Phenotypes of adults with congenital heart disease around the globe: a cluster analysis.

OBJECTIVE: To derive cluster analysis-based groupings for adults with congenital heart disease (ACHD) when it comes to perceived health, psychological functioning, health behaviours and quality of life (QoL). METHODS: This study was part of a larger worldwide multicentre study called APPROACH-IS; a cross sectional study which recruited 4028 patients (2013-2015) from 15 participating countries. A hierarchical cluster analysis was performed using Ward's method in order to group patients with similar psychological characteristics, which were defined by taking into consideration the scores of the following tests: Sense Of Coherence, Health Behavior Scale (physical exercise score), Hospital Anxiety Depression Scale, Illness Perception Questionnaire, Satisfaction with Life Scale and the Visual Analogue Scale scores of the EQ-5D perceived health scale and a linear analogue scale (0-100) measuring QoL. RESULTS: 3768 patients with complete data were divided into 3 clusters. The first and second clusters represented 89.6% of patients in the analysis who reported a good health perception, QoL, psychological functioning and the greatest amount of exercise. Patients in the third cluster reported substantially lower scores in all PROs. This cluster was characterised by a significantly higher proportion of females, a higher average age the lowest education level, more complex forms of congenital heart disease and more medical comorbidities. CONCLUSIONS: This study suggests that certain demographic and clinical characteristics may be linked to less favourable health perception, quality of life, psychological functioning, and health behaviours in ACHD. This information may be used to improve psychosocial screening and the timely provision of psychosocial care.

Smoking among adult congenital heart disease survivors in the United States: Prevalence and relationship with illness perceptions.

The relationship between smoking and illness perceptions among congenital heart disease (CHD) survivors is unknown. The primary aims of the present study were to compare the smoking prevalence among CHD survivors to a nationally representative U.S. sample and examine the relationship between smoking and illness perceptions. CHD survivors (N = 744) from six U.S. sites participated in the study. The smoking prevalence among CHD survivors (9.3%) was lower than the general population (15.3%). However, 23.3% of CHD survivors with severe functional limitations smoked. Smoking prevalence differed by U.S. region, with a greater proportion of those attending CHD care in the Midwest reporting smoking (11.8%). The illness perception dimensions of Concern and Emotional Response were independently associated with smoking. Differences in illness perceptions enhance our understanding of smoking among CHD survivors and may guide interventions promoting positive health behaviors. The protocol for the study from which the present analyses were conducted was recorded at ClinicalTrials.gov: NCT02150603.

Substantial Cardiovascular Morbidity in Adults With Lower-Complexity Congenital Heart Disease.

BACKGROUND: Although lower-complexity cardiac malformations constitute the majority of adult congenital heart disease (ACHD), the long-term risks of adverse cardiovascular events and relationship with conventional risk factors in this population are poorly understood. We aimed to quantify the risk of adverse cardiovascular events associated with lower-complexity ACHD that is unmeasured by conventional risk factors. METHODS: A multitiered classification algorithm was used to select individuals with lower-complexity ACHD and individuals without ACHD for comparison among >500 000 British adults in the UK Biobank. ACHD diagnoses were subclassified as isolated aortic valve and noncomplex defects. Time-to-event analyses were conducted for the primary end points of fatal or nonfatal acute coronary syndrome, ischemic stroke, heart failure, and atrial fibrillation and a secondary combined end point for major adverse cardiovascular events. Maximum follow-up time for the study period was 22 years with retrospectively and prospectively collected data from the UK Biobank. RESULTS: We identified 2006 individuals with lower-complexity ACHD and 497 983 unexposed individuals in the UK Biobank (median age at enrollment, 58 [interquartile range, 51-63] years). Of the ACHD-exposed group, 59% were male, 51% were current or former smokers, 30% were obese, and 69%, 41%, and 7% were diagnosed or treated for hypertension, hyperlipidemia, and diabetes mellitus, respectively. After adjustment for 12 measured cardiovascular risk factors, ACHD remained strongly associated with the primary end points, with hazard ratios ranging from 2.0 (95% CI, 1.5-2.8; P<0.001) for acute coronary syndrome to 13.0 (95% CI, 9.4-18.1; P<0.001) for heart failure. ACHD-exposed individuals with ≤2 cardiovascular risk factors had a 29% age-adjusted incidence rate of major adverse cardiovascular events, in contrast to 13% in individuals without ACHD with ≥5 risk factors. CONCLUSIONS: Individuals with lower-complexity ACHD had a higher burden of adverse cardiovascular events relative to the general population that was unaccounted for by conventional cardiovascular risk factors. These findings highlight the need for closer surveillance of patients with mild to moderate ACHD and further investigation into management and mechanisms of cardiovascular risk unique to this growing population of high-risk adults.

