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Background:Ascending aortic dilation is a feared complication in adults with repaired coarctation of the aorta, as the condition is associated with life-threatening complications such as aortic dissection and rupture. However, the data are currently limited regarding factors associated with ascending aortic dilation in these patients.Methods and results:From the national register of congenital heart disease, 165 adult patients (≥ 18 years old) with repaired coarctation of the aorta, and echocardiographic data on aortic dimensions, were identified (61.2% male, mean age 35.8 ± 14.5 years). Aortic dilation (aortic diameters > 2 SD above reference mean) was found in 55 (33.3%) of the 165 included patients, and was associated with manifest aortic valve disease in univariable logistic regression analysis (OR 2.44, 95% CI [1.23, 4.83]).Conclusions:Aortic dilation is common post-repair of coarctation of the aorta, and is associated with manifest aortic valve disease and thus indirectly with the presence of a bicuspid aortic valve. However, no association was found between aortic dilation and age or blood pressure.

Aims The majority of children with complex congenital heart disease (CHD) survive into adulthood due to advances in medical care. Adult patients with CHD have an increased incidence of diagnoses related to ageing such as heart failure, dementia, cancer and sarcopenia, despite a relatively low age. They also have a shorter life expectancy. It is unknown if their bone structures also show signs of premature ageing. We therefore investigated Bone Mineral Content (BMC) and bone mineral density (BMD) in an adult population with complex CHD. Methods The total body BMC and BMD was examined using dual energy X-ray absorptiometry (DXA) in 73 adults with complex CHD (mean age 35.8 ± 14.3, women n = 22) and 73 age and sex matched controls. Results The adults with complex CHD had lower total body BMC (2.6 ± 0.5 kg vs. 2.9 ± 0.5 kg, p Conclusion Adults with complex CHD have reduced total body BMC and BMD compared to healthy controls. These results are a sign of frailty that conforms with other previously reported signs of premature ageing. The risk of osteoporosis is low in our relatively young population, but it is assumed to increase with ageing. We recommend that clinicians pay close attention to risk factors for osteoporosis, and are generous in administering DXA-measurements in order to prevent future fractures among adults with complex CHD.

Abstract Background The aim of this retrospective study was to evaluate the relationship between right ventricular function derived from cardiac magnetic resonance imaging (CMR), echocardiography and exercise stress test performance, NT-proBNP (N-terminal proB-type natriuretic peptide) level and NYHA class in patients with a systemic right ventricle. Methods All patients with congenitally corrected transposition of the great arteries (ccTGA), or transposition of the great arteries after Mustard or Senning procedures, (TGA) followed at our centre who had undergone CMR, echocardiography, an exercise stress test and blood sampling, were included in the study. Results We examined 11 patients (six after the Senning procedure, one after the Mustard procedure, and four ccTGA) who have a median age of 32 years (22-67 years). A significant correlation was observed between the systemic ventricular function, expressed as the CMR-derived right ventricular ejection fraction and the right ventricular global longitudinal strain (r= -0.627; p=0.039). Conclusion We have demonstrated that in patients with ccTGA or TGA right ventricular global longitudinal strain may be useful in the evaluation of the systemic right ventricular function.

Cardiac resynchronization therapy may stabilize patients with severe heart failure awaiting heart transplant. Transvenous interventional implantation aided by intracardiac echocardiography is feasible in patients with adult congenital heart disease.

Investigate ventricular and atrial remodeling following atrial septal defect (ASD) closure and examine if pulmonary-to-systemic flow ratio (QP/QS) and right ventricular (RV) volume predict improvement, determined as percentage of predicted oxygen uptake (VO2%). Long-term cardiovascular magnetic resonance (CMR) data on atrial and ventricular remodeling after ASD-closure is limited and treatment effect on exercise capacity is debated. Sixteen patients undergoing transcatheter ASD closure and 16 age and sexmatched controls were studied. CMR was performed before treatment, the day after and 3 and 12 months later. Exercise test with gas analysis was performed before and 12 months after treatment. QP/QS decreased from 2.1 ± 0.5 to 1.4 ± 0.3 at day 1 and 1.1 ± 0.1 at 3 and 12 months. Left ventricular (LV) volumes increased and normalized on day 1 whereas left atrial volumes were unchanged. RV and right atrial volumes decreased the first 3 months. LV and RV volumes had not equalized at 12 months (RV/LV ratio 1.2 ± 0.1, P

Background:Patients with CHD exhibit reduced isometric muscle strength and muscle mass; however, little is known how these parameters relate. Therefore, the aim was to investigate the relation between isometric limb muscle strength and muscle mass for patients in comparison to age- and sex-matched control subjects.Methods:Seventy-four patients (35.6 ± 14.3 years, women n = 22) and 74 matched controls were included. Isometric muscle strength in elbow flexion, knee extension, and hand grip was assessed using dynamometers. Lean mass, reflecting skeletal muscle mass, in the arms and legs was assessed with dual-energy x-ray absorptiometry.Results:Compared to controls, patients had lower muscle strength in elbow flexion, knee extension, and hand grip, and lower muscle mass in the arms (6.6 ± 1.8 kg versus 5.8 ± 1.7 kg, p < 0.001) and legs (18.4 ± 3.5 kg versus 15.9 ± 3.2 kg, p < 0.001). There was no difference in achieved muscle force per unit muscle mass in patients compared to controls (elbow flexion 0.03 ± 0.004 versus 0.03 ± 0.005 N/g, p = 0.5; grip strength 0.008 ± 0.001 versus 0.008 ± 0.001 N/g, p = 0.7; knee extension 0.027 ± 0.06 versus 0.028 ± 0.06 N/g, p = 0.5). For both groups, muscle mass in the arms correlated strongly with muscle strength in elbow flexion (patients r = 0.86, controls, r = 0.89), hand grip (patients, r = 0.84, controls, r = 0.81), and muscle mass in the leg to knee extension (patients r = 0.64, controls r = 0.68).Conclusion:The relationship between isometric muscle strength and limb muscle mass in adults with CHD indicates that the skeletal muscles have the same efficiency as in healthy controls.

