New Insights into the Education of Children with Congenital Heart Disease with and without Trisomy 21.

Background and Objectives: Patients with congenital heart disease (CHD), especially as a concomitant syndromal disease of trisomy 21 (T21), are at risk for impaired neurodevelopment. This can also affect these patients' education. However, there continues to be a research gap in the educational development of CHD patients and T21 CHD patients. Materials and Methods: In total, data from 2873 patients from the German National Register for Congenital Heart Defects were analyzed. The data are based on two online education surveys conducted among patients registered in the National Register for Congenital Heart Defects (2017, 2020). Results: Of 2873 patients included (mean age: 14.1 ± 4.7 years, 50.5% female), 109 (3.8%) were identified with T21 (mean age: 12.9 ± 4.4 years, 49.5% female). T21 CHD participants had a high demand for early specific interventions (overall cohort 49.1%; T21 cohort 100%). T21 CHD children more frequently attended special schools and, compared to non-trisomy 21 (nT21) CHD patients, the probability of attending a grammar school was reduced. In total, 87.1% of nT21 CHD patients but 11% of T21 CHD patients were enrolled in a regular elementary school, and 12.8% of T21 CHD patients could transfer to a secondary school in contrast to 35.5% of nT21 CHD patients. Most of the T21 CHD patients were diagnosed with psychiatric disorders, e.g., learning, emotional, or behavioral disorders (T21 CHD patients: 82.6%; nT21 CHD patients: 31.4%; p < 0.001). Conclusions: CHD patients are at risk for impaired academic development, and the presence of T21 is an aggravating factor. Routine follow-up examinations should be established to identify developmental deficits and to provide targeted interventions.

Microcephaly is associated with impaired educational development in children with congenital heart disease.

Objectives: This study aims to evaluate the school careers of patients with congenital heart disease (CHD) and microcephaly. Methods: An exploratory online survey was conducted on patients from a previous study on somatic development in children with CHD in 2018 (n = 2818). A total of 750 patients participated in the online survey (26.6%). This publication focuses on 91 patients (12.1%) diagnosed with CHD and microcephaly who participated in the new online survey. Results: Microcephaly was significantly associated with CHD severity (p < 0.001). Microcephalic patients suffered from psychiatric comorbidity two times as often (67.0%) as non-microcephalic patients (29.8%). In particular, the percentage of patients with developmental delay, intellectual debility, social disability, learning disorder, or language disorder was significantly increased in microcephalic CHD patients (p < 0.001). A total of 85.7% of microcephalic patients and 47.6% of non-microcephalic patients received early interventions to foster their development. The school enrollment of both groups was similar at approximately six years of age. However, 89.9% of non-microcephalic but only 51.6% of microcephalic patients were enrolled in a regular elementary school. Regarding secondary school, only half as many microcephalic patients (14.3%) went to grammar school, while the proportion of pupils at special schools was eight times higher. Supportive interventions, e.g., for specific learning disabilities, were used by 52.7% of microcephalic patients and 21.6% of non-microcephalic patients. Conclusion: Patients with CHD and microcephaly are at high risk for impaired educational development. Early identification should alert clinicians to provide targeted interventions to optimize the developmental potential.

Erectile Dysfunction in Men With Adult Congenital Heart Disease: A Prevalent but Neglected Issue.

BACKGROUND AND OBJECTIVES: For adult men with congenital heart disease (ACHD), data on erectile dysfunction (ED) is limited. We aimed to assess the frequency of ED, its role in patient-physician communication and to identify parameters predicting ED. METHODS: Male ACHD ≥18 years registered at the German National Register for Congenital Heart Defects were invited to participate in an online questionnaire about sexual health. Participants with presumed ED according to International Index of Erectile Function Score were compared to patients without ED. RESULTS: The 371 patients responded to the questionnaire (83% with moderate to highly complex ACHD). The 43% presented with more than mild ED. When ED was present, patients complained about general anxiety to be sexually active more often (p<0.05) and underwent sexual activity less frequently compared to those without ED (p<0.05). Age ≥40 years (odds ratio [OR], 3.04; p=0.002), being single (OR, 6.82; p<0.0001), anxiety to be sexually active (OR, 2.64; p=0.0002) and psychiatric disease (OR, 4.33; p<0.0007) emerged as independent predictors for ED. Overall, patients sought medical advice in 6.7% of cases, whilst 29.6% would appreciate an active approach by the physician to address this sensitive topic. CONCLUSIONS: ED is affecting one third to one half of male ACHD according to a questionnaire-based analysis. Older age, being single, fear of sexual activity due to ACHD and psychiatric disorder emerged as independent predictors for ED. These parameters can easily be assessed to identify patients at risk. ED should be addressed proactively by health professionals.

