Publications by authors named "Ulrike M Bauer"

41 Publications

Maternal and neonatal complications in women with congenital heart disease: a nationwide analysis.

Eur Heart J 2021 Oct 12. Epub 2021 Oct 12.

Department of Cardiology III, Adult Congenital and Valvular Heart Disease, University Hospital Muenster, Albert-Schweitzer-Campus 1, 48149 Münster, Germany.

Aims: The aim of this study was to provide population-based data on maternal and neonatal complications and outcome in the pregnancies of women with congenital heart disease (CHD).

Methods And Results: Based on administrative data from one of the largest German Health Insurance Companies (BARMER GEK, ∼9 million members representative for Germany), all pregnancies in women with CHD between 2005 and 2018 were analysed. In addition, an age-matched non-CHD control group was included for comparison and the association between adult CHD (ACHD) and maternal or neonatal outcomes investigated. Overall, 7512 pregnancies occurred in 4015 women with CHD. The matched non-CHD control group included 6502 women with 11 225 pregnancies. Caesarean deliveries were more common in CHD patients (40.5% vs. 31.5% in the control group; P < 0.001). There was no excess mortality. Although the maternal complication rate was low in absolute terms, women with CHD had a significantly higher rate of stroke, heart failure and cardiac arrhythmias during pregnancy (P < 0.001 for all). Neonatal mortality was low but also significantly higher in the ACHD group (0.83% vs. 0.22%; P = 0.001) and neonates to CHD mothers had low/extremely low birth weight or extreme immaturity (<0.001) or required resuscitation and mechanical ventilation more often compared to non-CHD offspring (P < 0.001 for both). On multivariate logistic regression maternal defect complexity, arterial hypertension, heart failure, prior fertility treatment, and anticoagulation with vitamin K antagonists emerged as significant predictors of adverse neonatal outcome (P < 0.05 for all). Recurrence of CHD was 6.1 times higher in infants to ACHD mothers compared to controls (P < 0.0001).

Conclusions: This population-based study illustrates a reassuringly low maternal mortality rate in a highly developed healthcare system. Nevertheless, maternal morbidity and neonatal morbidity/mortality were significantly increased in women with ACHD and their offspring compared to non-ACHD controls highlighting the need of specialized care and pre-pregnancy counselling.
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http://dx.doi.org/10.1093/eurheartj/ehab571DOI Listing
October 2021

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.

J Clin Med 2021 Aug 25;10(17). Epub 2021 Aug 25.

Klinik für Angeborene Herzfehler und Kinderkardiologie, Universitätsklinikum Schleswig-Holstein, 24105 Kiel, Germany.

(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 ( = 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.
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http://dx.doi.org/10.3390/jcm10173807DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8432184PMC
August 2021

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

Int J Cardiol 2021 Nov 3;343:37-44. Epub 2021 Sep 3.

Competence Network for Congenital Heart Defects Berlin, Germany; Department of Congenital Heart Disease and Paediatric Cardiology, German Heart Centre Munich, TUM School of Medicine, Technical University of Munich, Germany; DZHK (German Center for Cardiovascular Research), partner site Munich Heart Alliance, Munich, Germany.

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.
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http://dx.doi.org/10.1016/j.ijcard.2021.08.047DOI Listing
November 2021

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.

J Clin Med 2021 Aug 6;10(16). Epub 2021 Aug 6.

Department of Cardiology III-Adult Congenital and Valvular Heart Disease, University Hospital Muenster, Albert-Schweitzer Campus 1, 48149 Münster, Germany.

(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.
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http://dx.doi.org/10.3390/jcm10163483DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8396998PMC
August 2021

Pulmonary valve prostheses: patient's lifetime procedure load and durability. Evaluation of the German National Register for Congenital Heart Defects.

Interact Cardiovasc Thorac Surg 2021 Aug 26. Epub 2021 Aug 26.

Department of Cardiac, Thoracic, Transplantation and Vascular Surgery, Hanover Medical School, Hanover, Germany.

