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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.

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: 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: 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.

BACKGROUND: When more patients with congenital heart disease reach adult age, increased incidence of long-term complications, including ischemic stroke, are expected. The aim of this study was to analyze cumulative incidence of ischemic stroke, associated factors, and case fatality in adult congenital heart disease.

METHODS AND RESULTS: The study is based on Swedish national registers on congenital heart disease and stroke. Patients with congenital heart disease were followed between 2001 and 2018 for first-ever ischemic stroke events (ischemic stroke due to patent foramen ovale excluded). Factors possibly associated with ischemic stroke were analyzed using Cox regression models. Out of 8914 adult patients with congenital heart disease, 108 suffered ischemic stroke over a mean period of 7.6±4.7 years. The mean age at ischemic stroke was 53.8 years, and the cumulative incidence was 0.15% at 1 year, 0.5% at 5 years, and 1.5% at 10 years. In multivariable analysis, age (hazard ratio [HR], 1.04 [95% CI, 1.03-1.06]), diabetes (HR, 2.9 [95% CI, 1.3-6.4]), ejection fraction <50% (HR, 1.9 [95% CI, 1.1-3.4]), atrial septal defect (HR, 3.0 [95% CI, 1.03-8.5]), and aortic valve lesions (HR, 4.8 [95% CI, 1.6-14.1]) were associated with increased risk. Among those with ischemic stroke, approximately half were on antithrombotic treatment (anticoagulants or antiplatelets) before admission. Case fatality was 6.5%.

CONCLUSIONS: The cumulative incidence of ischemic stroke was 1.5% after 10 years. In adult congenital heart disease, the type of heart lesion, diabetes, ejection fraction <50%, and age were important factors associated with ischemic stroke. Despite a relatively young age, mortality is a significant threat. At time of the ischemic stroke event, approximately half of the patients were on antithrombotic treatment.

Background: An association between impaired exercise capacity and risk of mortality has been reported among adults with congenital heart disease (CHD). Over the years, treatment methods have improved and may influence outcome. Hence, we report data from a national cohort reflecting a contemporary population.

Objectives: The purpose of this study was to investigate the association between exercise capacity (workload) and mortality in a large registry-based cohort.

Methods: Data on exercise capacity using cycle ergometer were retrieved from the national registry of CHD. The association between predicted exercise capacity (%ECpred) and mortality was analyzed using Cox regression.

Results: In total, 3,721 adults (>18 years, 44.6% women) with CHD were included. The median age was 27.0 years (IQR: 20.8-41.0 years) and mean %ECpred was 77% ± 20%. Over a mean follow-up of 9.4 ± 6.0 years, there were 214 (5.8%) deaths. The Multivariable Cox regression model showed that moderately and severely impaired exercise capacity (50-<70 %ECpred: HR: 2.1, 95% CI: 1.4-3.2, P < 0.001, and <50 %ECpred: HR: 3.5, 95% CI: 2.1-6.0, P < 0.001) and CHD complexity were associated with higher mortality (moderate complexity: HR: 1.9 95% CI: 1.2-3.0, P = 0.003, great complexity: HR: 2.3 95% CI: 1.3-4.2, P = 0.008) when adjusted for New York Heart Association class, physical activity, cardiovascular medication, sex, impaired systemic ventricular function, and age.

Conclusions: Impaired exercise capacity and CHD complexity are independently associated with all-cause mortality in patients with CHD. Exercise capacity is an easily accessible variable that may be a useful tool for risk assessment in adult patients with CHD, but this needs confirmation in prospective studies.

Background: Coarctation of the aorta coexists with other cardiac anomalies and has long-term complications, including recoarctation, which may require intervention after the primary coarctation repair. This study aims to clarify the prevalence of and risk factors for interventions related to the coarctation complex as well as late mortality in a large contemporary patient population.

Methods and Results: The Swedish National Register of Congenital Heart Disease was used, which comprised 683 adults with repaired coarctation of the aorta. Analysis was performed on freedom from intervention thereafter at the coarctation site, aortic valve, left ventricular outflow tract, or ascending aorta. One hundred ninety-six (29%) patients had at least 1 of these interventions. Estimated freedom from either of these interventions was 60% after 50 years. The risk of undergoing such an intervention was higher among men (hazard ratio, 1.6 [95% CI, 1.2-2.2]). Estimated freedom from another intervention at the coarctation site was 75% after 50 years. In women, there was an increase in interventions at the coarctation site after 45 years. Patients who underwent one of the previously mentioned interventions after the primary coarctation repair had poorer left ventricular function. Eighteen patients (3%) died during follow-up in the register. The standardized mortality ratio was 2.9 (95% CI, 1.7-4.3).

Conclusions: Interventions are common after coarctation repair. The risk for and time of interventions are affected by sex. Our results have implications for planning follow-up and giving appropriate medical advice to the growing population of adults with repaired coarctation of the aorta.

Background: On a group level, patients with repaired tetralogy of Fallot have impaired exercise capacity. Since exercise capacity is related to prognosis, and more patients survive into higher age, it is important to know the expected exercise capacity at different ages. Furthermore, factors associated with exercise capacity and the relation between exercise capacity and mortality need further evaluation.

Methods: The national register of congenital heart disease was searched for exercise tests in adults with repaired tetralogy of Fallot. The results from exercise tests were compared with national reference data.

Results: 314 patients were identified (median age 33.2 [IQR 24.8-44.7], 40.8% women). The mean percent of predicted workload was 74.8 (±19.6) % without change across ages. In multivariable analysis, NYHA class I (odds ratio [OR]4.2, 95% confidence interval [CI]1.7-10.0) and higher physical activity level (>3 h/week) (OR 3.6, 95%CI 1.8-7.3) were positively associated with higher exercise capacity, while ongoing cardiovascular therapy (OR 0.4, 95%CI 0.2-0.7) and male sex (OR 0.3, 95%CI 0.2-0.6) were negatively associated with higher exercise capacity. Both exercise capacity (HR 0.96, 95%CI 0.93-0.98) and heart rate reserve (HR 0.96, 95%CI 0.94-0.98) were associated with mortality.

Conclusions: In patients with repaired tetralogy of Fallot the exercise capacity was approximately 75% of expected, regardless of age. Patients with better NYHA class and high physical activity level had a higher exercise capacity. Low exercise capacity and low heart rate reserve were associated with higher mortality. Therefore, evaluating exercise capacity as part of follow up is of importance.