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Congenital heart disease
Original research
Outcomes among adult survivors of total cavopulmonary Fontan palliation for single ventricle
  1. Christopher Anigwe1,
  2. Vidhushei Yogeswaran2,
  3. Anita Moon-Grady1,3,
  4. Sophie McAllister1,
  5. Anika Aggarwal2,
  6. Sarah Blissett2,4,
  7. Ian S Harris2,
  8. Peter C Kouretas5,
  9. Vaikom S Mahadevan2,
  10. Aarthi Sabanayagam2,
  11. Anushree Agarwal2
  1. 1 School of Medicine, University of California San Francisco, San Francisco, California, USA
  2. 2 Department of Medicine, Division of Cardiology, University of California San Francisco, San Francisco, California, USA
  3. 3 Department of Pediatrics, Division of Cardiology, UCSF Benioff Children's Hospital, San Francisco, California, USA
  4. 4 Department of Medicine, Division of Cardiology, Western University, London, Ontario, Canada
  5. 5 Department of Surgery, Division of Pediatric Cardiothoracic Surgery, University of California San Francisco, San Francisco, California, USA
  1. Correspondence to Dr Anushree Agarwal, Department of Medicine, Division of Cardiology, University of California San Francisco, San Francisco, California 94143-0124, USA; anu.agarwal2{at}ucsf.edu

Abstract

Objective This tertiary centre study aims to identify factors associated with adverse outcomes in adult survivors with total cavopulmonary connection (TCPC) Fontan palliation for single ventricle.

Methods This retrospective review of medical records identified adult (≥18 years) survivors of TCPC Fontan palliation who were followed at a single tertiary centre between 1 January 2000 and 1 July 2019. Adverse outcomes were defined as arrhythmia, pacemaker/implantable cardioverter defibrillator placement, liver cirrhosis, protein losing enteropathy, hospitalisation for heart failure, thromboembolic complication and/or death.

Results 160 adult TCPC patients met the inclusion criteria: 117 (73.1%) extracardiac and 43 (26.9%) lateral tunnel. The median (IQR) duration of follow-up since TCPC palliation was 17.5 (11.8–21.3) years. An adverse outcome occurred in 87 (54.4%) patients. Adverse outcome-free survival rates at 10, 20 and 25 years post TCPC were 89% (95% CI 82% to 93%), 60% (95% CI 50% to 69%) and 24% (95% CI 15% to 35%), respectively. On multivariate analysis, extracardiac Fontan (HR 2.21, 95% CI 1.20 to 4.08, p=0.011) was observed to be an independent risk factor for adverse outcomes after adjusting for age, race, morphology of the systemic ventricle and history of fenestration.

Conclusions In this single-centre retrospective study of adult survivors of TCPC palliation, extracardiac Fontan was associated with an increased hazard for adverse outcomes. This finding could guide clinicians in developing risk modification strategies and management decisions to improve long-term outcomes in these patients.

  • outcome assessment
  • health care
  • risk factors
  • Fontan procedure

Data availability statement

Data are available upon reasonable request. The data used in these analyses include potentially identifying participant information and therefore are not publicly available due to legal and ethical restrictions. Please contact the corresponding author to obtain access to de-identified data.

https://doi.org/10.1136/heartjnl-2021-319760

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    Data availability statement

    Data are available upon reasonable request. The data used in these analyses include potentially identifying participant information and therefore are not publicly available due to legal and ethical restrictions. Please contact the corresponding author to obtain access to de-identified data.

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    Footnotes

    • Twitter @anigwedr

    • Contributors CA collected and analysed the data and wrote the paper. AAga contributed to statistical analysis. AAga, AAgg and SM helped in writing the paper. VY, AM-G, SB, ISH, PCK, VM, AS and AAga assisted with the critical review of the manuscript. AAga is the principal investigator of this study and the corresponding author of this paper. CA, VY and AAga had full access to all the data and take responsibility for the integrity of the data and the accuracy of the data analysis. AAga is responsible for the overall content as guarantor.

    • 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.