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This study aims to clarify the relation of development of aortopulmonary collateral arteries (APCs) with anatomical sub-types and the shunt types at Norwood procedure in patients with hypoplastic left heart syndrome (HLHS). A total of 140 patients with HLHS who completed 3 staged palliation between 2003 and 2019 were included. Incidence of APCs and corresponding interventions were examined using angiogram by cardiac catheterization, with respect to the anatomical sub-types and shunt types. Totally, APCs were observed in 87 (62%) of the patients; pre-stage II in 32 (23%), pre-stage III in 64 (46%), and after stage III in 40 (29%). The incidence of APCs before stage II was significantly higher in patients with aortic atresia/mitral atresia (AA/MA) compared with other sub-types (P = 0.022). Patients with right ventricle to pulmonary artery conduit (RVPAC) had a higher incidence of APCs originating from the descending aorta, compared with those with modified Blalock-Taussig shunt (20% vs 2%, P = 0.002). Interventions for APCs were performed in 58 (41%) patients; before stage II in 10 (7%), after stage II in 7 (5%), before stage III in 22 (16%), and after stage III in 32 (23%). Patients with AA/MA had more interventions before stage II (P = 0.019), and patients with aortic stenosis/mitral stenosis (AS/MS) had a lower incidence of interventions after stage III (P = 0.047). More than half of the patients with HLHS developed APCs. Before stage II, patients with AA/MA sub-type had a higher incidence of APCs, and those with RVPAC had significantly more APCs from the descending aorta. [Display omitted] [ABSTRACT FROM AUTHOR]

This study was intended to determine the impact of extracardiac anomalies on outcomes in patients with functional single ventricle who underwent staged palliation. We reviewed medical records of patients who underwent first-stage palliation at our center between 2001 and 2020. The prevalence and type of extracardiac anomalies were evaluated, and their impact on outcomes during staged palliation was analyzed. Among 602 patients who underwent first-stage palliation, 81 (14%) patients had associated with extracardiac anomalies. They were more frequently associated with prematurity (P =.03) and low birth weight below 2.5 kg (P <.01). Mortality between first-stage palliation and stage II was similar in patients with and without extracardiac anomalies (24.7% vs 17.1%, P =.10). However, mortality between stage II and stage III was significantly higher in patients with extracardiac anomalies compared with those without (22.2% vs 12.5%, P =.02). Mortality after stage III was also higher in patients with extracardiac anomalies compared with those without (4.9% vs 1.5%, P =.04). In the subgroup analysis of 81 patients with extracardiac anomalies, renal anomalies were identified as a significant risk factor for mortality (P =.03, hazard ratio 2.44). The incidence of extracardiac anomalies in this study was 14%, and patients with extracardiac anomalies were highly associated with prematurity and low birth weight. Presence of extracardiac anomalies was associated with higher mortality between stage II and stage III palliation and after stage III phase, but not before stage II. Among extracardiac anomalies, renal anomalies were identified as a risk factor for mortality. [ABSTRACT FROM AUTHOR]

Total anomalous pulmonary venous connection (TAPVC) with a functional single ventricle is a risk factor for mortality during staged palliation. This study aimed to assess TAPVC's impact on staged palliation outcomes. In a total of 602 patients with a functional single ventricle who underwent stage 1 palliation (S1P) at our center between 2001 and 2020, 39 (6.5%) patients were associated with TAPVC. Median age at S1P was 12.0 (interquartile range, 7-21) days with a body weight of 3.1 (interquartile range, 2.8-3.6) kg. Outcomes during staged palliation were compared with the remaining 563 patients without TAPVC. Risk factors for mortality were identified using a Cox proportional hazards regression model. Primary diagnosis in functional single-ventricle patients with TAPVC included hypoplastic left heart syndromes (n = 13), unbalanced atrioventricular septal defects (n = 12) tricuspid atresias (n = 2), double inlet left ventricle (n = 1), and others (n = 11). Types of TAPVC were supracardiac (n = 21), cardiac (n = 10), infracardiac (n = 6), and mixed (n = 2). Pulmonary venous obstruction (PVO) was associated in 19 (49%) patients. S1Ps included Norwood (n = 13), aortopulmonary shunt (n = 21), and pulmonary artery banding (n = 5). Thirty-day mortality after S1P was significantly increased in patients with TAPVC vs without TAPVC (43.6% vs 16.3%; P <.001). After bidirectional cavopulmonary shunt and total cavopulmonary connection procedures, mortality was low in both groups, and no statistically significant differences were found. Correction of TAPVC at the time of S1P was not found to be a significant risk factor in univariable Cox regression analysis. In univariate and multivariate analysis, PVO was identified as an independent risk factor for mortality in patients with TAPVC (P <.001). Overall survival is lower in TAPVC single-ventricle patients than in non-TAPVC patients. Most deaths after S1P were associated with TAPVC, but not after S2P. PVO is a mortality risk factor in TAPVC patients. [ABSTRACT FROM AUTHOR]

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The study objective was to compare the results after Norwood procedure between modified Blalock-Taussig shunt (MBTS) and right ventricle-to-pulmonary artery conduit (RVPAC) according to Sano in patients with hypoplastic left heart syndrome (HLHS) and aortic atresia (AA). A total of 146 neonates with HLHS and AA who underwent the Norwood procedure at our institution between 2001 and 2020 were divided into 2 groups according to shunt type (MBTS or RVPAC). Survival after the Norwood procedure was compared between the groups. Longitudinal right ventricular and tricuspid valve function in each group were evaluated using cubic splines method. RVPAC was performed in 103 patients and MBTS in 43 according to surgeon preference. There were no differences in the 30-day mortality rates (16.5% vs 16.3%, P  = 0.973). Survival at 0.5, 1 and 3 years was 79.6%, 74.6%, and 68.9% in RVPAC and 66.8%, 64.3%, and 58.5% in MBTS (P  =  0.293). Among 23 patients undergoing tricuspid valve procedure, different mechanisms of tricuspid regurgitation were observed between the groups. Longitudinal analysis revealed greater prevalence of late right ventricular dysfunction in RVPAC patients. In 77 patients who completed Fontan procedure, the postoperative N-terminal pro B-type natriuretic peptide value was significantly higher in RVPAC vs MBTS (554 vs 276 ng/L, P  =  0.007). No survival advantage of RVPAC over MBTS was observed in neonates with HLHS and AA undergoing the Norwood procedure. Longitudinal analysis demonstrated a greater prevalence of right ventricular dysfunction and higher N-terminal pro B-type natriuretic peptide values during late follow-up in patients with RVPAC. [ABSTRACT FROM AUTHOR]