Healthcare system inputs and patient-reported outcomes: a study in adults with congenital heart defect from 15 countries.

BACKGROUND: The relationship between healthcare system inputs (e.g., human resources and infrastructure) and mortality has been extensively studied. However, the association between healthcare system inputs and patient-reported outcomes remains unclear. Hence, we explored the predictive value of human resources and infrastructures of the countries' healthcare system on patient-reported outcomes in adults with congenital heart disease. METHODS: This cross-sectional study included 3588 patients with congenital heart disease (median age = 31y; IQR = 16.0; 52% women; 26% simple, 49% moderate, and 25% complex defects) from 15 countries. The following patient-reported outcomes were measured: perceived physical and mental health, psychological distress, health behaviors, and quality of life. The assessed inputs of the healthcare system were: (i) human resources (i.e., density of physicians and nurses, both per 1000 people) and (ii) infrastructure (i.e., density of hospital beds per 10,000 people). Univariable, multivariable, and sensitivity analyses using general linear mixed models were conducted, adjusting for patient-specific variables and unmeasured country differences. RESULTS: Sensitivity analyses showed that higher density of physicians was significantly associated with better self-reported physical and mental health, less psychological distress, and better quality of life. A greater number of nurses was significantly associated with better self-reported physical health, less psychological distress, and less risky health behavior. No associations between a higher density of hospital beds and patient-reported outcomes were observed. CONCLUSIONS: This explorative study suggests that density of human resources for health, measured on country level, are associated with patient-reported outcomes in adults with congenital heart disease. More research needs to be conducted before firm conclusions about the relationships observed can be drawn. TRIAL REGISTRATION: ClinicalTrials.gov: NCT02150603. Registered 30 May 2014.

Knowledge of Life-Long Cardiac Care by Adolescents and Young Adults with Congenital Heart Disease.

National management guidelines recommend that patients with moderate and complex congenital heart disease (CHD) receive life-long cardiac care (LLCC), guided in adulthood by an adult congenital heart disease (ACHD) specialist. However, the percentage of adult CHD patients who receive such care is quite low. Inadequate knowledge regarding LLCC may contribute to care interruption. We, therefore, sought to determine the knowledge of adolescents and young adults regarding LLCC. In this multi-center study, we administered a survey to patients 13-20 years of age with surgically repaired congenital heart disease. We assessed the understanding of both their need for LLCC and awareness of the type of recommended care providers. A total of 290/302 (96%) patients approached in the outpatient clinic setting (10 centers) agreed to study participation; mean age was 16.3 ± 2.3 years; patients were 62% male. While the need for LLCC was recognized by 78% of subjects, only 37% understood that an ACHD specialist in adulthood should guide this care. Only 37% of respondents stated that their current cardiology team had spoken to them about LLCC, but 90% wished to learn more. A substantial number of adolescents and young adults with moderate and complex CHD lack adequate knowledge about LLCC, but most have a desire to learn more about the type of care they will require in adulthood. Transition education/assessment ensuring successful transfer to adult-oriented care for this population should emphasize the importance of LLCC.

Regional variation in quality of life in patients with a Fontan circulation: A multinational perspective.

