metabolic biomarkers may have diagnostic and prognostic values for the clinical management of patients with PAH-CHD. Here, NPPB is linked to metabolic disease.