This study sought to determine long-term outcomes of a primary Damus-Kaye-Stansel (DKS) procedure in patients with a functional single ventricle and to compare the results with those of our historical control subjects who underwent pulmonary artery banding before the DKS procedure. The study reviewed the medical records of all patients who underwent the DKS procedure at the German Heart Center of Munich, Germany between December 1994 and December 2019. The DKS procedure was performed as initial palliation in 52 patients (primary DKS group) and as staged palliation after pulmonary artery banding in 24 patients (staged DKS group). The median follow-up period after the DKS procedure was 8.9 years in the primary DKS group and 8.0 years in the staged DKS group. The survival rates at 10 years after the DKS procedure were 89% in the primary DKS group and 68% in the staged DKS group (log-rank P = 0.04). Before total cavopulmonary connection, the pressure gradient through the systemic ventricular outflow tract was significantly lower in the primary DKS group than in the staged DKS group (P <.001). At last follow-up echocardiography, reduced ventricular function was observed in 1 patient in the primary DKS group and in 7 patients in the staged DKS group (P <.001). The degree of neoaortic regurgitation was significantly higher in the staged DKS group than in the primary DKS group (P <.001). A primary DKS procedure in patients with a functional single ventricle and potential systemic ventricular outflow tract obstruction is recommended to obtain favorable long-term survival with preserved ventricular function and competent semilunar valve function. [ABSTRACT FROM AUTHOR]

Thrombus formation is a feared complication following bidirectional cavopulmonary shunt (BCPS). We aimed to investigate the effect of thrombus formation on outcome. BCPS was performed in 525 patients at our center between 1998 and 2018. The impacts of thrombus formation on survival and probability of Fontan completion were analyzed, and risk factors for thrombus formation were examined. Thrombus formation occurred in 30 patients (5.7%). Compared with the remaining 495 patients, there was no significant difference in the median age at BCPS (4.9 vs 4.7 months; P = 0.587). However, unbalanced atrioventricular septal defects (17 vs 5%; P = 0.008) and preoperative ventricular dysfunction (23.3 vs 8%; P = 0.004) were more frequent in patients who developed a thrombus. Thrombolytic therapy was performed in all patients and surgical thrombus removal was required in 13 patients. In-hospital mortality was higher in patients with thrombus (30.0 vs 2.2%; P < 0.001). Of 505 hospital survivors, an estimated survival at 1 year after hospital discharge following BCPS was 84.4% (95% CI, 76.1-92.7%) in patients with thrombus and 96.8% (95% CI, 96.0-97.6%) in those without (P < 0.001). Cumulative incidence of Fontan completion at 3 years after BCPS was 52.8% (95% CI, 30.3-75.2%) in patients with thrombus and 90.1% (95% CI, 87.2-92.9%) in those without (P = 0.004). Higher left atrial pressure (OR = 1.165; P = 0.029) and longer cardiopulmonary bypass time (OR = 1.013, P = 0.001) at BCPS were independent risk factors for thrombus formation after BCPS. Thrombus formation after BCPS poses a significant risk for survival and Fontan completion. Preoperative higher left atrial pressure and longer cardiopulmonary bypass time are significant risk factors. [ABSTRACT FROM AUTHOR]

Our aim was to evaluate the results of stage 2 palliation by means of bidirectional cavopulmonary shunt (BCPS) and to identify risk factors for failed Fontan completion. Between 1998 and 2018, BCPS was performed on 525 patients with functional single ventricle. Patient demographics, surgical data, and echocardiographic and cardiac catheterization measurements were analyzed, and outcomes after BCPS were evaluated. The median age at BCPS was 4.7 months (interquartile range, 3 to 7.4). Hypoplastic left heart syndrome was the most frequent diagnosis. The median follow-up after BCPS was 3.4 years (interquartile range, 1.5 to 8.7); 407 patients underwent the Fontan procedure, 50 were waiting for Fontan completion, 47 died, 6 were considered not suitable for Fontan completion, and 15 were lost to follow-up. Of the 407 patients who underwent Fontan completion, there were 5 early deaths. Freedom from failed Fontan completion (mortality before, unsuitability for, and early mortality after the Fontan procedure) at 1, 2, and 3 years was 91.9%, 87.3%, and 86.1%, respectively. Risk factor analysis for failed Fontan completion revealed hypoplastic left heart syndrome (hazard ratio [HR] 4.1, P =.001), unbalanced atrioventricular septal defect (HR 10.1, P <.001), higher pulmonary artery pressure (HR 1.1, P =.040), and reduced ventricular function (HR 4.2, P =.001) as risks. Stage 2 palliation can be performed with minimal risk and provides excellent subsequent Fontan completion. Hypoplastic left heart syndrome, unbalanced atrioventricular septal defect, high pulmonary artery pressure, and reduced ventricular function at the time of BCPS were identified as risk factors for failure to successfully complete the Fontan procedure. [Display omitted] [ABSTRACT FROM AUTHOR]

Objective: To improve health-related physical fitness (HRPF) (primary outcome) and health-related quality of life (HRQoL) with a web-based motor intervention program in pediatric patients with congenital heart disease (CHD).Study Design: Overall, 70 patients (13.0 ± 2.6 years; 34% girls) aged 10-18 years with moderate or complex CHD severity were randomly allocated 1:1 to an intervention or control group. The intervention group trained 3 times per week for 20 minutes in a web-based exercise program over a period of 24 weeks. The control group followed lifestyle per usual. At baseline and follow-up HRPF was assessed via 5 tasks of the FITNESSGRAM and converted to a HRPF z score. HRQoL was assessed with KINDL self-report questionnaire.Results: In total, 61 patients completed the follow-up. There was no change in total HRPF z score (intervention group: 0.14 ± 0.38 vs control group: 0.09 ± 0.38, P = .560) and total HRQoL (intervention group: -1.73 ± 8.33 vs control group: 1.31 ± 7.85, P = .160) after the 24-week web-based exercise intervention. This was true for all subcategories of HRPF and HRQoL. There were no adverse events associated with the web-based exercise intervention.Conclusions: We found that 24 weeks of web-based exercise intervention with an aimed volume of 60 minutes of exercise per week was safe but did not improve HRPF and HRQoL in children with moderate or complex CHD.Trial Registration: Clinicaltrials.gov: NCT03488797. [ABSTRACT FROM AUTHOR]

