Anne-Marie Koop

282 ABSTRACT Background Over the last decades, the population of patients with congenital heart disease (CHD) and pulmonary hypertension (PH) has increased due to improved treatment options. In many of these patients the right ventricle (RV) remains persistently exposed to pressure, volume or combined overload, leading to RV remodelling including fibrosis, hypertrophy, dilatation and eventually RV failure. Early recognition of such remodelling processes may enable preservation of cardiac function and functional status by targeted interventions. This study aimed to explore whether multi-biomarker assessment can help in early identification of RV remodelling and its functional consequences in children with CHD and PH. Methods One-hundred and twenty-five children (median age 9.6years (IQR 5.0-14.2), 53%male) with (a historyof) various types of RVoverload underwent clinical, echocardiographic, and laboratory assessment of eight selected blood-derived biomarkers, reflecting various remodelling processes. Results Blood-derived multi-biomarker profiles were associated with both type and severity of RV overload. N-terminal pro-B-Type natriuretic peptide (NT-proBNP) and endothelin-1 (ET-1) were increased in children with RV pressure overload compared to a reference group with no residual overload, whereas none of the biomarkers were increased in children with RV volume overload. High sensitive Troponin T, NT- proBNP, ET-1, Mid-Regional Pro-Adrenomedullin, myeloperoxidase, and growth differentiation factor-15 were associated with RV hypertrophy. NT-proBNP inversely correlated with systolic RV function. Conclusions This study outlines the concept of the use of multi-biomarker assessment for early recognition of RV remodelling and activation of molecular processes of RV adaptation in relation to type and degree of RV overload and function.