Medicine and Healthcare
Risk of Heart Failure with ACHD
Study examines disease risk and care situation of adults with congenital heart defects
Scientific name of the study
Epidemiology, treatment and progression of heart failure in adults with congenital heart disease (ACHD): analysis of data from the National Registry of Congenital Heart Defects (NRAHF)
It is a major achievement in cardiac medicine that most children today reach adulthood even with a serious congenital heart defect. In Germany, there are now more adults with congenital heart defects than children. About two thirds of patients are adults.
But most of them are not cured. Adults with congenital heart defects are dependent on lifelong specialized aftercare. This is because the risk of secondary diseases and complications increases with age.
The Risk of Heart Failure
Deaths due to congenital heart defects have long since shifted from children to adults. Heart failure is one of the major risk factors. We want to find out how often adults with congenital heart disease (CHD) are affected, which anatomical and functional features are associated with it, how the disease has been prevented or treated, and with what long-term outcome.
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Good to know
Heart Failure is Preventable and Treatable
Heart failure in adults is one of the consequential risks of congenital heart disease. Regular follow-up by ACHD specialists can save lives. Around a quarter of people with a congenital heart defect will develop heart failure in adulthood. This includes those whose congenital heart defect was successfully treated in childhood.
The time of diagnosis and the course of the disease vary greatly depending on the type of defect. People often do not notice the symptoms at first because they have become accustomed to a reduced physical capacity over the course of their lives.
In addition, the nature of the heart defect sometimes precludes the use of proven preventative treatments with appropriate medications, as they pose another major risk. However, heart failure is often preventable.
If it is recognised in good time, it can usually be treated with medication. Regular check-ups with a specialized doctor at one of the German ACHD centers significantly reduces the risk of heart failure being detected too late and increases the chances of it being treated in time.
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©The National Registry has been collecting, maintaining, and updating data voluntarily provided by patients with congenital heart defects throughout Germany since 2003.
This is the first time that we have been able to get a clear picture of the risk factors for developing heart failure and to analyse the results of treatment options in more detail. This is because the data can be used to answer questions where the course of the disease, interventions, and drug treatments need to be monitored and compared over long periods of time.
Patient Groups at Risk
We want to find out which factors favour heart failure in ACHD and how the disease can be best treated and prevented.
Our initial focus is on patients with transposition of the great arteries (TGA) after atrial bypass surgery or with congenital corrected TGA (ccTGA), Ebstein's anomaly, or Tetralogy of Fallot (ToF), and its variants. These patient groups have a high incidence of heart failure.
For comparison, we are also analysing the data from patients born with an Atrial Septal Defect (ASD). This group of patients is considered cured if the ASD is closed in childhood.
The Other "Normal"
A congenital heart defect creates a different 'normality' from a healthy heart, even after successful corrective surgery. This requires intensive research and education, as it can lead to misinterpretations of exercise capacity, symptoms, and laboratory values, and thus to decisions that may do more harm than good.
We know from clinical experience that a very young age at the time of corrective surgery, but also a lack of treatment with appropriate cardiovascular medications, can lead to heart transplantation being deemed necessary for heart failure in early adulthood.
The use of ventricular assist devices and artificial hearts, which can temporarily take over the cardiac function until a suitable donor heart is available, is not always an option. The anatomical and functional nature of some heart defects does not allow this.
We therefore hope that the evaluation of long-term outcomes on a broad data base and a corresponding characterization of patients will also provide us with insights into the best treatment option in each case. The research situation in the growing group of ACHD patients is still unsatisfactory.
Registry Research Needed
While many people with acquired cardiovascular disease benefit from the results of randomized drug trials for the treatment of heart failure, this is not the case for people with congenital heart disease. Their inclusion in such trials is complex and limited due to the large number and variety of their individual diagnoses. The analysis of registry data is therefore important because it is the only way to draw important conclusions about the safety and efficacy of different forms of therapy.
Longer Life, better Quality of Life
Promising results from randomised trials of SGLT2 inhibitors have led to the approval of new drugs. The European Society of Cardiology has adapted its guidelines for the treatment of heart failure accordingly. Clinical experience suggests that this guideline-based drug treatment is also recommended for adults with impaired pumping function of the ventricle responsible for the systemic (body) circulation.
Our research project also aims to test this assumption. The results of the new registry study should help to improve care for people with congenital heart defects. The aim is to support adults with congenital heart defects and an increased risk of heart failure well into old age and significantly improve their quality of life.
In charge of the project:
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© UKM
Prof. Dr. Dr. med. Gerhard-Paul Diller
Gerhard-Paul Diller is Director of the Clinic for Cardiology III: Congenital Heart Defects (EMAH) and Valve Diseases at Münster University Hospital. He is a Visiting Professor at the Imperial College London. More
Gerhard-Paul Diller studied medicine at the Ludwig-Maximilians University in Munich and he received much of his clinical training in Great Britain, at the Royal Brompton Hospital, London. In his research, the certified ACHD expert focuses on adults with congenital heart disease and pulmonary hypertension. In addition, he holds a Master’s degree (M.Sc.) in Health Economics Policy and Management from the London School of Economics, London, UK. Gerhard-Paul Diller has been Director of the Clinic for Cardiology III: Congenital Heart Defects (EMAH) and Valvular Heart Disease at the University Hospital Münster since July 2024. Gerhard-Paul Diller is an executive board member of both the European Heart Rhythm Association and the ESC-Working-Group Grown Up Congenital Heart Disease. Furthermore, he is the deputy chair of the AG 9 Kongenitale Herzfehler (working group on congenital heart disease) of the German Cardiac Society (DGK). Gerhard-Paul Diller is a member of the Competence Network for Congenital Heart Defects where he is also a member of the Review Board.
