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.

Due to technological and medical advances the population of adults with congenital heart disease (ACHD) is growing. Worldwide, congenital heart disease (CHD) affects 1.35-1.5 million children each year and more than 90% reach adulthood. Given the heterogeneity of CHD, survivors are faced with not only complex medical but also psychological challenges which may manifest in mental health problems, such as depression, anxiety and posttraumatic stress disorder. This review focuses on the emotional dimension of CHD. More precisely, it summarizes the present state of research on the prevalence of emotional distress in ACHD. Theoretical models provide a framework for possible explanations of mental health issues in ACHD. Additionally, the review examines the relation between psychological processes and overall health considering the latest scientific findings on coping with chronic illness (illness identity). There is still insufficient knowledge on the psychosocial treatment of mental health issues in the growing population of ACHD. This review suggests a vital need to further investigate the psychological situation of ACHD on a large-scale basis in order to establish a holistic treatment approach to accommodate the patients' special needs.

Congenital heart diseases (CHD) are a leading cause of morbidity in children with a high impact on the psychological health of parents. Possible short-term and long-term psychological problems among parents are addressed in the current paper. The diagnosis of CHD paired with subsequent surgical and interventional treatment and prolonged hospital stays cause acute psychological distress and can lead to posttraumatic stress disorders (PTSD). As the disease course progresses, the impact on parents' health tends to decrease, but the risk of developing long-term psychological issues remains high. Studies have focused mainly on stress and other distressing symptoms without explicitly addressing the effects of a CHD diagnosis on the family system. Since the social environment may play an important role in parent's life, it may be useful to conduct studies to address these issues. In particular, the psychological situation of the father and the impact of the child's disease on the different dimensions of the father's life, such as parenting skills and influences on the parental relationship, have been largely neglected. Recent research has also disregarded the impact of CHD on siblings of the affected child. Research on chronic diseases in general has shown that the children's age and severity of the disease are related to an increased level of stress. Given the severity of CHD, anxiety and depression were higher in parents with children with more severe conditions. In addition, the results suggest that a positive construction of the parent-child relationship (attachment and bonding) is impaired, especially in mothers. Mothers reported worries and concerns about the challenging tasks they would face after learning about their child's CHD and how they can deal with their child's needs. It has also been shown that the child's illness has a negative impact on the whole family system, including the parent's relationship. Impairments on the parental relationship were perceived differently among mothers and fathers. Thus, there is high need for major changes to be identified, developed and implemented in the psychological care of parents with chronically ill children. So far, research has focused more on the psychological status of parents with chronically ill children, but less research has closely examined the effects of a child's CHD on its parent's mental health even though there is a high demand in additional support. A holistic treatment approach should include professional parental support, especially during children's hospitalization, information on the home care resources and services (especially respite services) and psychological support for parents.

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.

ObjectiveThe 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.DesignCross-sectional survey.ParticipantsBetween 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 measuresQOL 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.ResultsOverall, 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).ConclusionCurrent 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 numberDRKS00017699; Results.

Due to technological and medical advances the population of adults with congenital heart disease (ACHD) is constantly growing. Worldwide, congenital heart disease (CHD) affects 1.35-1.5 million children each year and the number of ACHD meanwhile exceeds the number of CHD children. It has been found that a substantial number of ACHD present problematic health behaviors, such as physical inactivity and bad nutritional habits. Recent studies document alarming rates of overweight and obesity among CHD patients which may consequently lead to further health complications in this population. The present article focuses on the distinct psychosocial effects resulting from the diagnosis of CHD and their impact on developing disordered eating patterns and excess weight. It seeks to identify unique risk factors and relevant explanations associated with the increasing prevalence of obesity among CHD patients. This review suggests a vital need to establish clinical guidelines for nutrition and weight management in this patient population as part of a holistic treatment approach.

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.

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 followup, 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.