Congenital heart defects are encountered in about 1% of newborns and the overall prevalence is increasing due to improved treatment options. The population of adults with congenital heart disease (ACHD) has exceeded that of children in the last two decades and continues to grow. Together with this, there has been an inevitable increase in those entering their 4th and 5th decades of adult life with CHD now exhibiting a greater burden of
cardiovascular diseases. Heart failure is a common, yet poorly understood late sequala of many complex congenital heart defects. ACHD-related heart failure encompasses one of the most urgent problems in de ACHD practice and robust data on the prevalence, timely recognition and optimal management strategies is currently missing. It is estimated that one
in 3 adult patients with a Fontan circulation have evidence of circulatory failure, with half of these progressing to death during intermediate-term follow up. In adult patients with a systemic right ventricle, ventricular systolic dysfunction is common affecting at least 37% by the time they reach adult life. Major adverse cardiovascular events including death, heart transplant and requirement for ventricular assist devices occurs in at least 8.8 per 1000
patient years. Numerous guidelines and position papers recognise the current paucity of evidence based strategies in managing ACHD-related heart failure, yet also highlight the difficulty in formulating any recommendations given the current paucity of data in this hemodynamically intricate and clinically complex patient population. There is an urgent need for coordination, collaboration and cooperation to address the growing challenges of ACHD-related heart failure.

List of members:

  • Dr. Anastasia D. Egorova, application and coordination (application and coordination, CAHAL, LUMC)
  • Dr. Berto J. Bouma (CAHAL, Amsterdam UMC)
  • Dr. Heleen van der Zwaan (UMCU)
  • Judith van de Meerakker, Patiëntenvereniging Aangeboren Hartafwijkingen

Start date: December 2023


1. Providing a framework and actively stimulating national collaboration and cooperation in the field of ACHD-related heart failure to better understand the clinical course, facilitate timely intervention and improve patient outcomes.
2. Inclusive multicenter participation in international registries, trials and consortia, broadly representing the Dutch ACHD patient population.
3. Promoting and expanding an (inter-)national research network and funding applications focusing on ACHD-related heart failure.
4. Working towards evidence based guidelines on the management of the ACHD-related heart failure. 
5. Facilitating a dialogue with health care stakeholders (amongst others insurance companies, pharmaceutical and heath care product and device companies) and patient organizations to improve the outcomes in ACHD-related heart failure and improve patient knowledge and empowerment.

Intended activities:
1. Organizing an ACHD-related heart failure symposium for medical professionals, patients and health care stakeholders to raise awareness of the growing clinical problem and the unmet need for dedicated management strategies.
2. Setting up a national ACHD-heart failure registry to evaluate the prevalence of ACHD-related heart failure, understand how the current heart failure pharmacotherapy is prescribed in this population (safety and prescription patterns) and reflect on the efficacy of the pharmacotherapy (QoL, clinical outcomes and survival).
3. Applying and coordinating joint (inter-)national research grant applications and participation in registries and trials including, but not limited to the ARTORIA-R and the Global Prospective CHD Registry. 

Partners (to be expanded): 

  • Concor, dr. Berto J. Bouma
  • Patiëntenvereniging Aangeboren Hartafwijkingen, Judith van de Meerakker
  • Global Prospective ACHD Registry, dr. Anastasia Egorova and dr. Gruschen Veldtman, NHS Golden Jubilee, Glasgow, UK
  • ARTORIA-R, dr. Christoph Sinning, University Medical Center Hamburg, Germany
  • L.E.I.C.A. Laboratory of Cardiovascular Science, dr. Nina C. Hauck-Weber and dr. Coert J. Zuurbier, Amsterdam University Medical Centers, location Amsterdam UMC, the Netherlands
  • ESC working group of Adult Congenital Heart Disease
  • NVVC working group on Adult Congenital Heart Disease

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