Introduction Ventricular septal defect (VSD) is one of the most common congenital heart lesions. Shunts with hemodynamic significance are usually closed early in life whereas small shunts are left without intervention. The need for late cardiac interventions in these populations is essentially unknown. The aim was to study the late cardiac interventions in adults with VSD. Methods The national register on congenital heart disease was searched for patients with VSD with or without associated simple cardiac defects but without complex lesions. For these patients, the last 10 years (over the age of 18) in the national patient register was searched for cardiac interventions. Results 774 patients (mean age 39.0±14.7 years, women =50.6%), 224 (28.9%) with previous closure of VSD, were identified. The total observed time was 6920 patient years. There were 43 interventions in 41 patients (5.3%) of whom 12 had a previous closure of VSD. Twelve patients had isolated closure of VSD, 18 closure of VSD together with other cardiac surgery (one of these had repeated cardiac surgery) and 8 had only other cardiac surgery. In 4 cases, a pacemaker was implanted, of whom one together with cardiac surgery. Conclusion The need for cardiac interventions is relatively common in adults with a VSD, also in those without previous closure of their shunt. Our data suggests that most patients with a VSD, closed or not, should be offered periodic follow-up as approximately one out of 20 patients may encounter a complication within 10 years. Funding Acknowledgement Type of funding source: Foundation. Main funding source(s): The Swedish Heart-Lung Foundation

Introduction: Interatrial communication is associated with an increased risk of decompression sickness (DCS) in scuba diving. It has been proposed that there would be a decreased risk of DCS after closure of the interatrial communication, i.e., persistent (patent) foramen ovale (PFO). However, the clinical evidence supporting this is limited. Methods: Medical records were reviewed to identify Swedish scuba divers with a history of DCS and catheter closure of an interatrial communication. Thereafter, phone interviews were conducted with questions regarding diving and DCS. All Swedish divers who had had catheter-based PFO-closure because of DCS were followed up, assessing post-closure diving habits and recurrent DCS. Results: Nine divers, all with a PFO, were included. Eight were diving post-closure. These divers had performed 6,835 dives (median 410, range 140–2,200) before closure, and 4,708 dives (median 413, range 11–2,000) after closure. Seven cases with mild and 10 with serious DCS symptoms were reported before the PFO closure. One diver with a small residual shunt suffered serious DCS post-closure; however, that dive was performed with a provocative diving profile. Conclusion: Divers with PFO and DCS continue to dive after PFO closure and this seems to be fairly safe. Our study suggests a conservative diving profile when there is a residual shunt after PFO closure, to prevent recurrent DCS events.

Objective: Congenitally corrected and surgical atrial redirected transposition of the great arteries (TGA) represents states where the morphological right ventricle serves as a systemic ventricle (S-RV). The S-RV is prone to failure, but data on medical treatment on this problem is limited. The purpose of this study was to evaluate the survival in adults with S-RV, with or without heart failure treatment. Design: The SWEDCON registry was used to collect data. All adults with S-RV and minimum follow-up of 1 year were included retrospectively. Medical treatment was defined as taking beta-blockers and/or ACE inhibitors and/or ARBs for more than 50% of the time. Results: We identified 343 patients with S-RV (median age: 21 years). Surgical atrial redirected TGA was present in 58% and congenitally corrected TGA in 42% of patients. The medically treated group (n = 126) had higher rates of impaired S-RV function, use of diuretics, pacemaker and higher NYHA functional class at baseline compared to controls. The proportion of patients with impaired functional class did not change over time in the medically treated group, but increased in controls (21% vs. 30%, p = .015). In Kaplan-Meier analysis, the mean follow-up was 10.3 years, no difference in survival was seen between the groups. Conclusions: Medical treatment may be beneficial in patients with S-RV and impaired functional class and appears to be safe in the long term. The treatment group had equal survival to controls, despite worse baseline characteristics, which might be a result of slower progression of disease in this group.

Background: Regional ventricular pumping mechanisms in patients with volume-loaded right ventricles (RV) are altered, but the cause is unknown. The aim was to determine whether these changes in ventricular pumping mechanisms are influenced by the RV dilatation itself or the aetiology behind it. Methods: Seventeen patients with atrial septal defects (ASD) and 10 healthy controls underwent cardiovascular magnetic resonance (CMR) at rest and during dobutamine/atropine stress. Sixteen patients underwent transcutaneous ASD closure. Follow-up CMR at rest was performed the following day. Thirty patients with RV overload due to pulmonary regurgitation (PR) underwent CMR at rest. Cine images were used to measure left ventricular (LV) and RV volumes as well as septal, longitudinal and lateral contributions to LV and RV stroke volume (SV). Results: At rest, septal contribution to LVSV was lower in ASD patients than controls (-1% versus 7%, P

Background The SWEDish registry of CONgenital heart disease is a Swedish national quality register for collecting data on pediatric and adult (GUCH) patients with congenital heart diseases that aims to improve medical practice and research. The aim of this study was to validate data in the register using data in medical records. Methods This cross-sectional observational study compared specific variables from SWEDCON with data retrieved from medical records for 182 pediatric and 186 adult patients who were randomly selected from four different hospitals. Results The absolute concordance for date of visit, diagnosis, information on surgery and catheter intervention was 71–98% between SWEDCON and medical journals. The specificity for cardiac examinations and specific medical therapy was 71–100%. The concordance for information for transition from pediatric care to GUCH or finishing regular check-ups was 92%. Conclusion The overall concordance between data in SWEDCON-pediatric as well as SWEDCON-GUCH with data in medical records was good, and data from SWEDCON is reliable to use for medical improvements and for research purposes.

