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Background: Adults with congenital heart disease (CHD) are at high risk of premature death, making advance care planning (ACP) crucial for aligning care with individual values and goals. Previous ACP research has focused primarily on the United States and Canada, highlighting the need for a global perspective. We aimed to describe the ACP practices, needs and preferences of adults with CHD around the globe and to investigate associations with patient-related factors.

Methods: This cross-sectional study, part of the APPROACH-IS II project, assessed ACP preferences, needs and practices using patient-reported surveys. Overall, 8,281 patients with CHD (median age 32 years; 54% women; 15% mild, 58% moderate, 27% complex CHD) from 53 centers in 32 countries, spanning 6 continents, were included.

Results: Over half (55%) of participants reported speaking to their physician about how their health might be in the future and 9% had preferences being documented in a plan. According to 66% of patients, the best time to initiate ACP is early in the disease trajectory. Most patients indicated being relatively comfortable talking to their physician about their future health and about death. ACP varied widely across different countries, with United States and Canada top of the class for most variables.

Conclusions: When looking at global ACP practices, needs and preferences, much room for improvement of ACP provision could be noticed. Also, a notable variation in ACP was observed worldwide.

Background: Closure of a patent foramen ovale (PFO) is frequently recommended in patients with cryptogenic stroke. Long-term outcomes in real-world settings remain unknown.

Objectives: This study analyzed standardized mortality ratio (SMR), subsequent stroke, and associated risk factors after PFO closure.

Methods: National registers on congenital heart disease and stroke were cross-linked to identify individuals who underwent PFO closure between 2001 and 2018. The ratio of observed to expected deaths was calculated (SMR). Data were analyzed using survival analysis and Cox regression.

Results; A total of 827 patients (60.5% males, median age 47.9 years, IQR: 40.0-55.6 at the time of PFO closure) were included and observed for a median duration of 8.0 years (IQR: 4.8-11.0). During follow-up, 23 patients died, SMR was 0.65 (95% CI: 0.41-0.98). A total of 34 ischemic strokes occurred, yielding an incidence rate of 0.51 events per 100 patient-years. Among the 34 patients who experienced a subsequent stroke, 27 were receiving antithrombotic therapy at the time of the event. New-onset atrial fibrillation following PFO closure was associated with an increased risk of subsequent ischemic stroke (HR: 8.2; 95% CI: 2.6-25.8), as was active/previous smoking (HR: 2.5; 95% CI: 1.2-5.1).

Conclusions: Patients undergoing PFO closure demonstrated a lower all-cause mortality compared to the general population. The observed rate of subsequent ischemic stroke was consistent with findings from previous randomized controlled trials. New-onset atrial fibrillation following PFO closure and active/previous smoking emerged as modifiable risk factors.

Background: Fatigue is a symptom that has been described among adult patients with congenital heart disease (CHD), but the prevalence and impact of fatigue on the patient's daily life has been poorly studied. This study (i) examines the prevalence of fatigue in patients aged over 40 years with moderately complex or complex congenital heart disease compared to controls and (ii) explores the relationship between fatigue, heart disease complexity, clinical characteristics and self-reported New York Heart Association Functional Class (self-reported NYHA class).

Methods: The Multidimensional Fatigue Inventory (MFI-20) was applied in 166 patients with moderately complex CHD or complex CHD (44 % females, median age 55.3 years, IQR 47.6–64.8) along with 89 controls (43 % female, median age 54.0, IQR 46.0–65.9). MFI-20 measured general fatigue, physical fatigue, mental fatigue, reduced motivation and reduced activity.

Results: Physical fatigue (severe to very severe) was more common in complex CHD compared with moderately complex CHD (25 % vs. 52 %, p = 0.006). Complex CHD was associated with severe to very severe physical fatigue (odds ratio 3.1 (95 % CI 1.1–9.1). Patients with complex CHD had higher levels of self-reported NYHA class than patients with moderately complex CHD. All dimensions of fatigue were positively associated with self-reported NYHA class.

