Heart Failure in Patients with a Systemic Right Ventricle: A Multicentre Study with Long-Term Follow-Up

European Journal of Heart Failure, vol. 27, pp. 3048–3057

Abstract

AIMS: Adult patients with transposition of the great arteries (TGA) and an systemic right ventricle (sRV) are at risk for heart failure (HF). In this study, we investigated risk factors for HF hospitalization and developed a novel risk stratification tool to optimize risk prediction for clinical practice. METHODS AND RESULTS: In this international multicentre study, 522 patients with TGA and an sRV, without history of HF hospitalization, were followed retrospectively for a median of 17.9 years (interquartile range [IQR] 12.9-22.1). HF hospitalization was defined as a hospital admission for HF signs and symptoms with initiation or increase of HF medication. Predictors for HF hospitalization were established using a Cox regression analysis and were used to build a 10-year risk score. Of the 522 patients, 70% had an atrial switch operation and 30% had a congenitally corrected TGA. The median age at time of enrolment was 23.7 years (IQR 19.9-32.1) and 64% were male. During follow-up, 127 patients (24.3%) had at least one HF hospitalization. A risk stratification tool was built using the following independent predictors associated with a 10-year risk of HF hospitalization: age, New York Heart Association functional class ≥II, QRS duration >120 ms, atrial fibrillation, moderate/severe right ventricular dysfunction, with a C-statistic of 0.868 (95% confidence interval 0.823-0.913). CONCLUSIONS: During follow-up, 24.3% of sRV patients had at least one HF hospitalization. Five simple, clinically-accessible variables can be used as a risk score tool to identify patients at higher risk of HF hospitalization.

Authors 16

1. Mohammad Mostafa Ansari Ramandi

   University Medical Center Groningen · University of Groningen

   Affiliation as printed

   Department of Cardiology, University of Groningen, Center for Congenital Heart Disease, University Medical Center Groningen , Groningen ,

   Department of Cardiology University of Groningen, Center for Congenital Heart Disease, University Medical Center Groningen Groningen The Netherlands

2. Werner Budts

   Universitair Ziekenhuis Leuven · KU Leuven

   Affiliation as printed

   Division of Cardiology, UZ Leuven, and Department of Cardiovascular Sciences, KU Leuven , Leuven ,

   Division of Cardiology, UZ Leuven, and Department of Cardiovascular Sciences KU Leuven Leuven Belgium

3. Jolien W. Roos‐Hesselink

   Erasmus MC

   Affiliation as printed

   Division of Cardiology, Department of Pediatrics, Erasmus Medical Center , Rotterdam ,

   Division of Cardiology, Department of Pediatrics Erasmus Medical Center Rotterdam The Netherlands

4. Willem A. Helbing

   Erasmus MC

   Affiliation as printed

   Division of Cardiology, Department of Pediatrics, Erasmus Medical Center , Rotterdam ,

   Division of Cardiology, Department of Pediatrics Erasmus Medical Center Rotterdam The Netherlands

5. Anastasia D. Egorova Aachen Department of Cardiology, CAHAL Center for Congenital Heart Disease Amsterdam Leiden

   Leiden University Medical Center

   Affiliation as printed

   Department of Cardiology, CAHAL, Center for Congenital Heart Disease Amsterdam-Leiden, Leiden University Medical Center , Leiden ,

   Department of Cardiology CAHAL, Center for Congenital Heart Disease Amsterdam‐Leiden, Leiden University Medical Center Leiden The Netherlands

6. Berto Jorrit Bouma

   Amsterdam UMC Location University of Amsterdam · Amsterdam University Medical Centers

   Affiliation as printed

   Department of Cardiology, CAHAL, Center for Congenital Heart Disease Amsterdam-Leiden, Amsterdam University Medical Center, AMC , Amsterdam ,

   Department of Cardiology CAHAL, Center for Congenital Heart Disease Amsterdam‐Leiden, Amsterdam University Medical Center, AMC Amsterdam The Netherlands

7. Anthonie L. Duijnhouwer

   Radboud University Medical Center

   Affiliation as printed

   Department of Cardiology, Radboud University Medical Center , Nijmegen ,

   Department of Cardiology Radboud University Medical Center Nijmegen The Netherlands

8. Roland R.J. van Kimmenade

   Radboud University Medical Center

   Affiliation as printed

   Department of Cardiology, Radboud University Medical Center , Nijmegen ,

   Department of Cardiology Radboud University Medical Center Nijmegen The Netherlands

9. Gertjan T. Sieswerda

   University Medical Center Utrecht

   Affiliation as printed

   Department of Cardiology, University Medical Center Utrecht , Utrecht ,

   Department of Cardiology University Medical Center Utrecht Utrecht The Netherlands

10. Thelma C. Konings

    Amsterdam University Medical Centers · Vrije Universiteit Amsterdam

    Affiliation as printed

    Department of Cardiology, Amsterdam UMC, Vrije Universiteit Amsterdam , Amsterdam ,

    Department of Cardiology Amsterdam UMC, Vrije Universiteit Amsterdam Amsterdam The Netherlands

11. Lodewijk J. Wagenaar

    Medisch Spectrum Twente

    Affiliation as printed

    Department of Cardiology, Thoraxcentrum Twente, Medisch Spectrum Twente Hospital , Enschede ,

    Department of Cardiology Thoraxcentrum Twente, Medisch Spectrum Twente Hospital Enschede The Netherlands

12. Joey F.A. Ubachs

    Maastricht University Medical Centre · Maastricht University

    Affiliation as printed

    Department of Cardiology, Maastricht University Medical Center , Maastricht ,

    Department of Cardiology Maastricht University Medical Center Maastricht The Netherlands

13. Rolf M.F. Berger

    University Medical Center Groningen · University of Groningen · Beatrix Kinderziekenhuis

    Affiliation as printed

    Center for Congenital Heart Diseases, Department of Pediatric Cardiology, Beatrix Children's Hospital, University of Groningen, University Medical Center Groningen , Groningen ,

    Center for Congenital Heart Diseases, Department of Pediatric Cardiology Beatrix Children's Hospital, University of Groningen, University Medical Center Groningen Groningen The Netherlands

14. Elke S. Hoendermis

    University Medical Center Groningen · University of Groningen

    Affiliation as printed

    Department of Cardiology, University of Groningen, Center for Congenital Heart Disease, University Medical Center Groningen , Groningen ,

    Department of Cardiology University of Groningen, Center for Congenital Heart Disease, University Medical Center Groningen Groningen The Netherlands

15. Adriaan A. Voors

    University Medical Center Groningen · University of Groningen

    Affiliation as printed

    Department of Cardiology, University of Groningen, University Medical Center Groningen , Groningen ,

    Department of Cardiology University of Groningen, University Medical Center Groningen Groningen The Netherlands

16. Joost P. van Melle corresponding

    University Medical Center Groningen · University of Groningen

    Affiliation as printed

    Department of Cardiology, University of Groningen, Center for Congenital Heart Disease, University Medical Center Groningen , Groningen ,

    Department of Cardiology University of Groningen, Center for Congenital Heart Disease, University Medical Center Groningen Groningen The Netherlands