Objectives: We evaluated 4384 procedures performed between 1957 and 2018, collected in the National Register for Congenital Heart Defects, conducted on 997 patients with 1823 pulmonary valve replacements (PVRs), including 226 implanted via catheter [transcatheter valve (TCV)]. Main study targets are as follows: TCV benefit, valve type durability, decade-wise treatment changes and procedure frequencies over the lifetime of a PVR patient.

Methods: We studied TCV impact on surgical valve replacement (via Kaplan-Meier); pulmonary valve type-specific performance (Kaplan-Meier and Cox regressions with age group as stratification or ordinary variable); procedure interval changes over the decades (Kaplan-Meier); procedure load, i.e. frequency of any procedure/surgical PVR/interventional or surgical PVR by patient age (multistate analyses).

Results: TCV performance was equivalent to surgical PVRs and extended durability significantly. Homografts were most durable; Contegras lasted comparably less in older; and Hancock devices lasted less in younger patients. Matrix P-valves showed poorer performance. Age group stratification improves the precision of valve-specific explantation hazard estimations. The current median interval between procedures is 2.6 years; it became significantly shorter in most age groups below 40 years. At 30 years, 80% of patients had undergone ≥3 procedures, 20% ≥3 surgical PVRs and 42% ≥3 surgical or interventional PVRs.

Conclusions: TCVs doubled freedom from explantation of conventional valves. Homografts' age group-specific explantation hazard ratio was lowest; Matrix P's hazard ratio was highest. Age-stratified Cox regressions improve the precision of prosthesis durability evaluations. The median time between procedures for PVR patients shortened significantly to 2.6 years. At 30 years, 42% had ≥3 PVRs.
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http://dx.doi.org/10.1093/icvts/ivab233DOI Listing
August 2021

Lack of specialist care is associated with increased morbidity and mortality in adult congenital heart disease: a population-based study.

Eur Heart J 2021 Jul 16. Epub 2021 Jul 16.

Department of Cardiology III-Adult Congenital and Valvular Heart Disease, University Hospital Muenster, Albert-Schweitzer Campus 1, Building A1, Münster 48149, Germany.

Aims: The aim of this study was to provide population-based data on the healthcare provision for adults with congenital heart disease (ACHD) and the impact of cardiology care on morbidity and mortality in this vulnerable population.

Methods And Results: Based on administrative data from one of the largest German Health Insurance Companies, all insured ACHD patients (<70 years of age) were included. Patients were stratified into those followed exclusively by primary care physicians (PCPs) and those with additional cardiology follow-up between 2014 and 2016. Associations between level of care and outcome were assessed by multivariable/propensity score Cox analyses. Overall, 24 139 patients (median age 43 years, 54.8% female) were included. Of these, only 49.7% had cardiology follow-up during the 3-year period, with 49.2% of patients only being cared for by PCPs and 1.1% having no contact with either. After comprehensive multivariable and propensity score adjustment, ACHD patients under cardiology follow-up had a significantly lower risk of death [hazard ratio (HR) 0.81, 95% confidence interval (CI) 0.67-0.98; P = 0.03) or major events (HR 0.85, 95% CI 0.78-0.92; P < 0.001) compared to those only followed by PCPs. At 3-year follow-up, the absolute risk difference for mortality was 0.9% higher in ACHD patients with moderate/severe complexity lesions cared by PCPs compared to those under cardiology follow-up.

Conclusion: Cardiology care compared with primary care is associated with superior survival and lower rates of major complications in ACHD. It is alarming that even in a high resource setting with well-established specialist ACHD care approximately 50% of contemporary ACHD patients are still not linked to regular cardiac care. Almost all patients had at least one contact with a PCP during the study period, suggesting that opportunities to refer patients to cardiac specialists were missed at PCP level. More efforts are required to alert PCPs and patients to appropriate ACHD care.
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http://dx.doi.org/10.1093/eurheartj/ehab422DOI Listing
July 2021

Systematic assessment of health care perception in adults with congenital heart disease in Germany.

