Continuous Long-Term Assessment of Heart Rate Variability in Adults with Cyanotic Congenital Heart Disease after Surgical Repair.

Background: Heart rate variability (HRV) is an established, non-invasive parameter for the assessment of cardiac autonomic nervous activity and the health status in general cardiology. However, there are few studies on HRV in adults with congenital heart defects (CHDs). The aim of the present study was to evaluate the use of long-term continuous HRV measurement for the assessment of global health status in adults with cyanotic CHD. Methods: This prospective study included 45 adults (40% female, mean age = 35.2 ± 9.2 [range: 19-58] years) after cardiac surgical repair. HRV parameters were calculated from continuous 24 h measurements using a Bittium Faros 180 sensor (Bittium Corp., Oulu, Finland). Results: Postoperative patients with transposition of the great arteries (TGA) (n = 18) achieved significantly higher values of standard deviation of NN intervals (SDNN) (175.4 ± 59.9 ms vs. 133.5 ± 40.6 ms; p = 0.013) compared with patients with other conotruncal anomalies (n = 22). Comparing patients with TGA after a Senning-Brom or Mustard operation (n = 13) with all other heart surgery patients (n = 32), significantly higher HRV parameters were found after atrial switch (root mean square of successive RR interval differences: 53.6 ± 20.7 ms vs. 38.4 ± 18.3 ms; p = 0.019; SDNN: 183.5 ± 58.4 ms vs. 136.3 ± 45.3 ms; p = 0.006). A higher SDNN was also measured after Senning-Brom or Mustard operations than after a Rastelli operations (n = 2) (SDNN: 183.5 ± 58.4 ms vs. 84.5 ± 5.2 ms; p = 0.037). When comparing atrial switch operations (n = 3) with Rastelli operations, the SDNN value was significantly shorter in the Rastelli group (p = 0.004). Conclusions: Our results suggest that continuous HRV monitoring may serve as a marker of cardiac autonomic dysfunction in adults with cyanotic CHD after surgical repair. Impaired cardiac autonomic nervous activity may be associated with an increased risk of adverse reactions in patients with repaired CHD. Therefore, a longitudinal assessment of HRV patterns and trends may provide a deeper insight into dynamic changes in their autonomic regulation and disease progression, lifestyle changes, or treatments. As each person has individual variability in heart rate, HRV may be useful in assessing intra-individual disease progression and may help to improve personalized medicine. Further studies are needed to better understand the underlying mechanisms and to explore the full potential of HRV analysis to optimize medical care for ACHDs.

Myocarditis Screening Methods in Athletes After SARS-CoV-2 Infection - a Systematic Review.

This review aims to elucidate the myocarditis incidence in SARS-CoV-2-positive athletes and to evaluate different screening approaches to derive sports cardiological recommendations after SARS-CoV-2 infection. The overall incidence of athletes (age span 17-35 years, 70% male) with myocarditis after SARS-CoV-2 infection was 1.2%, with a high variation between studies (which contrasts an incidence of 4.2% in 40 studies within the general population). Studies that used conventional screening based on symptoms, electrocardiogram, echocardiography, and cardiac troponin - only followed by cardiac magnetic resonance imaging in case of abnormal findings - reported lower myocarditis incidences (0.5%, 20/3978). On the other hand, advanced screening that included cardiac magnetic resonance imaging within the primary screening reported higher incidences (2.4%, 52/2160). The sensitivity of advanced screening seems to be 4.8 times higher in comparison to conventional screening. However, we recommend prioritization of conventional screening, as the economical load of advanced screening for all athletes is high and the incidence of myocarditis in SARS-CoV-2-positive athletes and the risk of adverse outcomes seems low. Future research will be important to analyze the long-term effects of myocarditis after infection with SARS-CoV-2 in athletes for risk stratification to optimally guide a safe return to sport.

Clinical aspects and targeted inspiratory muscle training in children and adolescents with Fontan circulation: a randomized controlled trial.