Objective: To compare daily physical activity of children with congenital heart disease (CHD) with healthy peers measured using wearables bracelets in a large cohort. Additionally, subjectively estimated and objectively measured physical activity was compared.Study Design: From September 2017 to May 2019, 162 children (11.8 ± 3.2 years; 60 girls) with various CHD participated in a self-estimated and wearable-based physical activity assessment. Step-count and moderate-to-vigorous physical activity were recorded with the Garmin vivofit jr. for 7 consecutive days and compared with a reference cohort (RC) of 96 healthy children (10.9 ± 3.8 years; 49 girls).Results: Children with CHD were active and 123 (75.9%) achieved 60 minutes physical activity on a weekly average according to the World Health Organization criteria as 81 (84.3%) of the healthy peers did (P = .217). After correction for age, sex, and seasonal effects, only slightly lower step count (CHD: 10 206 ± 3178 steps vs RC: 11 142 ± 3136 steps; P = .040) but no lower moderate-to-vigorous physical activity (CHD: 80.5 ± 25.6 minutes/day vs RC: 81.5 ± 25.3 minutes/day; P = .767) occurred comparing CHD with RC. In children with CHD higher age (P = .004), overweight or obesity (P = .016), complex severity (P = .046), and total cavopulmonary connection (P = .027) were associated with not meeting World Health Organization criteria. Subjective estimation of daily moderate-to-vigorous physical activity was fairly correct in half of all children with CHD.Conclusions: Even though the majority is sufficiently active, physical activity needs to be promoted in overweight or obese patients, patients with complex CHD severity, and in particular in those with total cavopulmonary connection. [ABSTRACT FROM AUTHOR]

The aim of this study was to report long-term outcomes after percutaneous pulmonary valve implantation (PPVI). Excellent short- and mid-term results after PPVI for right ventricular outflow tract (RVOT) dysfunction have been reported. Data on long-term results after PPVI are scarce. All 226 patients treated with PPVI at a single institution were prospectively enrolled in a database and included in the study. Follow-up information regarding outcomes was collected. Risk-factor analysis for death and reinterventions was performed. Follow-up data (up to 11 years) were available in 96% of the patients. At the end of the study period, 219 patients (92.8%) still lived with the initially implanted valve. Seven patients died, 2 of them from procedure-related complications. Seventeen patients (7.2%) needed valve replacement surgically (n = 11) or percutaneously (n = 6) for infectious endocarditis (n = 10) or valve degeneration (n = 7). In the remaining patients, the valves retained excellent function, and right ventricular dimensions and exercise capacity improved. A post-interventional RVOT gradient >15 mm Hg was a risk factor for death (hazard ratio: 7.57; 95% confidence interval: 1.26 to 45.38; p = 0.027) and for valve failure (hazard ratio: 3.76; 95% confidence interval: 1.43 to 9.93; p = 0.007). The best outcome was achieved with RVOT pre-stenting and a post-interventional gradient <15 mm Hg, resulting in an estimated event-free survival rate of 88% at 10 years. Patients after PPVI have excellent long-term outcomes. Right ventricular volumes, function, and exercise capacity improve significantly. A residual RVOT gradient <15 mm Hg was associated with the best outcome. [ABSTRACT FROM AUTHOR]

Heart failure with reduced ejection fraction (HFrEF) is common in the developed world and results in significant morbidity and mortality. Accurate risk assessment methods and prognostic variables are therefore needed to guide clinical decision making for medical therapy and surgical interventions with the ultimate goal of decreasing risk and improving health outcomes. The purpose of this review is to examine the role of cardiopulmonary exercise testing (CPET) and its most commonly used ventilatory gas exchange variables for the purpose of risk stratification and management of HFrEF. We evaluated five widely studied gas exchange variables from CPET in HFrEF patients based on nine previously used systematic criteria for biomarkers. This paper provides clinicians with a comprehensive and critical overview, class recommendations and evidence levels. Although some CPET variables met more criteria than others, evidence supporting the clinical assessment of variables beyond peak V̇O2 is well-established. A multi-variable approach also including the V̇E-V̇CO2 slope and EOV is therefore recommended. [ABSTRACT FROM AUTHOR]

We sought to identify the impact of pulmonary artery size on outcomes after nonfenestrated total cavopulmonary connection. In a subgroup of patients with right-sided bidirectional cavopulmonary shunt, the impact of each branch pulmonary artery size was individually determined. The medical records of all patients who underwent nonfenestrated total cavopulmonary connection between 2009 and 2021 were reviewed. The pulmonary artery index was calculated using angiography before the operation. A total of 247 patients were included in this study. A right-sided bidirectional cavopulmonary shunt was performed in 217 patients (88%). Median pulmonary artery index was 162 (133-207) mm2/m2 before total cavopulmonary connection. Chylothorax occurred in 55 patients (22%). Pulmonary artery index was an independent factor for chylothorax (odds ratio, 0.98, 95% confidence interval, 0.97-0.99, P <.001) with a cutoff value of 170 mm2/m2. In a subgroup of patients with right-sided bidirectional cavopulmonary shunt, the left pulmonary artery index was identified as an independent risk factor for longer stay in the intensive care unit (coefficient B –0.02, 95% confidence interval, –0.04 to –0.002, P =.034) and for adverse events (hazard ratio, 0.98, 95% confidence interval, 0.96-0.99, P =.011) with a cutoff value of 56 mm2/m2. The pulmonary artery index is significantly associated with the occurrence of chylothorax after nonfenestrated total cavopulmonary connection with a cutoff value of 170 mm2/m2. In patients with right-sided bidirectional cavopulmonary shunt, left pulmonary artery index has a significant predictive value for longer stay in the intensive care unit and adverse events with a cutoff value of 56 mm2/m2. [Display omitted] [ABSTRACT FROM AUTHOR]

Background Patients with congenital heart disease and native or palliated conditions are at risk to develop pulmonary hypertension (PH) in later life. Screening for PH is currently performed by regular echocardiographic follow-up, which appears to be difficult in several congenital conditions. This study evaluated the screening for PH in congenital heart disease by cardiopulmonary exercise testing (CPET). Methods We analyzed our database including all patients with congenital heart disease referred for CPET in our institution from June 2001 to September 2013 and identified 683 patients who had an accompanied heart catheterization less than 6 month after CPET. Those 130 patients with proven PH were compared with the other 563 patients with congenital heart disease but without PH. Results Peak oxygen uptake was the most discriminative variable, showing two thresholds at 16.3 mL/min per kg and 25.2 mL/min per kg. The highest specificity of 95% for PH was found in patients with a peak oxygen uptake of 16.3 mL/min per kg or less and a breathing reserve of 37.4% or less. In patients with a peak oxygen uptake exceeding 16.3 mL/min per kg, there was a high specificity of 86.3% but a low sensitivity of 53.1%. With 25.2 mL/min per kg as the threshold, the sensitivity for PH was only 10.0%. Conclusions Detection of PH in patients with congenital heart disease by CPET is difficult because of many falsely positive tests. However, a peak oxygen uptake higher than 25.2 mL/min per kg makes the diagnosis of PH unlikely. [ABSTRACT FROM AUTHOR]