Cardiovasc Diagn Ther 2021 Apr;11(2):481-491

Department of Congenital Heart Disease and Pediatric Cardiology, German Heart Center Munich, Technical University Munich, Germany.

Background: The number of adults with congenital heart disease (ACHD) is steadily rising due to increased survival rate and improved medical resources. Accordingly, more than 330,000 ACHD are currently living in Germany. Almost all of them require lifelong specialized medical follow-up for their chronic heart disease, often accompanied by residua, sequelae, or comorbidities. Primary care physicians (PCPs) are a crucial factor in directing patients to ACHD specialists or specialized institutions, but despite all efforts, the number of ACHD under specialized care is low, the lost-to-follow-up rate is high, and the morbidity and mortality is substantial. The present cross-sectional study was designed to systematically characterize the health care of ACHD from a patient perspective, including (I) use of medical care by ACHD, (II) medical counselling needs, and (III) perceived satisfaction with health care.

Methods: The German-wide analysis was based on a 25-item questionnaire designed to address different aspects of medical status and health issues of ACHD from their own perspective, performed between May of 2017 and July of 2020.

Results: A total of 4,008 ACHD (52% female; mean age 41.9±17.2 years) completed the questionnaire. The majority of ACHD (3,524, 87.9%) reported, that they consulted their PCP for non-cardiac health problems, and 49.7% (n=1,991) consulted their PCP also for medical problems associated with the underlying CHD. Almost all ACHD reported a need for medical advice concerning exercise capacity and daily life activities, occupational skills, pregnancy, rehabilitation, genetic counselling, insurance, and retirement. A total of 1,840 (45.9%) patients were not aware of the existence of certified ACHD specialists or centers. Moreover, 2,552 (67.6%) of those surveyed were uninformed about patient organizations for ACHD.

Conclusions: The present study demonstrates that ACHD are largely uninformed about the ACHD care structures available nationwide, although the patients have a great need for specialized follow-up, advice, and care.
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http://dx.doi.org/10.21037/cdt-20-825DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8102265PMC
April 2021

RNA expression profiles and regulatory networks in human right ventricular hypertrophy due to high pressure load.

iScience 2021 Mar 27;24(3):102232. Epub 2021 Feb 27.

Department of Pediatric Cardiology and Critical Care, Hannover Medical School, Hannover, Germany.

Right ventricular hypertrophy (RVH) occurs in high pressure afterload, e.g., tetralogy of Fallot/pulmonary stenosis (TOF/PS). Such RVH is associated with alterations in energy metabolism, neurohormonal and epigenetic dysregulation (e.g., microRNA), and fetal gene reprogramming in animal models. However, comprehensive expression profiling of competing endogenous RNA in human RVH has not been performed. Here, we unravel several previously unknown circular, long non-coding, and microRNAs, predicted to regulate expression of genes specific to human RVH in the non-failing heart (TOF/PS). These genes are significantly overrepresented in pathways related to regulation of glucose and lipid metabolism (SIK1, FABP4), cell surface interactions (THBS2, FN1), apoptosis (PIK3IP1, SIK1), extracellular matrix composition (CTGF, IGF1), and other biological events. This is the first unbiased RNA sequencing study of human compensated RVH encompassing coding and non-coding RNA expression and predicted sponging of miRNAs by non-coding RNAs. These findings advance our understanding of adaptive RVH and highlight future therapeutic targets.
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http://dx.doi.org/10.1016/j.isci.2021.102232DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7994198PMC
March 2021

Surgery during pregnancy - results of a German questionnaire.

Innov Surg Sci 2020 Mar 8;5(1-2):21-26. Epub 2020 Oct 8.

Klinik für Allgemein- und Viszeralchirurgie, DIAKOVERE Henriettenstift, Hannover, Germany.

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.
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http://dx.doi.org/10.1515/iss-2020-0025DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7798309PMC
March 2020

Acquired Comorbidities in Adults with Congenital Heart Disease: An Analysis of the German National Register for Congenital Heart Defects.