BACKGROUND: Impaired quality of life (QOL) is associated with congenital heart disease (CHD) and country of residence; however, few studies have compared QOL in patients with differing complexities of CHD across regional populations. The current study examined regional variation in QOL outcomes in a large multinational sample of patients with a Fontan relative to patients with atrial septal defects (ASDs) and ventricular septal defects (VSDs). METHODS: From the Assessment of Patterns of Patient-Reported Outcomes in Adults with Congenital Heart disease-International Study (APPROACH-IS), 405 patients (163 Fontan and 242 ASD/VSD) across Asia, Europe, and North America provided consent for access to their medical records and completed a survey evaluating QOL (0 to 100 linear analog scale). Primary CHD diagnosis, disease complexity, surgical history, and documented history of mood and anxiety disorders were recorded. Differences in QOL, medical complications, and mood and anxiety disorders between Fontan and ASD/VSD patients, and across geographic regions, were examined using analysis of covariance. Hierarchical regression analyses were conducted to identify variables associated with the QOL ratings. RESULTS: Patients with a Fontan reported significantly lower QOL, and greater medical complications and mood and anxiety disorders relative to patients with ASD/VSD. Inpatient cardiac admissions, mood disorders, and anxiety disorders were associated with lower QOL among patients with a Fontan, and mood disorders were associated with lower QOL among patients with ASD/VSD. Regional differences for QOL were not observed in patients with a Fontan; however, significant differences were identified in patients with ASD/VSD. CONCLUSIONS: Regional variation of QOL is commonplace in adults with CHD; however, it appears affected by greater disease burden. Among patients with a Fontan, regional variation of QOL is lost. Specific attempts to screen for QOL and mood and anxiety disorders among CHD patients may improve the care of patients with the greatest disease burden.

Transition and transfer of childhood cancer survivors to adult care: A national survey of pediatric oncologists.

BACKGROUND: Pediatric oncologists are responsible for ensuring that adolescent and young adult (AYA) childhood cancer survivors have the knowledge and skills necessary to manage their follow-up care in adult healthcare systems. PROCEDURES: To describe transition practices and barriers to transfer, we electronically surveyed U.S. Children's Oncology Group members: 507/1449 responded (35%) and 347/507 (68%) met eligibility criteria. RESULTS: Of 347 respondents, 50% are male, median years in practice 10 (range 5-22), 37% practice in freestanding children's hospitals. Almost all care for survivors up to age 21 years (96%), 42% care for survivors over age 25 years, and only 16% over age 30 years. While 66% of oncologists reported providing transition education to their patients, very few (8%) reported using standardized transition assessments. The most frequent barriers to transfer were perceived attachment to provider (91%), lack of adult providers with cancer survivor expertise (86%), patient's cognitive delay (81%), or unstable social situation (80%). Oncologists who continue to care for patients older than 25 years are more likely to perceive parents' attachment to provider (P = 0.037) and patients' social situation as barriers to transfer (P = 0.044).  Four themes emerged from a content analysis of 75 respondents to the open-ended question inviting comments on transition/transfer practices: desire for flexible transfer criteria; providers as barriers; provider lack of transition knowledge, skills, and resources; and desire for collaboration. CONCLUSIONS: Although most pediatric oncologists reported transferring AYA cancer survivors to adult care and providing some transition education, they endorse deficits in transition skills, emotional readiness, and institutional resources.

Transitions in Pediatric Gastroenterology: Results of a National Provider Survey.