Background: Since 1968, thousands of patients with a morphologically or functionally univentricular heart have been treated with a total cavopulmonary connection/Fontan operation. Because of the resulting passive pulmonary perfusion, blood flow is assisted by the pressure shift during respiration. Respiratory training is known to improve exercise capacity and cardiopulmonary function. However, there are limited data on whether respiratory training can also improve physical performance after Fontan surgery. The aim of the present study was to clarify the effects of six months of daily home-based inspiratory muscle training (IMT) aimed at increasing physical performance by strengthening respiratory muscles, improving lung function and peripheral oxygenation. Methods: In this non-blinded randomized controlled trial, the effects of IMT on lung capacity and exercise capacity were measured in a large cohort of 40 Fontan patients (25% female; 12.3±2.2 years) who were under regular follow-up by the outpatient clinic of the Department of Congenital Heart Defects and Pediatric Cardiology of the German Heart Center Munich. After a lung function test and a cardiopulmonary exercise test, patients were randomly assigned in a parallel arm design to either an intervention group (IG) or a control group (CG) via stratified and computer-generated letter randomization from May 2014 to May 2015. The IG completed a daily, telephone-monitored IMT of three sets of 30 repetitions for six months with an inspiratory resistive training device (POWERbreathe medic®), the CG continued their usual daily activities without an IMT until the second examination within the timeframe of November 2014 until November 2015. Results: After six months of IMT, lung capacity values in the IG (n=18) did not increase significantly compared to the CG [n=19; ΔFVC: IG: 0.21±0.16 l vs. CG 0.22±0.31 l; P=0.946 (CI: -0.16, 0.17); ΔFEV1: CG: 0.14±0.30 vs. IG: 0.17±0.20 P=0.707 (CI: -0.20, 0.14)]. Exercise capacity did not improve significantly, yet the maximum workload achieved trended to improve with an increase of 14% in the IG vs. 6.5% in the CG [P=0.113 (CI: -15.8, 1.76)]. There was a significant increase of oxygen saturation at rest in the IG compared to the CG [IG: 3.31%±4.09% vs. CG: 0.17%±2.92%, P=0.014 (CI: -5.60, -0.68)]. Compared to the CG, the mean oxygen saturation at peak exercise no longer dropped below 90% in the IG. This observation is thus not statistically significant, yet of clinical relevance. Conclusions: The results of this study show benefits of an IMT in young Fontan patients. Even if some data are not statistically significant, they may still be clinically relevant and may contribute to a multidisciplinary approach in patient care. IMT should therefore be an additional target and integrated into the training program to improve the prognosis of Fontan patients. Trial Registration: German Clinical Trials Register; DRKS.de; registration ID: DRKS00030340.

[Care of adults with congenital heart diseases in Germany-Leading role by internal medicine specialists and general practitioners]. / Versorgung von Erwachsenen mit angeborenen Herzfehlern in Deutschland ­ tragende Rolle von Internisten und Hausärzten.

BACKGROUND: The number of adults with congenital heart disease (ACHD) is steadily increasing. The present cross-sectional study was conceived to investigate ACHD care from the perspective of patients and family practitioners (specialists for general medicine and internal medicine, general practitioners). METHODS: Questionnaire-based cross-sectional study to analyze the real care situation of ACHD in Germany from the perspective of patients and primary care physicians (PCP). RESULTS: The questionnaire was completed by 4493 ACHD (53.7% female; 41.3 ± 16.9 years) and 1055 PCP. The majority of ACHD (79.8%) visited their PCP for noncardiac health problems but also for cardiac problems. Almost all ACHD had substantial needs for medical consultation (performance, employment etc.). Of the patients 2014 (44.8%) did not know of any certified ACHD specialists or specialized centers and 2816 (62.7%) respondents were not aware of any ACHD patient organization. Of the PCPs 87.5% had cared for ACHD of all severities due to defect-typical residual and resulting symptoms. Many were not aware of any certified ACHD specialists. Only 28.5% consulted an ACHD specialist. Only 23.5% were aware of ACHD patient organizations. CONCLUSION: General practitioners are a mainstay of ACHD care in Germany. The present study shows that ACHD and their general practitioners are largely uninformed about the specialized care structures available nationwide, despite the high level of need for specialist care. In order to keep the morbidity and mortality of affected patients low, solutions must be elaborated in future to involve and integrate primary care physicians more intensively into the already existing dedicated ACHD care structures, in cooperation with specialized pediatric cardiologists, cardiologists and centers.

Exercise capacity in patients with repaired Tetralogy of Fallot aged 6 to 63 years.

OBJECTIVES: This study aimed to provide a perspective for the interpretation of exercise capacity (peakVO2) in patients with repaired Tetralogy of Fallot (patients with rTOF) by describing the course of peakVO2 from patients aged 6-63 years. METHODS: A retrospective study was performed between September 2001 and December 2016 in the German Heart Centre Munich, Germany, and in the University Medical Centre Groningen, the Netherlands. A total of 1175 cardiopulmonary exercise tests (CPETs) were collected from 586 patients with rTOF, 46% female. Maximal exertion was verified using a respiratory exchange ratio ≥1.00. PeakVO2 was modelled using time-dependent multilevel models for repeated measurements (n=889 in 300 patients), and compared with subject-specific reference values calculated by the models of Bongers et al and Mylius et al. RESULTS: The peakVO2 of patients with rTOF was reduced at all ages. At the age of 6, the peakVO2 was 614 mL/min (70% of predicted (95% CI 67 to 73)). The reduced increase in peakVO2 during adolescence resulted in a significant lower maximum peakVO2 of 1209 mL/min at 25 years (65% predicted, p<0.001). A linear decline after 25 years was observed in patients and references, although patients showed an accelerated decline, with a -0.24% point of predicted (95% CI 0.11 to 0.38) per year without differences between sexes (p=0.263). CONCLUSIONS: This study provides a context for peakVO2 across ages in patients with rTOF under contemporary treatment strategies. It showed that the reduction in peakVO2 originates from childhood and declines over time. Sex differences in patients with rTOF were similar to natural existing sex differences.