Objectives This study sought to investigate the impact of tricuspid regurgitation (TR) on right ventricular function after percutaneous pulmonary valve implantation (PPVI). Background PPVI provides a less invasive alternative to surgery in patients with right ventricular-to-pulmonary artery (RV-PA) conduit dysfunction. Recovery of the right ventricle has been described after PPVI for patients with pulmonary stenosis and for those with pulmonary regurgitation. Additional TR enforces RV dysfunction by supplemental volume overload. Limited data are available on the potential of the right ventricle to recover in such a specific hemodynamic situation. Methods In a matched cohort study, we compared patients who underwent PPVI with additional TR with those without TR. Results The degree of TR improved in 83% of the patients. In our patients (n = 36) exercise capacity and right ventricular volume index improved similarly 6 months after PPVI in patients with and without important TR. None of them had significant TR in the long-term follow-up of median 78 months. Conclusions PPVI improves not only RV-PA-conduit dysfunction, but also concomitant TR. In patients with a dysfunctional RV-PA conduit and TR, the decision whether to fix TR should be postponed after PPVI. [ABSTRACT FROM AUTHOR]

Background After the Fontan-Björk modification for tricuspid atresia, some patients show pulsatile systolic pulmonary flow. We compared the hemodynamic findings and the clinical presentation of patients with and without pulsatile systolic flow after atrioventricular connection. Methods According to the pulmonary flow pattern by pulsed-wave Doppler assessment of transthoracic echocardiography, 41 patients after atrioventricular connection were divided into two groups: patients who showed dominant pulsatile systolic pulmonary flow (group P, n = 11), and patients who did not (group N, n = 30). Results Mean follow-up time was 27.8 ± 4.7 years in group P and 25.3 ± 3.8 years in group N ( p = 0.1). Patients in group P had significantly less frequently catheter ablation procedures for tachyarrhythmia (9% versus 50%, p = 0.03). No patient in group P had had cardiac decompensation, whereas 7 patients (23%) in group N had had an episode of cardiac decompensation ( p = 0.08). Cardiopulmonary exercise testing revealed that patients in group P showed higher oxygen uptake compared with patients in group N (25.0 ± 7.3 versus 19.6 ± 6.0 mL · kg −1 · min −1 , p = 0.03). Patients in group P showed higher systolic pulmonary artery pressure (21.3 ± 8.4 versus 16.8 ± 4.5 mm Hg, p = 0.05), higher right ventricular end-diastolic volume index (88.6 ± 30.2 versus 50.3 ± 28.5 mL · L −1 · m −2 , p = 0.03), and higher right ventricle to left ventricle ratio of end-diastolic volume index (1.4 ± 0.6 to 0.7 ± 0.3, p = 0.01). Conclusions Patients with pulsatile systolic flow in the pulmonary artery had better hemodynamic and better exercise performance compared with patients without pulsatile systolic flow after atrioventricular connection. A sufficient volume and function of the right ventricle is a prerequisite to create pulsatile systolic flow. [ABSTRACT FROM AUTHOR]

Objective Patients with repaired tetralogy of Fallot (ToF) have an increased long-term risk of cardiovascular morbidity and mortality. Risk stratification in this population is difficult. Initial evidence suggests that cardiopulmonary exercise testing (CPET) may be helpful to risk-stratify patients with repaired ToF. Methods and results We studied 875 patients after surgical repair for ToF (358 females, age 25.5 ± 11.7 year, range 7–75 years) who underwent CPET between 1999 and 2009. During a mean follow-up of 4.1 ± 2.6 years after CPET, 30 patients (3.4%) died or had sustained ventricular tachycardia (VT). 225 patients (25.7%) had other cardiac related events (emergency admission, surgery, or catheter interventions). On multivariable Cox regression-analysis, %predicted peak oxygen uptake ( V ˙ O 2 %) (p = 0.001), resting QRS duration (p = 0.030) and age (p < 0.001) emerged as independent predictors of mortality or sustained VT. Patients with a peak V ˙ O 2 ≤ 65% of predicted and a resting QRS duration ≥ 170 ms had a 11.4-fold risk of death or sustained VT. Ventilatory efficiency expressed as V ˙ E / V ˙ C O 2 slope (p < 0.001), peak V ˙ O 2 % (p = .001), QRS duration (p = .001) and age (p = 0.046) independently predicted event free survival. V ˙ E / V ˙ C O 2 slope ≥ 31.0, peak V ˙ O 2 % ≤ 65% and QRS duration ≥ 170 ms were the cut-off points with best sensitivity and specificity to detect an unfavorable outcome. Conclusions CPET is an important predictive tool that may assist in the risk stratification of patients with ToF. Subjects with a poor exercise capacity in addition to a prolonged QRS duration have a substantially increased risk for death or sustained ventricular tachycardia, as well as for cardiac-related hospitalizations. [ABSTRACT FROM AUTHOR]

Background: Anomalous left coronary artery from the pulmonary artery (ALCAPA) is a very rare disease. Accordingly, the incidence and distribution of myocardial scarring in long-term follow-up of patients after successful surgery of ALCAPA is unknown. Therefore, the aim of this study was to describe the distribution of left ventricular (LV) myocardial scarring by late gadolinium enhancement magnetic resonance in these patients. Methods: Fourteen consecutive patients over 12 years of age with ALCAPA without any further cardiac abnormalities, and after corrective surgery at our center, participated prospectively in this study. All patients underwent cardiac magnetic resonance including LV function analysis and late gadolinium enhancement magnetic resonance. Of these 14 patients, 12 patients also underwent cardiopulmonary exercise testing. Results: Four patients had no evidence of myocardial scarring. The median extent of the myocardial scar of all patients by late gadolinium enhancement magnetic resonance, expressed as a percentage of the total LV myocardial volume, was only 2% (range, 0% to 11%). Median LV ejection fraction was 0.55 (range, 0.31 to 0.73). Median LV end-diastolic volume indexed to body surface area was 84 mL/m2 (39 to 120 mL/m2). Median peak oxygen uptake during cardiopulmonary exercise was 24 mL · kg−1 · minute−1 (range, 19 to 51 mL/kg/minute). Conclusions: We conclude that despite often severely compromised LV function and evidence of scarring before corrective surgery of patients with ALCAPA, in long-term follow-up scar tissue is relatively scarce. [ABSTRACT FROM AUTHOR]