Secundum Type Atrial Septal Defect in Patients with Trisomy 21-Therapeutic Strategies, Outcome, and Survival: A Nationwide Study of the German National Registry for Congenital Heart Defects.

(1) Secundum type atrial septal defect (ASD II) is usually considered a relatively benign cardiac lesion amenable to elective closure at preschool age. Patients with trisomy 21 (T21), however, are known to have a higher susceptibility for pulmonary vascular disease (PVD). Therefore, T21 children may present with clinical symptoms earlier than those without associated anomalies. In addition, early PVD may even preclude closure in selected T21 patients. (2) We performed a retrospective analysis of the German National Register for Congenital Heart Defects including T21 patients with associated isolated ASD II. We report incidence, demographics, therapeutic strategy, outcome, and survival of this cohort. (3) Of 46,628 patients included in the registry, 1549 (3.3%) had T21. Of these, 156 (49.4% female) had an isolated ASD II. Fifty-four patients (34.6%) underwent closure at 6.4 ± 9.9 years of age. Over a cumulative follow-up (FU) of 1148 patient-years, (median 7.4 years), only one patient developed Eisenmenger syndrome and five patients died. Survival of T21 patients without PVD was not statistically different to age- and gender-matched controls from the normal population (p = 0.62), whereas children with uncorrected T21/ASD II (including patients with severe PVD, in whom ASD-closure was considered contraindicated) showed a significantly higher mortality. (4) The outcome of T21-patients with ASD II and without PVD is excellent. However, PVD, either precluding ASD-closure or development of progressive PVD after ASD-closure, is associated with significant mortality in this cohort. Thus T21 patients with ASD II who fulfill general criteria for closure and without PVD should be offered defect closure analogous to patients without T21.

Ventricular assist devices in paediatric cardiomyopathy and congenital heart disease: An analysis of the German National Register for Congenital Heart Defects.

BACKGROUND: Ventricular assist devices (VAD) are increasingly used in patients with end-stage heart failure due to acquired heart disease. Limited data exists on the use and outcome of this technology in children. METHODS: All children (<18 years of age) with VAD support included in the German National Register for Congenital Heart Defects were identified and data on demographics, underlying cardiac defect, previous surgery, associated conditions, type of procedure, complications and outcome were collected. RESULTS: Overall, 64 patients (median age 2.1 years; 45.3% female) receiving a VAD between 1999 and 2015 at 8 German centres were included in the analysis. The underlying diagnosis was congenital heart disease (CHD) in 25 and cardiomyopathy in 39 children. The number of reported VAD implantations increased from 13 in the time period 2000-2004 to 27 implantations in the time period 2010-2014. During a median duration of VAD support of 54 days, 28.1% of patients experienced bleeding complications (6.3% intracerebral bleeding), 14.1% thrombotic (10.9% VAD thrombosis) and 23.4% thromboembolic complications (including cerebral infarction in 18.8% of patients). Children with cardiomyopathy were more likely to receive a cardiac transplantation (79.5% vs. 28.0%) compared to CHD patients. Survival of cardiomyopathy patients was significantly better compared to the CHD cohort (p < 0.0001). Multivariate Cox-proportional analysis revealed a diagnosis of CHD (hazard ratio [HR] 4.04, p = 0.001), age at VAD implantation (HR 1.09/year, p = 0.04) and the need for pre-VAD extracorporeal membrane oxygenation (ECMO) support (HR 3.23, p = 0.03) as independent predictors of mortality. CONCLUSIONS: The uptake of VAD therapy in children is increasing. Morbidity and mortality remain high, especially in patients with congenital heart disease and those requiring ECMO before VAD implantation.

Optimizing Care for Adults with Congenital Heart Disease: Results of a Conjoint Analysis Based on a Nationwide Sample of Patients Included in the German National Register.