[Managing transition - Medical follow-up care for adults with congenital heart defects]. / Transition ­ Nachsorge für Erwachsene mit angeborenen Herzfehlern.

Currently, due to medical advances, more than 500 000 patients with congenital heart defects (CHD) are living in Germany, including more than 330 000 adults. In CHD, the aspect of transition from childhood and adolescence to adulthood is becoming increasingly important to ensure long-term success of the provided treatments. This is so important because despite primarily successful treatments, residua and sequels as well as (non-) cardiac comorbidities may influence the chronic course of the disease and lead to increased morbidity and mortality. Adults with congenital heart defects in Germany are insufficiently managed by existing specialized and accredited care structures, despite the great need for follow-up, and their care is largely provided by primary care physicians (general practitioners and internists) and non-specialized cardiologists.Optimized, future-oriented care requires regular follow-up and the possibility of interdisciplinary, integrated medical care of CHD in the long-term course.

Cardiac Rehabilitation in German Speaking Countries of Europe-Evidence-Based Guidelines from Germany, Austria and Switzerland LLKardReha-DACH-Part 1.

BACKGROUND: Although cardiovascular rehabilitation (CR) is well accepted in general, CR-attendance and delivery still considerably vary between the European countries. Moreover, clinical and prognostic effects of CR are not well established for a variety of cardiovascular diseases. METHODS: The guidelines address all aspects of CR including indications, contents and delivery. By processing the guidelines, every step was externally supervised and moderated by independent members of the "Association of the Scientific Medical Societies in Germany" (AWMF). Four meta-analyses were performed to evaluate the prognostic effect of CR after acute coronary syndrome (ACS), after coronary bypass grafting (CABG), in patients with severe chronic systolic heart failure (HFrEF), and to define the effect of psychological interventions during CR. All other indications for CR-delivery were based on a predefined semi-structured literature search and recommendations were established by a formal consenting process including all medical societies involved in guideline generation. RESULTS: Multidisciplinary CR is associated with a significant reduction in all-cause mortality in patients after ACS and after CABG, whereas HFrEF-patients (left ventricular ejection fraction <40%) especially benefit in terms of exercise capacity and health-related quality of life. Patients with other cardiovascular diseases also benefit from CR-participation, but the scientific evidence is less clear. There is increasing evidence that the beneficial effect of CR strongly depends on "treatment intensity" including medical supervision, treatment of cardiovascular risk factors, information and education, and a minimum of individually adapted exercise volume. Additional psychologic interventions should be performed on the basis of individual needs. CONCLUSIONS: These guidelines reinforce the substantial benefit of CR in specific clinical indications, but also describe remaining deficits in CR-delivery in clinical practice as well as in CR-science with respect to methodology and presentation.

'Well-being paradox' revisited: a cross-sectional study of quality of life in over 4000 adults with congenital heart disease.

OBJECTIVE: The present cross-sectional study investigated quality of life (QOL) in a large cohort of German adults with congenital heart disease (ACHDs) in association with patient-related and clinical variables. DESIGN: Cross-sectional survey. PARTICIPANTS: Between 2016 and 2019, a representative sample of 4014 adults with various forms of congenital heart defect (CHD) was retrospectively analysed. Inclusion criteria were confirmed diagnosis of CHD; participant aged 18 years and older; and necessary physical, cognitive and language capabilities to complete self-report questionnaires. PRIMARY AND SECONDARY OUTCOME MEASURES: QOL was assessed using the 5-level EQ-5D version (EQ-5D-5L). Sociodemographic and medical information was obtained by a self-devised questionnaire. Associations of QOL with patient-reported clinical and sociodemographic variables were quantified using multiple regression analysis and multiple ordinal logit models. RESULTS: Overall, ACHDs (41.8±17.2 years, 46.5% female) reported a good QOL comparable to German population norms. The most frequently reported complaints occurred in the dimensions pain/discomfort (mean: 16.3, SD: p<0.001) and anxiety/depression (mean: 14.3, p<0.001). QOL differed significantly within ACHD subgroups, with patients affected by pretricuspid shunt lesions indicating the most significant impairments (p<0.001). Older age, female sex, medication intake and the presence of comorbidities were associated with significant reductions in QOL (p<0.001). CHD severity was positively associated with QOL within the dimensions of self-care (OR 0.148, 95% CI 0.04 to 0.58) and mobility (OR 0.384, 95% CI 0.19 to 0.76). CONCLUSION: Current findings temper widely held assumptions among clinicians and confirm that ACHDs experience a generally good QOL. However, specific subgroups may require additional support to cope with disease-related challenges. The negative correlation of QOL with age is especially alarming as the population of ACHDs is expected to grow older in the future. TRIAL REGISTRATION NUMBER: DRKS00017699; Results.