OBJECTIVES: Transition and transfer to adult-oriented health care is an important yet challenging task for adolescents and young adults with chronic medical conditions. Transition practices vary widely, but a paucity of data makes determination of best practices difficult. We described North American pediatric gastroenterologists' preferences and present transition practice patterns and explored whether experience affected providers' perspectives. METHODS: An online survey was distributed via e-mail to members of the North American Society of Pediatric Gastroenterology, Hepatology and Nutrition. Participation was voluntary and answers were anonymous. Quantitative and qualitative analysis was performed. RESULTS: Almost three quarters of the 175 respondents describe providing transition or self-care management education, but only 23% use structured readiness assessments. Most respondents (88%) report having age cutoffs above which they no longer accept new referrals, with the most common age being 18 years (57%). One third report the ability to provide age-appropriate care to patients older than 21 years. Only 6% indicate that their practice or institution should provide care for individuals older than 25 years. Many (63%) indicate that their practice or institution has a policy regarding age of transfer, but most (79%) are flexible. Provider preferences for triggers to transfer to adult care diverge widely between age, milestones, and comorbidities. Overall, parent (81%) and patient (74%) attachment to pediatric health care providers are cited as the most common barriers to transition. CONCLUSIONS: Preferences and practices surrounding transition preparation and transfer to adult care vary widely, reflecting continued uncertainty regarding optimal transition strategies.

Provision of Transition Education and Referral Patterns from Pediatric Cardiology to Adult Cardiac Care.

ACC/AHA guidelines recommend a structured preparation for and transfer to adult-oriented cardiac care for adult survivors of pediatric onset heart disease (POHD). Given this, we sought to describe the transition and transfer practices for a cohort of young adults with POHD and to determine factors associated with successful transfer to adult-oriented cardiac care. We performed a single-center, retrospective chart review on patients ≥18 years of age, with POHD likely to require lifelong cardiac care, who were seen in outpatient pediatric cardiology (PC) between 2008 and 2011. Successful transfer was defined as the subsequent attendance at adult cardiology (AC) within 2 years of PC visit. We identified 118 patients who met study criteria. Mean age 22.4 ± 2.0 years, 59 % male, 64 % white and 40 % Hispanic. Mean transition education topics noted was 3.3 ± 1.8 out of 20 and covered the underlying cardiac disease (89 %), follow-up and current medications (56 %) and exercise limitations (34 %). Recommendations for follow-up were AC (57 %) and PC (33 %). Of those told to transfer to AC, 79 % successfully transferred. Characteristics of successful transfer included: prior cardiac surgery (p = 0.008), cardiac medication use (p = 0.006) and frequency of follow-up ≤1 year (p = 0.037). One-quarter of all subjects did not follow-up within at least 2 years. Despite published guidelines, transition education appears lacking and the approach to transfer to adult cardiac care is not consistent. Given the increased risk of morbidity and mortality in this patient population, standardization of transition education and transfer processes appear warranted.

Patient-centered medical home for patients with complex congenital heart disease.

PURPOSE OF REVIEW: Originally conceived by pediatricians as a model for the care of children with special healthcare needs, the 'patient-centered medical home' (PCMH) has been identified by the Affordable Care Act as a model for all future outpatient care delivery. Although few studies have demonstrated its efficacy in improving care for children with congenital heart disease (CHD), access to a PCMH is likely to improve CHD-patient outcomes, including global function and quality of life, while reducing healthcare costs. RECENT FINDINGS: To date, most patients with complex CHD have their care anchored in cardiology subspecialty-care clinics, which lack many of the attributes of a PCMH. Given that many of these patients have noncardiac morbidities, including neurocognitive impairment and multiorgan dysfunction, we believe such patients will benefit from a PCMH model of care based on primary care. SUMMARY: The PCMH based on primary care for patients with complex CHD could result in improved clinical outcomes, improved patient satisfaction and quality of life as well as decreased healthcare costs. Policy and practice reform are required to increase CHD-patient access to a PCMH, based on primary-care settings.

Cardiovascular outcomes after the arterial switch operation for D-transposition of the great arteries.