(1) Background: Congenital heart disease (CHD) requires lifelong specialized care. Failure to follow up and gaps in care are common in this group and lead to increased morbidity/mortality. We evaluated patients' perceived needs and expectations regarding specialized care using state-of-the-art statistical and market research techniques based on a nationwide sample of CHD patients. (2) Methods: A random sample of adults with CHD registered in the German National Register for Congenital Heart Defects were invited to answer an adaptive online questionnaire based on the conjoint analysis (CA) technique. CA determines the relative importance of various aspects of health care provision and allows individuals to trade between characteristics, thus recognizing limited resources. (3) Results: 637 patients participated (mean age 33.8 ± 12.6 years; 55.6% female; disease complexity: simple defect 12.6%, moderate complexity 40.3%, complex CHD 40.2%) in the analysis. Patients assigned the highest relative importance to aspects of patient-physician communication, physician qualifications, waiting time, medical care, and medical equipment. Comfort-related aspects such as driving time or hotel aspects of care received much lower scores. We identified four well-defined clusters of patients with differing expectation patterns: (i) time sensitive patients; (ii) excellence seeking patients; (iii) continuity seekers, and (iv) support seeking patients. (4) Conclusions: Adult CHD patients rank effective patient-physician interaction and communication as the most important factors. As we identified significant heterogeneity between CHD patients, centers should cater for individual preferences and integrate individual needs into treatment plans to prevent failure to follow up and ensure patient compliance.

Education of Children With Cyanotic Congenital Heart Disease After Neonatal Cardiac Surgery.

BACKGROUND: The aim of the study was to evaluate the educational achievement of patients diagnosed with univentricular heart physiology (UVHP) or transposition of the great arteries (TGA) after neonatal cardiac surgery. METHODS: An exploratory online survey was performed with patients registered with the National Register for Congenital Heart Defects in Germany. For this publication, a subgroup analysis was conducted among patients diagnosed with TGA (n = 173; 36.3%) and UVHP (n = 304; 63.7%). RESULTS: Median age of the sample at school enrollment was 6 years (range, 5-8 years). The majority were enrolled at a standard elementary school (n = 368 of 477; 77.1%), although patients with UVHP were enrolled significantly more often at a special needs school (n = 52 of 304; 17.1%, TGA patients n = 11/ of 173; 6.4%, P < .001). A total of 45.8% (n = 66 of 144) of the patients graduated with a high school diploma. A substantial number of patients had been diagnosed with behavioral or learning disorders (TGA patients n = 63 of 173 [36.4%], UVHP patients n = 148 of 304 [48.7%]) and received early supportive therapy or remedial teaching before (TGA patients n = 89 of 173 [51.4%], UVHP patients n = 209 of 304 [68.8%]) and/or during their school careers (TGA patients n = 54 of 173 [31.2%], UVHP patients n = 120 of 304 [39.5%]). CONCLUSIONS: A large proportion of patients who underwent neonatal cardiac surgery graduated with a high school diploma. These results are of great importance to congenital heart defect patients, affected families, and treating physicians. Nevertheless, study participants, especially patients with UVHP, face some academic challenges. We conclude that long-term follow-up examinations and regular developmental assessments may be beneficial.

Physical Activity Among Children With Congenital Heart Defects in Germany: A Nationwide Survey.

Objective: In children with congenital heart defects (CHD), a sedentary lifestyle should be avoided and usually WHO recommendations on physical activity (PA) are supposed to be followed. In order to obtain representative data of the actual amount of PA (and potential influencing factors) in children with CHD we performed a nationwide online survey. Methods: All patients aged 6-17 years registered in the German National Register for CHD were contacted by email and asked to participate in the survey using the comprehensive questionnaire of the "Motorik-Modul" from the German Health Interview and Examination Survey for Children and Adolescents (KiGGS), thus allowing the comparison with a representative age-matched subset of 3.385 participants of the KiGGS study. The questionnaire for CHD-patients was amended by specific questions regarding medical care, sports recommendations and PA restrictions. Results: Complete datasets of 1.198 patients (mean age of 11.6 ± 3.1 years) were available for evaluation. Compared to the reference group, CHD patients significantly less frequently reached the WHO recommended level of 60 min of daily PA (8.8 vs. 12%; p < 0.001). Enjoyment in sports was almost equally distributed across CHD and reference groups, and strongly correlated with the level of PA (r = 0.41; p < 0.001). Remarkably, 49.2% of children with complex CHD, 31.7% with moderate, and even 13.1% with simple CHD were advised by their physician to restrict PA. Conclusions: According to this nationwide survey, PA is markedly reduced in children with CHD. An important reason for this might be an unexpected high rate of physician-recommended restrictions on levels of PA.