BACKGROUND: Right ventricular (RV) dysfunction may be caused by either pressure or volume overload. RV function is conventionally assessed with echocardiography using tricuspid annular plane systolic excursion (TAPSE), RV fractional area change (RVFAC), tricuspid lateral annular systolic velocity (S') and RV index of myocardial performance (RIMP). The purpose of this study was to evaluate whether RV global longitudinal strain (RVGLS) and RV-free wall strain (RV-free) could add additional information to differentiate these two causes of RV overload.METHODS AND RESULTS: The study enrolled 89 patients with an echocardiographic trans-tricuspid gradient >30 mmHg. Forty-five patients with pulmonary arterial hypertension or chronic thromboembolic pulmonary hypertension (pressure overload) were compared with 44 patients with an atrial septum defect (volume overload). RV size was larger in the volume group (P

BackgroundArterial hypertension is common in adults with repaired coarctation of the aorta, and is associated with several severe complications.AimsThis study aimed to investigate the prevalence of poorly controlled (⩾140/90 mmHg) blood pressure among patients with diagnosed hypertension and to identify associated factors.MethodsIn the national register for CHD, adults with repaired coarctation of the aorta and diagnosed hypertension – defined as a registry diagnosis and/or use of anti-hypertensive prescription medication – were identified. Logistic regression analysis was used to identify variables associated with poorly controlled blood pressure.ResultsOf the 243 included patients, 27.2% were female, the mean age was 45.4±15.3 years, and 52.3% had poorly controlled blood pressure at the last registration. In a multivariable model, age (years) (OR 1.03, CI 1.01–1.06, p=0.008) was independently associated with poorly controlled blood pressure and so was systolic arm–leg blood pressure gradient in the ranges [10, 20] mmHg (OR 4.92, CI 1.76–13.79, p=0.002) to >20 mmHg (OR 9.93, CI 2.99–33.02, pConclusionsPoorly controlled blood pressure is common among patients with repaired coarctation of the aorta and diagnosed hypertension, despite what seems to be more intensive treatment. A systolic arm–leg blood pressure gradient is associated with poorly controlled blood pressure, even at low levels usually not considered for intervention, and may be an indicator of hypertension that is difficult to treat.

Background. Reconstruction of the right ventricular outflow tract with a conduit is an established surgical procedure in congenital heart disease and reinterventions are common. Objective. An increasing number of patients have a conduit, but there are few population-based studies of long-term outcomes after conduit surgery, reoperations, and transcatheter pulmonary valve replacement. Methods. In April 2015, all adult patients with a conduit were identified in the Swedish National Registry for Congenital Heart Disease (SWEDCON). Data on patients who died before age of 16 years are not included in the registry and thus not included in the study. Results. We found 574 patients with a mean age 36.1 years. The largest proportion had tetralogy of Fallot (45%). In total there were 762 operations and 50 transcatheter pulmonary valve replacements. Mean age at first conduit operation was 20.2 years. Long-term survival up to 48 years including perioperative mortality (n = 176) was significantly lower than after first conduit operation (70% vs 77% p = .04). Higher age at first conduit operation was associated with a reduced risk of reintervention, whereas male sex and complex malformations were associated with increased risk of reintervention. Conclusions. The mortality of repeated conduit reinterventions is low. The need for reintervention of conduits is considerable, and reintervention-free survival after the first conduit reintervention is poorer than after first conduit implantation. The findings in this study only applies for patients reaching 16 years of age.

Objective: The use of a conduit is an established surgical method for reconstruction of the right ventricular outflow tract in congenital heart disease; however, its limited durability makes reintervention almost inevitable. We studied the trends in new implantation, reoperation, and transcatheter pulmonary valve replacement (TPVR) from a Swedish national perspective. Design and results: The Swedish registry of congenital heart disease (SWEDCON) was used to collect data. From 2000–2014, there was an increase in adult patients with conduits from 122 to 536. There were 60 surgical conduit replacements, 40 TPVRs and 176 new conduit implantations in the study period. Perioperative mortality was