BACKGROUND: Data regarding long-term outcomes after the arterial switch operation for D-transposition of the great arteries are scarce. METHODS AND RESULTS: A single-institution retrospective cohort study was conducted to assess cardiovascular outcomes after an arterial switch operation between 1983 and 1999. Patients without follow-up visits within 3 years were contacted and secondary sources of information obtained. Overall, 400 patients, 154 (38.3%) with a ventricular septal defect, 238 (59.5%) with an intact septum, and 9 (2.3%) with a Taussig-Bing anomaly, were followed for a median of 18.7 years. In perioperative survivors, overall and arrhythmia-free survival rates at 25 years were 96.7±1.8% and 96.6±0.1%, respectively. Late mortality was predominantly a result of sudden deaths and myocardial infarction. At 25 years, 75.5±2.5% remained free from surgical or catheter-based reintervention. Freedom from an adverse cardiovascular event was 92.9±1.9% at 25 years. Independent predictors were a single right coronary artery (hazard ratio, 4.58; 95% confidence interval, 1.32-15.90), P=0.0166) and postoperative heart failure (hazard ratio, 6.93; 95% confidence interval, 1.57-30.62; P=0.0107). At last follow-up, the left ventricular ejection fraction was 60.3±8.9%, 97.3% had class I symptoms, and 5.2% obstructive coronary artery disease. Peak oxygen uptake was 35.1±7.6 mL/kg/min (86.1±15.1% predicted), with a chronotropic index <80% in 34.2%. At least moderate neoaortic and pulmonary regurgitation were present in 3.4% and 6.6%, respectively, and more than mild neoaortic and pulmonary stenosis in 3.2% and 10.3%. CONCLUSIONS: Long-term and arrhythmia-free survival is excellent after arterial switch operation. Although sequelae include chronotropic incompetence and neoaortic, pulmonary, and coronary artery complications, most patients maintain normal systolic function and exercise capacity.

Aortic root dilatation in adults with surgically repaired tetralogy of fallot: a multicenter cross-sectional study.

BACKGROUND: Although aortic root pathology has been described in patients with tetralogy of Fallot, the scope of the problem remains poorly defined. We sought to determine the prevalence and predictors of aortic root dilatation in adults with repaired tetralogy of Fallot. METHODS AND RESULTS: A multicenter cross-sectional study was conducted with standardized reassessment of echocardiographic parameters in 474 adults (≥18 years) with surgically repaired tetralogy of Fallot or pulmonary atresia with ventricular septal defect. The aortic root was measured in a parasternal long-axis view, in diastole, at the level of the sinus of Valsalva. Prevalence and predictors of an absolute diameter ≥40 mm and of an observed-to-expected ratio >1.5 were assessed. The aortic root dimension was ≥40 mm in 28.9% (95% confidence interval, 26.9%-30.9%). In multivariate analyses, the only independently associated factor was male sex (odds ratio, 4.48; 95% confidence interval, 1.55-12.89; P=0.006). The prevalence of an observed-to-expected aortic root dimension ratio >1.5 was 6.6% (95% confidence interval, 5.3%-7.9%). It was associated with pulmonary atresia and moderate or severe aortic regurgitation in univariate analyses, but no independent predictor was identified. The side of the aortic arch was not associated with aortic root dimension. The prevalence of moderate or severe aortic regurgitation was 3.5% (95% confidence interval, 2.7%-4.2%). CONCLUSIONS: Although nearly one third of adults with repaired tetralogy of Fallot have an aortic root diameter ≥40 mm, the prevalence of a dilated aortic root, when defined by an indexed ratio of observed-to-expected values, is low. Similarly, moderate or severe aortic regurgitation is uncommon.

Provider awareness alone does not improve transition readiness skills in adolescent patients with inflammatory bowel disease.

