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Tolerability and beneficial effects of sacubitril/valsartan on systemic right ventricular failure
  1. Marieke Nederend1,2,
  2. Philippine Kiès1,2,
  3. Madelien V Regeer1,2,
  4. Hubert W Vliegen1,2,
  5. Bart JA Mertens3,
  6. Daniëlle Robbers-Visser4,
  7. Berto J Bouma4,
  8. Laurens F Tops2,
  9. Martin J Schalij1,2,
  10. Monique R M Jongbloed1,2,5,
  11. Anastasia D Egorova1,2
  1. 1Center for Congenital Heart Disease Amsterdam Leiden (CAHAL), Leiden University Medical Center, Leiden, The Netherlands
  2. 2Cardiology, Leiden University Medical Center, Leiden, The Netherlands
  3. 3Biomedical Data Sciences, Leiden University Medical Center, Leiden, The Netherlands
  4. 4Center for Congenital Heart Disease Amsterdam Leiden (CAHAL), Amsterdam University Medical Center, Amsterdam, The Netherlands
  5. 5Anatomy & Embryology, Leiden University Medical Center, Leiden, The Netherlands
  1. Correspondence to Dr Anastasia D Egorova, Cardiology, Leiden University Medical Center, Albinusdreef 2, 2333 ZA, Leiden, The Netherlands; a.egorova{at}


Objective Patients with a systemic right ventricle (sRV) in the context of transposition of the great arteries (TGA) after atrial switch or congenitally corrected TGA (ccTGA) are prone to sRV dysfunction. Pharmacological options for sRV failure remain poorly defined. This study aims to investigate the tolerability and effects of sacubitril/valsartan on sRV failure in adult patients with sRV.

Methods In this two-centre, prospective cohort study, all consecutive adult patients with symptomatic heart failure and at least moderately reduced sRV systolic function were initiated on sacubitril/valsartan and underwent structured follow-up.

Results Data of 40 patients were included (40% female, 30% ccTGA, median age 48 (44–53) years). Five patients discontinued therapy during titration. Median follow-up was 24 (12–36) months. The maximal dose was tolerated by 49% of patients. No episodes of hyperkalaemia or renal function decline occurred. Six-minute walking distance increased significantly after 6 months of treatment (569±16 to 597±16 m, p=0.016). Serum N-terminal-prohormone brain natriuretic peptide (NT-proBNP) levels decreased significantly after 3 months (567 (374–1134) to 404 (226–633) ng/L, p<0.001). Small, yet consistent echocardiographic improvements in sRV function were observed after 6 months (sRV global longitudinal strain: −11.1±0.5% to −12.6±0.7%, p<0.001, and fractional area change: 20% (16%−24%) to 26% (19%−30%), p<0.001). The linear mixed-effects model illustrated that after first follow-up moment, no time effect was present for the parameters.

Conclusions Treatment with sacubitril/valsartan was associated with a low rate of adverse effects in this adult sRV cohort. Persisting improvement in 6-minute walking test distance, NT-proBNP levels and echocardiographic parameters of sRV function was observed in an on-treatment analysis and showed no differential response based on sex or anatomy.

  • heart failure, systolic
  • pharmacology
  • heart defects, congenital
  • transposition of great vessels

Data availability statement

Data are available upon reasonable request.

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Data availability statement

Data are available upon reasonable request.

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  • Contributors Substantial contributions to the conception or design of the work; or the acquisition, analysis, or interpretation of data for the work—MN, PK, MVR, HWV, BJM, DR-V, BB, LFT, MJS, MRMJ and ADE. Drafting the work or revising it critically for important intellectual content—MN, PK, MVR, HWV, BJM, DR-V, BB, LFT, MJS, MRMJ and ADE. Final approval of the version to be published—MN, PK, MVR, HWV, BJM, DR-V, BB, LFT, MJS, MRMJ and ADE. Agreement to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved—MN, PK, MVR, HWV, BJM, DR-V, BB, LFT, MJS, MRMJ and ADE. Guarantor: MN, PK, MVR, HWV, BJM, DR-V, BB, LFT, MJS, MRMJ and ADE.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Patient and public involvement Patients and/or the public were not involved in the design, or conduct, or reporting, or dissemination plans of this research.

  • Provenance and peer review Not commissioned; externally peer reviewed.

  • Supplemental material This content has been supplied by the author(s). It has not been vetted by BMJ Publishing Group Limited (BMJ) and may not have been peer-reviewed. Any opinions or recommendations discussed are solely those of the author(s) and are not endorsed by BMJ. BMJ disclaims all liability and responsibility arising from any reliance placed on the content. Where the content includes any translated material, BMJ does not warrant the accuracy and reliability of the translations (including but not limited to local regulations, clinical guidelines, terminology, drug names and drug dosages), and is not responsible for any error and/or omissions arising from translation and adaptation or otherwise.