Heart failure in congenital heart disease: management options and clinical challenges

Expert Rev Cardiovasc Ther. 2020 Aug;18(8):503-516. doi: 10.1080/14779072.2020.1797488. Epub 2020 Aug 24.


Introduction: The population of adults with congenital heart disease (ACHD) is rapidly expanding and one of the major complications is heart failure. Timely diagnosis and treatment are crucial, but strong evidence for effectiveness of heart failure treatment in ACHD is currently lacking. Components of the medical history, physical examination, and further diagnostic tests including ECG, echocardiography, cardiac magnetic resonance imaging, exercise testing, and biomarkers can identify patients at risk for early mortality or heart failure.

Areas covered: Although the number of studies guiding evidence-based treatment are expanding, many clinical questions have not been completely answered yet. Therefore, in this review we provide an overview of current available insights in epidemiology, diagnosis, risk stratification, and treatment options in ACHD patients, including non-medical therapies and advanced care planning.

Expert opinion: We strongly advocate expanding current use of biomarkers in the diagnostic process and timely initiation of discussing advanced treatment options and advanced care planning with patients and their loved ones. More research in multi-center collaborations is needed to study all aspects of care of adult congenital heart disease patients.

Keywords: Risk stratification; biomarkers; congenital Heart Defects; diagnosis; heart failure; predictors; prognosis; treatment.

Publication types

  • Review

MeSH terms

  • Adult
  • Echocardiography
  • Electrocardiography
  • Exercise Test
  • Heart Defects, Congenital / complications*
  • Heart Defects, Congenital / diagnosis
  • Heart Defects, Congenital / diagnostic imaging
  • Heart Defects, Congenital / therapy
  • Heart Failure / etiology*
  • Heart Failure / prevention & control
  • Humans
  • Magnetic Resonance Imaging