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Original research
Greater admissions, mortality and cost of heart failure in adults with congenital heart disease
  1. Danielle S Burstein1,
  2. Joseph W Rossano1,
  3. Heather Griffis2,
  4. Xuemei Zhang2,
  5. Rachel Fowler3,
  6. Benjamin Frischertz3,
  7. Yuli Y Kim1,4,
  8. JoAnn Lindenfield3,
  9. Jeremy A Mazurek4,
  10. Jonathan B Edelson1,
  11. Jonathan N Menachem3
  1. 1Division of Pediatric Cardiology, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA
  2. 2Healthcare Analytics Unit, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA
  3. 3Division of Cardiology, Vanderbilt University Medical Center, Nashville, Tennessee, USA
  4. 4Division of Cardiovascular Medicine, Hospital of the University of Pennsylvania, Philadelphia, Pennsylvania, USA
  1. Correspondence to Dr Danielle S Burstein, Division of Pediatric Cardiology, Childrens Hospital of Philadelphia, Philadelphia, PA 19103, USA; bursteind{at}


Objective Limited contemporary data exist regarding outcomes and resource use among adults with congenital heart disease and heart failure (ACHD-HF). This study compared outcomes, emergency department (ED) and hospital resource use, and advanced heart failure (HF) therapies in ACHD-HF versus non-ACHD with HF (HF-non-ACHD).

Methods The Nationwide Emergency Department Sample and Nationwide Inpatient Sample were used to analyse outcomes and resource use among ACHD-HF ED visits and hospitalisations from 2006 to 2016. ACHD-HF was stratified by single-ventricle (SV) and two-ventricle (2V) disease.

Results A total of 76 557 ACHD-HF visits (3.6% SV physiology) and 31 137 414 HF-non-ACHD visits were analysed. ACHD-HFs were younger (SV 33 years (IQR 25–44), 2V 62 years (IQR 45–76); HF-non-ACHD 74 years (IQR 63–83); p<0.001). ACHD-HFs had higher ED admissions (78% vs 70%, p<0.001), longer hospital length of stay (5 days (IQR 2–8) vs 4 days (IQR 2–7), p<0.001) and greater hospital costs ($49K (IQR 2K–121K) vs $32K (17K–66K), p<0.001). Mortality was significantly higher among ACHD-HFs with SV physiology (6.6%; OR 1.6, 95% CI 1.1 to 2.3) or 2V physiology (6.3%; OR 1.4, 95% CI 1.3 to 1.5) versus HF-non-ACHD (5.5%). ACHF-HF hospitalisations increased more (46% vs 6% HF-non-ACHD) over a 10-year period, but the proportion receiving ventricular assist device (VAD) (ACHD-HF −2% vs HF-non-ACHD 294%) or transplant (ACHD-HF −37% vs HF-non-ACHD 73%) decreased.

Conclusion ACHD-HFs have significant ED and hospital resource use that has increased over the past 10 years. However, advanced HF therapies (VAD and transplantation) are less commonly used compared with those without adult congenital heart disease.

  • heart failure
  • heart defects
  • congenital

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  • Contributors DSB conceptualised and designed the study, drafted the initial manuscript, and reviewed and revised the manuscript. HG and XZ designed the data collection instruments, collected the data, carried out the initial analysis, and reviewed and revised the manuscript. JR, BF, YK, JL, JAM, JE and RF reviewed and interpreted the data analysis, and reviewed and revised the manuscript. JNM conceptualised and designed the study, coordinated and supervised data collection, and critically reviewed the manuscript for important intellectual content. All authors approved the final manuscript as submitted and agreed to be accountable for all aspects of the work. The corresponding author has the right to grant on behalf of all authors and does grant on behalf of all authors, an exclusive licence on a worldwide basis to the BMJ Publishing Group Ltd and its licensees to permit this article (if accepted) to be published in HEART editions and any other BMJPGL products to exploit all subsidiary rights.

  • Funding This work is supported in part by the Cardiac Center Clinical Research Core at the Children’s Hospital of Philadelphia. No extramural funding was used to support this work.

  • Competing interests None declared.

  • Patient consent for publication Not required.

  • Ethics approval The institutional review board of the Children’s Hospital of Philadelphia deemed this study exempt from review under 45 CFR 46.102(f), as patient data were deidentified.

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

  • Data availability statement All data relevant to the study are included in the article or uploaded as supplementary information.

  • 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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