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Background: Coronary artery disease (CAD) will increasingly determine outcome in the aging adult congenital heart disease (CHD) population. We aimed to determine sex-specific incidence of CAD in adult CHD patients throughout adulthood, compared to the general population. Methods and results: We followed 11,723 adult CHD patients (median age 33 years; 49% male; 57% mild, 34% moderate, 9% severe CHD) from the Dutch CONCOR registry, and two age-sex-matched persons per patient from the general population for first CAD event in national registers (period 2002–2012). Incidence rates were estimated using smoothed hazard functions. CAD risk during follow-up, stratified by CHD severity, was compared using proportional subdistribution hazards regression. In ACHD patients, 103 CAD events (43 women) occurred over 60,456 person-years. Rates per 1000person-years increased from 0.3(95% confidence interval: 0.1–0.6) at age 20 to 5.8(3.7–8.9) at 70 years in female, and from 0.5(0.3–1.0) to 7.8(5.1–11.8) in male patients. Compared to the general population, relative risk was 12.0(2.5–56.3) in women and 4.6(1.7–12.1) in men aged 20 years. Relative risk declined with age, remaining significant up to age ~65 years in women and ~50 years in men. In patients with mild, moderate and severe CHD, CAD risk was 1.3(0.9–1.9), 1.6(1.0–2.5) and 2.9(1.3–6.9) times increased compared to the general population, respectively. Conclusions: We found increased CAD risk in adult CHD patients, with greater relative risk at younger age, in women and those with more severe CHD. These results underline the importance of screening for and treatment of CAD risk factors in these patients.

Aims Heart failure is the main threat to long-term health in adults with transposition of the great arteries (TGA) corrected by an atrial switch operation (AtrSO). Current guidelines refrain from recommending heart failure medication in TGA-AtrSO, as there is insufficient data to support the hypothesis that it is beneficial. Medication is therefore prescribed based on personal judgements. We aimed to evaluate medication use in TGA-AtrSO patients and examine the association of use of renin–angiotensin–aldosterone system (RAAS) inhibitors and β-blockers with long-term survival. Methods and results We identified 150 TGA-AtrSO patients [median age 30 years (interquartile range 25–35), 63% male] included in the CONCOR registry from five tertiary medical centres with subsequent linkage to the Dutch Dispensed Drug Register for the years 2006–2014. Use of RAAS inhibitors, β-blockers, and diuretics increased with age, from, respectively, 21% [95% confidence interval (CI) 14–40], 12% (95% CI 7–21), and 3% (95% CI 2–7) at age 25, to 49% (95% CI 38–60), 51% (95% CI 38–63), and 41% (95% CI 29–54) at age 45. Time-varying Cox marginal structural models that adjusted for confounding medication showed a lower mortality risk with use of RAAS inhibitors and β-blockers in symptomatic patients [hazard ratio (HR) = 0.13 (95% CI 0.03–0.73); P = 0.020 and HR = 0.12 (95% CI 0.02–0.17); P = 0.019, respectively]. However, in the overall cohort, no benefit of RAAS inhibitors and β-blockers was seen [HR = 0.93 (95% CI 0.24–3.63); P = 0.92 and HR = 0.98 (0.23–4.17); P = 0.98, respectively]. Conclusion The use of heart failure medication is high in TGA-AtrSO patients, although evidence of its benefit is limited. This study showed lower risk of mortality with use of RAAS inhibitors and β-blockers in symptomatic patients only. These findings can direct future guidelines, supporting use of RAAS inhibitors and β-blockers in symptomatic, but not asymptomatic patients.

Aims Adult congenital heart disease(ACHD) patients are at increased risk ofsudden cardiac deathandout-of-hospital cardiac arrest(OHCA). Currently, insufficient data exist on outcome, causes and circumstances ofOHCAof ACHD patients resuscitated for OHCA. We investigate these parameters in ACHD patients in comparison to OHCA in the general population. Methods and results We identified ACHD patients with OHCA by linking data from a Dutch nationwide registry of ACHD patients (CONCOR, n = 15,727), and ARREST, a cohort of OHCA cases (n = 17,868). 62 ACHD patients with OHCA were identified.Ventricular septal defect(n = 11),bicuspid aortic valve(n = 10) andatrial septal defect(n = 8) were the most common diagnoses. We included OHCA cases from the general population as controls. ACHD patients were younger than controls (n = 11,624) at the time of OHCA (47 (SD ± 17) years vs. 66 (SD ± 15) years, respectively, p ischemia. Survival was better in ACHD patients than in controls (44% vs. 19%, p resuscitation, public location and shockable rhythm. Conclusions OHCA in ACHD patients occurs at young age, is rarely caused by ischemia and occurs mainly in patients with simple congenital defects.Risk stratificationefforts should therefore not be restricted to ACHD patients with severe congenital defects.