OBJECTIVE: Adolescent patients with chronic health conditions must gradually assume responsibility for their health. Self-management skills are needed for a successful transfer from adolescent to adult health care, but the development of these skills could be resource intensive. Pediatric providers are already instrumental in teaching patients about their health and may improve these skills. The aim of the study was to evaluate whether informal education of pediatric providers regarding transition improves inflammatory bowel disease (IBD) patient self-management skills. METHODS: Consecutive patients with IBD older than 10 years who presented to the outpatient setting were administered a survey regarding self-management behaviors in 2008 and 2011. During this time, several conferences on transition were presented to the providers. RESULTS: In 2008, 294 patients completed the survey (82%) compared with 121 patients (89%) in 2011. The patient groups were comparable with respect to sex (boys 50% vs 42%), mean age (16.7 vs 16.2 years), and type of IBD (Crohn 68% vs 66%). The 13- to 15-year-olds reported calling in refills (11%, 8%, respectively), scheduling clinic appointment (0, 1%), preparing questions (13%, 5%), and taking the main role in talking during clinic visits (15%, 24%). The 16- to 18-year-olds reported calling in refills (13%, 27%), scheduling clinic appointments (9%, 6%), preparing questions (9%, 16%), and taking the main role in talking in clinic visits (36%, 45%). Responsibility for behaviors gradually increases with age, but did not differ significantly between 2008 and 2011. CONCLUSIONS: Increasing awareness around transition readiness for pediatric providers had an insignificant effect on the self-management skills of patients with IBD. A more formal or structured approach is likely required to improve transition skills in adolescent patients.

Heart failure caused by congenital left-sided lesions.

There are diverse mechanisms by which congenital left-sided cardiac lesions can precipitate heart failure. Left heart outflow obstruction can impose abnormal pressure load on the left ventricle, inducing adverse remodeling, hypertrophy, and diastolic and systolic dysfunction. Abnormalities in left ventricular inflow can increase pulmonary venous pressure and predisposing to pulmonary edema. In addition, inborn abnormalities in left ventricular myocardial structure and function can impair both systolic and diastolic function and manifest as heart failure later in life. In this article, the different mechanisms, outcomes, and treatments of heart failure in patients with congenital left-sided lesions are discussed.

Transition of pediatric patients to adult care: an analysis of provider perceptions across discipline and role.

The importance of successfully transitioning pediatric patients to adult care is increasingly recognized as more children with chronic diseases are living to adulthood. The aim of this study was to investigate the current state of provider perceptions across disciplines regarding transition of pediatric patients to adult care. Focus groups made up of providers of various roles and experience levels were conducted. A total of six major themes were identified. We conclude that pediatric providers share common concerns about transitioning pediatric patients to adult care. We reinforce many of the issues raised in the literature and also discuss a sense of professional ego that was identified as a barrier to successful transition, which is not widely reported in other studies.

[Summary: International consensus statement on nomenclature and classification of the congenital bicuspid aortic valve and its aortopathy, for clinical, surgical, interventional and research purposes]. / Resumen: Consenso internacional para la nomenclatura y clasificación de la válvula aórtica bicúspide congénita y su aortopatía, con fines clínicos, quirúrgicos, intervencionistas y de investigación.

This consensus of nomenclature and classification for congenital bicuspid aortic valve and its aortopathy is evidence-based and intended for universal use by physicians (both pediatricians and adults), echocardiographers, advanced cardiovascular imaging specialists, interventional cardiologists, cardiovascular surgeons, pathologists, geneticists, and researchers spanning these areas of clinical and basic research. In addition, as long as new key and reference research is available, this international consensus may be subject to change based on evidence-based data1.

Este consenso de nomenclatura y clasificación para la válvula aórtica bicúspide congénita y su aortopatía está basado en la evidencia y destinado a ser utilizado universalmente por médicos (tanto pediatras como de adultos), médicos ecocardiografistas, especialistas en imágenes avanzadas cardiovasculares, cardiólogos intervencionistas, cirujanos cardiovasculares, patólogos, genetistas e investigadores que abarcan estas áreas de investigación clínica y básica. Siempre y cuando se disponga de nueva investigación clave y de referencia, este consenso internacional puede estar sujeto a cambios de acuerdo con datos basados en la evidencia1.

Patient-reported outcomes in the aging population of adults with congenital heart disease: results from APPROACH-IS.