ObjectiveThe optimal timing for transplantation is unclear in patients with Eisenmenger syndrome (ES). We investigated post-transplantation survival and transplantation-specific morbidity after heart–lung transplantation (HLTx) or lung transplantation (LTx) in a cohort of Nordic patients with ES to aid decision-making for scheduling transplantation.MethodsWe performed a retrospective, descriptive, population-based study of patients with ES who underwent transplantation from 1985 to 2012.ResultsAmong 714 patients with ES in the Nordic region, 63 (9%) underwent transplantation. The median age at transplantation was 31.9 (IQR 21.1–42.3) years. Within 30 days after transplantation, seven patients (11%) died. The median survival was 12.0 (95% CI 7.6 to 16.4) years and the overall 1-year, 5-year, 10-year and 15-year survival rates were 84.1%, 69.7%, 55.8% and 40.6%, respectively. For patients alive 1 year post-transplantation, the median conditional survival was 14.8 years (95% CI 8.0 to 21.8), with 5-year, 10-year and 15-year survival rates of 83.3%, 67.2% and 50.0%, respectively. There was no difference in median survival after HLTx (n=57) and LTx (n=6) (14.9 vs 10.6 years, p=0.718). Median cardiac allograft vasculopathy, bronchiolitis obliterans syndrome and dialysis/kidney transplantation-free survival rates were 11.2 (95% CI 7.8 to 14.6), 6.9 (95% CI 2.6 to 11.1) and 11.2 (95% CI 8.8 to 13.7) years, respectively. The leading causes of death after the perioperative period were infection (36.7%), bronchiolitis obliterans syndrome (23.3%) and heart failure (13.3%).ConclusionsThis study shows that satisfactory post-transplantation survival, comparable with contemporary HTx and LTx data, without severe comorbidities such as cardiac allograft vasculopathy, bronchiolitis obliterans syndrome and dialysis, is achievable in patients with ES, with a conditional survival of nearly 15 years.

Adults with complex congenital heart disease (CHD) have reduced aerobic capacity and impaired muscle function. We therefore hypothesized that patients have a lower skeletal muscle mass and higher fat mass than controls.Body composition was examined with full body Dual-Energy x-ray Absorptiometry (DXA) in 73 patients with complex CHD (mean age 35.8 ± 14.3, women n = 22) and 73 age and sex matched controls. Patients fulfilling criteria for low skeletal muscle mass in relation to their height and fat mass were defined as sarcopenic.Male patients (n = 51) were shorter (177.4 ± 6.6 cm vs. 180.9 ± 6.7 cm, p = 0.009) and weighed less (76.0 ± 10.8 kg vs. 82.0 ± 12.4 kg, p = 0.01) than controls. Also, patients had a lower appendicular lean mass-index (ALM-index) (7.57 ± 0.97 kg/mThe body composition was altered toward lower skeletal muscle mass in patients with complex CHD. Approximately half of the patients were classified as sarcopenic. Contrary to men, the women had increased body fat and a higher BMI. Further research is required to assess the cause, possible adverse long-term effects and whether sarcopenia is preventable or treatable.

Adults with complex congenital heart disease (CHD) show reduced aerobic exercise capacity and impaired skeletal muscle function compared with healthy peers. Peripheral muscle factors are presumed to be important contributors to the aerobic capacity, but the mechanisms are poorly understood. The aim of the present study was to investigate differences between adults with CHD and controls in muscle oxygenation kinetics at rest, and during and after exercise.Seventy-four patients with complex CHD (mean age 35.6 ± 14.3 years, female n = 22) were recruited. Seventy-four age- and sex-matched subjects were recruited as controls. Muscle oxygenation was successfully determined on the anterior portion of the deltoid muscle using near-infrared spectroscopy in 65 patients and 71 controls. Measurements were made at rest, during isotonic shoulder flexions (0-90°) to exhaustion, and during recovery.The patients with CHD performed fewer shoulder flexions (40 ± 17 vs 69 ± 40; P0.001), had lower muscle oxygen saturation (StOIn comparison with age- and sex-matched controls, adults with complex CHD had slower oxygenation kinetics. This altered skeletal muscle metabolism might contribute to the impaired skeletal muscle endurance capacity shown and thereby also to the reduced aerobic capacity in this population.

Aims In adults with coarctation of the aorta (CoA), hypertension (HTN) is a common long-term complication. We investigated the prevalence of HTN and analyzed factors associated with HTN. Methods and results In the national register for congenital heart disease, 653 adults with repaired CoA were identified (mean age 36.9±14.4years); 344 (52.7%) of them had HTN, defined as either an existing diagnosis or blood pressure (BP) ≥140/90mmHg at the clinical visit. In a multivariable model, age (years) (odds ratio [OR] 1.07, CI 1.05-1.10), sex (male) (OR 3.35, CI 1.98-5.68), and body mass index (kilograms per square meter) (OR 1.09, CI 1.03-1.16) were independently associated with having HTN, and so was systolic arm-leg BP gradient where an association with HTN was found at the ranges of (10, 20] and >20mmHg, in comparison to the interval ≤10mmHg (OR 3.58, CI 1.70-7.55, and OR 11.38, CI 4.03-32.11). This model remained valid when all patients who had increased BP (≥140/90mmHg) without having been diagnosed with HTN were excluded from the analyses. Conclusions Hypertension is common in patients with previously repaired CoA and is associated with increasing age, male sex, and elevated body mass index. There is also an association with arm-leg BP gradient, starting at relatively low levels that are usually not considered for intervention.