Aims To assess medication use in adult congenital heart disease (ACHD) patients compared to the age- and sex-matched general population, identify patterns of pharmacotherapy, and analyse associations between pharmacotherapy and adverse outcomes in ACHD. Methods and results Data of 14 138 ACHD patients from the CONCOR registry [35 (24–48) years, 49% male] and age- and sex-matched referents (1:10 ratio) were extracted from the Dutch Dispensed Drug Register for the years 2006–14. Adult congenital heart disease patients had more cardiovascular and non-cardiovascular drugs than referents (median 3 vs. 1, P Conclusion Both cardiovascular and non-cardiovascular medication use is high in ACHD with twice as much polypharmacy compared with the matched general population. Patients with polypharmacy had a four-fold increased risk of mortality and adverse drug events. Recognition of distinct medication patterns can help identify patients at highest risk. Drug regimens need repeating evaluation to assess the appropriateness of all prescriptions. More high-quality studies are needed to improve ACHD care with more evidence-based pharmacotherapy.

Background Reported long-term outcome measures vary greatly between studies in Fontan patients making comprehensive appraisal of mortality hazard challenging. We sought to create a clinical risk score to assist monitoring of Fontan patients in the outpatient setting. Methods A systematic review was conducted to evaluate risk factors for long-term (beyond the first postoperative year) mortality in Fontan patients. Studies were eligible for inclusion if ≥90 patients were included or ≥20 long-term mortalities we reported. Risk factors for long-term mortality were determined. The pooled hazard ratios were used to create components of a clinical score for long-term mortality using meta-analysis techniques. Results Twenty-eight studies were included. The total number of patients was 6707 with an average follow-up of 8.23 ± 5.42 years. There were 1000 deaths. Thirty-five risk factors for late mortality were identified and classified into 9 categories and their relative hazards were used to derive the initial components of a weighted, practical and clinically based Fontan risk score (ranging from 0 to 100). The final score included 8 risk factors: anatomic risk factors, elevated preoperative pulmonary artery pressure, atriopulmonary Fontan, heart failure symptoms, arrhythmia, moderate/severe ventricular dysfunction or atrioventricular valve regurgitation, protein losing enteropathy, and end organ disease (cirrhosis or renal insufficiency). Conclusion In patients with Fontan circulation, the influence of readily available risk factors can be quantified in an integer score to predict long-term mortality. Prospective validation and refinement of this risk score will be undertaken.

Background: Proximal aortic dilatation in certain congenital heart diseases (CHD) prompts concerns about dissection and consideration of prophylactic surgery. To evaluate contemporary prophylactic practice, we determined incidence of aortic dissection and prophylactic surgery in aortopathy-associated CHD, compared to Marfan syndrom (MFS) and controls.Methods and results: We followed patients from the CONCOR adult CHD registry (2002-2015), with a native proximal aorta and aortopathy-associated CHD, comprising bicuspid aortic valve/aortic stenosis ('BAV/AS'; n = 2239) and aortic coarctation/conotruncal defects/univentricular heart/ventricular septal defect ('At-risk CHD'; n = 5439). As reference, we selected MFS (n = 356) and 'Control' (atrial septal defect, pulmonary stenosis; n = 2940) patients. Cumulative incidences of dissection and prophylactic proximal aortic replacement considered competing events - were determined, and compared corrected for age and sex. Median follow-up was 6.7 years. Ten-year dissection-incidence was 0.3% (95% CI: 0.0-0.7) in BAV/AS and 0.2% (0.0-0.3) in At-risk CHD, both significantly lower than in MFS (4.1%; 1.8-6.4) and similar to Controls (0.1%; 0.0-0.3). Ten-year prophylactic-surgery incidence was 9.3% (7.6-11.0) in BAV/AS and 0.7% (0.5-1.0) in At-risk CHD, both significantly lower than in MFS (21.3%; 16.3-26.3) and higher than in Controls (0.1%; 0.0-0.3).Conclusions: In contemporary practice, aortic-dissection incidence is low in adults with aortopathy-associated CHDs, while prophylactic-surgery incidence is high in BAV/AS. To reduce surgical burden, BAV/AS patients could benefit from more individualised prophylactic-surgery algorithms.