Universitätsklinikum Münster
Klinik für Kardiologie III: Angeborene Herzfehler (EMAH) und Klappenerkrankungen
Albert-Schweitzer-Campus 1
48149 Münster -
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PD Dr. med. Christoph Sinning
Christoph Sinning is a specialist in internal medicine and cardiology. The ACHD specialist works as a senior physician and head of echocardiography at the University Medical Centre Hamburg Eppendorf (UKE). More
Christoph Sinning studied medicine at the University of Bonn. A specialist in internal medicine and cardiology with an additional qualification in ACHD cardiology, he habilitated in internal medicine/cardiology in 2015 on "Biomarkers for risk stratification in cardiology" and received his Venia legendi. Since 2017, he has been a senior physician in the Department of General and Interventional Cardiology and head of the echocardiography laboratory at the University Medical Center Hamburg-Eppendorf (UKE). Christoph Sinning has been a Fellow of the European Society of Cardiology (FESC) since 2017 and a Fellow of the European Association of Cardiovascular Imaging (FEACVI) since 2019.
Universitätsklinikum Hamburg-Eppendorf
Universitäres Herz- und Gefäßzentrum Hamburg
Martinistraße 52
Gebäude O 70
20246 Hamburg -
Dr. med. Lucie Kretzler
Lucie Kretzler is a specialist in internal medicine and cardiology and head of the Cardiovascular Assessment Module at the Clinical Study Centre (CSC) of the Berlin Institute of Health (BIH). More
Lucie Kretzler studied medicine at the Charles University in Prague. During her residency at the HELIOS Clinic in Pirna from 2012 to 2017, she completed a doctoral programme at the Technical University of Dresden, graduating magna cum laude. The topic of her dissertation was "Analysis of baseline characteristics and clinical outcomes of patients undergoing percutaneous left atrial appendage occlusion at Pirna Hospital". From 2017 to 2020, Lucie Kretzler worked as a specialist in internal medicine and cardiology at the Charité Campus Virchow-Klinikum. Since 2020, she has been head of the Cardiovascular Assessment Module at the Clinical Study Centre (CSC) of the Berlin Institute of Health (BIH).
Deutsches Herzzentrum der Charité
Klinik für Kardiologie, Angiologie und Intensivmedizin | CBF
Hindenburgdamm 30
Campus Benjamin Franklin (Hauptgebäude Behandlungsbau, Haus VB)
12200 Berlin -
© Alex Orrow
Prof. Dr. med. Anselm Uebing
Anselm Uebing, born in 1969, is Director of the Clinic for Congenital Heart Defects and Pediatric Cardiology at the University Medical Center Schleswig-Holstein, Campus Kiel. More
Anselm Uebing studied medicine at Heinrich Heine University in Düsseldorf. Thereafter he completed his training as a pediatrician and pediatric cardiologist at University Hospital in Kiel. From 2004 to 2006 Anselm Uebing received clinical and scientific training in the treatment of adults with a congenital heart at the Royal Brompton Hospital in London and specialized as a "London -Toronto Fellow” in Adult Congenital Heart Disease. From 2006 to 2010, he worked as a senior physician at the Clinic for Congenital Heart Defects and Pediatric Cardiology in Kiel, Germany. In 2010, he moved to the Royal Brompton Hospital London, where he became head of the cardiac catheterization laboratory in the adult congenital heart defect unit. In 2017 he became head of the Department of Pediatric and Adolescent Medicine - Pediatric Cardiology at Münster University Hospital. Since April 2019, Anselm Uebing has been Director of the Clinic for Congenital Heart Defects and Pediatric Cardiology at Christian Albrechts University of Kiel (CAU). Anselm Uebing is also a DZHK (Deutsches Zentrum für Herz-Kreislauf-Forschung, German Centre for cardiovascular research) scientist at the Hamburg/Kiel/Lübeck site.
Universitätsklinikum Schleswig-Holstein - Campus Kiel
Klinik für angeborene Herzfehler und Kinderkardiologie
Arnold-Heller-Str. 3
24105 Kiel -
© Wolfram Scheible für Nationales Register
Dr. med. Ulrike Bauer
Ulrike Bauer, born in 1958, is the Scientific Managing Director of the National Register for Congenital Heart Defects and the Competence Network for Congenital Heart Defects. More
Ulrike Bauer studied human medicine at the Humboldt University of Berlin. After finishing her doctorate on echocardiography in congenital heart disease, she was a resident for internal medicine at the county hospital in Chemnitz, after which she transferred to the Institute for Cardiovascular Diagnostics at the Charité Berlin. From there she transferred to the department of pediatric cardiology at the German Heart Center, Berlin (Deutsches Herzzentrum Berlin). Under the guidance of Prof. Dr. Peter E. Lange, Ulrike Bauer started setting up a nationwide register for congenital heart disease. The initiative by Prof. Dr. Peter E. Lange lead to the founding of the association of the National Register for Congenital Heart Defects. It was jointly supported by the board of trustees of Deutsches Herzzentrum Berlin and the cardiac societies, as well as by parent and patient associations. The same year was also the kick-off for creating the Competence Network for Congenital Heart Defects, which could took place thanks to government grants. Ulrike Bauer is a member of the German Society of Paediatric Cardiology (DGK), the European Society of Cardiology (ESC), the German Cardiac Society (DGK) and the Association for European Paediatric and Congenital Cardiology (AEPC).
Kompetenznetz Angeborene Herzfehler e. V.
Network Headquarters
Augustenburger Platz 1
13353 Berlin
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