Conclusions: Patients over the age of 40 with complex CHD were three times more likely to report severe to very severe physical fatigue and reported higher levels of self-reported NYHA class than patients with moderately complex CHD. This highlights the importance of considering CHD complexity in clinical practice.

Background: Decades of progress in care and treatment for congenital heart disease (CHD) has gradually shifted the research focus from initial survival to long-term prognosis and the ageing of adults with CHD. Knowledge about the ageing adult with CHD will guide interventions to safeguard the quality of life across the life course. The present study compares the prevalence of frailty and cognitive dysfunction between adults with CHD and a control group.

Methods: Using a multicentre design, we compared adults with moderate or complex CHD aged ≥40 years, equally distributed across the age groups 40-49, 50-59, and >60 years, with age- and sex-matched controls. We assessed frailty phenotypes using the Fried method and cognitive dysfunction using the Montreal Cognitive Assessment tool.

Results: In total, 156 adults with CHD (56.0 ± 10.4 years, 54.4% male) and 86 controls (55.6 ± 11.2 years, 55.8% male) were included in the study. Adults with CHD and controls did not differ in terms of mean score on the Montreal Cognitive Assessment (mean score 27.1 vs 26.9, P = 0.59). Similarly, there was no statistical difference in the prevalence of prefrailty/frailty between adults with CHD and controls (36.5% vs 29.0%, P = 0.26).

Conclusions: Prevalence rates of cognitive dysfunction and frailty were similar between adults with CHD and age-matched controls. As more patients, particularly those with complex heart lesions, reach older ages, the prevalence of cognitive impairment and frailty may change.

Contexte: Les progrès réalisés au fil des décennies dans les soins et le traitement des cardiopathies congénitales ont fait en sorte que la recherche, d'abord centrée sur la survie à court terme, s'est graduellement orientée vers l'étude du pronostic à long terme et du vieillissement des adultes atteints de cardiopathies congénitales. Les connaissances sur l'adulte vieillissant atteint de cardiopathie congénitale guideront les interventions visant à préserver la qualité de vie tout au long de la vie. La présente étude compare la prévalence de la fragilité et du dysfonctionnement cognitif entre des adultes atteints de cardiopathie congénitale et un groupe témoin.

Méthodologie: Au moyen d'une étude multicentrique, nous avons comparé des adultes atteints d'une cardiopathie congénitale modérée ou complexe, âgés de 40 ans ou plus, répartis également dans les groupes d'âge de 40 à 49 ans, de 50 à 59 ans et de plus de 60 ans, à des témoins appariés selon l'âge et le sexe. Nous avons évalué les phénotypes de fragilité à l'aide de la méthode Fried et les dysfonctionnements cognitifs à l'aide du MoCA (Montreal Cognitive Assessment).

Résultats: Au total, 156 adultes atteints de cardiopathie congénitale (56,0 ± 10,4 ans, dont 54,4 % étaient des hommes) et 86 témoins (55,6 ± 11,2 ans, dont 55,8 % étaient des hommes) ont été inclus dans l'étude. Les adultes atteints de cardiopathie congénitale et les témoins n'étaient pas différents pour ce qui est du score moyen au MoCA (score moyen de 27,1 vs 26,9, p = 0,59). De même, il n'y avait pas de différence statistique dans la prévalence de la préfragilité/fragilité entre les adultes atteints de cardiopathie congénitale et les témoins (36,5 % vs 29,0 %, p = 0,26).

Conclusions: Les taux de prévalence de dysfonction cognitive et de fragilité étaient similaires chez les adultes atteints de cardiopathie congénitale et les témoins de même âge. À mesure qu'augmentera le nombre de patients atteignant un âge plus avancé, en particulier ceux qui présentent des lésions cardiaques complexes, la prévalence des troubles cognitifs et de la fragilité pourrait changer.

Background: The global prevalence of congenital heart disease (CHD) is increasing. Research on patient-reported outcomes (PROs) predominantly originates from high-income countries, resulting in an incomplete understanding of the true global burden of CHD from the patient perspective. Therefore, we described perceived health, psychological distress and quality of life (QoL) in a large sample of adults with CHD from the globe and explored the relationship between PROs and the income level of the countries.

