Plasma copeptin levels in children with pulmonary arterial hypertension associated with congenital heart disease.

The study aimed to evaluate the plasma copeptin levels in children with pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD), to assess the predictive value of plasma copeptin level for adverse outcomes, and to correlate its levels with various data in these patients. We included 25 children with PAH-CHD as group I and 25 children with CHD and no PAH as group II. Twenty-five healthy children of matched age and sex served as the control group. Patients were evaluated by echocardiography and right heart catheterization. The plasma level of copeptin was also measured. All patients were followed up for death or readmission for 1 year. Plasma copeptin levels were significantly higher in group I compared to group II and the control group and were correlated with increasing severity of PAH. The best cutoff of plasma copeptin level to predict poor prognosis in group I was ≥24.2 ng/ml with a sensitivity of 90% and a specificity of 80%. There was a statistically significant positive correlation between plasma copeptin levels and mean pulmonary pressure, pulmonary vascular resistance, and pulmonary blood flow, while there was a statistically significant negative correlation between plasma copeptin levels and right ventricular diastolic function.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 that copeptin is an accurate dependable biomarker for predicting poor outcomes in children with PAH-CHD who needed extensive further intervention.