Sacubitril/valsartan in the treatment of systemic right ventricular failure

Tjitske E Zandstra, Marieke Nederend, Monique R M Jongbloed, Philippine Kiès, Hubert W Vliegen, Berto J Bouma, Laurens F Tops, Martin J Schalij, Anastasia D Egorova, Tjitske E Zandstra, Marieke Nederend, Monique R M Jongbloed, Philippine Kiès, Hubert W Vliegen, Berto J Bouma, Laurens F Tops, Martin J Schalij, Anastasia D Egorova

Abstract

Objective: Pharmacological options for patients with a failing systemic right ventricle (RV) in the context of transposition of the great arteries (TGA) after atrial switch or congenitally corrected TGA (ccTGA) are not well defined. This study aims to investigate the feasibility and effects of sacubitril/valsartan treatment in a single-centre cohort of patients.

Methods: Data on all consecutive adult patients (n=20, mean age 46 years, 50% women) with a failing systemic RV in a biventricular circulation treated with sacubitril/valsartan in our centre are reported. Patients with a systemic RV ejection fraction of ≤35% who were symptomatic despite treatment with β-blocker and ACE-inhibitor/angiotensin II receptor-blockers were started on sacubitril/valsartan. This cohort underwent structural follow-up including echocardiography, exercise testing, laboratory investigations and quality of life (QOL) assessment.

Results: Six-month follow-up data were available in 18 out of 20 patients, including 12 (67%) patients with TGA after atrial switch and 6 (33%) patients with ccTGA. N-terminal pro-B-type natriuretic peptide (NT-pro-BNP) decreased significantly (950-358 ng/L, p<0.001). Echocardiographic systemic RV fractional area change and global longitudinal strain showed small improvements (19%-22%, p<0.001 and -11% to -13%, p=0.014, respectively). The 6 min walking distance improved significantly from an average of 564 to 600 m (p=0.011). The QOL domains of cognitive function, sleep and vitality improved (p=0.015, p=0.007 and p=0.037, respectively).

Conclusions: We describe the first patient cohort with systemic RV failure treated with sacubitril/valsartan. Treatment appears feasible with improvements in NT-pro-BNP and echocardiographic function. Our positive results show the potential of sacubitril/valsartan for this patient population.

Keywords: complex congenital heart disease; congenital heart disease; heart failure; transposition of the great arteries.

Conflict of interest statement

Competing interests: None declared.

© Author(s) (or their employer(s)) 2021. Re-use permitted under CC BY. Published by BMJ.

Figures

Figure 1
Figure 1
Treatment and follow-up protocol. *Depending on previous dose of ACEi/ARB. †If potassium >5.5 mmol/L, increase in creatinine >310 μmol/L (or eGFR 2), hypotension or signs of decompensation. ACEi, ACE inhibitors; ARB, angiotensin II-receptor blockers.
Figure 2
Figure 2
(A) Percentage of change in NT-pro-BNP at 6 months compared with the levels of individual patients at baseline. (B) Absolute NT-pro-BNP levels at baseline and 6 months, showing median (horizontal line) with IQR (box), lower and upper extreme (whiskers) and outliers (values represented with ● and ■). NT-pro-BNP, N-terminal pro-B-type natriuretic peptide.

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