Methods: Assessment of Patterns of Patient-Reported Outcomes in Adults with Congenital Heart Disease-International Study II (APPROACH-IS II) represents an international cross-sectional investigation of PROs in 8415 patients from 53 centres across 32 countries. Patients completed questionnaires to measure perceived health status (RAND-12 Health Survey; EuroQOL-5D Visual Analog Scale); depressive symptoms (Patient Health Questionnaire-8, PHQ-8); anxiety (Generalized Anxiety Disorder Scale-7) and QoL (Linear Analog Scale). Gross National Income per capita in US dollars was used for stratifying countries according to income levels.

Results: Large intercountry disparities in PROs were observed. Switzerland demonstrated the highest mean scores for physical functioning, self-rated health and QoL, while Senegal had the lowest scores. Patients from Malta demonstrated the highest mean scores for mental health, and Senegal had the lowest scores. With regard to depressive symptoms and anxiety, Pakistan had the lowest mean scores, while Turkey had the highest scores. Patients from high-income nations reported significantly better physical functioning, mental functioning and QoL.

Conclusion: Large intercountry disparities in PROs were observed. APPROACH-IS II is a pioneering international endeavour that comprehensively evaluated PROs among adults with CHD, drawing participants from nations with different income levels.

Background: Impaired muscle function, aerobic capacity, and fatigue are common in individuals with Fontan circulation. Knowledge regarding the effects of strength training in this population is limited. Therefore, the study aimed to investigate the effects of strength training on dynamic muscle function, aerobic capacity, and fatigue in adults with Fontan circulation compared to matched controls.

Methods: In this pilot non-randomised controlled trial, nine patients with Fontan circulation (median age 28.9 years [IQR: 23.4-35.0], 44.4% women) and nine age- and sex-matched controls completed a 10-week strength training intervention. Dynamic muscle function was assessed through shoulder flexion, heel rise, elbow flexion, and knee extension tests. Aerobic capacity was evaluated using cardiopulmonary exercise testing, and fatigue using the questionnaire Multidimensional Fatigue Inventory. All assessments were conducted pre- and post-intervention. Within-group changes were analysed using the Wilcoxon signed rank test and between-group differences using the Mann-Whitney U test.

Results: Patients showed improvements in all muscle function tests post-intervention (shoulder flexions 39.3% [IQR: 18.9-69.7], p = 0.008; heel rise 26.7% [IQR:17.5-58.1], p = 0.008; elbow flexions 57.1% [IQR: 50.0-173.8], p = 0.007; knee extensions 66.7% [24.3-92.9], p = 0.008). The improvements were at comparable levels to controls. Only controls reported reduced fatigue (-19.4% [IQR: -28.7, -10.5], p = 0.01), while patients showed no change (-5.9% [IQR: -25.5, 3.2], p = 0.1). Aerobic capacity remained unchanged. No severe adverse events occurred.

Conclusion: Strength training is safe and improves dynamic muscle function in patients with Fontan circulation, with changes comparable to those of healthy controls. However, the effect of strength training on fatigue and aerobic capacity requires further investigation. ClinicalTrials.gov, ID: NCT05454254, https://clinicaltrials.gov.

Background: Advances in medical and surgical care have improved survival in patients with transposition of the great arteries (TGA), shifting focus towards quality of life (QoL). This study evaluates QoL in adults with TGA, including congenitally corrected TGA (ccTGA) and patients with dextro-TGA (d-TGA), by comparing patients with a systemic right ventricle (sRV) and systemic left ventricle (sLV), while identifying mediating factors.

Methods: This cross-sectional study, part of the APPROACH-IS II study, included 798 adults with TGA from 42 centers worldwide. QoL was assessed using a linear analogue scale (0–100). Regression models identified variables associated with QoL, and mediation analysis examined the effect of sRV on QoL.

