Frailty and Cognitive Function in Middle-Aged and Older Adults With Congenital Heart Disease.

BACKGROUND: Life expectancy of patients with congenital heart disease (CHD) has increased rapidly, resulting in a growing and aging population. Recent studies have shown that older people with CHD have higher morbidity, health care use, and mortality. To maintain longevity and quality of life, understanding their evolving medical and psychosocial challenges is essential. OBJECTIVES: The authors describe the frailty and cognitive profile of middle-aged and older adults with CHD to identify predictor variables and to explore the relationship with hospital admissions and outpatient visits. METHODS: Using a cross-sectional, multicentric design, we included 814 patients aged ≥40 years from 11 countries. Frailty phenotype was determined using the Fried method. Cognitive function was assessed by the Montreal Cognitive Assessment. RESULTS: In this sample, 52.3% of patients were assessed as robust, 41.9% as prefrail, and 5.8% as frail; 38.8% had cognitive dysfunction. Multinomial regression showed that frailty was associated with older age, female sex, higher physiologic class, and comorbidities. Counterintuitively, patients with mild heart defects were more likely than those with complex lesions to be prefrail. Patients from middle-income countries displayed more prefrailty than those from higher-income countries. Logistic regression demonstrated that cognitive dysfunction was related to older age, comorbidities, and lower country-level income. CONCLUSIONS: Approximately one-half of included patients were (pre-)frail, and more than one-third experienced cognitive impairment. Frailty and cognitive dysfunction were identified in patients with mild CHD, indicating that these concerns extend beyond severe CHD. Assessing frailty and cognition routinely could offer valuable insights into this aging population.

Evaluation of a nurse-led multi-component transition program for adolescents with congenital heart disease.

OBJECTIVE: To evaluate the effectiveness of the transition program for adolescents with congenital heart disease (CHD) 'Transition With a Heart' (TWAH) on disease-related knowledge, quality of life (QoL), transition experiences, and gaps in follow-up. METHODS: A study with pre-posttest and control group (post-test) using consecutive sampling, including adolescents with moderate to severely complex CHD, without intellectual disability, aged≥ 12 y, and parents. After weighting, t-tests were performed. A multivariable regression analysis explored the outcomes' determinants. RESULTS: In the intervention group, 28 adolescents and 25 parents were included, and 53 adolescents and 18 parents as controls. Adolescents' knowledge significantly increased after completing TWAH (from 59.8% to 75.7%;p < 0.01). Their knowledge was positively correlated with TWAH (ß = +13.3;p < 0.01). Adolescents' transition experiences were also positively related to TWAH (general experience: ß = +5.5;p < 0.01; transfer satisfaction: ß = +0.8; p < 0.01). Adolescents' QoL was mainly determined by CHD complexity and not by TWAH. No one showed gaps in follow-up. TWAH was not associated with parents' transition experiences. CONCLUSION: Implementing TWAH substantially improved adolescents' disease-related knowledge and transition experiences. PRACTICE IMPLICATIONS: The results regarding transition experiences need to be confirmed by further research. The TWAH design with the person-tailored educational program, skills training, and the transition coordinator can be used in settings with other chronic diseases.

Preferences for disease-related information and transitional skills among adolescents with congenital heart disease in the early transitional stage.

PURPOSE: The transition towards adult-focused healthcare comprises a complex process requiring careful, individualized guidance of adolescents with congenital heart disease (CHD). Detailed data on their preferences regarding disease-related information and acquirable transitional skills are mostly lacking. We examined the preferences of CHD adolescents in the early transition stage. METHODS: A cross-sectional descriptive study was performed with adolescents recruited from a transition program. Two questionnaires assessing needs for information and transitional skills were used. Only questionnaires completed in the early transition stage were included. RESULTS: Forty-nine adolescents participated (mean age 15.9 ± 1.2 years, 43% girls). 59% requested information about their heart and previous and/or future surgeries/interventions, 45% about sports and medication, and a maximum of 27% about psychosocial topics. More girls than boys requested surgical information (76% versus 46%; p = 0.04). Adolescents with severely complex CHD more often requested information about medication than those with moderately complex CHD (63% versus 28%; p = 0.02). Older adolescents were less likely to request surgical information (OR = 0.53; 95%CI [0.26-0.88]; p = 0.03). Up to 83% of the adolescents perceived their skills as insufficient, but less than 14% was interested in acquiring skills.    Conclusion: Adolescents were mainly interested in medical topics, followed by lifestyle information. The informational needs tended to decrease with age. Timely gauging individual needs and delivering information, ideally in the early teens, appear important when providing person-tailored transitional care in CHD. Paradoxically, although the transitional skills were low-perceived, there was limited motivation to improve them. This paradox requires further investigation to better tailor transition interventions.     Clinical trial registration: Not applicable. WHAT IS KNOWN: • Transition towards adult life and care requires careful patient guidance. A person-tailored approach is strongly encouraged. However, details on preferences regarding disease-related information and transitional skills are scarce in adolescents with CHD. WHAT IS NEW: • This study showed that adolescents with CHD in early transition mainly need medical-related information. Their informational needs tend to decrease with age necessitating timely gauging for interest and delivering information. Adolescents report a low need to acquire transitional skills despite low self-esteemed skills levels.