Provision of medical health care for adults with congenital heart disease associated with aortic involvement.

BACKGROUND: All patients with congenital heart disease (CHD) are chronically ill from their cardiac disease. Despite the increasing evidence that aortic alterations are becoming relevant, the importance of aortopathy in CHD has long been underestimated. This study was conducted to determine the health status of patients and/or the provision of health services of adults with CHD (ACHD) with manifest aortopathy or at risk thereof. METHODS: In a questionnaire-based cross-sectional survey, the "real life"-care of ACHD was analysed, comparing patients with risk of developing aortopathy and/or manifest aortopathy. RESULTS: Of the 563 enrolled ACHD (49.6% female, mean age 35.8±12.1, 18-86 years) 56.8% (n=320) had a risk of developing aortopathy and/or manifest aortopathy. Of the 320 patients at risk, 187 (33.2% of the total number) had a proven aortopathy. Within this subgroup, the basic medical care for CHD-independent medical problems was given by primary medical care providers [family doctors/general practitioners (GP) in 89.4% (n=286), internists in 13.4% (n=43), physicians of another specialty in 2.5% (n=8)]. Almost all primary medical care providers knew about the CHD of their patients. Even for CHD-specific health problems, the basic medical care of risk patients was provided by a family doctor or GP in 56.6% (n=181) and by an internist in 18.4% (n=59). 30.0% (n=96) primarily consulted another specialist, including cardiologists. Only 32.8% of ACHD at risk had ever been referred to a CHD specialist by a GP for cardiac problems related to their CHD. In contrast, the need for advice was high for ACHD with aortopathy and related mainly to physical activity, employment and education, pregnancy, rehabilitation or health and life insurance. Only 35.5% of patients at risk indicated that their information on specific care structures for ACHD was sufficient, and a further 38.1% of patients were aware of patient organizations. CONCLUSIONS: Even today, aortic involvement in ACHD is an often-overlooked condition, although considerable negative effects on morbidity and mortality exist. As aortopathy gains in importance with increasing age and complexity of CHD, almost all affected ACHD need lifelong medical advice and access to modern, scientifically based care concepts. According to the study-results, primary care providers and also patients are mostly insufficiently informed about the specialized ACHD facilities. The future goal is therefore to create a better awareness of CHD problems among both primary care physicians and the patients concerned.

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

Quality of life in patients with Marfan syndrome: a cross-sectional study of 102 adult patients.

BACKGROUND: Marfan syndrome (MFS) is a genetically determined multiorgan disease that leads to severe physiological and psychological impairments in adult life. Little consensus exists regarding quality of life (QOL) in individuals with MFS. The present study sought to investigate QOL in a representative cohort of adults with MFS. METHODS: Patient-reported outcome measures from a representative sample of 102 adults with MFS (39.3±13.1 years of age; 40.2% female) were retrospectively analyzed and compared with those from adults with different congenital heart defects (CHD), at the German Heart Center Munich. QOL was assessed using the updated five-level version of the EQ-5D. RESULTS: Differences between both populations were analyzed. Subjects affected by MFS reported an overall reduced QOL. Compared to CHD patients, individuals with MFS scored significantly lower in the dimensions of pain/discomfort, anxiety/depression, mobility and usual activities (P<0.05). CONCLUSIONS: Patients with MFS are at high risk for impaired QOL, especially in mental and physical domains. Psychosocial consequences of MFS cost resources for both, patients and professionals. Current findings highlight the great importance of additional psychological support to cope with disease-related challenges. Increased attention should be directed towards enhancing their subjective wellbeing to potentially improve their QOL and long-term health outcomes.

Medical treatment of pulmonary hypertension in adults with congenital heart disease: updated and extended results from the International COMPERA-CHD Registry.

