Cardiovascular risk factors in adults with congenital heart defects - Recognised but not treated? An analysis of the German National Register for Congenital Heart Defects.

BACKGROUND: As adult congenital heart disease (ACHD) patients are aging, a high prevalence of cardiovascular risk factors is encountered similar to the general population. Currently, data regarding the primary and secondary prevention of acquired cardiovascular disease in ACHD is lacking. METHODS: The German National Register of Congenital Heart Defects was systematically screened for ACHD patients with established cardiovascular risk factors or documented acquired cardiovascular conditions. Data were analyzed with regard to the according medical treatment. RESULTS: Overall, 539 patients were included (mean age 38.4 ±â€¯17.7 years, 49.2% female). Diabetes was present in 57 pts. (10.6%), arterial hypertension in 113 pts. (21.0%), hyperlipidaemia in 81 pts. (15.0%) and obesity in 271 pts. (50.2%). 31 pts. (5.8%) were smokers. Coronary artery disease was established in 16 pts. (3.0%), peripheral vascular disease in 9 pts. (1.7%), and cerebrovascular accidents in 141 pts. (26.2%). Out of the patients with coronary artery disease only 81.3% received antithrombotic treatment. Only 18.8% were prescribed a statin. Of the pts. with peripheral arterial disease, 44.4% received an antiplatelet drug, and only 22.2% were on a statin. Patients with arterial hypertension received antihypertensive drugs in 66.4%. CONCLUSIONS: Primary and secondary prevention of acquired cardiovascular disease in ACHD is underutilized. This highlights the importance of educating primary physicians as well as ACHD physicians about the need of primary and secondary prevention for acquired cardiovascular disease.

Congenital heart disease patients' and parents' perception of disease-specific knowledge: Health and impairments in everyday life.

BACKGROUND: Children and adolescents with congenital heart disease (CHD) and their families require qualified combined medical and psychosocial information, care, and counseling. This study aimed to analyze CHD patients' and parents' perception of disease-specific knowledge, state of health, and impairments experienced in everyday life, as well as factors influencing these perceptions. MATERIALS AND METHODS: Analyses were based on a survey among patients/parents recruited via the German National Register for Congenital Heart Defects (NRCHD). The total sample (N = 818) was divided into four groups: "Children" (176 patients), "Adolescents" (142 patients), "Adults" (269 patients), and "Parents" (231 parents). The patients were stratified into those with simple and those with complex CHD. Descriptive and univariate analyses were performed. RESULTS: Patients' age and CHD severity were related to self-assessed state of health (P = .04 and P = .02). In addition, CHD severity was associated with worse impairment in everyday life (P < .001). Psychosocial support was related to the self-assessed state of health (P = .01) and the reported impairment in everyday life (P < .001). CONCLUSIONS: Patients' age, CHD severity, and psychosocial support seem to be related to self-assessed state of health and impairments in everyday life. To evaluate causality beyond associations, the development of patients' and parents' assessments and quality of life during the phase of transition from childhood to adulthood could be investigated by prospective long-term studies.

Eisenmenger syndrome and long-term survival in patients with Down syndrome and congenital heart disease.

OBJECTIVE: To characterise patients with trisomy 21 (Down syndrome, DS) based on the data of the German National Register for Congenital Heart Defects, to identify changes in the availability of surgical therapy over time and to analyse the impact of these changes on developing Eisenmenger syndrome (ES) as well as survival. METHODS: Out of 1549 patients with DS with congenital heart disease in the National Register for Congenital Heart Defects, 894 patients (55% female, mean age 17.5 years) had a post-tricuspid shunt lesion (atrioventricular septal defect 69.5%, ventricular septal defect 27.7%, patent arterial duct 2.6%) and were included in the current study. RESULTS: The likelihood of being treated interventionally or surgically before the age of 1 year increased significantly over time. In parallel, the likelihood of developing ES decreased over time (53% birth cohort during 1950s/1960s vs 0.5% birth cohort during 2000-2009, p<0.0001). Overall survival after 1, 10, 20 and 40 years was 96.8%, 94.1%, 92.6% and 75.5%, respectively. Patients with ES had a significantly worse survival compared with those without ES (HR 18.1; 95% CI 7.2 to 45.4; p<0.0001). CONCLUSIONS: The availability of surgical correction was associated with a decrease in the likelihood of developing ES. Patients with DS still have reduced survival prospects compared with the general population, but this effect is largely driven by patients developing ES who still have a very poor prognosis.