The congenital heart disease (CHD) population now comprises an increasing number of older persons in their 6th decade of life and beyond. We cross-sectionally evaluated patient-reported outcomes (PROs) in persons with CHD aged 60 years or older, and contrasted these with PROs of younger patients aged 40-59 years and 18-39 years. Adjusted for demographic and medical characteristics, patients ≥60 years had a lower Physical Component Summary, higher Mental Component Summary, and lower anxiety (Hospital Anxiety and Depression Scale-Anxiety) scores than patients in the two younger categories. For satisfaction with life, older persons had a higher score than patients aged 40-59 years. Registration: ClinicalTrials.gov NCT02150603.

Time in Therapeutic Range With Vitamin K Antagonists in Congenital Heart Disease: A Multicentre Study.

BACKGROUND: Vitamin K antagonists (VKAs) are frequently prescribed to patients with congenital heart disease (CHD) for atrial arrhythmias or Fontan palliation, but there is a paucity of data regarding time spent in the therapeutic range (TTR). We sought to determine the TTR in patients with CHD and atrial arrhythmias or Fontan palliation prescribed VKAs and explore associations with thromboembolic and bleeding events. METHODS: A multicentre North American cohort study was conducted on patients with CHD who received VKAs for sustained atrial arrhythmia or Fontan palliation. TTR was calculated using the Rosendaal linear interpolation method. Generalized estimating equations were used to explore factors associated with time outside the therapeutic range. RESULTS: A total of 567 patients, aged 33 ± 17 years, 56% female, received VKAs for 11.5 ± 8.4 years for atrial arrhythmias (63.0%) or Fontan palliation (58.0%). CHD was simple, moderate, and complex in 10.8%, 20.3%, and 69.0%, respectively. Site investigators perceived good control over international normalized ratio (INR) levels in most patients (75.3%), with no or minor compliance or adherence issues (85.6%). The mean TTR was 41.9% (95% confidence interval [CI], 39.0%-44.8%). Forty-seven (8.3%) and 34 (6.0%) patients had thromboembolic and bleeding events, respectively. Thromboembolic events were associated with a higher proportion of time below the therapeutic range (31.3% vs 19.1%, P = 0.003) and bleeding complications with a higher proportion of time above the therapeutic range (32.5% vs 19.5%, P = 0.006). CONCLUSIONS: Patients with CHD who receive VKAs spend < 42% of their time with INR levels in the therapeutic range, with repercussions regarding thromboembolic and bleeding complications.

Heart Failure and Patient-Reported Outcomes in Adults With Congenital Heart Disease from 15 Countries.

Background Heart failure (HF) is the leading cause of mortality and associated with significant morbidity in adults with congenital heart disease. We sought to assess the association between HF and patient-report outcomes in adults with congenital heart disease. Methods and Results As part of the APPROACH-IS (Assessment of Patterns of Patient-Reported Outcomes in Adults with Congenital Heart disease-International Study), we collected data on HF status and patient-reported outcomes in 3959 patients from 15 countries across 5 continents. Patient-report outcomes were: perceived health status (12-item Short Form Health Survey), quality of life (Linear Analogue Scale and Satisfaction with Life Scale), sense of coherence-13, psychological distress (Hospital Anxiety and Depression Scale), and illness perception (Brief Illness Perception Questionnaire). In this sample, 137 (3.5%) had HF at the time of investigation, 298 (7.5%) had a history of HF, and 3524 (89.0%) had no current or past episode of HF. Patients with current or past HF were older and had a higher prevalence of complex congenital heart disease, arrhythmias, implantable cardioverter-defibrillators, other clinical comorbidities, and mood disorders than those who never had HF. Patients with HF had worse physical functioning, mental functioning, quality of life, satisfaction with life, sense of coherence, depressive symptoms, and illness perception scores. Magnitudes of differences were large for physical functioning and illness perception and moderate for mental functioning, quality of life, and depressive symptoms. Conclusions HF in adults with congenital heart disease is associated with poorer patient-reported outcomes, with large effect sizes for physical functioning and illness perception. Registration URL: https://clinicaltrials.gov; Unique identifier: NCT02150603.

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.