Sacubitil/valsartan in systemic right ventricular failure

Background In patients with heart failure with low ejection fraction (EF) (≤35%) sacubitril/valsartan is proven to be beneficial. We aimed to assess the effects of sacubitril/valsartan in adult congenital heart disease patients with failing systemic right ventricle (sRV) in the setting of congenitally corrected transposition of the great arteries (TGA) or TGA after atrial switch. Purpose To evaluate the feasibility and effect of treatment with sacubitril/valsartan in patients with heart failure in the setting of a sRV on optimal medical heart failure treatment. Methods Data of patients with sRV failure (estimated RVEF ≤35%) treated with sacubitril/valsartan were analyzed. Patients were clinically evaluated at baseline and at six months after initiation of the highest tolerated dose. Results Fourteen patients completed six-month follow-up thus far, of which 6 female (42.8%) and 2 (14.3%) with congenitally corrected transposition of the great arteries (TGA). After 6 months, 9 patients (64.3%) were using the highest dose (97/103 mg twice daily) of sacubitril/valsartan. NT-proBNP was significantly lower after 3 months and after 6 months compared with baseline (64% of baseline, p=0.006 and 53% of baseline, p=0.001, respectively). The kidney function did not change significantly (creatinine at baseline 83.6±12.0 and at 6 months 88.5±13.87, p=0.141) and serum potassium remained within normal limits (4.28±0.36 and 4.49±0.30, p=0.007). There was no significant decrease in systolic blood pressure (p=0.960). The echocardiographic sRV fractional area change (FAC) improved (p=0.002) and Global Longitudinal Strain (GLS) showed a trend towards improvement (p=0.058). During the 6 months of treatment, the exercise capacity remained stable, there was no increase in diuretic use, no serious adverse events and no heart failure related admissions occurred. Conclusion Treatment of sRV heart failure patients with sacubitril/valsartan leads to a significant reduction in NT-proBNP and significant increase in FAC. All patients remained clinically stable and no heart failure related admissions or serious adverse events occurred. Changes after 6 months Funding Acknowledgement Type of funding source: None