Pulmonary hypertension after shunt closure in patients with simple congenital heart defects.

BACKGROUND: Patients with simple shunt lesions, such as atrial septal defect (ASD), ventricular septal defect (VSD) and persistent arterial duct (PDA) remain at risk of developing pulmonary hypertension (PH) even after correction of their cardiac defect. We aimed to assess the contemporary prevalence of PH in a well characterized nationwide group of patients based on the German National Register for Congenital Heart Defects. METHODS AND RESULTS: We included all patients >16 years of age with an isolated diagnosis of ASD, VSD or PDA. Only patients with previous surgical or interventional closure of the defect were included. Patients with genetic syndromes were excluded. Out of 49,597 CHD patients in the register we identified 825 patients with closed, isolated simple defects (52% ASD, 41% VSD, 7% PDA). Of these, 25 (3%) developed PH after a median follow-up of 16 years from defect closure. The risk of PH increased significantly with age at follow-up (p < 0.0001) and age at repair (p < 0.0001) on logistic regression analysis Patients with PH were significantly more likely to be symptomatic (59% vs. 9% in NYHA class ≥2, p < 0.0001) and had significantly higher mortality (hazard ratio 13.4, p < 0.0001) compared to the remaining patients. CONCLUSIONS: Based on data from the German National Register CHD Register we report a PH prevalence of 3.0% in patients with corrected, simple lesions. Patients with PH were more symptomatic and had significantly increased mortality risk. Life-long surveillance and low threshold for workup is recommended to ascertain diagnosis of PH, which has important prognostic and clinical implications.

Surgery during pregnancy - results of a German questionnaire.

OBJECTIVES: Worldwide, not only the number of female medical students, but also of female surgeons increases. Simultaneously, younger generations take a closer look to their work-life balance. With this in mind, it seems necessary to evaluate the expectations of female surgeons in particular with respect to pregnancy during their surgical career. METHODS: Therefore, a nationwide survey was conducted in Germany from July to December 2016 under the auspices of the German Society of Surgery as well as the Professional Board of German Surgeons. The questionnaire involved 2,294 female surgeons and 1,843 complete records were evaluated. RESULTS: Of the analyzed answers, 62% of the women (n=781) were operating during pregnancy. The joy of surgery (91.6%), followed by team spirit (57.1%), were the main motivations to perform operations while pregnant. Operative activity decreased from 30.8% in the first 3 months of pregnancy to 21.5% during the last three months. Regarding the possible complaints, e.g., leg edema, back pain, premature labor and vaginal bleeding, there were no significant differences between the women with or without activity in the operating room. Sick leave due to pregnancy (1-10 days) was stated by 40.4% of respondents. CONCLUSION: Despite strong legal regulations for pregnant surgeons, the survey showed that most female surgeons are eager to operate despite their pregnancy. The results also demonstrate no significant differences regarding complications during pregnancy- or pregnant-dependent absence from work. Hospitals and surgical departments are asked to establish proper working conditions for pregnant surgeons and pregnancy should not be an obstacle for a career in surgery.

Educational achievement of children with congenital heart disease: Promising results from a survey by the German National Register of Congenital Heart Defects.

