Background—: Adult congenital heart disease (ACHD) patients have ongoing morbidity and reduced long-term survival. Recently, the importance of specialized follow-up at tertiary ACHD centers has been highlighted. We aimed to assess survival prospects and clarify causes of death in a large cohort of patients at a single, tertiary center. Methods and Results—: We included 6969 adult patients (age 29.9±15.4 years) under follow-up at our institution between 1991 and 2013. Causes of death were ascertained from official death certificates. Survival was compared with the expected survival in the general age- and sex-matched population, and standardized mortality rates were calculated. Over a median follow-up time of 9.1 years (interquartile range, 5.2–14.5), 524 patients died. Leading causes of death were chronic heart failure (42%), pneumonia (10%), sudden-cardiac death (7%), cancer (6%), and hemorrhage (5%), whereas perioperative mortality was comparatively low. Isolated simple defects exhibited mortality rates similar to those in the general population, whereas patients with Eisenmenger syndrome, complex congenital heart disease, and Fontan physiology had much poorer long-term survival ( P <0.0001 for all). The probability of cardiac death decreased with increasing patient’s age, whereas the proportion of patients dying from noncardiac causes, such as cancer, increased. Conclusions—: ACHD patients continue to be afflicted by increased mortality in comparision with the general population as they grow older. Highest mortality rates were observed among patients with complex ACHD, Fontan physiology, and Eisenmenger syndrome. Our data provide an overview over causes of mortality and especially the spectrum of noncardiac causes of death in contemporary ACHD patients.

We aimed to investigate the distribution of exercise capacity across the spectrum of adult congenital heart disease (ACHD) using own data and the published experience and to provide diagnosis, gender-, and age- specific reference values. Publications describing exercise capacity in ACHD patients using cardiopulmonary exercise testing (CPET) were identified (n = 2286 patients in 23 papers). In addition, we included 2129 patients who underwent CPET at our own institution. The majority of patients (80%) had reduced peak oxygen uptake (peak VO₂) compared with normal values (defined as <90% of predicted peak VO₂). There were significant differences in peak VO₂ between subgroups of patients, with the lowest values seen in patients with Eisenmenger syndrome and complex heart disease. However, even in patients with simple lesions, peak VO₂ was on average significantly reduced compared with normal values. Based on a large number of observations we herewith provide gender- and age-specific peak VO₂ centile plots for the most common lesions (Tetralogy of Fallot, systemic right ventricle, Ebstein anomaly and Fontan–palliation) and relate disease-specific exercise capacity to that required for specific activities of daily life, sports, and occupations. We provide age-, gender-, and diagnosis-specific data on peak VO₂ levels across the spectrum of ACHD allowing to compare the exercise capacity of individual patients with that of their peer patients. These data should be helpful in interpreting CPET results, guiding therapy, and advising patients on activities of daily life, sports participation, and choice of occupation.

Background— Limited data exist with which to stratify risk in adult congenital heart disease (ACHD). An increased ventilatory response to exercise, expressed as ventilation per unit of carbon dioxide production (V̇ e /V̇ co ₂ slope), is an established predictor of impaired survival in acquired heart disease. We sought to establish the distribution, relation to cyanosis, and prognostic value of the V̇ e /V̇ co ₂ slope across a wide spectrum of ACHD patients. Methods and Results— Five hundred sixty ACHD patients of varying diagnoses and 50 healthy controls underwent cardiopulmonary exercise testing at a single laboratory between 2001 and 2004. Patient age was 33.2±12.9 years (mean±SD). Peak oxygen consumption was 23.5±9.0 mL · kg ⁻¹ · min ⁻¹ . V̇ e /V̇ co ₂ slope for all patients was 36.3±15.3. The slope was raised in all ACHD groups compared with controls and was 73% higher in cyanotic patients. Cyanosis, with or without pulmonary arterial hypertension, was the strongest predictor of abnormal V̇ e /V̇ co ₂ slope. The V̇ e /V̇ co ₂ slope was the most powerful univariate predictor of mortality in the noncyanotic group and the only independent predictor of mortality among exercise parameters on multivariate analysis. In cyanotic patients, no parameter was predictive of death. Conclusions— Ventilatory response to exercise is abnormal across the spectrum of ACHD. Cyanosis is a powerful stimulus for such exaggerated ventilatory patterns irrespective of the presence of pulmonary arterial hypertension. Increased V̇ e /V̇ co ₂ slope is the strongest exercise predictor of death in noncyanotic ACHD patients.