Background: The purpose of this study was to determine the effect of stress on left-to-right shunting in patients with atrial septal defect (ASD) and to investigate if the degree of shunting, cardiac output (CO), and right ventricular (RV) volumes are related to exercise capacity. Methods: Twenty-six patients with a secundum ASD and 16 healthy volunteers were studied with rest/stress cardiac magnetic resonance using 20 μg/kg/min dobutamine and 0.25-0.75 mg atropine to quantify CO, pulmonary to systemic flow ratio (QP/QS), and left ventricular (LV) and RV volumes. Peak oxygen uptake (VO2 peak) was determined on ergospirometry. Results: In patients with ASD the QP/QS decreased from 2.0 ± 0.2 at rest to 1.5 ± 0.1 (P < 0.001) during dobutamine stress (n = 20) and shunt volume per heartbeat decreased from 70 ± 9 to 38 ± 9 mL (P < 0.001). However, absolute shunt volume per minute was unchanged (5.1 ± 0.8 vs. 4.5 ± 1.0 L/min, P = 0.32) explained by a higher increase in systemic CO during stress (90 ± 11%) compared with pulmonary CO (43 ± 7%, P < 0.001). In ASD patients, VO2 peak correlated with aortic CO during stress (r = 0.77) and QP/QS at rest (r = -0.48) but not during stress (P = 0.09). VO2 peak did not correlate with RV volumes in patients. Conclusion: Pulmonary to systemic flow ratio and shunt volume per heartbeat decrease during stress in ASD patients. This may be explained by an enhanced LV diastolic function during stress and may have implications to detect disturbances in LV compliance in ASD patients. A high systemic CO during stress is a strong predictor of exercise capacity. (Less)

Ventricular septal defects (VSDs), if haemodynamically important, are closed whereas small shunts are left without intervention. The long-term prognosis in congenital VSD is good but patients are still at risk for long-term complications. The aim of this study was to clarify the incidence of infective endocarditis (IE) in adults with VSD.The Swedish registry for congenital heart disease (SWEDCON) was searched for adults with VSD. 779 patients were identified, 531 with small shunts and 248 who had the VSD previously closed. The National Patient Register was then searched for hospitalisations due to IE in adults during a 10-year period.Sixteen (2%) patients were treated for IE, 6 men and 10 women, with a mean age of 46.3±12.2 years. The incidence of IE was 1.7-2.7/1000 years in patients without previous intervention, 20-30 times the risk in the general population. Thirteen had small shunts without previous intervention. There was no mortality in these 13 cases. Two patients had undergone repair of their VSD and also aortic valve replacement before the episode of endocarditis and a third patient with repaired VSD had a bicuspid aortic valve, all of these three patients needed reoperation because of their IE and one patient died. No patient with isolated and operated VSD was diagnosed with IE.A small unoperated VSD in adults carries a substantially increased risk of IE but is associated with a low risk of mortality.

Atrial septal defect (ASD) is a congenital heart lesion that often remains undiagnosed until adulthood. The reasons for this may be multifactorial. It is, however, known that closure of a hemodynamically significant ASD improves exercise capacity. This study aimed to explore whether the aerobic capacity in late adolescence is associated with time to diagnosis and intervention in adult men with late diagnosis of an atrial shunt.The Swedish Military Conscription Service Register contains data on exercise tests performed in late adolescence. By linking these data with the National Patient Register, 254 men with a later intervention for an ASD were identified.Interventions were performed at a mean of 26.5 ± 7.9 years after the initial exercise tests. The mean absolute workload among those with a later diagnosed ASD was similar to those without a later diagnosed ASD (274 ± 51 W vs. 276 ± 52 W, p = 0.49). Men with a higher exercise capacity (≥1 SD) had their intervention earlier (21.9 ± 8.6 years vs. 27.5 ± 7.4 years, p 0.001).The aerobic exercise capacity was similar in adolescent men with later interventions for ASD compared to the reference population. Furthermore, those with high exercise capacity appeared to be diagnosed earlier. Thus, low exercise capacity may not be a feature of ASD during adolescence, but rather develop later in life as a natural progression of the disease.

Background Limited data are available on the increase in cardiac troponin after transcatheter closure of interatrial septal defects, and the mechanism is not fully understood. The aim with the study was to examine retrospectively whether transcatheter closure of the interatrial septum leads to myocardial injury, and to determine the mechanism. Methods Troponin T (TnT) serum concentrations were determined before and 1 day after transcatheter closure of an atrial septal defect (ASD) or patent foramen ovale (PFO) in a retrospective study in adults. Fifty-one patients were included, 36 ASD and 15 PFO (female/male 35/16), mean age 52 ± 16 y (range 17–80 y). Results An increase in TnT was observed 1 day after the procedure (p = 0.000) in 44 patients (92%). The increase in TnT was significantly correlated to the size of the ASD (r = 0.424, p = 0.010), as well as to the size of the implanted ASD device (r = 0.542, p = 0.001), and the duration of the ASD intervention (r = 0.348, p = 0.035). In cases of PFO, the increase in TnT was not correlated with the size of the PFO or the PFO device, or the duration of the intervention. No correlations were found between the increase in TnT and the patient’s age, body weight or body surface area. Conclusions Transcatheter closure of the interatrial septum causes an increase in TnT indicating a minor and clinically insignificant myocardial injury. The findings indicate that mechanical trauma caused by inserting the ASD device may play an important role in this elevation. The impact of the size of the device on the degree of TnT elevation reflects the amount of myocardium affected by the device. Trial registration NCT03099967. Registered 4 April 2017, retrospectively registered.

