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Background: Exercise capacity (EC) is a key prognostic indicator in adults with congenital heart disease (ACHD), closely associated with all-cause mortality. Thus, EC may serve a valuable tool for risk-stratification in ACHD. We propose a novel risk score incorporating EC, age, and lesion complexity to improve mortality risk prediction in ACHD.

Methods: This observational study utilized data from the Swedish National Register of Congenital Heart Disease. Adults aged 18 to 60 years who had completed at least one bicycle ergometer exercise test were included. EC was classified according to percentage of predicted capacity (% ECpred): normal (>70%), moderately impaired (50–70%), severely impaired (<50%). Patients were followed for ten years or until death. Odds ratios for mortality were used to derive a 5-point risk-score to estimate 10-year mortality, with the following point allocation: % ECpred 50–70 = 1 point, <50% = 2 points; age 25–40 years = 1 point, 40–60 years = 2 points; moderate/severe lesion complexity = 1 point. Internal validation of risk score resulted in a median AUC of 0.72 for training data, and 0.70 for test data.

Results: In total, 1,525 patients (mean age 31.1 ± 11.8 years; 44% women) were included, where 104 deaths occurred during follow-up. Risk scores ranged from 0 to 5, corresponding to 10-year mortality rates of 1%, 3%, 5%, 10%, 18%, and 34%, respectively.

Conclusion: A simple risk score incorporating exercise capacity, age, and congenital heart lesion complexity can effectively stratify ACHD patients to 10-year risk of all-cause mortality following an exercise test.

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: 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.

Introduction: In aortic valve disease, several interventions are available. In young people who are still growing, or considering the risks of long-term anticoagulation, the Ross procedure remains an alternative for aortic valve replacement. This procedure entails the transposition of the patient’s pulmonary valve to the aortic position, with placement of a homograft in the pulmonary position. However, long-term prognosis remains largely unknown.

Methods: The Swedish national registry of congenital heart disease was searched for adult patients with a history of Ross operation.

Results: 82 patients (mean age 40.4±15.8 years) were identified, with a mean age at the time of the Ross procedure of 23.6±14.7 years. After a mean follow-up of 16.8±5.5 years, 24.4% of patients underwent a first reoperation involving either the neoaortic valve or the pulmonary homograft, at a mean age of 32.0±13.9 years. The cumulative incidence of reoperation was approximately 15% at 10 years and 30% at 20 years post-Ross procedure. Among the 20 reinterventions, 17 (85.0%) involved the pulmonary valve and 8 the neoaortic valve; five patients underwent procedures on both valves. Two patients (2.3%) died during follow-up.

Forty-eight patients in the cohort had undergone primary Ross surgery. This subgroup was older at the time of data extraction (mean age 46.7±15.7 years) compared with those who underwent secondary Ross surgery (mean age 31.3±10.7 years), that is, typically following previous interventions. The secondary Ross group demonstrated better left ventricular function, with ejection fraction >50% in 91.7% of cases, compared with 69.8% in the primary group (p=0.041).

Conclusion: One in four patients undergoing the Ross procedure requires a reintervention, commonly involving the pulmonary valve. Long-term mortality was low. In selected patients, the Ross procedure remains a viable option; however, late morbidity must be considered. Our findings suggest that secondary Ross surgery is associated with better long-term outcomes, particularly regarding left ventricular function, although the underlying mechanisms remain unclear.

Introduction: Pharmacological therapy has transformed the management of heart failure, yet pivotal trials have primarily focused on acquired left ventricular disease. Evidence for renin-angiotensin-aldosterone system (RAAS) inhibition in systemic right ventricle (sRV) remains scarce, and current practice is largely based on extrapolation from left ventricular studies and expert consensus. This meta-analysis evaluates the efficacy and safety of RAAS blockade in patients with a failing sRV.

Methods: A PubMed search identified studies investigating RAAS inhibition in sRV failure. Reported outcomes included changes in NT-pro-BNP, right ventricular ejection fraction (RVEF), and peak oxygen uptake (VO2-max). Data were analyzed using conventional statistics and visualized with forest plots.

Results: Ten eligible studies were included, comprising a pooled cohort of 325 patients, of which 261 received active treatment. The population was predominantly male (67.1%), with mean cohort ages ranging from 25.2 to 48.5 years. Mean treatment durations spanned 3.5 to 37.2 months. RAAS inhibition was associated with a reduction in NT-pro-BNP (Cohen's d = –0.28, 95% CI –0.49 to –0.07, p = 0.01). However, no improvements in RVEF or VO2-max were observed. The therapy was generally well-tolerated. The overall mortality incidence was 5.6 deaths per 1000 person-years in the active treatment groups.

Conclusion: The evidence for RAAS inhibition in patients with sRV is limited but suggests a beneficial reduction in NT-pro-BNP, indicating a hemodynamic effect but without improvements in RVEF or VO2-max. When combined with extrapolated data from large left ventricular heart failure trials, these findings support that RAAS inhibition is safe and may be considered in patients with sRV failure.

