RT Journal Article
SR Electronic
T1 Sacubitril/valsartan in the treatment of systemic right ventricular failure
JF Heart
JO Heart
FD BMJ Publishing Group Ltd and British Cardiovascular Society
SP 1725
OP 1730
DO 10.1136/heartjnl-2020-318074
VO 107
IS 21
A1 Tjitske E Zandstra
A1 Marieke Nederend
A1 Monique R M Jongbloed
A1 Philippine Kiès
A1 Hubert W Vliegen
A1 Berto J Bouma
A1 Laurens F Tops
A1 Martin J Schalij
A1 Anastasia D Egorova
YR 2021
UL http://heart.bmj.com/content/107/21/1725.abstract
AB 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.All data relevant to the study are included in the article or uploaded as supplementary information. Additional data (statistical analysis plans) are available on reasonable request.