Results: Among participants (median age 34 years, 44.9% women), 504 patients (63.2%) had an sRV (ccTGA or d-TGA with atrial switch), while 294 patients (36.8%) had an sLV (ccTGA with double switch operation or Rastelli and d-TGA with arterial switch or Rastelli). Patients with an sRV reported lower QoL (75 [IQR: 60-85]) compared to those with an sLV (80 [IQR: 70-90], p <0.001). The negative effect of sRV on QoL was mediated for 59% by ventricular dysfunction (B=-2.37, 95%CI: [-3.38- -1.21], p <0.001). Poorer QoL was independently associated with Asian race, employment status (job-seeking, unemployed, or disabled), less social support, New York heart association functional class ≥II, ventricular dysfunction, a greater number of interventional catheterizations and depression/anxiety.

Conclusions: TGA patients with an sRV experience lower QoL than those with an sLV, mediated mainly by ventricular dysfunction.Keywords

Background: In congenital aortic stenosis (CAS), commissurotomy is an option in patients not suitable to receive a valve prosthesis. However, there is often a need for future additional interventions on the aortic valve. The fate of the aortic valve is, however, essentially unknown. This study reports the need for reinterventions after surgical commissurotomy, based on a national register.

Materials and methods: The national register on congenital heart diseases (CHDs) was searched for patients with CAS, simple or associated with other CHD and an index commissurotomy with later data from follow-up.

Results: 300 patients with CAS and an index commissurotomy (mean age at the operation 7.4±7.8 years, 72.4% males) were identified. After an observation time of 27.4±10.0 years, 54.7% of the patients had a reintervention that occurred 14.2±10.1 years after the index operation. The cumulative incidence of reintervention was approximately 25% 10 years after and 60% 30 years after the index intervention. The prevalence of left ventricular hypertrophy (LVH) was higher among those that needed reintervention (41.3 vs 26.8%, p=0.023). Furthermore, eight patients died with a cumulative incidence of 7% 30 years after the index intervention, where most were (7/8) without reintervention (p=0.025). There were no additional important differences between patients with and without reintervention. The prevalence of left ventricular dysfunction and New York Heart Association class >1 was low.

Conclusion: Most (54.7%) patients with a commissurotomy, more than half of them within 30 years and eventually all will need a reintervention. This is important information to patients, especially for women in childbearing age. The higher prevalence of LVH in the group with reintervention needs attention during follow-up. Furthermore, those without reintervention, for unknown reasons, had a higher mortality. Our data strengthen the arguments for close outpatient follow-up among patients with a previous commissurotomy.

Aims: The prevalence of adults with congenital heart disease (ACHD) is rising due to improved paediatric care. In parallel, updated data on prognosis in adult life are needed.

Objectives: The aim was to calculate the standardized mortality ratio (SMR) and death rates in ACHD compared to the general population.

Methods and results: Data were obtained from the national register of congenital heart disease. The general Swedish population served as a reference. SMR was calculated as the ratio between observed and expected deaths. 9089 patients (median age 28 years, interquartile range [IQR] 20-45, 47% females) were followed for a median of 8 years (IQR 4-14). 525 deaths occurred during observation. The SMR increased by lesion complexity: atrial septal defect [1.3 (95% CI: 1.1-1.5)]; ventricular septal defect [2.0 (1.4-2.7)]; congenital aortic valve disease [2.2 (1.6-2,9)]; Ebstein's anomaly [3.2 (1.8-5.2)]; tetralogy of Fallot [3.8 (2.6-5.2)]; congenitally corrected transposition of the great arteries [5.6 (2.9-9.6)]; Eisenmenger syndrome [8.7 (5.5-13.1)]; transposition of the great arteries with a previous atrial redirection operation [12.3 (6.8-20.1)]; and Fontan physiology [22.5 (12.5-37.0)]. Calculations were also performed by severity (mild, moderate, and severe) and age by six age groups. SMR was generally higher in younger age, and the difference in mortality from the general population was estimated to be lower for older age groups. The mortality distribution and death rate per 1000 person-years have also been calculated for each lesion.

Conclusion: The mortality in ACHD remains increased compared to the general population and reflects the severity of the lesion. In higher ages, the observed mortality is more in line with the general population, probably because of survival of the least affected patients, and that few persons with severe lesions have reached advanced age.