BACKGROUND: Survival rates of children with congenital heart disease (CHD) have increased significantly in the decade. There is now increased interest in the long-term outcome and quality of life of these children. AIMS: To assess the educational achievement of patients with CHD in Germany. STUDY DESIGN: Cross-sectional study using an online survey. The recruitment of study participants was carried out via the database of the German National Register for Congenital Heart Defects (NRCHD). SUBJECTS: Patients born between 1992 and 2011 were enrolled in the study. For 2609 study participants (female = 1870 (71.7%); 1072 (41.1%) patients; 1537 (58.9%) parents), who participated in the survey, detailed information regarding the underlying CHD diagnosis and clinical data was available. OUTCOME MEASURES: Age at enrollment, secondary school form, school year repetition, school degree. RESULTS: The large majority of study participants were enrolled at a conventional elementary school (83.4%) and started school at the age of 6 years or below (73.3%). In total 45.7% of graduated study participants graduated with the qualification necessary to study at any university. In terms of analysis of the different CHD severity subgroups 57.3% of patients with a mild CHD, 47.5% with a moderate CHD and only 35.1% suffering from a severe CHD attained a high school diploma. CONCLUSIONS: In our study, the majority of participating CHD patients had a standard school career. These initial results are of great importance to affected families and treating physicians as they show that, in general, a normal school career is possible for all CHD patients.

Patients with congenital heart defect and their families support genetic heart research.

BACKGROUND: Congenital heart disease (CHD) affects up to 1% of live births the etiology remains relatively poorly understood. Thus, cardiac research is needed to understand the underlying pathomechanisms of the disease. About 51 000 CHD patients are registered in the German National Register for Congenital Heart Defects (NRCHD). Patients and relatives were interviewed online about their willingness to support genetic heart research in order to donate a biological sample. METHODS: Study participants were recruited via the database of the NRCHD. Seven thousand nine hundred eighty-nine patients were invited to participate in the study. Participants have been asked to rate three questions on a ten-staged Likert scale about their willingness to provide a saliva/blood sample and their motivation to ask family members to support genetic heart research. RESULTS: Overall, 2035 participants (patients/relatives) responded the online survey (25.5%). Two-thirds of the participants are willing to donate a saliva sample. Whereas the motivation to provide a blood sample is slightly lower (patients: 63.8%, relatives: 60.6%). Female relatives are more fain to provide a saliva sample as well as a blood sample compared to men (saliva sample: P < .001, blood sample: P < .01). The motivation to ask an additional family member for a biological sample was significantly higher in relatives (59.2%) compared to patients (48.4%). CONCLUSIONS: The motivation to provide biological samples is high reflecting the need for genetic research to unravel the pathomechanism of CHD. A future aim should be to offer an individual risk assessment for each patient based on the underlying genetics.

Cardiac resynchronization therapy in congenital heart disease: Results from the German National Register for Congenital Heart Defects.

BACKGROUND: Cardiac resynchronization therapy (CRT) is an established option for patients with heart failure. Limited data exists on indications and outcome of CRT in contemporary congenital heart disease (CHD) patients. METHODS AND RESULTS: All patients with CRT registered in the German National Register for Congenital Heart Defects were systematically identified. We analysed data on demographics, type of congenital defect as well as repair, associated conditions, indication for CRT, heart failure medication, combination with a defibrillator or pacemaker and outcome. Overall, 65 patients with CRT were identified. The most common congenital diagnoses were Tetralogy of Fallot (n = 11), congenitally corrected transposition of the great arteries (ccTGA) (n = 9) and double outlet right ventricle (n = 6). The majority of patients (n = 48, 87%) had conventional antibradycardia pacing or ICD indications. Of these, the majority (n = 44) underwent an upgrade to a CRT system to avoid the detrimental consequences of longstanding conventional ventricular single-site pacing, whereas four patients required an ICD due to heart failure and a history of malignant ventricular tachycardia. During a median follow-up of 6.9 years 19 patients developed complications: 16 patients experienced pacemaker lead dysfunction and 3 patients pacemaker infection. CONCLUSIONS: The current study based on a large national register for CHD shows that CRT is feasible and can be used as an adjunct in the heart failure treatment of selected CHD patients. Uptake of this therapy proved to be low in this nationwide study and CRT implantation was largely used in patients with a pre-existing pacing indication or those requiring an ICD.

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.

Educational level and employment status in adults with congenital heart disease.

Purpose Through this study we aimed to assess the educational level and employment status of adults with CHD in Germany. METHODS: Data were acquired from an online survey carried out in 2015 by the German National Register for Congenital Heart Defects. A total of 1458 adults with CHD participated in the survey (response rate: 37.6%). For 1198 participants, detailed medical information, such as main cardiac diagnosis and information from medical reports, was available. RESULTS: Of the participants surveyed (n=1198), 54.5% (n=653) were female, and the mean age was 30 years. The majority of respondents (59.4%) stated that they had high education levels and that they were currently employed (51.1%). Patients with simple CHD had significantly higher levels of education (p<0.001) and were more likely to be employed (p=0.01) than were patients with complex CHD. CONCLUSIONS: More than half of the participants had high education levels and the majority were employed. The association between CHD and its severity and individuals' educational attainment should be investigated more closely in future studies.

