Anne-Marie Koop

8 295 Timely identification of type and severity of RV pressure overload and the adaptive status of the RV is believed to be of important in pursuing preservation of functional status. Pressure overload is a debilitating condition for the thin walled RV and is known to induce rapid deterioration of RV function, especially in acute onset or severe pressure overload. The current study shows that in children with CHD, NT-proBNP is a marker of RV pressure overload and its severity. NT-proBNP is released by cardiomyocytes due to myocardial stretch, resulting in RAAS activation with increased natriuresis, diuresis and vasodilation, and widely used as a marker of cardiac decompensation. The current study assessed NT-proBNP as part of the multi-biomarker panel and showed that NT-proBNPwas highly affected by pressure overload compared to other biomarkers. The progressively increased plasma levels in RV pressure overload were also found for ET-1 and reflect a state of progressive inotropic andmitogenic abilities. Increased plasma levels of ET-1 have been related to PAH and ET-1 has been shown to be involved in the pathogenesis of this disease. 36,37 In the current study ET-1 was increased predominantly in patients with PAH (data not shown), indicating that increased ET-1 plasma levels in children with CHD may increase awareness for the presence of PAH. In addition to a measure for the severity of RV pressure load, NT-proBNP can serve as a surrogate of systolic RV function, as demonstrated by its correlation with TAPSE, that was independent of age and sex. This is in line with studies in patients with TOF, a systemic RV, and PH. 38–41 If hsTnT is increased simultaneously, this indicates severe RV pressure overload accompanied with cell decay, a consideration that may direct to expeditious interventions. In adults with PAH due to CHD, increased plasma levels of cardiac troponin are shown to be associated with poor outcome 42,43 and hsTnT has been identified as a marker for risk stratification. 44 To our knowledge, we are the first to explicitly relate cardiac troponin to the degree of RV pressure overload in children with CHD.