Congenital heart disease (CHD) is a prevalent but heterogeneous substrate for infective endocarditis (IE). Risk depends on CHD type, repair status, and the presence of prosthetics. Overall, clinical presentation, diagnosis, microbiology, and treatment of CHD-associated IE do not differ from IE in general. However, right-sided infections are more frequent, and echocardiographic sensitivity may be reduced. IE should be suspected in all patients with CHD with ongoing fever or other suggestive symptoms. Care is best provided in specialized CHD centres. While treatment remains challenging, overall outcome of CHD-associated IE is relatively good. Primary prevention is important in this population, and the value of proper dental health and hygiene should be stressed in patient education.

ObjectiveTo determine factors associated with coronary artery disease (CAD) and ischaemic stroke in ageing adult congenital heart disease (ACHD) patients.MethodsWe performed a multicentre case–control study, using data from the national CONgenital CORvitia (CONCOR) registry to identify ACHD patients within five participating centres. Patients with CAD were matched (1:2 ratio) with ACHD patients without CAD on age, CHD defect group and gender. Patients with ischaemic stroke (or transient ischaemic attack) were matched similarly. Medical charts were reviewed and a standardised questionnaire was used to determine presence of risk factors.ResultsOf 6904 ACHD patients, a total of 55 cases with CAD (80% male, mean age 55.1±12.4 years) and 56 cases with stroke (46% male, mean age 46.9±15.2) were included and matched with control patients. In multivariable logistic regression analysis, traditional atherosclerotic risk factors (hypertension (OR 2.45; 95% CI 1.15 to 5.23), hypercholesterolaemia (OR 3.99; 95% CI 1.62 to 9.83) and smoking (OR 2.25; 95% CI 1.09 to 4.66)) were associated with CAD. In contrast, these risk factors were not associated with ischaemic stroke. In multivariable analysis, stroke was associated with previous shunt operations (OR 4.20; 95% CI 1.36 to 12.9), residual/unclosed septal defects (OR 2.38; 95% CI 1.03 to 5.51) and left-sided mechanical valves (OR 2.67; 95% CI 1.09 to 6.50).ConclusionsTraditional atherosclerotic risk factors were associated with CAD in ACHD patients. In contrast, ischaemic stroke was related to factors (previous shunts, septal defects, mechanical valves) suggesting a cardioembolic aetiology. These findings may inform surveillance and prevention strategies.

Background: Post-repair atrial septal defects (ASD) patients are frequently discharged from follow-up, but the extent of pulmonary symptoms long-term post-repair is unknown. Methods and Results: The national CONgenital CORvitia registry was linked to the national Drug Registry to investigate all ambulatory-dispensed pulmonary inhalants for 2006–2014. ASD patients were compared with age-and sex-matched referents from the general population. A total of 1,959 adult patients (age 42±17 years; 66% female; 1,223 [62%] repaired) were included. Compared with the referents, ASD patients had more inhalant use, even at long-term post-repair follow-up (OR=1.81 [95% CI 1.62–2.03]; P

Background: Adults with congenital heart disease (ACHD) are affected by atrial arrhythmias (AA). To elucidate the impact of AA on prognosis, we aimed to determine the impact of AA on death, heart failure and stroke in ACHD patients in a prospective nationwide clinical registry. Methods: All patients aged >= 18 years included in the CONCOR registry per October 1st 2015 were analysed. Prior AA was defined as atrial fibrillation, atrial flutter or unspecified AA before inclusion in CONCOR and new-onset AA as a first documented AA during follow-up. The outcomes were death, first stroke and first admission for heart failure (HF). Results: The study cohort comprised 14,224 patients (baseline median age 33.6 [IQR 23-47], male 49.5%, AA n = 1501, complex defect 10.3%, repaired defect 58.9%). Median follow-up was 6.5 years [IQR 3-10]. Adjusting for age, sex, repair status and defect severity, patients with prior AA had higher mortality and more HF admissions, but no increased risk of stroke compared to those without AA (HR = 2.11; 95% CI = 1.79-2.49; p