Aims To characterize contemporary Eisenmenger patients at a large centre for adult congenital heart disease, assess survival prospects, and identify predictors of death in this population.

Aims The population of adults with congenital heart disease (ACHD) is growing and ageing. Limited information about the diagnostic spectrum of this emerging population, its resource utilization at tertiary ACHD centres, and especially about prognostic parameters is available. Methods and results Retrospective cohort study on all ACHD patients ≥60 years of age under active follow-up. All-cause mortality was the primary outcome measure. Out of a total population of 7315 ACHD patients, 375 [190 females (50.7%), mean age 64.8 ± 5.9 years] fulfilled the inclusion criteria. During a median follow-up of 5.5 (IQR 3.1–8.6) years, 55 of the 375 patients died. The number of interventions ( P = 0.0006), the number and length of hospitalization ( P < 0.0001), and the number of outpatient clinic visits ( P < 0.0001) were significantly higher in patients ≥60 compared with patients aged between 20 and 60 years. Patients ≥60 years of age with moderate or severe congenital heart defects had worse survival prospects than their age- and gender-matched comparison population. On multivariate Cox analysis, coronary artery disease [hazard ratio (HR): 5.04; 95%CI: 1.88–13.51, P = 0.0014], symptoms of heart failure (HR: 2.36; 95%CI: 1.05–5.29, P < 0.05), NYHA class (HR: 1.96; 95%CI: 1.18–3.26, P < 0.01), and moderate to severe reduction in systemic ventricular systolic function (HR: 1.90; 95%CI: 1.20–2.99, P < 0.001) were the strongest prognostic factors. Conclusion There is a growing number of elderly ACHD patients with high mortality rates and a higher utilization of healthcare resources compared with younger patients. Acquired morbidities, such as coronary artery disease, seem to be key determinants of outcome in this older population in conjunction with the underlying congenital heart disease.

Background—: Sudden cardiac death and life-threatening ventricular arrhythmia remain a concern in adult patients with repaired tetralogy of Fallot. Longitudinal left ventricular (LV) function is sensitive in detecting early myocardial damage and may have prognostic implications in this setting. Methods and Results—: We included 413 tetralogy of Fallot patients (age, 36±13 years; QRS duration, 148±27 milliseconds; LV ejection fraction, 55±10%). A composite end point of sudden cardiac death/life-threatening ventricular arrhythmia (sustained ventricular tachycardia, resuscitated sudden cardiac death, or appropriate implantable cardioverter-defibrillator discharge) was used. During a median follow-up of 2.9 years, 5 patients died suddenly, 9 had documented sustained ventricular tachycardia, and another 5 had appropriate implantable cardioverter-defibrillator shocks. On univariate Cox analysis, QRS duration (hazard ratio [HR], 1.02 per 1 ms; P =0.046), right atrial area (HR, 1.05 per 1 cm ² ; P =0.02), right ventricular fractional area change (HR, 0.94 per 1%; P =0.02), right ventricular outflow tract diameter (HR, 1.08 per 1 mm; P =0.01), mitral annular plane systolic excursion (HR, 0.84 per 1 mm; P =0.03), and LV global longitudinal 2-dimensional strain (HR, 0.87 per 1%; P =0.03) were related to the combined end point. On bivariable analysis, mitral annular plane systolic excursion and LV global longitudinal 2-dimensional strain were related to outcome independently of QRS duration ( P =0.002 and P =0.01, respectively). In addition, a combination of echocardiographic variables, including right atrial area, right ventricular fractional area change, and LV global longitudinal 2-dimensional strain or mitral annular plane systolic excursion, was also found to be significantly related to outcome ( P <0.001; c statistic, 0.70). Conclusions—: LV longitudinal dysfunction was associated with greater risk of sudden cardiac death/life-threatening ventricular arrhythmias. In combination with echocardiographic right heart variables, also available from routine echocardiography, these measures provide important outcome information and should be considered a useful adjunct to established markers such as QRS duration in the estimation of prognosis in this challenging population.