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Congenital heart disease in adult patients
Medical therapy for heart failure in adult congenital heart disease: does it work?
  1. Pastora Gallego1,2,
  2. Jose Maria Oliver3,4
  1. 1 Adult Congenital Heart Disease Unit, Hospital Universitario Virgen del Rocío, Sevilla, Andalucía, Spain
  2. 2 Instituto de Biomedicina de Sevilla, Sevilla, Andalucía, Spain
  3. 3 Hospital General Universitario Gregorio Marañón, Madrid, Madrid, Spain
  4. 4 La Fundacion para la Investigacion Biomedica del Hospital Gregorio Marañón, Madrid, Spain
  1. Correspondence to Dr Pastora Gallego, Adult Congenital Heart Disease Unit, Hospital Universitario Virgen del Rocío, Sevilla 41013, Spain; antoniap.gallego.sspa{at}

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Learning objectives

  • To understand similarities and disparities of heart failure pathophysiology between adult congenital heart disease as compared with acquired heart disease.

  • To be aware of the available evidence to better guide medical heart failure therapies depending on clinical phenotypes.

  • To highlight the role of non-pharmacological treatment strategies for adult congenital heart disease–related heart failure.


Heart failure (HF) is currently recognised as one of the most clinically important events resulting from the prolonged survival to adulthood of patients with congenital heart disease (ACHD). Hospitalisations involving HF have increased 91% over the last two decades,1 with an impact on rehospitalisations and deaths during follow-up. According to data extracted from the Dutch CONCOR Registry,2 patients with CHD first admitted for HF have a fivefold higher mortality risk than those with no admissions; more recently, a 25% risk of death within 1 year following first HF admission has been reported by Lal et al.3 Consequently, a steady increase in the proportion of HF-related deaths with ageing has been described and HF is currently cited as the leading cause of cardiac death in patients with ACHD.4

Despite the contribution of HF to lifelong mortality and morbidity, management strategies remain challenging. Correction of residual lesions may be of benefit for treating ventricular dysfunction. However, the patients with ACHD have been traditionally excluded from HF trials and recruiting a sufficient number of patients for specifically ACHD-dedicated clinical studies with hard endpoints may be very difficult considering the heterogeneity of this patient group. Thus, these studies are frequently unpowered to detect effects of the drugs and HF medical therapy frequently rests on the extrapolation of acquired HF guidelines.5 6 Otherwise, the interplay among the unique CHD haemodynamics, biventricular or univentricular heart function, early and/or ongoing cyanosis, surgical and residual sequelae, and multisystemic affectation makes …

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  • Contributors PG and JMO fulfil the authorship criteria of the ICMJE and approved either the manuscript or this submission.

  • 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 statement This research was done without patient involvement. Patients were not invited to comment on the study design and were not consulted to develop patient relevant outcomes or interpret the results. Patients were not invited to contribute to the writing or editing of this document for readability or accuracy.

  • Patient consent for publication Not required.

  • Provenance and peer review Commissioned; internally peer reviewed.

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