Secundum atrial septal defect (ASDII) is a common congenital heart defect that causes shunting of blood between the systemic and pulmonary circulations. Patients with an isolated ASDII often remain asymptomatic during childhood and adolescence. If the defect remains untreated, however, the rates of exercise intolerance, supraventricular arrhythmias, right ventricular dysfunction and pulmonary arterial hypertension (PAH) increase with patient age, and life expectancy is reduced. Transcatheter and surgical techniques both provide valid options for ASDII closure, the former being the preferred method. With the exception of those with severe and irreversible PAH, closure is beneficial to, and thus indicated in all patients with significant shunts, regardless of age and symptoms. The symptomatic and survival benefits conferred by defect closure are inversely related to patient age and the presence of PAH, supporting timely closure after diagnosis. In this paper we review the management of adult patients with an isolated ASDII, with a focus on aspects of importance to the decision regarding defect closure and medical follow-up.

Item does not contain fulltext AIMS: The identification of sex differences in the prognosis of adults with a secundum atrial septal defect (ASD2) could help tailor their clinical management, as it has in other cardiovascular diseases. We investigated whether disparity between the sexes exists in long-term outcome of adult ASD2 patients. METHODS AND RESULTS: Patients with ASD2 classified as the primary defect were selected from the Dutch national registry of adult congenital heart disease. Survival stratified by sex was compared with a sex-matched general population. In a total of 2207 adult patients (mean age at inclusion 44.8 years, 33.0% male), 102 deaths occurred during a cumulative follow-up of 13 584 patient-years. Median survival was 79.7 years for men and 85.6 years for women with ASD2. Compared with the age- and sex-matched general population, survival was lower for male, but equal for female patients (P = 0.015 and 0.766, respectively). Logistic regression analyses showed that men had a higher risk of conduction disturbances (OR = 1.63; 95% CI, 1.22-2.17) supraventricular dysrhythmias (OR = 1.41; 1.12-1.77), cerebrovascular thromboembolic events (OR = 1.53; 1.10-2.12), and heart failure (OR = 1.91; 1.06-3.43). CONCLUSION: In contrast to women, adult men with an ASD2 have worse survival than a sex-matched general population. Male patients also have a greater risk of morbidity during adult life. Sex disparity in survival and morbidity suggests the need for a sex-specific clinical approach towards these patients.

Aims Adult congenital heart disease (ACHD) predisposes to infective endocarditis (IE). Surgical advancements have changed the ACHD population, whereas associated prosthetic material may constitute additional IE targets. We aimed to prospectively determine contemporary incidence, risk factors, and predictors of IE in a nationwide ACHD cohort, focusing on the presence of prosthetics. Methods and results We identified 14 224 patients prospectively followed in the CONCOR ACHD registry (50.5% female, median age 33.6years). IE incidence was determined using Poisson regression, risk factors and predictors using Cox regression. Overall incidence was 1.33 cases/1000 person-years (124 cases in 93 562 person-years). For risk-factor analysis, presence of prosthetics was forced—as separate time-updated variables for specific prosthetics—into a model with baseline characteristics univariably associated with IE. Valve-containing prosthetics were independently associated with greater risk both short- and long term after implantation [0–6 months: hazard ratio (HR) = 17.29; 7.34–40.70, 6–12 months: HR = 15.91; 6.76–37.45, beyond 12 months: HR = 5.26; 3.52–7.86], non-valve-containing prosthetics, including valve repair, only in the first 6 months after implantation (HR = 3.34; 1.33–8.41), not thereafter. A prediction model was derived and validated using bootstrapping techniques. Independent predictors of IE were baseline valve-containing prosthetics, main congenital heart defect, multiple defects, previous IE, and sex. The model had fair discriminative ability and provided accurate predictions up to 10 years. Conclusions This study provides IE incidence estimates, and determinants of IE risk in a nationwide ACHD cohort. Our findings, essentially informing IE prevention guidelines, indicate valve-containing prosthetics as a main determinant of IE risk whereas other prosthetics, including valve-repair, are not associated with increased risk long term after implantation.

