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.</p

Sense of coherence in adults with congenital heart disease in 15 countries: Patient characteristics, cultural dimensions and quality of life

BACKGROUND: Previous studies have found that sense of coherence (SOC) is positively related to quality of life (QoL) in persons with chronic conditions. In congenital heart disease (CHD), the evidence is scant. AIMS: We investigated (i) intercountry variation in SOC in a large international sample of adults with CHD; (ii) the relationship between demographic and clinical characteristics and SOC; (iii) the relationship between cultural dimensions of countries and SOC; and (iv) variation in relative importance of SOC in explaining QoL across the countries. METHODS: APPROACH-IS was a cross-sectional, observational study, with 4028 patients from 15 countries enrolled. SOC was measured using the 13-item SOC scale (range 13-91) and QoL was assessed by a linear analog scale (range 0-100). RESULTS: The mean SOC score was 65.5±13.2. Large intercountry variation was observed with the strongest SOC in Switzerland (68.8±11.1) and the lowest SOC in Japan (59.9±14.5). A lower SOC was associated with a younger age; lower educational level; with job seeking, being unemployed or disabled; unmarried, divorced or widowed; from a worse functional class; and simple CHD. Power distance index and individualism vs collectivism were cultural dimensions significantly related to SOC. SOC was positively associated with QoL in all participating countries and in the total sample, with an explained variance ranging from 5.8% in Argentina to 30.4% in Japan. CONCLUSION: In adults with CHD, SOC is positively associated with QoL. The implementation of SOC-enhancing interventions might improve QoL, but strategies would likely differ across countries given the substantial variation in explained variance

Development of quality indicators of transfer and transition in adolescents and young adults with congenital heart disease.

BACKGROUND Quality indicators are crucial in evaluating and comparing the quality of healthcare services. In the case of congenital heart disease, transition programmes for adolescents have been recommended to ensure uninterrupted healthcare and lifelong care. It is necessary to establish quality indicators in order to facilitate the evaluation of programme quality and to allow comparison between different centres. The objective of this study is therefore to develop a set of quality indicators for the transition of adolescents with congenital heart disease. METHODS The RAND/UCLA appropriateness method was employed in a four-step process to develop a set of quality indicators. First, a literature search was conducted on the dimensions of transitional care, based on which a preliminary set of quality indicators was developed. Second, experts were contacted, and an expert panel was established. Third, the panel members were asked to rate the appropriateness of the quality indicators in a two-round process. Finally, in the fourth step, we evaluated the data by measuring the median and Disagreement Index. RESULTS The expert panel consisted of 16 members, congenital cardiologists, nurses, transition experts, patients and research experts. The preliminary set of quality indicators comprised 16 items, categorized in process and structure criteria. Based on the panel's feedback, the set was refined to 12 quality indicators, which were rated as relevant and feasible. CONCLUSIONS This study represents the first attempt to develop quality indicators for transitional care services for adolescents with congenital heart disease. The set of 12 quality indicators was developed based on existing evidence and expert opinion. Further testing is needed to assess the feasibility of these quality indicators in daily practice. If successfully implemented, these quality indicators could allow comparison and facilitate benchmarking of transitional care services for adolescents with congenital heart disease

Validity, reliability and responsiveness of the "Schedule for the Evaluation of Individual Quality of Life – Direct Weighting" (SEIQoL-DW) in congenital heart disease

BACKGROUND: The 'Schedule for the Evaluation of Individual Quality of Life – Direct Weighting' (SEIQoL-DW) is an instrument developed to measure individual quality of life. Although this instrument has been used in numerous studies, data on validity and reliability are sparse. This study aimed to examine aspects of validity, reliability and responsiveness of the SEIQoL-DW on data obtained in adults with congenital heart disease, by using the new standards of psychological testing. METHODS: We evaluated validity evidence based on test content, internal structure, and relations to other variables, as well as the stability and responsiveness of the SEIQoL-DW. Evidence was provided by both theoretical considerations and empirical data. Empirical data were acquired from two studies. Firstly, using a cross-sectional study design, we included 629 patients with congenital heart disease. Secondly, 130 of the 629 initially included patients readministered the questionnaires approximately one year after the first data collection. In addition to the SEIQoL-DW, linear analog scales were used to assess overall quality of life and perceived health. RESULTS: We found that the SEIQoL-DW is not a valid measure of quality of life, but rather assesses determinants that contribute to individuals' quality of life. The SEIQoL-DW consistently proved to be valid and reliable to assess those determinants. However, responsiveness in patients with congenital heart disease may be problematic. CONCLUSION: Based on theoretical and empirical considerations, the SEIQoL-DW cannot be considered as a quality of life instrument. Nonetheless, it is a valid and reliable instrument to explore determinants for patients' quality of life

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Delivery of care for adult patients with congenital heart disease in Europe: results from the Euro Heart Survey

Aims The increasing number of adults with congenital heart disease (CHD) has prompted the development of recommendations for the management of these patients and for the organization of their healthcare. The aim of this report is to describe the delivery of care in Europe for adults with congenital cardiac anomalies. Methods and results As part of the Euro Heart Survey on Adult Congenital Heart Disease, we obtained data from 71 voluntarily participating centres that detailed their care practices for these patients. Forty-eight of these centres were specialist centres and 23 were non-specialist centres. We found that only 19% of the specialist centres complied with defined standards for optimal care structure. The criteria that appeared to be most difficult for all centres to achieve were performing 50 congenital heart operations or more per year and involving nurse specialists in the care of these patients. Conclusion This survey indicated that the provision of care in Europe for adults with congenital heart defects is suboptimal. To fully realize the benefits of cardiac surgery performed in infants and children, continuous effort must be applied by healthcare professionals in order to implement the recommendations on the organization of care for these patient

Individual quality of life in adults with congenital heart disease: a paradigm shift

Aims During the last decade, a paradigm shift has emerged in the measurement of quality of life, from the use of standard questionnaires towards a more individualized approach. Therefore, this study examined individual quality of life in adults with congenital heart disease and explored potential differences with those reported by matched, healthy control subjects. Methods and results We examined 579 adults with congenital heart disease. A subsample of 514 of these patients was matched for age, gender, educational level, and employment status with 446 healthy counterparts. Individual quality of life was assessed using the Schedule for the Evaluation of Individual Quality of Life-Direct Weighting (SEIQoL-DW). Twelve domains affecting patients' quality of life were identified. Family, job/education, friends, health, and leisure time were the most prominent quality of life domains. Significantly fewer patients than control subjects considered financial means and material well-being and future to be important determinants of quality of life. Conclusion Assessment of quality of life in adults with congenital heart disease that focusses on the individual is appropriate for obtaining in-depth information on issues relevant for patients' quality of life. This represents a paradigm shift in the measurement of this concep