BACKGROUND: Pulmonary arterial hypertension (PAH) is common in congenital heart disease (CHD). Because clinical-trial data on PAH associated with CHD (PAH-CHD) remain limited, registry data on the long-term course are essential. This analysis aimed to update information from the COMPERA-CHD registry on management strategies based on real-world data. METHODS: The prospective international pulmonary hypertension registry COMPERA has since 2007 enrolled more than 10,000 patients. COMPERA-CHD is a sub-registry for patients with PAH-CHD. RESULTS: A total of 769 patients with PAH-CHD from 62 specialized centers in 12 countries were included into COMPERA-CHD from January 2007 through September 2020. At the last follow-up in 09/2020, patients [mean age 45.3±16.8 years; 512 (66%) female] had either post-tricuspid shunts (n=359; 46.7%), pre-tricuspid shunts (n=249; 32.4%), complex CHD (n=132; 17.2%), congenital left heart or aortic valve or aortic disease (n=9; 1.3%), or miscellaneous CHD (n=20; 2.6%). The mean 6-minute walking distance was 369±121 m, and 28.2%, 56.0%, and 3.8% were in WHO functional class I/II, III or IV, respectively (12.0% unknown). Compared with the previously published COMPERA-CHD data, after 21 months of follow-up, the number of included PAH-CHD patients increased by 91 (13.4%). Within this group the number of Eisenmenger patients rose by 39 (16.3%), the number of "Non-Eisenmenger PAH" patients by 45 (26.9%). Currently, among the 674 patients from the PAH-CHD group with at least one follow-up, 450 (66.8%) received endothelin receptor antagonists (ERA), 416 (61.7%) PDE-5 inhibitors, 85 (12.6%) prostacyclin analogues, and 36 (5.3%) the sGC stimulator riociguat. While at first inclusion in the COMPERA-CHD registry, treatment was predominantly monotherapy (69.3%), this has shifted to favoring combination therapy in the current group (53%). For the first time, the nature, frequency, and treatment of significant comorbidities requiring supportive care and medication are described. CONCLUSIONS: Analyzing "real life data" from the international COMPERA-CHD registry, we present a comprehensive overview about current management modalities and treatment concepts in PAH-CHD. There was an trend towards more aggressive treatment strategies and combination therapies. In the future, particular attention must be directed to the "Non-Eisenmenger PAH" group and to patients with complex CHD, including Fontan patients. TRIAL REGISTRATION: www.clinicaltrials.gov, study identifier: Clinicaltrials.gov NCT01347216.

Implantable loop recorder for monitoring patients with congenital heart disease.

BACKGROUND: Patients with congenital heart defects (CHD) are prone to residua, sequels and complications from the underlying anomaly, where cardiac arrhythmias are one of the major causes for hospitalization, morbidity and mortality. The importance of the subcutaneous implantable loop recorder (ILR) for the detection and documentation of significant arrhythmias has increased over the last years. To date, however, there is little data on ILR use in the CHD population. METHODS: In this single center, retrospective observational study, all CHD-patients with an ILR were identified who were under care of the German Heart Center Munich between February 2015 and January 2019. The primary endpoint of the study was the detection or exclusion of significant arrhythmias during follow-up in CHD-patients who had received an ILR. The secondary endpoint was to determine whether ILR findings influenced patient management, defined as initiation or adjustment of medication, cardioversion, electrophysiologic study, catheter ablation, or implantation of cardiac implantable electronic devices (CIEDs) such as pacemakers (PM) or implantable cardioverter-defibrillators. RESULTS: An ILR was implanted in 33 CHD-patients (mean age, 43±20 years; 42.4% female) with CHD. During a mean observation period of 697±433 days, clinically relevant arrhythmias, correlating with the patients' complaints and symptoms, were detected in 19 patients (59.4%), encompassing supraventricular tachycardia (n=10), supraventricular or ventricular ectopic beats (n=10), non-sustained ventricular tachycardia (n=2), ventricular tachycardia (n=2), and bradycardia (n=2). In 9 patients (28.1%) the detected arrhythmia was considered an event requiring treatment. Treatment modalities included catheter ablation (n=5), modification of antiarrhythmic drug regime (n=2), adaptation of anticoagulation therapy (n=2), or implantation of a subcutaneous ICD (n=1). Regarding the occurrence of cardiac arrhythmias or a related need for therapeutic intervention, no significant differences were identified with respect to WHO functional class, the presence of pulmonary arterial hypertension or reduced resting peripheral oxygen saturation. CONCLUSIONS: In symptomatic CHD-patients at risk for life-threatening cardiac events, ILR has a considerable complementary diagnostic value for the detection and differentiation of benign and malignant arrhythmias. Considering the overall low risk of complications, ILR implantation should be considered in patients with CHD of any complexity who need medium or long-term arrhythmia monitoring, especially if short-term Holter monitoring cannot provide sufficient diagnostic certainty.

Lessons from the short- and mid-term outcome of medical rehabilitation in adults with congenital heart disease.