J Clin Med 2021 Jan 16;10(2). Epub 2021 Jan 16.

Department of Congenital Heart Disease and Paediatric Cardiology, German Heart Centre Munich, TUM School of Medicine-Technical University of Munich, 80636 Munich, Germany.

Background: As adults with congenital heart disease (ACHD) are getting older, acquired comorbidities play an important role in morbidity and mortality. Data regarding their prevalence in ACHD that are representative on a population level are not available.

Methods: The German National Register for Congenital Heart Defects was screened for ACHD. Underlying congenital heart disease (CHD), patient demographics, previous interventional/surgical interventions, and comorbidities were retrieved. Patients <40 years of age were compared to those ≥40 years.

Results: A total of 4673 patients (mean age 33.6 ± 10.7 years, female 47.7%) was included. At least one comorbidity was present in 2882 patients (61.7%) altogether, and in 56.8% of patients below vs. 77.7% of patients over 40 years of age ( < 0.001). Number of comorbidities was higher in patients ≥40 years (2.1 ± 2.1) than in patients <40 years (1.2 ± 1.5, < 0.001). On multivariable regression analysis, age and CHD complexity were significantly associated with the presence and number of comorbidities.

Conclusions: At least one acquired comorbidity is present in approximately two-thirds of ACHD. Age and complexity of the CHD are significantly associated with the presence of comorbidities. These findings highlight the importance of addressing comorbidities in ACHD care to achieve optimal long-term outcomes.
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http://dx.doi.org/10.3390/jcm10020314DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7830982PMC
January 2021

Mortality and morbidity in patients with congenital heart disease hospitalised for viral pneumonia.

Heart 2020 Oct 27. Epub 2020 Oct 27.

Adult Congenital and Valvular Heart Disease Center, Department of Cardiology and Angiology, University Hospital Muenster, Muenster, Germany.

Objectives: Data on the clinical outcome of patients with congenital heart disease (CHD) affected by severe viral pneumonia are limited. We analysed morbidity and mortality of viral pneumonia and evaluated the association between medical conditions, medication, vaccination and outcome specifically in patients with CHD requiring hospitalisation for viral pneumonia.

Methods: Based on data from one of Germany's largest health insurers, all cases of viral pneumonia requiring hospital admission (2005-2018) were studied. Mortality, and composites of death, transplantation, mechanical circulatory support, ventilation or extracorporeal lung support served as endpoints.

Results: Overall, 26 262 viral pneumonia cases occurred in 24 980 patients. Of these, 1180 cases occurred in patients with CHD. Compared with patients without CHD, mortality rate was elevated in patients with CHD. As a group, patients with CHD aged 20-59 years even exceeded mortality rates in patients without CHD aged >60 years. No mortality was observed in patients with CHD with simple defects <60 years of age without associated cardiovascular risk factors. On multivariable logistic regression analysis, age, CHD complexity, chromosomal anomalies, cardiac medication, use of immunosuppressants and absence of vaccination for influenza emerged as risk factors of adverse outcome.

Conclusions: We present timely data on morbidity and mortality of severe viral pneumonia requiring hospital admission in patients with CHD. Need for mechanical ventilation and risk of death in CHD increase early in life, reaching a level equivalent to non-CHD individuals >60 years of age. Our data suggest that except for patients with isolated simple defects, patients with CHD should be considered higher-risk individuals when faced with severe viral pneumonia.
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http://dx.doi.org/10.1136/heartjnl-2020-317706DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8223651PMC
October 2020

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

Ann Thorac Surg 2021 Nov 16;112(5):1546-1552. Epub 2020 Oct 16.

Department of Congenital Heart Disease/Pediatric Cardiology, Deutsches Herzzentrum Berlin, Germany; DZHK (German Centre for Cardiovascular Research), partner site Berlin, Germany.

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.
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http://dx.doi.org/10.1016/j.athoracsur.2020.07.072DOI Listing
November 2021

Utility of deep learning networks for the generation of artificial cardiac magnetic resonance images in congenital heart disease.

