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Conclusion Plasma copeptin levels are elevated in children with PAH-CHD and found to be a good predictive marker for the severity of PAH and poor prognosis in these children. What is Known •PH is a life-threatening condition that can lead to right ventricular failure and death. •We need a non-invasive easy biomarker that can identify PH children with unfavorable prognosis who needed further intervention. What is New •It is the first study that assessed the prognostic value of plasma copeptin levels in children with PAH-CHD. •We found t