270: Effects of advanced therapies on echocardiographic and Doppler measures, compared with clinical evaluation and BNP in patients with Eisenmenger syndrome

Abstract

Management options for patients with Eisenmenger syndrome (ES) were limited to palliative measures or transplantation before the advent of advanced therapies (AT). Little data is available on the impact of disease targeting therapies on BNP and echocardiographic variables in patients with ES. We wanted to investigate the effects of AT on echocardiographic measures and BNP in patients with ES. We prospectively included 63 patients with ES who were started on AT (46% were treated with endothelin receptor antagonists, 44.4% with PDE5 inhibitors and 9.6% with an association. Clinical, 6 min walk test and BNP data were collected at baseline (before introduction of AT) and after a mean period of 2years of sustained therapy. Mean age was 42±13 years, most of patients were WHO III (97%) whereas a vast majority had post-tricuspid defects. At baseline, echocardiographic findings were consistent with abnormalities of the RV function and adaptation: overall dilated RV (mean inlet 44.9±6.2 mm), reduced TAPSE (16.4± 3.5 mm). AT was responsible for an improvement in WHO functional class (p=0.001) and walked distance (+35.1 m; p=0.01), however, it was not associated with any change in BNP (p=0.85). RV systolic function and adaptation was improved (decreased systolic/diastolic duration ratio and total isovolumic time, p<0.001 and p=0.003; decreased RV dP/dT, p=0.05; increased TAPSE and tricuspid Sm, p=0.01 and p=0.03), however, no significant right ventricular structural remodeling was observed. Agreement between the echocardiographic score (previously described as associated with outcomes in ES: reduction in TAPSE, RA area, RA/LA ratio or S/D ratio) and clinical response (decrease in WHO and improved 6MWT) was moderate but higher than BNP. Our results support the notion that therapy is associated with improved symptoms, RV systolic function and adaptation but not with reduction in BNP concentrations