BACKGROUND: The number of adults with congenital heart disease (ACHD) is steadily increasing. Over their life-time, many of the affected patients require medical rehabilitation after interventional or surgical treatment of residua, sequels or complications of their congenital heart defect (CHD). However, up to now only scarce data exist about indication, performance and outcomes of cardiac rehabilitation in ACHD. METHODS: The course and outcome of rehabilitation after previous interventional or surgical treatment in ACHD was analyzed in a retrospective cohort study. RESULTS: Two hundred and five ACHD {54% female; mean age 34±12 [16-68] years} with mild (23.9%), moderate (35.1%) or severe CHD (41.0%), of whom 32% had complex CHD, 21% right-ventricular outflow tract obstructions, 20% pre-tricuspid shunts, 18% left heart or aortic anomalies, 9% post-tricuspid shunts and 2% other congenital cardiac anomalies were included into analysis. The main indications for rehabilitation were a preceding surgical (92%) or interventional (3%) treatment of the underlying CHD immediately before rehabilitation. During rehabilitation, no severe complications occurred. The number of patients in function class I/II increased from 189 to 200 and decreased in class III/IV from 16 to 5. Cardiac medication could be reduced or stopped after rehabilitation in 194 patients, with the exception of ACE-inhibitors. There was an improvement in cardiovascular risk factors. While before medical treatment 77% (n=157) patients were capable of working, the number increased to 82% [168] at the end of rehabilitation. Throughout a follow-up 9.3% (n=19) of patients needed further cardiac interventions. CONCLUSIONS: The current study provided for the first time comprehensive data on the course of rehabilitation in a large cohort of ACHD after surgical or interventional treatment. The overall outcome of ACHD after rehabilitation was uneventful and favorable. Further research is required to assess the clinical long-term outcome, the impact of rehabilitation on the quality of life, disease coping and employment. The results of this study can serve as a benchmark for the development of specific rehabilitation programs in ACHD.

Facts about the General Medical Care of Adults with Congenital Heart Defects: Experience of a Tertiary Care Center.

Background: Due to the increase in survival rates for congenital heart disease (CHD) in the last decades, over 90% of patients today reach adulthood. Currently, there are more than 300,000 adults with CHD (ACHD) living in Germany. They have an increased need for specialized medical care, since almost all ACHD have chronic heart disease and suffer from specific chronic symptoms, risks, and sequelae. Primary care physicians (PCPs) play a crucial role in referring patients to ACHD specialists or specialized institutions. This cross-sectional study is intended to clarify the real-world care of ACHD from the PCP's perspective. Methods: This analysis, initiated by the German Heart Centre Munich, was based on a 27-item questionnaire on actual ACHD health care practice in Germany from the PCP's perspective. Results: In total, 767 questionnaires were considered valid for inclusion. The majority of the PCPs were general practitioners (95.9%), and 84.1% had cared for ACHD during the past year. A majority (69.2%) of the PCPs had cared for patients with simple CHD, while 50.6% and 33.4% had cared for patients with moderate and severe CHD, respectively, in all age groups. PCPs treated almost all typical residual symptoms and sequelae, and advised patients regarding difficult questions, including exercise capacity, pregnancy, genetics, and insurance matters. However, 33.8% of the PCPs did not even know about the existence of certified ACHD specialists or centers. Only 23.9% involved an ACHD-specialized physician in their treatment. In cases of severe cardiac issues, 70.8% of the PCPs referred patients to ACHD-certified centers. Although 52.5% of the PCPs were not sufficiently informed about existing structures, 64.2% rated the current care situation as either "very good" or "good". Only 26.3% (n = 190) of the responding physicians were aware of patient organizations for ACHD. Conclusions: The present study showed that the majority of PCPs are not informed about the ACHD care structures available in Germany. The need for specialized ACHD follow-up care is largely underestimated, with an urgent need for optimization to reduce morbidity and mortality. For the future, solutions must be developed to integrate PCPs more intensively into the ACHD care network.

Pulmonary Hypertension in Adults with Congenital Heart Disease: Real-World Data from the International COMPERA-CHD Registry.