Last year of life of adults with congenital heart diseases: causes of death and patterns of care.

AIMS: Although life expectancy in adults with congenital heart diseases (CHD) has increased dramatically over the past five decades, still a substantial number of patients dies prematurely. To gain understanding in the trajectories of dying in adults with CHD, the last year of life warrants further investigation. Therefore, our study aimed to (i) define the causes of death and (ii) describe the patterns of healthcare utilization in the last year of life of adults with CHD. METHODS AND RESULTS: This retrospective mortality follow-back study used healthcare claims and clinical data from BELCODAC, which includes patients with CHD from Belgium. Healthcare utilization comprises cardiovascular procedures, CHD physician contacts, general practitioner visits, hospitalizations, emergency department (ED) visits, intensive care unit (ICU) admissions, and specialist palliative care, and was identified using nomenclature codes. Of the 390 included patients, almost half of the study population (45%) died from a cardiovascular cause. In the last year of life, 87% of patients were hospitalized, 78% of patients had an ED visit, and 19% of patients had an ICU admission. Specialist palliative care was provided to 17% of patients, and to only 4% when looking at the patients with cardiovascular causes of death. CONCLUSIONS: There is a high use of intensive and potentially avoidable care at the end of life. This may imply that end-of-life care provision can be improved. Future studies should further examine end-of-life care provision in the light of patient's needs and preferences, and how the healthcare system can adequately respond.

Rationale, design and methodology of APPROACH-IS II: International study of patient-reported outcomes and frailty phenotyping in adults with congenital heart disease.

BACKGROUND: In recent years, patient-reported outcomes (PROs) have received increasing prominence in cardiovascular research and clinical care. An understanding of the variability and global experience of PROs in adults with congenital heart disease (CHD), however, is still lacking. Moreover, information on epidemiological characteristics and the frailty phenotype of older adults with CHD is minimal. The APPROACH-IS II study was established to address these knowledge gaps. This paper presents the design and methodology of APPROACH-IS II. METHODS/DESIGN: APPROACH-IS II is a cross-sectional global multicentric study that includes Part 1 (assessing PROs) and Part 2 (investigating the frailty phenotype of older adults). With 53 participating centers, located in 32 countries across six continents, the aim is to enroll 8000 patients with CHD. In Part 1, self-report surveys are used to collect data on PROs (e.g., quality of life, perceived health, depressive symptoms, autonomy support), and explanatory variables (e.g., social support, stigma, illness identity, empowerment). In Part 2, the cognitive functioning and frailty phenotype of older adults are measured using validated assessments. DISCUSSION: APPROACH-IS II will generate a rich dataset representing the international experience of individuals in adult CHD care. The results of this project will provide a global view of PROs and the frailty phenotype of adults with CHD and will thereby address important knowledge gaps. Undoubtedly, the project will contribute to the overarching aim of improving optimal living and care provision for adults with CHD.

Influenza Vaccination in Patients With Congenital Heart Disease in the Pre-COVID-19 Era: Coverage Rate, Patient Characteristics, and Outcomes.

