The National Register for Congenital Heart Defects
The Ability to Carry Out Reliable Research
The situation is aggravated by the fact that this disease, which is the most frequent primary congenital disease, is heterogeneous. This complicates research in this field, which is, however, urgently needed by the patients. To help solve this issue, the National Register for Congenital Heart Defects was set up at the Competence Network for Congenital Heart Defects. It is a research infrastructure that is internationally unique.
Clinical Relevance is on the Rise
Already today, there is an estimated number of 300,000 children, adolescents and adults with congenital heart disease in Germany. In the coming years, the clinical relevance of congenital heart disease is expected to continue increasing. For ideal medical care up until high age patients rely on research yielding results that are swiftly utilized in practice.
Development of Clinical Relevance
According to extrapolations, the clinical relevance of congenital heart disease will continue rising in the coming years.
Estimated clinical relevance of CHD in the next years
- Live births with CHD 0.8% of all live births
- Children with CHD 90% of live births with CHD
- 18-year-old with CHD 80% of children with CHD
- Adults with CHD
Source: Competence Network for Congenital Heart Defects. Estimated values from 2000
However, single scientific institutions hardly manage to acquire a sufficient number of data and samples as needed for significant research on congenital heart disease. For this purpose, the numbers of patients with single diagnoses are too small. This is why the National Register for Congenital Heart Defects with an associated biorepository was created. It offers a research infrastructure for multicenter research in this field.
Research Results that are Representative and Reproducible
The National Register for Congenital Heart Defects (NRAHF) collects and manages data regarding the course of disease, life expectancy, quality of life, healthcare situation, as well as biosamples from patients and their relatives on a permanent basis. The established research infrastructure, which is unique in the world, enables, for instance, linking phenotype data to courses of disease and biosamples. As a result, scientists can access a reliable basis for their research projects with their research results assuredly being representative and reproducible.
Today, roughly 55,000 patients and relatives take part in the National Register for Congenital Heart Defects. Their data and donated samples are used for research worldwide. The National Register’s data and sample management supports researchers in a way that allows them to completely focus on their research questions, thus relieving research institutions.
Researching for Optimal Healthcare
Today’s research for tomorrow’s healthcare. This is the maxim guiding all the work of the National Register for Congenital Heart Defects. For the first time, this patient database allows comprehensive studies investigating matters such as the prevalence of congenital heart disease, as well as the patients’ health-related social situation and quality of life. Numerous of the recent research projects focus on unanswered questions regarding the causes, treatment, long-term course and life with congenital heart disease. Research that is based on a broad collection of data and samples is also required for the development of pharmaceuticals and medical devices suitable for children.
The National Register for Congenital Heart Defects is one of the centerpieces of the Competence Network for Congenital Heart Defects. The patient database is run as a non-profit registered association by the three large German cardiac societies, i. e. the German Society of Paediatric Cardiology (DGPK), the German Cardiac Society (DGK) and the German Society for Thoracic and Cardiovascular Surgery (DGTHG).
Further Numbers and Facts
Good to Know
Development of the Survival Rate
Each year, about 6,000 children are born with congenital heart disease in Germany. Congenital malformations of the heart or the great arteries differ widely. The spectrum ranges from mild and simple defects, which hardly put any strain on the cardiovascular system, to very severe heart defects that cause death if left untreated. Thanks to progress in pediatric cardiology, cardiac surgery and anesthesia, over 90 percent of the patients reach adulthood today. However, in most cases, patients remain chronically ill throughout their life. Secondary diseases often emerge after surgery. These lead to an impaired quality of life, exercise capacity and ability to work and can even be life threatening. In Germany, there are currently approximately 300,000 affected children, adolescents and adults. Their number is increasing steadily since there is no actual cure.
Überlebensrate der Kinder mit angeborenen Herzfehlern
Source: Competence Network for Congenital Heart Defects.
Frequency of Single Congenital Heart Defects
There are great differences in the frequency with which single heart defects occur. There are mild heart defects and severe heart defects that occur more frequently; but there are also mild and severe heart defects that are very rare. The following table gives an overview of the proportion of single diagnoses amongst congenital heart disease in total.collapse