INTRODUCTION: Survival and healthcare for patients with congenital heart disease have improved, and the number of pregnancies among women of childbearing age with congenital heart disease has increased. Our aim was to investigate obstetric outcomes in a large retrospective, national registry study of women with congenital heart disease compared to controls.

MATERIAL AND METHODS: The study included women over 18 years of age from the Swedish Registry of Congenital Heart Disease. Each case was matched with 10 controls from Statistics Sweden, based on the mother's birth year and birth county and all were subsequently linked to the Swedish Medical Birth Register. We included 7998 pregnancies in women with congenital heart disease and 84 799 in controls during 1973-2020.

RESULTS: The mean age at delivery for women with congenital heart disease and controls was 28.7 (±5.0) and 28.7 (±5.1) years, respectively. Women with congenital heart disease smoked less, had a shorter gestation and a higher incidence of delivery by Cesarean section compared to controls. The likelihood of Cesarean section was increased in women with congenital heart disease compared to controls: odds ratio 1.45 (95% confidence interval (CI) 1.37-1.54). Compared to controls, women with congenital heart disease had an increased likelihood of giving birth to small-for-gestational-age neonates: odds ratio 1.40 (95% CI 1.23-1.58). The association regarding small-for-gestational-age remained after adjusting for body mass index, age, smoking, comorbid diseases and preeclampsia. Women with congenital heart disease had an increased likelihood of prematurity compared to controls: odds ratio 1.47 (95% CI 1.35-1.59). The likelihood of Cesarean section, small-for-gestational-age neonates and prematurity was higher in women with severe congenital heart disease than mild/moderate congenital heart disease, both compared to controls.

CONCLUSIONS: In this large national case-control study in women with congenital heart disease, we showed an increased likelihood of giving birth prematurely by Cesarean section, and having a small-for-gestational-age neonate compared to matched controls. The likelihood seems even higher in women with severe congenital heart disease. Further research is needed to explore the underlying reasons for the high rates of Cesarean section in women with congenital heart disease.

Background and Aims: The life expectancy of adult patients with congenital heart disease (ACHD) has improved, thus shifting the research focus towards age-related comorbidities to continue to improve patient outcomes. This study aimed to investigate all-cause mortality and recurrent acute myocardial infarction (AMI) in adults with and without congenital heart disease.

Methods: A nationwide case control study was conducted between 2000 and 2022. Patients with ACHD (n = 214) and controls (n = 275 377) who experienced their first AMI were identified. Of these, each patient (n = 213) with ACHD was matched with 10 controls (n = 2092) based on age, sex, hypertension, diabetes, hyperlipidaemia, and history of percutaneous coronary intervention or coronary artery bypass grafting. Mortality and recurrent AMI were assessed using unadjusted and adjusted Cox regression for matching and other clinical covariates.

Results: Patients with ACHD were younger (58 ± 14 years) than controls (70 ± 12 years) before the matching (P < .001). The mean follow-up time was 6.5 and 7.3 years for patients with ACHD and controls, respectively. There was no significant difference in mortality or recurrent AMI rates at 1 year between patients with ACHD and matched controls. The mortality rate was higher in ACHD at 10 years of follow-up (hazard ratio 1.4, 95% confidence interval 1.0–1.9) but did not remain after adjustment.

Conclusions: This study suggests that survival rates and the incidence of recurrent AMI in ACHD patients are similar to those of controls. Since patients with ACHD share similar cardiovascular risk factors as the general population, promoting healthy lifestyles and proactive risk management is crucial to mitigate acquired heart disease.

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: Sexual health and functioning are significant, yet often overlooked, components of psychosocial well-being of adults with congenital heart disease (ACHD). Prior research reports inconsistent findings, limited data on older adults with CHD, and few studies have compared patients with controls from the general population. This study investigated sexual functioning in middle-aged and elderly ACHD patients and compared it with controls.

Methods: This was a Swedish sub-study of the international APPROACH-IS II project . Patients 40 years or older with moderate or severe CHD were recruited along with controls from the general Swedish population. The design was case control. Erectile dysfunction (ED) in males was assessed using the International Index of Erectile Function. Sexual dysfunction (SD) in females was assessed using the Female Sexual Function Index.

Results: Overall, 146 individuals were included in the study; 90 patients with CHD and 56 controls. Among males, the prevalence of ED was higher in patients (n=57) than in controls (n=37), (21% vs. 2.7%, p=0.01). ED was associated with increasing age (OR=1.11; 95% CI 1.03-1.20). Among females, no difference in the prevalence of SD was observed between patients (n= 33) and controls (n=19) (27% vs. 30%, p=0.76)

Conclusion: Sexual dysfunction was more common in male patients than controls, whereas no differences were observed between female patients and controls. Given that approximately one in five males and one in four females with CHD reported sexual dysfunction, increased empirical and clinical attention is warranted.

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.