Background: Physical activity improves health, exercise tolerance and quality of life in adults with congenital heart disease (CHD), and exercise training is in most patients a high-benefit low risk intervention. However, factors that influence the confidence to perform exercise training, i.e. exercise self-efficacy (ESE), in CHD patients are virtually unknown. We aimed to identify factors related to low ESE in adults with CHD, and potential strategies for being physically active. Methods: Seventy-nine adults with CHD; 38 with simple lesions (16 women) and 41 with complex lesions (17 women) with mean age 36.7 ± 14.6 years and 42 matched controls were recruited. All participants completed questionnaires on ESE and quality of life, carried an activity monitor (Actiheart) during four consecutive days and performed muscle endurance tests. Results: ESE in patients was categorised into low, based on the lowest quartile within controls, (≤29 points, n = 34) and high (>29 points, n = 45). Patients with low ESE were older (42.9 ± 15.1 vs. 32.0 ± 12.4 years, p = 0.001), had more complex lesions (65% vs. 42%, p = 0.05) more often had New York Heart Association functional class III (24% vs. 4%, p = 0.01) and performed fewer shoulder flexions (32.5 ± 15.5 vs. 47.7 ± 25.0, p = 0.001) compared with those with high ESE. In a logistic multivariate model age (OR; 1.06, 95% CI 1.02–1.10), and number of shoulder flexions (OR; 0.96, 95% CI 0.93–0.99) were associated with ESE. Conclusion: In this study we show that many adults with CHD have low ESE. Age is an important predictor of low ESE and should, therefore, be considered in counselling patients with CHD. In addition, muscle endurance training may improve ESE, and thus enhance the potential for being physically active in this population. Keywords: Exercise self-efficacy, Adult congenital heart disease, Quality of life, Muscle function, Physical activity

Background: Many adults with congenital heart disease (CHD) have reduced aerobic exercise capacity and impaired muscle function. However, it is largely unknown which factors have influence on the c ...

OBJECTIVE: Improved diagnostic tools, timely closure of the shunt and a better understanding of the complexity of Eisenmenger syndrome (ES) have led to improved care and treatment in tertiary centres. These may have decreased the incidence of ES and improved survival of patients with ES, although evidence is still lacking. The aim of this study was to investigate temporal changes in incidence, prevalence and mortality in patients with ES for 35 years in the Nordic region.METHODS: This was a retrospective population-based study including 714 patients with ES. Survival analysis was performed based on all-cause mortality and accounting for immortal time bias.RESULTS: The incidence of ES decreased from 2.5/million inhabitants/year in 1977 to 0.2/million inhabitants/year in 2012. Correspondingly, prevalence decreased from 24.6 to 11.9/million inhabitants. The median survival was 38.4 years, with 20-year, 40-year and 60-year survival of 72.5%, 48.4%, and 21.3%, respectively. Complex lesions and Down syndrome were independently associated with worse survival (HR 2.2, pCONCLUSIONS: The incidence and prevalence of ES in the Nordic region have decreased markedly during the last decades. Furthermore, the median age at death increased throughout the study period, indicating prolonged life expectancy in the ES population. However, increasing age represents decreased incidence, rather than improved survival. Nonetheless, longevity with ES is still shorter than in the background population.

Aims Eisenmenger syndrome (ES) is associated with considerable morbidity and mortality. Therapeutic strategies have changed during the 2000s in conjunction with an emphasis on specialist follow-up. The aim of this study was to determine the cause-specific mortality in ES and evaluate any relevant changes between 1977 and 2015. Methods and results This is a retrospective, descriptive multicentre study. A total of 1546 patients (mean age 38.7 ± 15.4 years; 36% male) from 13 countries were included. Cause-specific mortality was examined before and after July 2006, ‘early’ and ‘late’, respectively. Over a median follow-up of 6.1 years (interquartile range 2.1–21.5 years) 558 deaths were recorded; cause-specific mortality was identified in 411 (74%) cases. Leading causes of death were heart failure (34%), infection (26%), sudden cardiac death (10%), thromboembolism (8%), haemorrhage (7%), and peri-procedural (7%). Heart failure deaths increased in the ‘late’ relative to the ‘early’ era (P = 0.032), whereas death from thromboembolic events and death in relation to cardiac and non-cardiac procedures decreased (P = 0.014, P = 0.014, P = 0.004, respectively). There was an increase in longevity in the ‘late’ vs. ‘early’ era (median survival 52.3 vs. 35.2 years, P Conclusion The study shows that despite changes in therapy, care, and follow-up of ES in tertiary care centres, all-cause mortality including cardiac remains high. Patients from the ‘late’ era, however, die later and from chronic rather than acute cardiac causes, primarily heart failure, whereas peri-procedural and deaths due to haemoptysis have become less common. Lifelong vigilance in tertiary centres and further research for ES are clearly needed.

Background: Eisenmenger syndrome is associated with substantial morbidity and mortality. There is no consensus, however, on mortality risk stratification. We aimed to investigate survival and predictors of death in a large, contemporary cohort of Eisenmenger syndrome patients. Methods: In a multicenter approach, we identified adults with Eisenmenger syndrome under follow-up between 2000 and 2015. We examined survival and its association with clinical, electrocardiographic, echocardiographic, and laboratory parameters. Results: We studied 1098 patients (median age, 34.4 years; range, 16.1–84.4 years; 65.1% female; 31.9% with Down syndrome). The majority had a posttricuspid defect (n=643, 58.6%), followed by patients with a complex (n=315, 28.7%) and pretricuspid lesion (n=140, 12.7%). Over a median follow-up of 3.1 years (interquartile range, 1.4–5.9), allowing for 4361.6 patient-years observation, 278 patients died and 6 underwent transplantation. Twelve parameters emerged as significant predictors of death on univariable analysis. On multivariable Cox regression analysis, only age (hazard ratio [HR], 1.41/10 years; 95% confidence interval [CI], 1.24–1.59; P P =0.041), oxygen saturation at rest (HR, 0.53/10%; 95% CI, 0.43–0.65; P P =0.013), and presence of pericardial effusion (HR, 2.41; 95% CI, 1.59–3.66; P Conclusions: There is significant premature mortality among contemporary adults with Eisenmenger syndrome. We report, herewith, a multivariable mortality risk stratification model based on 5 simple, noninvasive predictors of death in this population.

