Psychosocial well-being in postpartum women with congenital heart disease.

Background: Improved treatment options for congenital heart disease (CHD) lead to a growing number of women with CHD at reproductive age. Due to physical and psychological burden, pregnancies in women with CHD often count for high-risk. Resulting emotional distress can adversely impact pregnancy, motherhood and fetal health. The present study aims to retrospectively investigate mental outcomes and indices of adjustment in women with CHD before, during and after pregnancy. The novel concept of illness identity is applied to explain how patients experience and integrate their CHD into their identities. Methods: Patient-reported outcome measures on mental functioning and illness identity were assessed in a sample of 121 postpartum women with CHD [mean age: 42.7±9.2 (range, 27-81) years] at the German Heart Centre Munich between August and November 2021 in a cross-sectional design. Descriptive analyses, correlations and linear regression models were calculated. Results: Retrospectively assessed prevalence of emotional distress before giving birth was high (47.0%) and peaked shortly after childbirth in terms of elevated symptoms of postpartum depression and trauma. During the course of maternity, emotional distress decreased significantly (24.1%, P<0.001). Overall, postpartum women demonstrated high scores in functional illness identity states (i.e., acceptance and enrichment) and low scores in dysfunctional states (i.e., rejection and engulfment). CHD severity was not directly associated with mental outcomes (P>0.05), whereas maternal cardiovascular risk, according to the WHO classification, was significantly associated with a higher prevalence of postpartum trauma (t=2.485, P=0.015). Conclusions: Postpartum mental health problems, such as (postpartum) depression, anxiety, and posttraumatic stress can become a serious burden which might be detrimental to the mother's well-being and her infant's development. Present findings emphasise the urgent need for a holistic approach focusing on pregnant women with CHD starting at the prepartum stage to prevent adverse consequences and promote maternal well-being. Illness identity might become an important target construct for clinical practice as it may positively and enduringly influence mental well-being of pregnant women with CHD.

Quality of life in patients with Fabry's disease: a cross-sectional study of 86 adults.

Background: Fabry disease (FD) is a multi-organ disorder associated with severe physical and psychological impairments, particularly in adulthood. To date, comprehensive data on the psychological burden of FD are lacking. The present study assessed quality of life (QOL) in a representative cohort of adults with FD. Methods: Patient-reported outcome measures were retrospectively analyzed in 86 adults with FD (49.6±16.6 years; 62.8% female) and compared to adults with congenital heart defects (ACHD) which is another lifelong disease and affliction. QOL was assessed using the European Quality of Life 5 Dimensions 5 Levels questionnaire (EQ-5D-5L). Results: Subjects affected by FD reported an overall reduced QOL (EQ-VAS: 71.8±20.0). Most frequently reported complaints occurred within the dimensions pain/discomfort (69.7%), daily activities (48.9%) and anxiety/depression (45.4%). Compared to ACHD, individuals with FD scored significantly lower in the areas of pain/discomfort, usual activities and mobility (all P<0.05). Older age and female sex were particularly associated with diminished QOL (P=0.05). Conclusions: Patients with FD are at high risk for impaired QOL. They require additional support to cope with disease-related challenges. Increased attention should be directed towards improving their subjective well-being to potentially increase their QOL and long-term health outcomes.

[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.

Quality of life in adults with congenital heart disease with and without pulmonary hypertension: a comparative study.

Background: Pulmonary vascular disease and pulmonary hypertension (PH) belong to the most relevant complications of congenital heart disease (CHD) in the long-term course. Although PH might lead to a severely impaired quality of life (QOL), there are no current studies comparing QOL in adults with CHD (ACHD) with and without PH. Therefore, this study aimed to systematically examine QOL in ACHD with and without PH in order to generate a more differentiated understanding of their overall health-status and to employ newly gained findings into general care of this particular patient population. Methods: In this comparative cross-sectional study, a representative sample of 803 adults with various forms of CHD with or without PH was analyzed. Data collection took place between September 2017 until February 2018 in a tertiary care center for ACHD. Medical data was retrieved from medical records. QOL was assessed using the EQ-5D-5L questionnaire. Descriptive methods, chi-square and t-tests were used to compare QOL of patients with and without PH. Results: Of 803 patients, 752 patients had no PH [93.6%; 47.3% female; mean age: 34.9±11.83 (range, 18-86) years], 51 were identified with manifest PH [6.4%; 55.8% female; mean age: 41.9±12.17 (range, 21-69) years]. PH patients showed significantly worse overall QOL [mean no PH: 86.78±13.30 (8.2-100) vs. mean PH: 81.79±12.77 (43.6-100); 95% confidence interval (CI): 1.43 to 9.46; P=0.013], worse crosswalk index [mean no PH: 93.29±12.86 (-11, 100) vs. mean PH: 89.73±11.77 (47-100); 95% CI: 0.08 to 7.56; P=0.043], and worse VAS [mean no PH: 80.12±16.50 (15-100) vs. mean PH: 72.56±16.80 (40-100); 95% CI: 3.29 to 12.17; P=0.004]. However, after adjusting for age, only the VAS stayed significant (P=0.039). The QOL within the dimensions mobility (P<0.001), self-care (P=0.002), and usual activities (P=0.007), were significantly decreased in PH patients. Overall, anxiety and depression (11.08%) as well as pain and discomfort (11.03%) were the most impaired dimensions in both patient groups. Conclusions: Since QOL is a significant predictor of outcome, PH-CHD patients need an early provision of psychosocial, health promoting support in addition to dedicated care and targeted PH treatment. It is therefore pivotal to timely identify unique psychosocial impairments in order to enhance quantity and QOL in this particularly vulnerable patient population.

[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.

'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.

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.

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.

Overweight and obesity: an emerging problem in patients with congenital heart disease.

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.

Current research status on the psychological situation of parents of children with congenital heart disease.

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.

Current research status on the psychological situation of adults with congenital heart disease.

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.