Abstract: Objective: Even after repair of aortic coarctation without restenosis there is a high incidence of arterial hypertension. This study was performed to assess the contribution of several inherited gene polymorphisms, which are known to be related to essential hypertension. Patients and methods: 122 patients aged 17–72years, 46 women, and 2–27years after repair of isolated aortic coarctation without restenosis were investigated. Genomic polymorphism of angiotensin converting enzyme (ACE I/D), angiotensinogen (AGT, c.704C>T), angiotensin II receptor type 1 (AGTR1, c.1166A>C), aldosterone synthase (CYP11B2, c.−344C>T), endothelin 1 (EDN1, EDN1/ex5-c.5665G>T), G protein (GNB3, c.825C>T), G protein-coupled receptor kinase 4 (GRK4, c.679C>T), fibrillin 1 (FBN1, VNTR(TAAA)) and two polymorphisms each of the ß1 adrenoreceptor (ADRB1, c.145G>A and c.1165C>G), ß2 adrenoreceptor (ADRB2, c.46A>G and c.79C>G), and endothelial NO synthase (NOS3, intron 4 I/D and NOS3, c.894G>T) were determined by PCR amplification and fragment length analysis. Patients were classified “normotensive”, if they were not on antihypertensive drugs and showed normal blood pressure both on ambulatory measurement and exercise test. Results: None of the investigated genomic polymorphism could be related to hypertension. Only patients with the ACE I/I genotype had a less pronounced nocturnal dipping and patients with a ADRB1 c.1165 C/C genotype had a higher systolic and mean blood pressure at night. Conclusions: Development of late hypertension after aortic coarctation repair could not be related to the investigated genomic polymorphism. The correlation of the ACE I/D and the ADRB1 c.1165C>G polymorphism to nocturnal dipping and blood pressure at nighttime needs further confirmation. [Copyright &y& Elsevier]

Objective: The purpose of this longitudinal study was to assess improvements in exercise performance and quality of life in patients with Ebstein anomaly after surgical intervention. Methods: In 21 patients with Ebstein anomaly (between 6 and 59 years of age; 16 female, 5 male) who underwent surgery for tricuspid regurgitation and, if present, closure of an interatrial shunt, a cardiopulmonary exercise test and a quality-of-life assessment by the Medical Outcomes Study 36 item short form was performed prior to and 6 to 18 months after surgery. Results: After surgery, peak oxygen uptake increased from 68.4% of predicted to 77.3% of predicted (P = .009), and ventilatory efficiency (Ve/Vco 2 slope) improved from 32.5 to 29.3 (P = .001). In 14 patients with additional interatrial shunt closure, oxygen saturation improved from 95% to 99% at rest (P = .003) and from 88% to 99% under peak exercise (P = .003). Improvements in Ve/Vco 2 slope were similar in patients who had undergone primary surgery (P = .005) or reoperation (P = .018). Increase in exercise capacity was also similar in both groups but failed significance in both (primary surgery, P = .064; reoperation, P = .063). There was no difference between tricuspid valve repair and replacement in the short-term follow-up. Self-estimated quality of life was fairly good prior to and after surgery. Only in the single question about health transition at follow-up did the patients confirm an improved situation after surgery. Conclusions: Patients with Ebstein anomaly and severe tricuspid regurgitation draw clinical benefit from surgical intervention as measured on exercise testing. This holds true for primary surgery and for reoperation. [Copyright &y& Elsevier]

Background To investigate aortic dimensions in women with Turner syndrome (TS) in relation to aortic valve morphology, blood pressure, karyotype, and clinical characteristics. Methods and results: A cross sectional study of 102 women with TS (mean age 37.7; 18-62 years) examined by cardiovascular magnetic resonance (CMR- successful in 95), echocardiography, and 24-hour ambulatory blood pressure. Aortic diameters were measured by CMR at 8 positions along the thoracic aorta. Twenty-four healthy females were recruited as controls. In TS, aortic dilatation was present at one or more positions in 22 (23%). Aortic diameter in women with TS and bicuspid aortic valve was significantly larger than in TS with tricuspid valves in both the ascending (32.4 ± 6.7 vs. 26.0 ± 4.4 mm; p < 0.001) and descending (21.4 ± 3.5 vs. 18.8 ± 2.4 mm; p < 0.001) aorta. Aortic diameter correlated to age (R = 0.2 - 0.5; p < 0.01), blood pressure (R = 0.4; p < 0.05), a history of coarctation (R = 0.3; p = 0.01) and bicuspid aortic valve (R = 0.2-0.5; p < 0.05). Body surface area only correlated with descending aortic diameter (R = 0.23; p = 0.024). Conclusions Aortic dilatation was present in 23% of adult TS women, where aortic valve morphology, age and blood pressure were major determinants of the aortic diameter. [ABSTRACT FROM AUTHOR]

Objectives: The goal of this study was to assess the prognostic value of the cardiopulmonary exercise test (CPET) in patients who received a Mustard and Senning (M/S) operation. Background: Patients who received an M/S operation have increased long-term risk of cardiovascular morbidity and mortality. Limited information is available on how to stratify risk in this population. Methods: Between 1996 and 2007, 274 adults (age 26.3 ± 8.9 years, range 16 to 50 years) who had received a Mustard (n = 144) or Senning (n = 130) operation in infancy were studied with CPET. During a follow-up of 3.9 ± 2.3 years (range 0.2 to 10.8 years), 12 patients died at an age of 36 ± 14 years, and 46 patients required a cardiac-related emergency (<24 h from the onset of symptom/condition) hospital admission at an age of 30 ± 11 years. Results: At multivariate Cox analysis, the slope of ventilation per unit of carbon dioxide output (VE/VCO2 slope) (hazard ratio: 1.088, p < 0.0001) and percentage of predicted peak oxygen uptake (Vo 2%) (hazard ratio: 0.979, p = 0.0136) were the strongest predictors of death/cardiac-related emergency hospital admission among demographic, clinical, and exercise variables. A VE/VCO2 slope ≥35.4 (hazard ratio: 10.7, 95% confidence interval [CI]: 7.8 to 24.6), and a peak Vo 2% ≤52.3% (hazard ratio: 3.4, 95% CI: 2.5 to 8.2) were associated with an increased 4-year risk of death/cardiac-related emergency hospital admission. Patients who had both a VE/VCO2 slope ≥35.4 and a peak Vo 2% ≤52.3% of predicted value were at highest risk (4-year event rate: 78.8%). Conclusions: CPET provides important prognostic information in adults with M/S operation. Subjects with enhanced ventilatory response to exercise or those with poor exercise capacity have a substantially higher 4-year risk of death/cardiac-related emergency hospital admission. [Copyright &y& Elsevier]