Background— The Fontan procedure has improved survival in children with functionally univentricular hearts. With time, however, complications such as reduced exercise capacity are seen more frequently. Exercise intolerance is multifactorial, but pulmonary vascular resistance probably plays a crucial role. Elevated pulmonary vascular resistance has been associated with raised levels of endothelin-1, which are common both before and after Fontan operations. Treatment with endothelin-1 receptor antagonists could theoretically improve cardiopulmonary hemodynamics and exercise capacity. The aim of this study was therefore to examine the efficacy and safety of bosentan in Fontan patients. Methods and Results— Seventy-five adolescents and adults were randomized 1:1 to 14 weeks of treatment with bosentan or placebo. Cardiopulmonary exercise test, functional class, blood samples, and quality-of-life questionnaires were evaluated at baseline and at the end of treatment. Sixty-nine patients (92%) completed the study. Peak oxygen consumption increased 2.0 mL·kg −1 ·min −1 (from 28.7 to 30.7 mL·kg −1 ·min −1 ) in the bosentan group compared with 0.6 mL·kg −1 ·min −1 (from 28.4 to 29.0 mL·kg −1 ·min −1 ) in the placebo group ( P =0.02). Cardiopulmonary exercise test time increased by 0.48 minute (from 6.79 to 7.27 minutes) versus 0.08 minute (from 6.94 to 7.02 minutes; P =0.04). Nine bosentan-treated patients improved 1 functional class, whereas none improved in the placebo group ( P =0.0085). Side effects were mild and occurred equally in both groups. No serious adverse effects were seen, and no patients had liver enzyme levels above the 3-fold upper limit. Conclusions— Bosentan improves exercise capacity, exercise time, and functional class in Fontan patients without serious adverse events or hepatotoxicity. Clinical Trial Registration— URL: http://www.clinicaltrials.gov . Unique identifier: NCT01292551.

ObjectivePatients with congenital heart disease may be operated with surgical reconstruction of the right ventricular outflow tract (RVOT). Reintervention is common in this group of patients. The aim of this study was to examine longitudinal self-reported health measured by the EQ-5D questionnaire. DesignData were collected from the Swedish Registry of Congenital Heart Disease. EQ-5D, which measures health outcome, was introduced into the registry in 2005. All adult patients with previous surgical reconstruction of the RVOT who had EQ-5D data from their first and latest visit were analyzed. ResultsAmong 103 patients (65 male and 38 female), mean age 31 (range 19-78 years), the diagnoses were: tetralogy of Fallot (n=66); truncus, transpositions, and double outlet right ventricle (n=23); and Ross-operated congenital aortic valve disease (n=14). Time from first to latest visit was 3 years (range 1-7 years). Eighteen patients underwent 26 reinterventions in the observational period from the first to latest visit, including operations, percutaneous interventions, pacemaker implantations, and ablations. Health perception, mean EQ-5D visual analog scale, VAS, declined from 84.4 (standard deviation (SD)=14.6) to 78.6 (SD=18.3) at the latest visit, P=.001. The decline is almost exclusively seen in patients without reinterventions. Low EQ-VAS was associated with symptoms and New York Heart Association class II-IV. Patient-reported problems in the EQ-5D dimension usual activities were more common in the patients having reinterventions (25%) than those without reintervention (7%), P=.04. ConclusionIn this longitudinal cohort study of patients with previous surgical reconstruction of the RVOT, health perception declined over time. The decline was not observed in patients undergoing any additional interventions.

Adults with congenital heart disease (ACHD) usually have reduced aerobic exercise capacity compared with controls. However, their skeletal muscle function is less studied.In this cross-sectional study, unilateral isotonic shoulder flexion, unilateral isotonic heel-lift, maximum inspiratory pressure (MIP) and maximum expiratory pressure (MEP) were tested in 85 patients with ACHD (35 women, mean age 36.8 ± 14.8 years), classed as either 'complex' (n = 43) or 'simple' (n = 42), and 42 age and gender matched controls (16 women, mean age 36.9 ± 14.9). Maximum number of shoulder flexions and heel-lifts were measured. MIP/MEP was tested using a handheld respiratory pressure meter. Exercise self-efficacy, measuring confidence in performing exercise training, was evaluated.Adults with complex lesions performed fewer shoulder flexions compared with controls and patients with simple lesions (28.2 ± 11.1 vs. 63.6 ± 40.4, p 0.001 and 28.2 ± 11.1 vs. 54.9 ± 24.9, p 0.001), as well as fewer heel-lifts compared with controls and patients with simple lesions (17.6 ± 7.7 vs. 26.3 ± 12.8, p 0.001 and 17.6 ± 7.7 vs. 23.2 ± 7.0, p = 0.024), lower MIP than controls (80.7 ± 26.7 vs. 111.1 ± 29.9 cm H2O, p 0.001) and lower MEP compared with controls (110.8 ± 39.9 vs. 141.8 ± 39.5, p 0.001). Their exercise self-efficacy was lower than controls (28.0 ± 8.3 vs. 33.4 ± 6.1, p = 0.002). In a linear regression model complex heart lesions were independently associated with impaired limb muscle function.Adults with complex congenital heart disease have impaired skeletal muscle function compared with patients with simple lesions and healthy controls. They also had lower confidence in performing exercise training. Thus, this population might have a potential for rehabilitation focusing on improving muscle function and confidence in performing exercise training.

