'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

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