There are contradictory reports whether exercise capacity is reduced in patients on long-term follow-up after coarctation repair. Data from unselected patient groups are missing. In a cross-sectional, long-term follow-up study of a tertiary congenital cardiology referral center, 260 patients (30.2 ± 11.4 years old, 84 women), after surgical repair for isolated aortic coarctation (age at surgery 11.5 ± 11.2 years), underwent a symptom-limited exercise test. Peak workload was 180 ± 52 W, significantly less than the age- and height-related reference values (p <0.0005). A peak workload under 80% of expected was found in 200 patients (77%). Exercise performance of the patients was independent from age at surgery, type of surgery, or the systolic brachial-ankle blood pressure difference. The only exercise-limiting factor found was the chronic administration of diuretics to treat hypertension (p = 0.005). Exercise hypertension, defined as a systolic blood pressure >2 SD above the load-dependent reference value, was found in 73 patients (28%). It was independently related to the systolic brachial-ankle blood pressure difference (p <0.0005) and diuretics administration (p = 0.037). In conclusion, most patients after coarctation repair have a reduced exercise performance. This reduction is not related to the surgical results. Particularly, as these patients are at risk of early atherosclerosis, exercise should be promoted as primary prevention after restenosis, aortic or cerebral aneurysms, and severe exercise hypertension are ruled out. [Copyright &y& Elsevier]

Background: Previous studies have shown that older Fontan patients and those with an underlying morphologically right ventricle have lower exercise capacity. We sought to assess the natural history of exercise capacity after the Fontan operation in individual patients, and to identify the factors influencing the rate of decrease of exercise capacity over time. Methods: We studied, longitudinally, 53 Fontan patients with cardiopulmonary exercise tests (average 3.2 ± 1.1 tests for each patient). Age at the first test was 14 ± 6 years. Time intervals between tests ranged from 1 to 16 years (average, 7.7 ± 4.0 years). The morphology of the functionally single ventricle was left in 29 patients (55%) and right in 24 patients (45%). Twenty-two patients had undergone a total cavopulmonary connection (TCPC) at a mean age of 5.9 ± 2.8 years. Thirty-one patients had undergone an atriopulmonary or an atrioventricular connection at an age of 6.9 ± 4.4 years. Exercise capacity was expressed as percentage of predicted peak oxygen uptake (Vo 2). Results: Overall, peak Vo 2 decreased at a rate of −2.6 ± 2.7%/year. Ventricular morphology (r = 0.525, p = 0.0001) and type of Fontan operation (r = 0.381, p = 0.0057) appeared as the only predictors of the rate of decrease of peak Vo 2 at multivariate analysis. Patients with an underlying left ventricular morphology (−1.7 ± 2.0 vs −3.7 ± 3.2%/year, p = 0.005), and those who underwent a TCPC (−1.9 ± 1.8 vs −3.3 ± 3.2%/year, p = 0.042), had the lowest rate of decrease in peak Vo 2. Conclusions: Exercise capacity progressively declines in Fontan subjects. The decline of exercise capacity seems to be slower in patients with an underlying left ventricular morphology and in those who received a TCPC. [Copyright &y& Elsevier]

Objective: Recent studies have demonstrated that there is a loss of aortic compliance in patients after coarctation repair. The clinical effect of this and other mechanisms apart from restenosis on the rate of arterial hypertension is unknown. Methods: From 1974 through 2000, 404 patients born before January 1, 1985, underwent surgical intervention for isolated aortic coarctation. From those 382 who are still alive, 273 patients aged 16 to 73 years (1–27 years after surgical intervention) underwent a structured clinical investigation according to a prospective protocol, including blood pressure measurement at all limbs, ambulatory blood pressure measurement, and symptom-limited exercise testing. Results: Sixty-seven (25%) patients were already taking antihypertensive drugs, and another 63 (23%) patients had an increased ambulatory blood pressure. Still another 26 (10%) patients had a blood pressure during exercise exceeding 2 standard deviations of reference values. Only 117 (43%) patients had a normal blood pressure reaction. From those 156 patients with hypertension, only 21 (13%) had a systolic brachial–ankle blood pressure difference of greater than 20 mm Hg, suggesting restenosis. In the patient group without restenosis (n = 245), independent risk factors for hypertension were repair with prosthetic material, male sex, a residual brachial–ankle blood pressure difference, and older age at follow-up. Conclusions: The majority of patients were hypertensive at long-term follow-up after coarctation repair. This is caused by restenosis, defined by a gradient of greater than 20 mm Hg, in only a few patients. Even in those without prosthetic material or minimal-grade restenosis, there is a substantial incidence of arterial hypertension. [Copyright &y& Elsevier]

Objectives: There are reports associating an increased incidence of Wolff-Parkinson-White syndrome with tricuspid atresia. Here we report on electrophysiologic studies in patients with tricuspid atresia and Wolff-Parkinson-White syndrome after the Fontan operation. In these patients the atrial arrhythmia often seen in patients undergoing the Fontan operation triggered atrioventricular re-entrant tachycardia or caused life-threatening arrhythmias. Methods: Five patients with tricuspid atresia after palliation with a modified Fontan operation (atrioinfundibular connections) and Wolff-Parkinson-White syndrome are presented. Results: Four of these patients had symptomatic paroxysmal orthodromic atrioventricular re-entrant tachycardia and a history of syncope; one of them additionally had atrial flutter with 2:1 conduction to the ventricle. A fifth patient presented with a life-threatening broad-complex tachycardia. In electrophysiologic studies an accessory pathway was localized in the right septal area in 3 patients. In 2 patients the accessory atrioventricular pathways were created by means of surgical intervention, connecting the right atrial appendage to the right ventricular outflow tract. All patients could be managed successfully by means of catheter ablation. Conclusions: In patients with tricuspid atresia, there are congenital and surgically acquired accessory pathways responsible for the increased rate of Wolff-Parkinson-White syndrome. Both types of accessory pathways can and should be treated by means of catheter ablation because atrial arrhythmia often seen in patients undergoing the Fontan operation can trigger atrioventricular re-entrant tachycardia or cause life-threatening tachycardia. Congenital accessory pathways should be excluded carefully before surgical intervention for total cavopulmonary anastomosis in patients with tricuspid atresia. [Copyright &y& Elsevier]