Learning objectives 1. Review the epidemiology, histology, pathophysiology and genetics of congenital heart disease-associated aortopathies and genetic aortopathy syndromes. 2. Review and describe 1. appropriate imaging strategies for diagnosis and follow-up 2. appropriate pharmacotherapeutic strategies and indications for aortic surgery 3. considerations relevant to pregnancy 4. considerations relevant to sports participation in patients with congenital heart disease-associated aortopathy or a genetic aortopathy syndrome. Progressive proximal aortic (ie, aortic root and ascending aortic) dilatation is frequently found in adults with unrepaired or repaired congenital heart disease (CHD). With the growing number of patients with adult CHD (ACHD), physicians increasingly find themselves challenged with the management of proximal aortic dilatation in this heterogeneous patient population. Data on the natural history of CHD-associated aortic dilatation, including risk of dissection, rupture and aortic insufficiency, are scarce. These issues, essentially informing decisions regarding surveillance, pharmacotherapy and surgery, are better defined for genetic aortopathy syndromes, particularly Marfan syndrome (MFS). Moreover, histopathological similarities between CHD-associated and MFS-associated aortopathies have been found.1 This review outlines CHD–associated thoracic aortic dilatation, with special focus on management and indications for surgery. Genetic aortopathy syndromes, including MFS, are addressed as these also require cardiologic follow-up of the proximal aorta. Moreover, data from studies in patients with MFS may inform management of proximal aortic dilatation in the setting of other diseases and syndromes. International guidelines on aortic disease,2–4 ACHD5 6 and valvular heart disease7 are complementary with respect to management of CHD-associated aortic dilatation, and the proposed management represents an integration of these guidelines. The thoracic aorta is subdivided into the root, ascending aorta, arch and descending aorta (figure 1). Aortic diameter is dependent on age and body size, the latter accounting for generally larger diameters in men. The aorta is considered dilated when its diameter exceeds the upper limit of established normal values, and aneurysmal when …

Reported long-term outcome measures vary greatly between studies in Fontan patients making comprehensive appraisal of mortality hazard challenging. We sought to create a clinical risk score to assist monitoring of Fontan patients in the outpatient setting.A systematic review was conducted to evaluate risk factors for long-term (beyond the first postoperative year) mortality in Fontan patients. Studies were eligible for inclusion if ≥90 patients were included or ≥20 long-term mortalities we reported. Risk factors for long-term mortality were determined. The pooled hazard ratios were used to create components of a clinical score for long-term mortality using meta-analysis techniques.Twenty-eight studies were included. The total number of patients was 6707 with an average follow-up of 8.23 ± 5.42 years. There were 1000 deaths. Thirty-five risk factors for late mortality were identified and classified into 9 categories and their relative hazards were used to derive the initial components of a weighted, practical and clinically based Fontan risk score (ranging from 0 to 100). The final score included 8 risk factors: anatomic risk factors, elevated preoperative pulmonary artery pressure, atriopulmonary Fontan, heart failure symptoms, arrhythmia, moderate/severe ventricular dysfunction or atrioventricular valve regurgitation, protein losing enteropathy, and end organ disease (cirrhosis or renal insufficiency).In patients with Fontan circulation, the influence of readily available risk factors can be quantified in an integer score to predict long-term mortality. Prospective validation and refinement of this risk score will be undertaken.

Congenital heart disease (CHD) is a substrate for infective endocarditis (IE). With advancements in surgical care, the CHD population has expanded over the past decades, while use of prosthetic material provides additional IE targets. Studies on CHD-associated IE in the era of commonplace cardiac prosthetics are scarce. Right-sided IE is relatively common in CHD. Streptococcus and Staphylococcus species are the predominant causative agents. The threshold for suspecting IE should be low. Reduced sensitivity of echocardiography, especially in the setting of complex anatomy or prosthetics, may warrant use of alternative imaging modalities. While treatment remains challenging, the outcome of CHD-associated IE is relatively good. Prevention of IE in this population should focus on good dental health and hygiene.

Acquired cardiovascular diseases, such as coronary artery disease (CAD) and cerebrovascular accident (CVA) or transient ischemic attack (TIA), were reported main determinants of mortality in elderly patients with mainly anatomically classified simple congenital heart disease (CHD) [(1,2)][1].