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Advanced heart failure and transplant in congenital heart disease
  1. Leigh Reardon1,2,
  2. Jeannette Lin1
  1. 1 Medicine, University of California Los Angeles, Los Angeles, California, USA
  2. 2 Pediatrics, University of California Los Angeles, Los Angeles, California, USA
  1. Correspondence to Dr Jeannette Lin, Cardiology, University of California Los Angeles, Los Angeles, CA 90095, USA; JeannetteLin{at}

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Learning objectives

  • Recognise that most children with congenital heart disease live into adulthood and heart failure (HF) is the leading cause of death in adulthood.

  • Describe appropriate evaluation and diagnostic testing in patients with adult congenital heart disease (ACHD); recognise that potential reversible causes of cardiac failure should be addressed early.

  • Be aware that there is a higher early mortality in patients with ACHD after heart transplantation than other cohorts; however, long-term survival exceeds that of patients with non-congenital aetiologies of HF.


Due to advances in medical and surgical palliations, >90% of children born with congenital heart disease (CHD) are expected to survive into adulthood.1 2 Recent studies estimate a prevalence of CHD in the adult population of approximately 3000 per million individuals in developed countries.2 Although survival has improved significantly, patients with adult congenital heart disease (ACHD) often suffer from heart failure (HF) due to residual haemodynamic or anatomic abnormalities and sequelae of their defects or surgeries. HF is unfortunately the leading cause of death in patients with ACHD and accounts for approximately 20% of deaths in this population.3

Congenital heart defects vary widely in their complexity and prognosis. For example, patients with an isolated ventricular septal defect (VSD) may not require any intervention if the defect closes spontaneously or is very small, may develop HF and require catheter-based or surgical closure in early infancy if the defect is moderate or large, or may develop severe pulmonary hypertension and Eisenmenger syndrome if the defect is large and uncorrected. Care of the patient with ACHD thus requires knowledge of the spectrum of congenital diagnoses, interventions and both natural and postintervention histories of the diagnoses.

Patients with ACHD with HF often evade early detection and care. Some patients may have gaps in knowledge about their conditions,4 and many have …

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  • LR and JL contributed equally.

  • 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.

  • Patient consent for publication Not required.

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

  • Data availability statement There are no data in this work

  • Author note References which include a * are considered to be key references.