Dynamics in prevalence of Down syndrome in children with congenital heart disease.

We assessed the dynamics in the prevalence of children with congenital heart disease (CHD) and Down syndrome in Germany with regard to phenotype, severity, and gender. Data from patients with CHD and Down syndrome born between 1980 and 2014 were analyzed, who are registered with the German National Register for Congenital Heart Defects. One thousand six hundred eighteen CHD patients with Down syndrome were identified. The prevalence of children born with both Down syndrome and CHD was constant from 2005 to 2009 but increased from 2010 to 2014. Regarding CHD groups, complex and simple lesions have become more equal since 2005. The number of simple lesions with shunt has a peak prevalence in the period of 2010-2014. Atrioventricular septal defect was the most common CHD phenotype, but temporal changes were found within the group of CHD phenotypes over the observation period. CONCLUSION: Our findings suggest a growing number of CHD and Down syndrome, which may be the result of improved medical management and progress in educational, social, and financial support. This development is noteworthy as it adds new aspects to present discussions in the media and political settings. What is known: • Congenital heart disease is regarded to be the most important clinical phenomenon in children with Down syndrome, due to its significant impact on morbidity and mortality. • New developments in prenatal diagnostic and therapy management of congenital heart disease continue to influence the number of patients diagnosed with congenital heart disease and Down syndrome. What is New: • This study provides essential data giving the first overview of the dynamics in the prevalence of congenital heart disease and Down syndrome over an extended length of time up to 2015 in a large patient cohort, taking recent developments into account. • Our data suggest a growing prevalence of congenital heart disease and Down syndrome, which may be the result of improved medical management for Down syndrome patients and progress in educational, social, and financial support for their families; this development is noteworthy as it adds new aspects to the present discussion in the media and political settings.

Are adults with congenital heart disease informed about their risk for infective endocarditis and treated in accordance to current guidelines?

BACKGROUND: Adults with congenital heart disease (ACHD) have an increased risk for infective endocarditis (IE). In the last decade, the recommendations for IE prophylaxis have changed substantially. The knowledge level of patients about IE and IE prophylaxis has not been studied. METHODS: Patients recruited via the German National Register for Congenital Heart Defects were invited to an online survey about IE. Patients were divided into two groups based on ESC guidelines: high IE risk (antibiotic prophylaxis recommended) and low IE risk (prophylaxis not recommended). RESULTS: Overall, 1458 patients participated and out of these 1211 (age 30.5±11.8years, female=54.2%) with detailed clinical information were further analyzed. 343 patients had a high IE risk, whereas 868 had a low risk. Overall, 74.5% (n=902) stated to know what IE is (low IE risk: 71.3%, high IE risk: 82.5%) Out of these who stated to know what IE is (n=902), 76.5% (n=690) chose the correct answer in a multiple choice question (low IE risk: 76.4%; high IE risk: 76.7%). Antibiotic prophylaxis was known to 66.2% (low IE risk: 59.9%; high IE risk: 82.2%). Out of these who stated to know what antibiotic prophylaxis is (n=802), 83.8% (n=672) chose the correct answer in a multiple choice question (low IE risk: 82.9%; high IE risk: 85.5%). CONCLUSIONS: This study reveals important knowledge gaps regarding IE and antibiotic prophylaxis in ACHD patients. A discussion about IE and antibiotic prophylaxis should take place with every ACHD patient during regular clinical contacts to close this knowledge gap.

Changing prevalence of severe congenital heart disease: Results from the National Register for Congenital Heart Defects in Germany.