The prognosis in adults with congenital aortic valve disease is usually favourable; nevertheless, a number of medical and social factors might hamper long-term prognosis and quality of life. With a focus on physical exercise level, data from the Swedish National Registry on Congenital Heart Disease (SWEDCON) were analysed and variables associated with health-related quality of life in adults with congenital aortic valve disease were identified.In this registry study, SWEDCON was searched for adult patients with isolated congenital aortic valve disease and valid EuroQol-5Dimensions health questionnaire (EQ-5D) data.This study identified 315 patients. The majority (n = 202, 64%) reported best possible health status (EQ-5D(index) = 1) whereas 113 (35%) reported some impairment (EQ-5D(index) 1) with mean EQ-5D(index) 0.73 ± 0.17. In a multivariate logistic regression model, self-reported physical exercise 3 h/week was independently associated with best possible health status (EQ-5D(index) = 1; p = 0.013). Moreover presence of cardiovascular symptoms (p 0.001), active smoking (p = 0.002), history of valve surgery (p = 0.017), low educational level (p = 0.022), and higher systolic blood pressure (p = 0.029) were independently associated with impaired health status (EQ-5D(index) 1).Physical exercise3 h/week was, as a single variable, associated with best possible health status in adults with congenital aortic valve disease. In contrast, a number of medical and social factors are associated with worse self-reported health status. Among these, symptoms, smoking, and educational level are potential targets for modification and intervention. There is a need for studies investigating the effect of increased level of physical exercise in patients with congenital aortic valve disease.

Background Patients with cyanotic congenital heart disease (CCHD) have haemostatic abnormities associated with bleeding and thrombo-embolic events. The haemostatic abnormalities are not fully understood, but recent studies indicate that elevated haematocrit and fibrinogen function may be of importance. The aim of this study was to characterise the haemostatic profile and examine the potential role of haematocrit on clot formation and strength in CCHD patients. Furthermore to examine whether CCHD patients with history of haemoptysis have diminished fibrinogen function compared to those without haemoptysis. Methods In a prospective study 75 adult CCHD patients had haematocrit, platelet count, and plasma fibrinogen concentration examined. Furthermore thrombelastography(TEG) as well as TEG Functional Fibrinogen(TEG FF) assay evaluating fibrinogen function(FLEV) was performed. Data were compared with historical data regarding previous haemoptysis in CCHD patients. Results Haematocrit was 57±8% and platelet counts in the lower normal range. TEG revealed a hypocoagulable condition with impaired clot formation. TEG values were correlated to haematocrit, indicating that elevated haematocrit causes impaired clot formation and strength. Despite high levels of plasma fibrinogen, TEG FF demonstrated that FLEV was diminished and negatively correlated to haematocrit. Furthermore CCHD patients with previous history of haemoptysis had significantly lower FLEV compared to CCHD patients without haemoptysis. Conclusion Patients with CCHD are hypocoagulable mainly due to impaired fibrinogen function. Despite a low platelet count, platelet function does not seem to be severely affected in CCHD patients. Haemostasis, and especially fibrinogen function, is negatively affected by elevated haematocrit, and fibrinogen function is diminished in CCHD patients with haemoptysis.

Secundum atrial septal defect (ASD2) is one of the most common cardiac malformations diagnosed in adult life. Catheter closure has made treatment possible even in patients of high age. However, published outcome data for elderly patients is limited. The aim of this study was to report, on a national basis, the long-term outcome of ASD2 catheter closure in the elderly.We report the clinical and echocardiographic outcome of catheter closure of ASD2 in 148 patients aged 65-87years. Data was obtained from a national registry, medical records and a questionnaire. The proportion of patients in NYHA I increased from 34% to 61% (p0.001) one year after closure and remained stable at the latest follow-up 4,4 (SD 2,6) years post-closure. The proportion of patients with moderate/severe enlargement of the right ventricle and atrium fell from 77% and 76% to 25% and 40%, respectively, (p0.001) and right ventricular systolic pressure dropped significantly. Improvement of NYHA class was associated with reduced right ventricular systolic pressure but not with remodelling of the right heart. NYHA deteriorated in 9 patients, despite reduced right ventricular size. Overall, the prevalence of atrial fibrillation was unchanged after closure. Major complication rate was 2% and there was no procedure- or device-related mortality.Catheter closure of ASD2 in the elderly is a worthwhile procedure since it improves symptoms and has a low complication rate. However, a subset of patients do not improve, in which we suggest that concealed left ventricular dysfunction may play a causative role.

BACKGROUND: There is a lack of educational material for adults with congenitally malformed hearts. Computer-based education has shown to have significant effects on knowledge and management of chronic diseases. AIM: The aim of this study was to develop and evaluate a computer-based educational program for adults with congenitally malformed hearts. METHODS: The program was developed by a multidisciplinary team. Data were collected by questionnaires, observations, and structured interviews. RESULTS: The final product was a computer-based educational program consisting of ten separate modules, one for each particular malformation. The program was experienced as stimulating and easy to use. The appearance and quantity of the text was graded as good and the content as relevant and very useful. CONCLUSION: This is the first computer-based program developed for adults with congenitally malformed hearts. The evaluation found the program to have great potential as an important tool for improving care. Further studies are needed to test the outcomes of the program on knowledge, perceived control over the heart condition, anxiety/depression. and health-related quality of life. PRACTICE IMPLICATIONS: The program may be used as a complement to verbal information and every adult with a congenitally malformed hearts can receive individualized information from a personal CD.