Objectives: Closure of isolated secundum atrial septal defect is generally recommended at the age of 4 to 5 years. However, there are children with isolated secundum atrial septal defect in whom early closure should be performed. We aimed to assess the underlying conditions that led to earlier closure in this special patient group and to analyze the outcome. Methods and Results: From January 1990 through August 2002, 24 infants with isolated secundum atrial septal defect underwent surgical closure within the first year of life. All children were symptomatic. Signs of pulmonary hyperperfusion, such as tachydyspnea, failure to thrive, recurrent respiratory infections, or heart failure, were present. Four infants required artificial ventilation. Ten patients had additional problems, such as prematurity with chronic lung disease, hepato-omphalocele and congenital diaphragmatic hernia, which were present in 1 patient each. Eleven patients had defined dysmorphic syndromes. All but 1 infant underwent preoperative invasive hemodynamic evaluation. Thirteen patients had pulmonary hypertension preoperatively. The follow-up time was 46 ± 33 months (range, 4–125 months). At follow-up, pulmonary artery pressure proved to be normal in 11 of the 13 children who had pulmonary hypertension previously. One patient died of persistent pulmonary hypertension. Clinical performance, growth, and development improved in nearly all patients. All ventilator-dependent children could be weaned shortly after atrial septal defect closure. Conclusions: If lungs are compromised, even a minor left-to-right shunt might be poorly tolerated in infancy. In these children early surgical closure of an isolated secundum atrial septal defect should be performed to support thrive and growth and to prevent the onset of irreversible changes of the pulmonary vasculature. [Copyright &y& Elsevier]

A longer length of stay (LOS) in the intensive care unit (ICU) after the total cavopulmonary connection (TCPC) is thought to be a predictive sign of late Fontan failure. This study was performed to determine the clinical risk factors for ICU LOS. In total, 483 patients who underwent a TCPC between May 1994 and December 2016 were included the study. Patients' main diagnosis, morphologic characteristics, palliative procedures, hemodynamic parameters, and perioperative variables, were analyzed to identify risk factors influencing ICU stay based on Cox regression. Causes of longer ICU LOS and the impact of ICU LOS on late outcomes were evaluated. Age at TCPC, type of TCPC, and fenestration at TCPC did not affect the ICU LOS. With multivariable model, hypoplastic left heart syndrome (P =.001) and anomalous systemic venous drainage (P <.001) were identified as independent morphologic risk factors for prolonged ICU LOS. Of hemodynamic variables, preoperative high transpulmonary gradient (P =.037), and low aortic oxygen saturation (P =.031) were risks for longer ICU LOS. Of postoperative variables, pleural effusion (P <.001), chylothorax (P =.001), ascites (P <.001), and infection (P =.028) were risks for longer ICU LOS. The ICU LOS was found to be significantly associated with late mortality (P <.001) and late cardiac reoperation (P =.007). Patients with hypoplastic left heart syndrome and anomalous systemic venous drainage had longer ICU LOS. Extended cyanosis and elevated pulmonary artery pressure affect the ICU LOS. Special care should be provided during the initial postoperative phase in patients with such risk factors. Patients with anomalous systemic venous drainage (SVD), dextrocardia, hypoplastic left heart syndrome (HLHS), dominant right ventricle (RV), and single tricuspid valve (TV) had longer intensive care unit (ICU) length of stay (LOS). Preoperative extended cyanosis, demonstrated by greater hemoglobin level and lower arterial oxygen saturation (SO 2), elevated pulmonary artery (PA) pressure, increased transpulmonary gradient, and presence of aortopulmonary collaterals (APCs), affected the ICU LOS. Special care should be provided during the initial postoperative phase in patients with such risk factors. TCPC , Total cavopulmonary connection. [ABSTRACT FROM AUTHOR]

In our study, we sought to analyse the mid-term results after interventional aortic coarctation (CoA) stenting with sequential dilation of the stent. The data of all 218 patients, who are above the age of 6 years and underwent CoA-stent implantation in our hospital, were retrospectively analysed on the rate of re-interventions, complications and arterial hypertension at a follow-up time of 31 months. To avoid any aortic complications, stents were deployed primarily not in full size and a second cardiac catheterisation for further dilatation was scheduled within 6–12 months after the stent implantation. The median peak invasive systolic pressure gradient declined significantly from 26.2 mmHg to 2.7 mmHg after stenting. There was one procedure related death due to an aortic rupture after stent implantation. There were in total 33 (15.1%) procedure-related complications including femoral artery complications, stent fracture and stent dislocation (in 9, 9 and 7 patients, respectively). In 85 patients a re-dilatation and in 25 patients a second stent-implantation was necessary at the first re-intervention. The systolic blood pressure declined significantly from 144 mmHg to 131 mmHg after stenting. The number of patients being normotensive changed from 18% before stenting to 78.5% after stenting with adjusted antihypertensive medication. Aortic stenting is an effective means for CoA treatment. With sequential dilation of the stent, a very low rate of life-threatening procedural complications and mortality can be achieved. CoA stenting with proper antihypertensive medications results in better control of blood pressure. • Endovascular stent implantation is effective means for aortic coarctation treatment. • With the sequential dilation of the CoA-stent, a low rate of mortality can be achieved. • It also has a very low rate of life-threatening aortic complications. • It should be considered for patients with high risk for aortic complications. [ABSTRACT FROM AUTHOR]

Objectives Patients with a single ventricle infrequently undergo total cavopulmonary connection as preadolescents, adolescents, or adults. The purpose of this study was to clarify the characteristics of this cohort and to analyze the factors influencing outcomes. Methods Between 1994 and 2015, 50 of 460 patients underwent total cavopulmonary connection as preadolescents, adolescents, or adults (group A). The patients' characteristics and operative results were compared with those of the remaining 410 patients aged less than 9 years who underwent total cavopulmonary connection (group B). Post–total cavopulmonary connection echocardiogram reports (n = 4862) were used to evaluate longitudinal ventricular function, and ejection fraction was characterized using nonlinear mixed-effects models and compared between groups. Results The median follow-up time was 10.3 (2.8-15.5) years. The differences between groups in 30-day mortality ( P = .20), intensive care unit stay ( P = .20), and incidence of prolonged effusion ( P = .08) were not significant. The estimated survival at 15 years was lower in group A (86.5%) than in group B (94.0%, P = .04) patients. Long-term systemic ventricular ejection fraction, analyzed with linear mixed-effect models, was significantly reduced in group A than in group B patients ( P < .001). At a median postoperative period of 8.4 (7.1-10.5) years, the peak oxygen uptake as measured by exercise capacity testing was lower in group A than in group B patients, respectively (22.3 ± 6.5 [n = 25] vs 30.6 ± 8.1 [n = 100] mL/kg/min, P < .001). Conclusions The total cavopulmonary connection procedure was performed in preadolescent, adolescent, and adult patients with no significant difference in 30-day or hospital mortality compared with those in young children. However, long-term survival and ventricular performance were reduced in this older cohort. [ABSTRACT FROM AUTHOR]