OBJECTIVE: To assess the prevalence of congenital heart disease (CHD) in Germany in relation to phenotypes, severity and gender. DESIGN: Cross-sectional registry study. SETTING: We analyzed data from patients with CHD born between 1996 and 2015. PATIENTS: A total of 26 630 patients, registered with the NRCHD, were born between 1996 and 2015. 10 927 patients were excluded from the current analysis due to prior registration with the NRCHD under the German PAN Prevalence Study, which showed a potential bias in the inclusion of this patient population (proportion of mild cardiac lesions was comparatively high due to improved diagnostic capability for earlier identifying minor lesions). At least 15 703 patients with demographic data and detailed medical information were included in the current study. INTERVENTIONS: None. OUTCOME MEASURES: Prevalence of CHD in Germany differentiated into gender, severity, and phenotype. RESULTS: In total, 15 703 patients with CHD (47.1% female) were included in this study. The five most common phenotypes were found to be ventricular septal defect (19.2%), atrial septal defect (13.0%), Tetralogy of Fallot (9.3%), univentricular heart (9.4%), and coractation of the aortae (7.0%). The prevalence of CHD in regard to severity changed over the duration of the observation period. From 1996 to 2007, the number of simple CHD rose steadily (P < .001), whereas the number of severe CHD has grown significantly since 2008/2009 (P < .001). In regard to gender, the prevalence of simple CHD was higher in females, whereas complex lesions were more common in males (P < .001). CONCLUSIONS: Our study shows a growing number of registered severe CHD in the recent decade in Germany. This development is noteworthy as it implicates a growing demand for first intensive hospital care, expert pediatric cardiologic aftercare, and consequently higher economic impact for this patient population.

Transition in Patients with Congenital Heart Disease in Germany: Results of a Nationwide Patient Survey.

BACKGROUND: A growing number of adults with congenital heart disease (ACHD) pose a particular challenge for health care systems across the world. Upon turning into 18 years, under the German national health care system, ACHD patients are required to switch from a pediatric to an adult cardiologist or an ACHD-certified provider. To date, reliable data investigating the treatment situation of ACHD patients in Germany are not available. MATERIALS AND METHODS: An online survey was conducted in collaboration with patient organizations to address the life situation and the conditions of health care provision for ACHD patients in Germany. ACHD patients were recruited from the database of the National Register for Congenital Heart Defects (NRCHD) and informed about the survey via email, websites, and social networks. A total of 1,828 ACHD patients (1,051 females) participated in this study. The mean age was 31.7 ± 11.7 years. Participants were surveyed about treating physicians and the institution mainly involved in the treatment of their CHD. In addition, participants were asked questions to assess the level of trust toward their treating physician and their familiarity with the term "ACHD-certified provider." RESULTS: Among the surveyed patients, 25.4% stated that they attended a specific ACHD clinic at a heart center regularly, 32.7% were treated in a private practice setting by a pediatric cardiologist, 32.4% in a private practice (adult) cardiology setting, and 9.5% were treated by an "other physician." Only 24.4% of the male and 29.7% of the female ACHD patients were familiar with the term "ACHD-certified provider." CONCLUSION: The transfer from pediatric cardiology to ACHD care requires further attention as many adult patients have not transferred to certified ACHD providers. The question of whether ACHD patients in Germany are offered consistent and adequate care should also be investigated in more detail. The answers regarding the ACHD certification are particularly disappointing and indicative of a large information gap and inadequate education in clinical practice.

Is having a job a protective factor? Employment status and state of medical care as subjectively perceived by adults with CHD in Germany.

BACKGROUND: Most patients born with CHD nowadays reach adulthood, and thus quality of life, life situation, and state of medical care aspects are gaining importance in the current era. The present study aimed to investigate whether patients' assessment depends on their means of occupation. The findings are expected to be helpful in optimising care and for developing individual treatment plans. METHODS: The present study was based on an online survey conducted in cooperation with patient organisations. Participants were recruited from the database of the German National Register for Congenital Heart Defects. In total, 1828 individuals (777 males, 1051 females) took part. Participants were asked to rate aspects such their state of health on a six-tier scale (1=worst specification). Response behaviour was measured against the background of occupational details. RESULTS: Training for or pursuing a profession was found to be significantly associated with participants' rating of five of the six examined aspects (p<0.05). Sex seemed to play an important part in four of the six aspects. CONCLUSIONS: An optimal treatment plan for adults with CHD should always consider aspects such as sex and employment status. To work out such an optimal and individual treatment plan for each adult CHD patient, an objective tool to measure patients' actual CHD-specific knowledge precluding socially accepted response bias would be very useful.