BMC Med Imaging 2020 10 8;20(1):113. Epub 2020 Oct 8.

Department of Cardiology III - Adult Congenital and Valvular Heart Disease, University Hospital Muenster, Albert-Schweitzer Campus 1, Muenster, Germany.

Background: Deep learning algorithms are increasingly used for automatic medical imaging analysis and cardiac chamber segmentation. Especially in congenital heart disease, obtaining a sufficient number of training images and data anonymity issues remain of concern.

Methods: Progressive generative adversarial networks (PG-GAN) were trained on cardiac magnetic resonance imaging (MRI) frames from a nationwide prospective study to generate synthetic MRI frames. These synthetic frames were subsequently used to train segmentation networks (U-Net) and the quality of the synthetic training images, as well as the performance of the segmentation network was compared to U-Net-based solutions trained entirely on patient data.

Results: Cardiac MRI data from 303 patients with Tetralogy of Fallot were used for PG-GAN training. Using this model, we generated 100,000 synthetic images with a resolution of 256 × 256 pixels in 4-chamber and 2-chamber views. All synthetic samples were classified as anatomically plausible by human observers. The segmentation performance of the U-Net trained on data from 42 separate patients was statistically significantly better compared to the PG-GAN based training in an external dataset of 50 patients, however, the actual difference in segmentation quality was negligible (< 1% in absolute terms for all models).

Conclusion: We demonstrate the utility of PG-GANs for generating large amounts of realistically looking cardiac MRI images even in rare cardiac conditions. The generated images are not subject to data anonymity and privacy concerns and can be shared freely between institutions. Training supervised deep learning segmentation networks on this synthetic data yielded similar results compared to direct training on original patient data.
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http://dx.doi.org/10.1186/s12880-020-00511-1DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7542728PMC
October 2020

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

Front Pediatr 2020 30;8:170. Epub 2020 Apr 30.

Division of Pediatric Cardiology, Children's Hospital, University of Ulm, Ulm, Germany.

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. 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. 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%; < 0.001). Enjoyment in sports was almost equally distributed across CHD and reference groups, and strongly correlated with the level of PA ( = 0.41; < 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. 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.
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http://dx.doi.org/10.3389/fped.2020.00170DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7203217PMC
April 2020

Prediction of prognosis in patients with tetralogy of Fallot based on deep learning imaging analysis.

Heart 2020 07 11;106(13):1007-1014. Epub 2020 Mar 11.

Department of Cardiology III - Adult Congenital and Valvular Heart Disease, University Hospital Muenster, Muenster, Germany.

Objective: To assess the utility of machine learning algorithms for automatically estimating prognosis in patients with repaired tetralogy of Fallot (ToF) using cardiac magnetic resonance (CMR).

Methods: We included 372 patients with ToF who had undergone CMR imaging as part of a nationwide prospective study. Cine loops were retrieved and subjected to automatic deep learning (DL)-based image analysis, trained on independent, local CMR data, to derive measures of cardiac dimensions and function. This information was combined with established clinical parameters and ECG markers of prognosis.

Results: Over a median follow-up period of 10 years, 23 patients experienced an endpoint of death/aborted cardiac arrest or documented ventricular tachycardia (defined as >3 documented consecutive ventricular beats). On univariate Cox analysis, various DL parameters, including right atrial median area (HR 1.11/cm², p=0.003) and right ventricular long-axis strain (HR 0.80/%, p=0.009) emerged as significant predictors of outcome. DL parameters were related to adverse outcome independently of left and right ventricular ejection fraction and peak oxygen uptake (p<0.05 for all). A composite score of enlarged right atrial area and depressed right ventricular longitudinal function identified a ToF subgroup at significantly increased risk of adverse outcome (HR 2.1/unit, p=0.007).