Cause of death in adults with congenital heart disease - An analysis of the German National Register for Congenital Heart Defects.

BACKGROUND: Due to the great advances in the care of patients with congenital heart disease (CHD), mortality has decreased significantly over the last decades. Nonetheless, mortality for adults with congenital heart disease (ACHD) is still higher than for the general population. An analysis regarding causes of death in a nationwide contemporary cohort of ACHD is lacking. METHODS: A well-characterized cohort of the German National Register for Congenital Heart Defects was screened for patients over the age of 18 years who died between January 2001 and January 2015. Data relating to the cardiac diagnosis, symptoms, operations, interventions, comorbidities, and causes of death were analyzed. RESULTS: During a median follow-up of 3.67 years (IQR 1.32-9.41), 239 (9.2%) out of 2596 patients died during the study period (110 female (46%), mean age at death 39.8 ± 17.8 years). The majority of these deaths was CHD-related (171 patients (71.5%)). Leading causes of death were heart failure (n=66, 27.6%), and sudden cardiac death (n=55, 23.0%). Deceased patients had a more complex CHD and more extracardiac comorbidities compared with living patients. CONCLUSIONS: Causes of death of ACHD patients in a large contemporary cohort from a national register are in the majority still CHD-related, with heart failure being the leading cause of death. Additionally, extracardiac comorbidities gain increasing importance.

Current therapy and outcome of Eisenmenger syndrome: data of the German National Register for congenital heart defects.

AIMS: We aimed to assess the contemporary outcome of Eisenmenger syndrome (ES), delineate the use of disease targeting therapies (DTT) in these patients and to investigate the effect of treatment on outcome in the community. METHODS AND RESULTS: Patients with ES were systematically identified from the German National Register for Congenital Heart Defects. Data on underlying diagnosis, medical therapy, and survival were collected. The impact of DTT on survival was assessed using time-dependant Cox analysis. Overall, 153 ES patients were included (mean age 34.0 ± 13.3 years, 46% females). Of these, 88 (57.5%) were treated with at least one DTT (76.1% Bosentan, 20.5% Sildenafil) while 17.6% were on dual DTT. In addition, 24.8% of patients received digoxin, 10.5% angiotensin-converting enzyme-inhibitors/angiotensin receptor blockers, and 17.6% ß-blockers. Moreover, 17.6% of patients were treated with oral anticoagulants, while 23.5% of patients received Aspirin. The survival rate at 1, 5, and 10 years of follow-up was only 92, 75, and 57% in the entire cohort, and was even worse in treatment naive ES patients (survival rate 86, 60, and 34% at 1, 5, and 10 years). Use of DTT was independently associated with a better survival (hazard ratio 0.42, P= 0.015). CONCLUSION: This study illustrates the alarmingly poor survival prospects of Eisenmenger patients by community-based data even in the current era with advanced DTT and in a country with a wealthy health system. Treatment naive ES patients had especially high mortality rates approaching 60-70% at 10 years of follow-up. Treatment with DTT was associated with better survival.

Three parties, one direction: Research priorities in adults with congenital heart disease. What do professionals, patients and relatives want to know?

BACKGROUND: Patients, their relatives, as well as medical specialists from the fields of paediatric cardiology, cardiology and cardiac surgery were surveyed to investigate the current research needs in the field of congenital heart disease (CHD) focussing specifically on the needs of those affected. MATERIAL AND METHODS: An online survey including four groups of patients with CHD (Fontan circulation, transposition of the great arteries [TGA] after atrial switch [AS] and after arterial switch operation [ASO], tetralogy of Fallot [TOF]) was performed. Each questionnaire comprised twelve topics. Persons affected (patients and relatives) were surveyed by means of CHD group specific questionnaires. Participants were recruited through the German National Register for Congenital Heart Defects. RESULTS: N=596 affected persons (Fontan circulation: n=189; TGA after AS: n=64; TGA after ASO: n=90; TOF: n=253) and 75 physicians (57.3% paediatric cardiologists, 28.0% cardiologists, 10.7% cardiac surgeons, 4.0% other) participated. In general, those affected assume a greater need for research than physicians. Regarding the CHD related topics to be the focus of future research, those affected largely agreed with the participating physicians, although with a different ranking of research topics. CONCLUSIONS: Based on the results of our study the challenges immanent in routine care for the CHD patient groups investigated can be identified. Accordingly, these topics should be prioritized in the research of the coming years.