BACKGROUND: Influenza vaccination is the most commonly recommended immune prevention strategy. However, data on influenza vaccination in patients with congenital heart disease (CHD) are scarce. In this study, our goals were to: (1) measure vaccination coverage rates (VCRs) for influenza in a large cohort of children, adolescents, and adults with CHD; (2) identify patient characteristics as predictors for vaccination; and (3) investigate the effect of influenza vaccination on hospitalization. METHODS: A nationwide cohort study in Belgium included 16,778 patients, representing 134,782 vaccination years, from the Belgian Congenital Heart Disease Database Combining Administrative and Clinical Data (BELCODAC). Data over 9 vaccination years (2006-2015) were used, and patients were stratified into 5 age cohorts: 6 months to 4 years; 5-17 years; 18-49 years; 50-64 years; and 65 years and older. RESULTS: In the respective age cohorts, the VCR was estimated to be 6.6%, 8.0%, 23.9%, 46.6%, and 72.8%. There was a steep increase in VCRs as of the age of 40 years. Multivariable logistic regression showed that higher anatomical complexity of CHD, older age, presence of genetic syndromes, and previous cardiac interventions were associated with significantly higher VCRs. Among adults, men had lower and pregnant women had higher VCRs. The association between influenza vaccination and all-cause hospitalization was not significant in this study. CONCLUSIONS: The influenza VCR in people with CHD is low, especially in children and adolescents. Older patients, particularly those with complex CHD, are well covered. Our findings should inform vaccination promotion strategies in populations with CHD.

Needs and Experiences of Adolescents with Congenital Heart Disease and Parents in the Transitional Process: A Qualitative Study.

PURPOSE: Most patients with congenital heart disease (CHD) need lifelong cardiac follow-up. Transitioning to adulthood and transferring to adult-focused care are often challenging. We explored the experiences and needs of adolescents with CHD and parents during the entire transitional process, including the post-transfer period. DESIGN AND METHODS: We performed a qualitative study according to the phenomenological approach, focusing on adolescents with CHD and parents. Semi-structured interviews were carried out with patients (n = 9) and parents (n = 12) after being transferred to adult care facilities. Data were analyzed with inductive thematic analysis. Data collection and -analysis of both samples were done separately in a first step, after which results were merged to discover common themes. RESULTS: Five common themes were identified: 1) Having mixed feelings about leaving pediatric care; 2) Being prepared and informed; 3) Shifting responsibilities and roles; 4) Being accompanied during consultations; and 5) Gaining trust in new healthcare providers. CONCLUSION: Adolescents with CHD and parents express a need for adequate preparation and personalized guidance to reduce anxiety and uncertainty during transition. The process may benefit from focusing on improving the adolescents´ transitional skills and disease-related knowledge, which may, in turn, facilitate handing over responsibilities and adapting to new roles by the parents. Adolescents appreciate the presence of parents during the consultation, albeit with reduced input. Finally, a transition coordinator and a joint transfer consultation involving the pediatric cardiologist seem paramount for a fluent transitional process, especially in establishing new treatment relationships.

Different levels of care for follow-up of adults with congenital heart disease: a cost analysis scrutinizing the impact on medical costs, hospitalizations, and emergency department visits.

AIM: To scrutinize the economic impact of different care levels, such as shared care, in the follow-up of adult congenital heart disease (ACHD) patients. METHODS: The BELgian COngenital heart disease Database combining Administrative and Clinical data (BELCODAC) was analyzed. Patients (N = 6579) were categorized into five care levels based on their cardiac follow-up pattern between 2006 and 2010. Medical costs, hospitalizations, and emergency department visits were measured between 2011 and 2015. RESULTS: In patients with moderate lesions, highly specialized cardiac care (HSC; exclusive follow-up by ACHD specialists) and shared care with predominantly specialized cardiac care (SC+) were associated with significantly lower medical costs and resource use compared to shared care with predominantly general cardiac care (SC-) and general cardiac care (GCC). In the patient population with mild lesions, HSC was associated with better economic outcomes than SC- and GCC, but SC+ was not. HSC was associated with fewer hospitalizations (- 33%) and less pharmaceutical costs (- 46.3%) compared to SC+. Patients with mild and moderate lesions in the no cardiac care (NCC) group had better economic outcomes than those in the GCC and SC- groups, but post-hoc analysis revealed that they had a different patient profile than patients under cardiac care. CONCLUSION: More specialized care levels are associated with better economic outcomes in patients with mild or moderate lesions in cardiac follow-up. Shared care with strong involvement of ACHD specialists might be a management option to consider. Characteristics of patients without cardiac follow-up but good medium-term economic prospects should be further scrutinized.

Development of a transition program for adolescents with congenital heart disease.