Conclusions: We present data on the utility of machine learning algorithms trained on external imaging datasets to automatically estimate prognosis in patients with ToF. Due to the automated analysis process these two-dimensional-based algorithms may serve as surrogates for labour-intensive manually attained imaging parameters in patients with ToF.
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http://dx.doi.org/10.1136/heartjnl-2019-315962DOI Listing
July 2020

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

Int J Cardiol 2020 06 2;308:28-32. Epub 2020 Jan 2.

Department of Cardiology III - Adult Congenital and Valvular Heart Disease University Hospital Muenster, Germany.; Competence Network for Congenital Heart Defects, Berlin, Germany.

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.
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http://dx.doi.org/10.1016/j.ijcard.2019.12.070DOI Listing
June 2020

Sex differences in publication volume and quality in congenital heart disease: are women disadvantaged?

Open Heart 2019;6(1):e000882. Epub 2019 Apr 3.

Department of Cardiology III - Adult Congenital and Valvular Heart Disease, University Hospital Muenster, Muenster, Germany.

Background: Women are underrepresented in leading medical positions and academia. The gender-gap in authorship of congenital heart disease (CHD) publications remains unknown. As determinants of gender equity in this field are poorly characterised, we aimed to quantify and characterise publications in CHD and to assess factors associated with female representation in research.

Methods And Results: We identified 35 118 CHD publications between 2006 and 2015 for which author gender could be ascertained. Overall, 25.0% of all authors were female. Women accounted for 30.2% and 20.8% of all first and senior authorship positions with great geographic heterogeneity. While globally female first and senior authorship increased by 0.8% and 0.6%/year, some geographic regions showed no improvement in gender representation. Significant predictors of female first authorship on logistic regression analysis were country gross domestic product, human development index, gender inequality index and a female senior author (p<0.0001 for all). Publications with a female lead author tended to be published in journals with a higher impact factor (IF) and to attract more citations compared with those with a male author. Mixed gender authorship was associated with higher IF and number of citations. Women were less disadvantaged when the analysis was confined to original research.

Conclusions: While modest improvement in female authorship over time was noted, women remain underrepresented in contemporary academic CHD. Manuscripts with mixed gender authorship had higher IF and more citations. The main predictor of female first authorship was a female senior author. These data should inform policy recommendations regarding gender parity.
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http://dx.doi.org/10.1136/openhrt-2018-000882DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6519433PMC
February 2021

Improving medical care and prevention in adults with congenital heart disease-reflections on a global problem-part II: infective endocarditis, pulmonary hypertension, pulmonary arterial hypertension and aortopathy.

Cardiovasc Diagn Ther 2018 Dec;8(6):716-724

Department of Pediatric Cardiology and Congenital Heart Disease, German Heart Center Munich, Technical University Munich, Munich, Germany.

Despite relevant residua and sequels, follow-up care of adults with congenital heart disease (ACHD) is too often not performed by/in specialized and/or certified physicians or centers although major problems in the long-term course may develop. The most relevant encompass heart failure, cardiac arrhythmias, heart valve disorders, pulmonary vascular disease, infective endocarditis (IE), aortopathy and non-cardiac comorbidities. The present publication emphasizes current data on IE, pulmonary and pulmonary arterial hypertension and aortopathy in ACHD and underlines the deep need of an experienced follow-up care by specialized and/or certified physicians or centers, as treatment regimens from acquired heart disease can not be necessarily transmitted to CHD. Moreover, the need of primary and secondary medical prevention becomes increasingly important in order to reduce the burden of disease as well as the socioeconomic burden and costs in this particular patient group.
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http://dx.doi.org/10.21037/cdt.2018.10.16DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6331381PMC
December 2018

Improving medical care and prevention in adults with congenital heart disease-reflections on a global problem-part I: development of congenital cardiology, epidemiology, clinical aspects, heart failure, cardiac arrhythmia.

Cardiovasc Diagn Ther 2018 Dec;8(6):705-715

Department of Pediatric Cardiology and Congenital Heart Disease, German Heart Center Munich, Technical University Munich, Munich, Germany.