Objective The study objective was to determine the mechanisms of atrioventricular valve regurgitation in single-ventricle physiology and their influence on outcomes after total cavopulmonary connection. Methods Among 460 patients who underwent a total cavopulmonary connection, 101 (22%) had atrioventricular valve surgery before or coincident with total cavopulmonary connection. Results Atrioventricular valve morphology showed 2 separated in 33 patients, mitral in 11 patients, tricuspid in 41 patients, and common in 16 patients. Patients with a tricuspid and a common atrioventricular valve underwent atrioventricular valve surgery frequently, 27% and 36%, respectively. Atrioventricular valve regurgitation was due to 1 or more of the following mechanisms: dysplastic leaflet (62), prolapse (53), annular dilation (27), cleft (22), and chordal anomaly (14). Structural anomalies were observed in 89 patients (88%). The procedure was atrioventricular valve repair in 81 patients, atrioventricular valve closure in 16 patients, and atrioventricular valve replacement in 4 patients. Among 81 patients who underwent initial repair, repeat repair was required in 20 patients, atrioventricular valve replacement was required in 7 patients, and atrioventricular valve closure was required in 3 patients. Among patients undergoing atrioventricular valve surgery, overall survival after total cavopulmonary connection (88% vs 95% at 15 years, P = .01), freedom from atrioventricular valve reoperation after total cavopulmonary connection (75% vs 99% at 15 years, P < .01), and grade of atrioventricular valve regurgitation at a median follow-up of 6.6 years ( P < .01) were worse than in those who did not require atrioventricular valve surgery. Conclusions Atrioventricular valve regurgitation in univentricular heart is more frequently associated with a tricuspid or a common atrioventricular valve, and structural anomalies are the primary cause. Significant atrioventricular valve regurgitation requiring surgery influences survival after total cavopulmonary connection, especially when atrioventricular valve replacement was needed. Surgical management based on mechanisms of regurgitation is mandatory. [ABSTRACT FROM AUTHOR]

Abstract Aims Since the number of adults with congenital heart disease (ACHD) is increasing, age-related cardiovascular diseases become a relevant risk for ACHD. While previous studies investigated isolated risk factors only, this study examines the cardiovascular risk of ACHD based on the PROCAM scores. Methods and results From January 2017 to April 2018, 551 ACHD aged 30 years or older (43.9 ± 9.9 years, 48.3% female) were analyzed for their risk factors of major cardiovascular events within the next ten years using the PROCAM quick check and PROCAM health check. Compared to their individual reference, ACHD had a significantly lower absolute cardiovascular event risk in PROCAM quick check (ACHD: 2.5 ± 4.9%, reference: 3.8 ± 5.2%, p <.001) and PROCAM health check (ACHD: 1.8 ± 3.5%, reference: 3.9 ± 5.3%, p <.001). The relative risk of ACHD was 37% lower than in the general population calculated with the PROCAM quick test, and 57% lower with the PROCAM health check. Only 3.4% of the ACHD had a LDL cholesterol higher than 190 mg/dl, 8.3% had a HDL cholesterol lower than 40 mg/dl, and 26.0% had triglyceride higher than 150 mg/dl. Diabetes mellitus was prevalent in 4.0% of the ACHD and 10.9% were current smokers. Conclusion According to the PROCAM risk score, ACHD have a lower 10-year risk for major cardiovascular events compared to a healthy reference population. Whether this lower rate of the established risk factors leads to a lower rate of acquired cardiovascular disease has to be clarified in this particular population. Highlights • Adult patients with CHD have a lower 10-year risk for major cardiovascular events compared to a healthy reference • 8.3% of the ACHD had a HDL cholesterol lower than 40 mg/dl, and 26.0% had triglyceride higher than 150 mg/dl • Diabetes mellitus was prevalent in 4.0% of the ACHD and 10.9% were current smokers [ABSTRACT FROM AUTHOR]

Abstract: Objective: This study aimed to compare situational and trait anxiety levels in patients with congenital heart disease (CHD) with that in healthy controls and to investigate their correlation to the perceived health status. Patients and methods: From November 2007 to December 2009 in total 879 patients (405 female, 15–71years) with various CHD and 40 healthy controls completed the State-Trait Anxiety Inventory (STAI) to assess state and trait anxiety, the depression scaling instrument CES-D, and the health-related quality of life questionnaire SF-36. Results: In patients with CHD no increased anxiety as a trait could be found (Mann–Whitney Utest, p=.186). Only situational anxiety in the setting of an outpatient department in a tertiary center was increased compared to healthy controls (Mann–Whitney U test, p=.004). Anxiety was closely related to depression and to all of the nine SF-36 dimensions (r=−.149 to r=−.745, p<.001). Conclusions: In general, patients with CHD show a rather normal anxiety pattern compared to healthy controls. These symptoms of anxiety were strongly correlated with the perceived health status. Only situational anxiety levels at the hospital are increased and still remain an important challenge for doctors and psychologists. [Copyright &y& Elsevier]

Abstract: Objective: In patients with congenital heart disease quality of life is only marginally associated with exercise capacity. The aim of this study was to determine the prevalence of depression and its impact on quality of life and exercise capacity. Patients and methods: From November 2007 to October 2009 a total of 767 patients (352 female, 14–67years) with various congenital heart diseases (37 palliated/native cyanotic, 57 Fontan, 74 Transposition of the Great Arteries (TGA) after atrial switch, 50 other TGA, 136 Fallot, 38 Ebstein, 47 Pulmonic stenosis/regurgitation, 68 aortic coarctation, 103 aortic stenosis, 90 isolated shunts, 67 other) completed the health-related quality of life questionnaire SF-36 and the German translation of the “Center for Epidemiologic Studies Depression Scale” (CES-D) to assess depressive symptoms. Afterwards a cardiopulmonary exercise test was performed. Results: Only 66 patients (8.6%) showed depressive symptoms fulfilling the CES-D definition for depression. The total prevalence of depression was lower than in the general population (Wilcoxon test, p<0.001) and did not differ substantially in between the diagnostic subgroups (Kruskal–Wallis test, p=0.195). CES-D score was correlated to all of the nine dimensions of quality of life (r=−0.170 to r=−0.740, p<0.001) and less pronounced to exercise capacity (r=−0.164, p<0.001). Correlation of peak oxygen uptake to quality of life was weaker than the CES-D scores in all subscales of life quality. Conclusions: Patients with congenital heart disease are rarely depressive. However, even minor depressive symptoms have a stronger impact on quality of life than limited exercise capacity as seen in many patients. [Copyright &y& Elsevier]