Thanks to advances in care, most children with congenital heart disease nowadays survive into adulthood. The majority of patients remain at high risk for future complications. Hence, life-long follow-up is mandatory. Care needs of patients evolve, especially when reaching adulthood. A structured transition period to adult care is advocated. Currently, a fully detailed and structured transition program is not available for patients with congenital heart disease. The aim is to describe the development and design of a multicomponent transition program for adolescents with congenital heart disease, called "Transition with a heart." Transition with a heart was developed based on the Dutch program "On your own feet," starting at the age of 12 years and continuing after transfer. The most vital core components include a general and individualized flowchart, adolescent-centered communication, a joined transfer consultation, and an appointed transition coordinator. Adolescents are gradually informed about their condition and potential late consequences in adult life and stimulated to take medical care in their own hands.Conclusion: Transition with a heart is a practical, multicomponent, comprehensive transition program developed to cover the essential aspects of transitional care for adolescents with congenital heart disease (i.e., continuity of care, disease knowledge, and self-management skills). Interventions were selected from the highest sources of scientific evidence currently available.Clinical trial registration: Not applicableWhat is Known:• Transition towards adult life and health care is a complex process, requiring careful patients' guidance. Various task forces have described the need and potential benefits of transition programs in young people with chronic conditions. Details about the practical development and content of such programs in congenital heart disease are, however, currently lacking.What is New:• This method paper presents the development and design of a person-centered multicomponent transition program for adolescents with congenital heart disease comprising interventional components covering the most important aspects of transitional care: promoting autonomy, disease knowledge, and continuity of care.

Opinions of general and adult congenital heart disease cardiologists on care for adults with congenital heart disease in Belgium: a qualitative study.

BACKGROUND: The growing adult congenital heart disease (CHD) population requires efficient healthcare organisation. It has been suggested that clinically appropriate care be provided for individual patients on the least complex level possible, in order to alleviate saturation of special care programmes. METHODS: Semi-structured interviews with 10 general and 10 adult CHD cardiologists were conducted to elucidate opinions on healthcare organisation in Belgium. A particular focus was placed on the potential role of general cardiologists. The software program NVivo 12 facilitated thematic analysis. RESULTS: A discrepancy existed between how general cardiologists thought about congenital care and what adult CHD cardiologists considered the minimum knowledge required to adequately treat patients. Qualitative data were categorised under the following themes: knowledge dissemination, certification, (de)centralisation of care, the role of adult CHD cardiologists, the role of dedicated nurse specialists, and patient referral. It appeared to be pivotal to organise care in such a way that providing basic care locally does not impede the generation of sufficient patient volume, and to continue improving communications between different care levels when there is no referral back. Moreover, practical knowledge is best disseminated locally. Cardiologists' opinions on certification and on the role of dedicated nurse specialists were mixed. CONCLUSION: On the basis of the results, we propose five recommendations for improving the provision of care to adults with CHD. A multidimensional approach to defining the role of different healthcare professionals, to improving communication channels, and to effectively sensitising healthcare professionals is needed to improve the organisation of care.

Sexuality of patients with chronic heart failure and their spouses and the need for information regarding sexuality.

BACKGROUND: Psychological and physiological effects of chronic heart failure (CHF) can influence sexuality. Both CHF patients and their partners may experience changes and have questions about sexuality. Despite this, healthcare professionals give little information regarding sexuality. This may be due to the paucity of literature describing patients' concerns and questions about sexuality and CHF. AIMS: The aims of this study were to describe the sexuality of patients and their partners and to describe their needs for information regarding sexuality. METHODS: A prospective, descriptive cross-sectional design was used. A convenience sample was obtained in three hospitals (Belgian and the Netherlands). Patients and partners completed two questionnaires: Sexual Adjustment Scale (SAS) and the Needs of Sexual Counseling Scale for Chronic Heart Failure (NSCS-CHF). RESULTS: There were 52 patients and 32 partners who participated in the study. No or slight disturbance was seen in sexual discussions and intimacy. Marked or serious disturbance was reported in sexual activity. A mixed profile was seen in sexual performance, sexual interest and sexual pleasure. The majority of patients with CHF and their partners want information regarding sexuality. The NSCS-CHF clearly identified the information needs of patients and partners and most needs concerned the areas of symptoms, the relationship and relaxation. CONCLUSION: The questionnaires were easy to complete and changes in sexuality and information needs were identified. The NSCS-CHF needs further validation as a research instrument. Healthcare professionals can use both questionnaires to bring up the topic of sexuality and to identify topics that require further discussion or counselling with patients and partners.