Today most patients with congenital heart defects (CHD) survive into adulthood. Unfortunately, despite relevant residua and sequels, follow-up care of adults with congenital heart disease (ACHD) is not performed in specialized and/or certified physicians or centres. Major problems in the long-term course encompass heart failure, cardiac arrhythmias, heart valve disorders, pulmonary vascular disease, infective endocarditis, aortopathy and non-cardiac comorbidities. Many of them manifest themselves differently from acquired heart disease and therapy regimens from general cardiology cannot be transferred directly to CHD. It should be noted that even simple, postoperative heart defects that were until recently considered to be harmless can lead to problems with age, a fact that had not been expected so far. The treatment of ACHD has many special features and requires special expertise. Thereby, it is important that treatment regimens from acquired heart disease are not necessarily transmitted to CHD. While primary care physicians have the important and responsible task to set the course for adequate diagnosis and treatment early and to refer patients to appropriate care in specialized ACHD-facilities, they should actively encourage ACHD to pursue follow-up care in specialized facilities who can provide responsible and advanced advice. This medical update emphasizes the current data on epidemiology, heart failure and cardiac arrhythmia in ACHD.
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http://dx.doi.org/10.21037/cdt.2018.10.15DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6331379PMC
December 2018

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

Early Hum Dev 2019 01 15;128:27-34. Epub 2018 Nov 15.

Department of Congenital Heart Disease - Paediatric Cardiology, Deutsches Herzzentrum Berlin, Germany; DZHK (German Centre for Cardiovascular Research), partner site Berlin, Germany.

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.
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http://dx.doi.org/10.1016/j.earlhumdev.2018.11.003DOI Listing
January 2019

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

Congenit Heart Dis 2018 Sep 11;13(5):685-689. Epub 2018 Sep 11.

Department for Congenital Heart Disease and Pediatric Cardiology, DZHK (German Centre for Cardiovascular Research), University Hospital Schleswig-Holstein - Campus Kiel, Germany.

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.
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http://dx.doi.org/10.1111/chd.12630DOI Listing
September 2018

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

Int J Cardiol 2019 Feb 4;277:79-84. Epub 2018 Aug 4.

Department of Paediatric Cardiology and Congenital Heart Disease, German Heart Centre Munich, Technical University of Munich, Munich, Germany. Electronic address:

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 ± 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.
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http://dx.doi.org/10.1016/j.ijcard.2018.08.009DOI Listing
February 2019

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

Congenit Heart Dis 2018 May 25;13(3):377-383. Epub 2018 Jan 25.

National Register for Congenital Heart Defects, DZHK (German Center for Cardiovascular Research), Berlin, Germany.

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.
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http://dx.doi.org/10.1111/chd.12581DOI Listing
May 2018

Somatic Development in Children with Congenital Heart Defects.

J Pediatr 2018 01;192:136-143.e4

Department of Pediatric Cardiology, Saarland University Medical Center, Homburg/Saar, Germany.

Objectives: Somatic development is impaired in children with congenital heart defects (CHDs), and head circumference seems to be a strong predictor of neurodevelopmental prognosis. The aim of this study was to generate up-to-date reference values for the somatic development (head circumference, body weight, and length/height) of children with CHDs.

Study Design: Our study population consisted of all patients included in the PAN study (Prävalenz angeborener Herzfehler bei Neugeborenen in Deutschland), which was conducted prospectively over a 3-year study period by the Competence Network for Congenital Heart Defects. All children with mild, moderate, and severe CHDs born in 2006-2009 in Germany were enrolled. For computing of z-scores, only children with the following characteristics were included: appropriate for gestational age, nonsyndromic disease, term or post-term delivery, and no cardiac surgery.

Results: There were 2818 patients included. New z-scores for the described somatic measures of children with mild, moderate, and severe CHDs were computed. Comparisons with the KiGGS study (Gesundheit von Kindern und Jugendlichen in Deutschland) and the Berlin Longitudinal Study revealed significantly lower measurements for all measures-most notably in children with severe CHDs and/or cardiac surgery. In our cohort, no catch-up growth was seen after cardiac surgery.

