Endangered patients with congenital heart defect during transition—Germany-wide evaluation of medical data from National Register for Congenital Heart Defects (NRCHD)

Background: Appropriate care over the entire lifespan is essential in the population with congenital heart defect since the number of patients with congenital heart defect is increasing steadily worldwide. More than 90% survive into adulthood nowadays. The transition from pediatric to adult care in patients with congenital heart defect is a major challenge in clinical practice and often fails. Patients with congenital heart defect are generally at higher risk for different acquired secondary diagnoses. This cross-sectional retrospective study analysed data from the German National Register for Congenital Heart Defects to gain insight into the clinically relevant health-status of the transition population among congenital heart defect patients in Germany. Methods: Adolescents and young adults with congenital heart defect between the ages of 15 to 25 years (which have been defined as the transition generation) were identified using the National Register of Congenital Heart Defects medical database. Out of 55,687 patients with congenital heart defect, 8,834 adolescents and young adults with congenital heart defect [4,063 female (46.0%); 20.3±3.1 years] were included in the statistical analyses. Statistical analyses were conducted using the student’s t-test, χ²-test and Fisher’s exact test. Results: Severity of congenital heart defect: simple (23.4%), moderate (45.1%) and complex (31.5%). Most common congenital heart defect: atrial septal defects (14.9%) followed by ventricular septal defects (12.8%) and tetralogy of Fallot (10.5%). Most frequent acquired cardiac diagnosis: arrhythmia (25.5%) followed by secondly pulmonary hypertension (4.5%) and thirdly systemic arterial hypertension (3.6%). Almost 10% had chromosomal abnormalities and other genetic syndromes. Patients had neurological defects overall with 7.3%, followed by musculoskeletal defects with 6.9% and psychological disorders with 5.6%. Conclusions: Adolescents and young adults with congenital heart defect need to bridge the gap between pediatric and adult cardiology as they already show up to 4 cardiac and up to 7 extracardiac acquired secondary diagnoses during the transition period. Otherwise, early detection of an acquired secondary diagnosis, which affects the lives of young adults with congenital heart defect, fails with all its consequences.