INTRODUCTION: Pulmonary hypertension (PH) is a common complication in patients with congenital heart disease (CHD), aggravating the natural, post-operative, or post-interventional course of the underlying anomaly. The various CHDs differ substantially in characteristics, functionality, and clinical outcomes among each other and compared with other diseases with pulmonary hypertension. OBJECTIVE: To describe current management strategies and outcomes for adults with PH in relation to different types of CHD based on real-world data. METHODS AND RESULTS: COMPERA (Comparative, Prospective Registry of Newly Initiated Therapies for Pulmonary Hypertension) is a prospective, international PH registry comprising, at the time of data analysis, >8200 patients with various forms of PH. Here, we analyzed a subgroup of 680 patients with PH due to CHD, who were included between 2007 and 2018 in 49 specialized centers for PH and/or CHD located in 11 European countries. At enrollment, the patients´ median age was 44 years (67% female), and patients had either pre-tricuspid shunts, post-tricuspid shunts, complex CHD, congenital left heart or aortic disease, or miscellaneous other types of CHD. Upon inclusion, targeted therapies for pulmonary arterial hypertension (PAH) included endothelin receptor antagonists, PDE-5 inhibitors, prostacyclin analogues, and soluble guanylate cyclase stimulators. Eighty patients with Eisenmenger syndrome were treatment-naïve. While at inclusion the primary PAH treatment for the cohort was monotherapy (70% of patients), with 30% of the patients on combination therapy, after a median observation time of 45.3 months, the number of patients on combination therapy had increased significantly, to 50%. The use of oral anticoagulants or antiplatelets was dependent on the underlying diagnosis or comorbidities. In the entire COMPERA-CHD cohort, after follow-up and receiving targeted PAH therapy (n = 511), 91 patients died over the course of a 5-year follow up. The 5-year Kaplan-Meier survival estimate for CHD associated PH was significantly better than that for idiopathic PAH (76% vs. 54%; p < 0.001). Within the CHD associated PH group, survival estimates differed particularly depending on the underlying diagnosis and treatment status. CONCLUSIONS: In COMPERA-CHD, the overall survival of patients with CHD associated PH was dependent on the underlying diagnosis and treatment status, but was significantly better as than that for idiopathic PAH. Nevertheless, overall survival of patients with PAH due to CHD was still markedly reduced compared with survival of patients with other types of CHD, despite an increasing number of patients on PAH-targeted combination therapy.

Assessment of the Psychological Situation in Adults with Congenital Heart Disease.

BACKGROUND: Due to advances in the diagnosis and treatment of congenital heart disease (CHD), the number of adults who are surviving with congenital heart disease (ACHD) is constantly growing. Until recently, the psychological effects of CHD had been widely neglected. Current research provides evidence for an increased risk of emotional distress in ACHD. The concept of illness identity attempts to explain how patients experience and integrate their CHD into their identities. The present study investigated illness identity in relation to clinical parameters and psychological functioning. Psychometric properties of the German version of the Illness Identity Questionnaire (IIQD) were examined. METHODS: Self-reported measures on illness identity and psychological functioning (HADS-D) were assessed in a representative sample of 229 ACHD (38 ± 12.5 (18-73) years; 45% female) at the German Heart Center Munich. Descriptive analyses and multiple regression models were conducted. Confirmatory factor analysis was performed to validate the IIQD. RESULTS: The IIQD demonstrated good reliability. The originally-postulated four-factor structure could not be replicated. Anatomic disease complexity and functional status significantly influenced illness identity. Illness identity accounted for unique variances in depression and anxiety: Maladaptive illness identity states (i.e., , engulfment and rejection) were associated with higher emotional distress, whereas adaptive illness (i.e., , acceptance and enrichment) identity states were linked to lower emotional distress. CONCLUSIONS: Illness Identity emerged as a predictor of emotional distress in ACHD. Findings raise the possibility that interventions designed to target a patient's illness identity may improve psychological well-being and cardiac outcomes in ACHD.

The value of hand grip strength (HGS) as a diagnostic and prognostic biomarker in congenital heart disease.

BACKGROUND: In patients with congenital heart disease (CHD), there is little data on the diagnostic and prognostic relevance of hand grip strength (HGS) for clinical assignment, while in the general population the loss of muscle strength and mass is an important risk factor in cardiovascular disease which is conversely associated with morbidity and all-cause mortality. This study aimed to assess the degree of muscle dysfunction using HGS as a biomarker in a large group of patients with CHD who often develop muscle dysfunction. METHODS: In total, 385 patients (27.6±13.1 years, 43% female) were included and assigned to 5 diagnostic groups: complex anomalies (n=131), left heart anomalies/aortopathies (n=107), right heart/pulmonary artery anomalies (n=92), primary left to-right-shunts (n=42) and miscellaneous CHD (n=13). Patients with Fontan circulation, chronic cyanosis, morphologic right systemic ventricle, arterial switch operation, or Ebstein's anomaly were analyzed separately. A control group (CG) consisted of 124 healthy individuals (30.1±12.1 years, 42% female). HGS was measured with a Jamar Hydraulic Hand Dynamometer. RESULTS: HGS was reduced in CHD patients compared to controls (35.2±14.6 versus 43.7±14.4 kg). Most impairments were present in females (26.1±7.6 kg). Patients with cyanosis had lower HGS values compared to acyanotic CHD patients (P=0.03). Patients with left heart lesions had the highest HSG values (40.7±14.7 kg), while patients with primary left-to-right shunt lesions had the lowest HSG values (30.9±11.3 kg). Within specific groups of cardiac anomalies, patients with Fontan circulation showed the lowest (P=0.033) and patients with a morphologic right system ventricle showed higher results (P=0.004). The late mid-term survival was favorable, and 7 patients (1.8%) died in a median interval of 422 days (range, 206-1,824 days) after HGS-testing. CONCLUSIONS: This study provides the most comprehensive data on the use of HGS in CHD to date. Grip strength is an easily applicable, repeatedly usable and a cost-effective diagnostic tool to gain a quick, quantifiable assessment of the patient's current muscle function as an expression of cardiac fitness. Considering the low number of patients who died in the observation period, HGS may not be a suitable tool for survival assessment or identification of patients at risk. However, HGS is well suited to determine muscle function and strength and thereby to identify and to follow-up patients who have an increased cardiovascular risk.

