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Congenital heart disease
Original research
Effect of medical treatment on heart failure incidence in patients with a systemic right ventricle
  1. Magalie Ladouceur1,2,
  2. Teresa Segura de la Cal3,
  3. Bamba Gaye2,
  4. Eugenie Valentin2,
  5. Reaksmei Ly1,2,
  6. Laurence Iserin1,
  7. Antoine Legendre1,4,
  8. Elie Mousseaux2,5,
  9. Wei Li6,
  10. Isma Rafiq6,
  11. Aleksander Kempny6,
  12. Ana Barradas-Pires6,
  13. Sonya V Babu-Narayan6,7,
  14. Michael A Gatzoulis6,
  15. Konstantinos Dimopoulos6,7
  1. 1 Adult Congenital Heart Disease Unit, Hôpital Européen Georges Pompidou, Centre de référence des Malformations Cardiaques Congénitales Complexes, Assistance Publique—Hôpitaux de Paris, Paris University, Paris, France
  2. 2 Centre de Recherche Cardiovasculaire de Paris, U970, INSERM, Paris, France
  3. 3 Adult Congenital Heart Disease and Pulmonary Hypertension Unit, Hospital 12 de Octubre, Madrid, Spain
  4. 4 Pediatric Cardiology, Centre de référence des Malformations Cardiaques Congénitales Complexes, Necker, AP-HP, Paris, France
  5. 5 Department of Cardiovascular Radiology, Hôpital Européen Georges Pompidou, Assistance Publique—Hôpitaux de Paris, Paris University, Paris, France
  6. 6 Adult Congenital Heart Centre, National Centre for Pulmonary Hypertension, Royal Brompton Hospital, London, UK
  7. 7 National Heart and Lung Institute, Imperial College School of Medicine, London, UK
  1. Correspondence to Dr Magalie Ladouceur, APHP, Paris, France; magalie.ladouceur{at}aphp.fr

Abstract

Background To date, clinical trials have been underpowered to demonstrate a benefit from ACE inhibitors (ACEis) or angiotensin II receptor blockers (ARBs) in preventing systemic right ventricle (sRV) failure and disease progression in patients with transposition of the great arteries (TGA). This observational study aimed to estimate the effect of ACEi and ARB on heart failure (HF) incidence and mortality in a large population of patients with an sRV.

Methods Data on all patients with an sRV under active follow-up at two tertiary centres between January 2007 and September 2018 were studied. The effect of ACEi and ARB on the incidence of HF and mortality was estimated using a propensity score weighting approach to control confounding.

Results Among the 359 patients with an sRV (32.2 (IQR 26.4–38.3) years, 59.3% male, 66% complete TGA with atrial switch repair and 34% congenitally corrected TGA), 79 (22%) had a moderate to severe sRV dysfunction and 138 (38%) were treated with ACEi or ARB. Fourteen (3.6%) patients died, 8 (2.1%) underwent heart transplantation and 46 (11.8%) had a new HF event over a median follow-up of 7.1 (IQR 4.0–9.4) years. On multivariate Cox analysis with adjustment using propensity score weighting approaches, ACEi or ARBs treatment was not significantly associated with a lower HF incidence or mortality in patients with an sRV.

Conclusions Despite significant neurohormonal activation described in patients with an sRV, there is still no evidence of a beneficial effect of ACEi or ARB on morbidity and mortality in this population.

  • transposition of the great arteries
  • heart failure
  • heart failure

Data availability statement

No data are available.

https://doi.org/10.1136/heartjnl-2020-318787

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    Data availability statement

    No data are available.

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    Footnotes

    • Twitter @Mag_Ladouceur, @TeresaSeguraCal

    • Contributors ML: contributed to conception or design; contributed to acquisition, analysis and interpretation; drafted the manuscript; critically revised the manuscript; gave final approval; and agrees to be accountable for all aspects of work ensuring integrity and accuracy. TSdlC, BG, AB-P, WL, LI, IR and AK: contributed to interpretation; critically revised the manuscript; and gave final approval. EV: contributed to the analysis of the data (statistical analysis). RL and AL: drafted the manuscript and gave final approval. EM, SVB-N and MAG: critically revised the manuscript and gave final approval. KD: contributed to conception or design; contributed to analysis and interpretation; drafted the manuscript; critically revised the manuscript; gave final approval; and agrees to be accountable for all aspects of work ensuring integrity and accuracy.

    • Funding This work was supported by the Fédération Française de Cardiologie, Assistance Publique des Hôpitaux de Paris and by the Fundación Alfonso Martín Escudero.

    • Competing interests None declared.

    • 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.

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