Conclusion: Children with severe CHDs demonstrated the most abnormal pattern in growth, including head circumference before and after cardiac surgery, which is indicative of accompanying brain pathology unrelated to operative injury.
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http://dx.doi.org/10.1016/j.jpeds.2017.09.059DOI Listing
January 2018

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

Eur J Pediatr 2018 Jan 10;177(1):107-115. Epub 2017 Nov 10.

Department of Congenital Heart Disease-Paediatric Cardiology, Deutsches Herzzentrum Berlin, Augustenburger Platz 1, 13353, Berlin, Germany.

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.
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http://dx.doi.org/10.1007/s00431-017-3041-6DOI Listing
January 2018

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

Cardiol Young 2018 Jan 13;28(1):32-38. Epub 2017 Sep 13.

1Department of Congenital Heart Disease - Paediatric Cardiology,Deutsches Herzzentrum Berlin,Germany.

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.
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http://dx.doi.org/10.1017/S104795111700138XDOI Listing
January 2018

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

Int J Cardiol 2017 Oct 15;245:105-108. Epub 2017 Jul 15.

Department of Paediatric Cardiology and Congenital Heart Disease, German Heart Centre Munich, Technical University of Munich, Munich, Germany; Competence Network for Congenital Heart Defects, Berlin, Germany, DZHK (German Centre for Cardiovascular Research), Berlin, Germany. Electronic address:

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.
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http://dx.doi.org/10.1016/j.ijcard.2017.07.040DOI Listing
October 2017

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

Congenit Heart Dis 2017 Dec 18;12(6):787-793. Epub 2017 Jul 18.

Department of Congenital Heart Disease - Pediatric Cardiology, German Heart Center Berlin, Berlin, 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.
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http://dx.doi.org/10.1111/chd.12515DOI Listing
December 2017

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

Front Pediatr 2017 19;5:115. Epub 2017 May 19.

National Register for Congenital Heart Defects, Berlin, Germany.

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 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.
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http://dx.doi.org/10.3389/fped.2017.00115DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5437851PMC
May 2017

Toward evidence-based diagnosis of myocarditis in children and adolescents: Rationale, design, and first baseline data of MYKKE, a multicenter registry and study platform.

Am Heart J 2017 May 24;187:133-144. Epub 2017 Feb 24.

Congenital Heart Disease-Pediatric Cardiology, Deutsches Herzzentrum Berlin, Berlin; DZHK (German Center for Cardiovascular Research), Partner Site Berlin, Berlin.

The aim of this registry is to provide data on age-related clinical features of suspected myocarditis and to create a study platform allowing for deriving diagnostic criteria and, at a later stage, testing therapeutic interventions in patients with myocarditis.

Study Design And Results: After an initial 6-month pilot phase, MYKKE was opened in June 2014 as a prospective multicenter registry for patients from pediatric heart centers, university hospitals, and community hospitals with pediatric cardiology wards in Germany. Inclusion criteria consisted of age<18 years and hospitalization for suspected myocarditis as leading diagnosis at the discretion of the treating physician. By December 31, 2015, fifteen centers across Germany were actively participating and had enrolled 149 patients. Baseline data reveal 2 age peaks (<2 years, >12 years), show higher proportions of males, and document a high prevalence of severe disease courses in pediatric patients with suspected myocarditis. Severe clinical courses and early adverse events were more prevalent in younger patients and were related to severely impaired leftventricular ejection fraction at initial presentation.

Summary: MYKKE represents a multicenter registry and research platform for children and adolescents with suspected myocarditis that achieve steady recruitment and generate a wide range of real-world data on clinical course, diagnostic workup, and treatment of this group of patients. The baseline data reveal the presence of 2 age peaks and provide important insights into the severity of disease in children with suspected myocarditis. In the future, MYKKE might facilitate interventional substudies by providing an established collaborating network using common diagnostic approaches.
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http://dx.doi.org/10.1016/j.ahj.2017.02.027DOI Listing
May 2017
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