Hemostatic abnormalities in adult patients with Marfan syndrome.

BACKGROUND: Aortic root ectasia might induce hemostatic disorders in patients with Marfan syndrome (MFS) via altered blood flow and rheology. The aim of this study was to explore the hemostasis in patients with MFS compared with healthy controls. METHODS: In this cross-sectional case-control study we included patients with verified MFS (n=51) and sex- and age-matched healthy controls (n=50). Main criteria were the aortic root in echocardiography and cardiac magnetic resonance imaging (MRI), and the coagulation status. RESULTS: When compared with healthy controls, patients with MFS showed significantly increased diameters of the aortic roots (43.0±7.72 vs. 28.8±3.74 mm, P<0.001) and aortic Z-scores (4.36±2.77 vs. 0.948±1.09, P<0.001), considerably higher values of Multiplate® tests (e.g., MP-ADP: 878.4±201.7 vs. 660.4±243.6 AU*min, P<0.001) and PFA-100® tests (PFA Col/ADP: 102.5±45.5 vs. 91.1±46.2 s, P<0.05), PTT (30.0±3.91 vs. 28.7±2.50 s, P<0.05) and D-dimers (0.488±0.665 vs. 0.254±0.099 mg/L, P<0.001). In MFS von Willebrand factor (VWF) activity (81.9%±41.8% vs. 106.3%±41.5%, P<0.05) and antigen (93.8%±43.9% vs. 118.8%±47.8%, P<0.05) and factor VIII activity (108.9%±29.6% vs. 126.7%±28.4%, P<0.05) were reduced. Significant positive correlations were found between aortic diameters and D-dimers (all P<0.05), as well as PFA Col/ADP (all P<0.01) in MFS patients. Factor VIII activity correlated significantly negatively with the diameter of the aortic root in MFS (r=-0.55, P<0.05). CONCLUSIONS: In conclusion, our study reveals hemostatic deviations in patients with MFS. Further studies are necessary to understand the causal relationship and the exact pathomechanism.

Awareness of oral health in adults with congenital heart disease.

BACKGROUND: Poor oral health is known to be a risk factor for infective endocarditis (IE) and acquired cardiovascular disease. Many patients with congenital heart disease (CHD) are at risk for developing IE. Excellent oral health is deemed essential to prevent IE, a potentially life-threatening condition, and therefore is important in adults with congenital heart defects (ACHD). As data on oral heath in ACHD are scarce, the current study aims to assess the prevalence of caries among ACHD and the knowledge of patients about oral health as a risk factor for IE and cardiovascular disease. METHODS: A cross-sectional study included ACHD who subsequently presented at the German Heart Center Munich. The participants completed a questionnaire assessing oral health and risk awareness. Additionally, as an indicator of caries prevalence, a dentist documented the DMFT index (sum of decayed, missing and filled teeth). RESULTS: The median age of the 112 participants was 31.5 (range, 18-77) years. Forty-three patients (38%) were unaware of a correlation between oral health and heart disease. Twelve participants (11%) did not know about poor oral health being a risk factor for cardiovascular diseases. Less than half of the surveyed patients knew the term endocarditis. An additional twelve patients (11%) reported caveats against dental treatment because of their CHD. In twenty-three patients (21%), caries was found at the dentine level. The mean DMFT index, representing caries prevalence, was 7.91 (±6.54). There was no correlation between the Bethesda criteria of CHD and the DMFT index (P=0.69). The DMFT index was higher in patients at high risk for IE (P<0.01). However, this difference in the caries score was mainly due to more extracted and filled teeth in the high-risk patients for IE, whereas the number of decayed teeth did not differ significantly (P=0.09). CONCLUSIONS: The awareness of the importance of optimized oral health in ACHD needs to be improved.