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OBJECTIVES: This study aims to evaluate the clinical outcome following total cavopulmonary connection (TCPC) and to identify factors affecting early and late outcome. METHODS: Between May 1994 and March 2015, 434 patients underwent TCPC with 50 lateral tunnels and 374 extracardiac conduits. The clinical outcome, exercise capacity and liver examination results were retrospectively reviewed. RESULTS: Thirty-day survival was 98.2%, and the estimated survival rate at 15 years was 92.3%. Freedom from tachyarrhythmia at 15 years was 91.0%. Other late morbidities included bradyarrhythmia in 17, protein-losing enteropathy (PLE) in 15, thromboembolism in 3 and plastic bronchitis in 3 patients. At last follow-up, normal systemic ventricular function (ejection fraction >50%) was observed in 88.2%. Atrioventricular valve (AVV) regurgitation was mild or less in 90% of patients with systemic left ventricle, in 63% of those with systemic right ventricle and 58% of the patients with unbalanced atrioventricular septal defect or common inlet ventricles. Cardiopulmonary exercise capacity showed impaired peak oxygen uptake (71% of normal) in a sub-group of 120 patients at a mean of 9 years postoperatively. Biochemistry of 338 patients at last follow-up revealed a gamma-glutamyl transferase value beyond normal in 90 patients (26%), with a positive correlation between the level and the time after the initial operation (P < 0.01). Pre-TCPC high transpulmonary gradient emerged as a predictor for delayed hospital recovery (P = 0.002), late mortality (P = 0.016) and reoperation (P = 0.015) in multivariable analysis. CONCLUSIONS: Contemporary TCPC can be performed with low risk and provides excellent survival in the long-term. Classic morbidities of the original Fontan procedure, such as Fontan pathway revision, tachyarrhythmia and thromboembolism seem mitigated. However, exercise limitations, PLE and liver dysfunction remain an issue. AVV insufficiency and ventricular dysfunction are still a concern. [ABSTRACT FROM AUTHOR]

Aims: Patients with congenital heart disease usually show diminished exercise capacity and quality of life. However, there is only little information about daily activity, a marker for lifestyle, exercise capacity, and the prevention of arteriosclerosis. This study investigated exercise capacity, quality of life, daily activity, and their interaction with univentricular heart physiology after total cavopulmonary connection (TCPC). [ABSTRACT FROM PUBLISHER]

Objective Multiple studies have demonstrated the predictive value of arterial stiffness parameters like augmentation index (AIx) for cardiovascular events, the onset of hypertension, and the progression of heart failure. There is evidence that arterial stiffness is increased in some diagnostic subgroups of patients with congenital heart defects (CHD). This study aims to investigate AIx in a large cross-sectional cohort of patients with CHD. Patients and methods We prospectively examined 1125 consecutive patients with various congenital heart defects (27.3 ± 12.1 years, 464 female) referred for routine cardiopulmonary exercise testing (CPET) in our institution, and 322 healthy volunteers (29.4 ± 18.4 years, 165 female). AIx was estimated in supine position using the oscillometric Vicorder device (SMT medical, Würzburg, Germany). Afterward patients performed a CPET. Results In multivariable regression, presence of a CHD emerged as independent risk factor for higher AIx (p < .001). AIx was also higher in older (p < .001), smaller (p < .001) and heavier (p < .001) patients and in females (p = .008). Patients with aortic stenosis (p < .001), Tetralogy of Fallot (p < .001), transposition of the great arteries after atrial switch (p < .001) or Rastelli procedure (p = .013) and after Fontan procedure (p = .002) had higher AIx. Higher peak oxygen uptake (p < .001) and an ACE-inhibitor (p = .088) were associated with a lower AIx. Conclusions AIx is increased in patients with CHD. Several diagnostic subgroups are at risk. A better understanding of pathophysiologic mechanisms, genetic predisposition, the role of surgical aortic scars or implanted conduits/patches and medication is needed to define the value of AIx for further cardiovascular risk assessment in this cohort. [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]

Abstract: Objective: Patients with cyanotic congenital heart disease without corrective surgery or palliation survive into adulthood, if they have a balanced pulmonary blood flow facilitated by pulmonary stenosis (PS) or Eisenmenger syndrome (ES). Both groups show cyanosis, diminished exercise performance and impaired quality of life. This study aimed to compare the functional outcome of those two cohorts directly. Patients and methods: In total fifty-eight cyanotic patients with cardiac shunts (28 male, 30 female, aged 14–55years) were investigated, twenty-three of them with PS and thirty-five of them with ES. They completed the health related quality of life questionnaire SF-36 and performed a symptom limited cardiopulmonary exercise test. Results: At exercise, oxygen saturation decreased severely and similarly in both groups (PS: 90% to 65% vs. ES: 87 % to 64%). Moreover, hemoglobin levels were comparable in both subgroups. Exercise capacity was markedly reduced, but more diminished in ES (PS: 20.3 (11.9;24.6) ml/min/kg vs. ES: 11.3 (9.7;14.5) ml/min/kg; p <0.001) and ventilatory inefficiency expressed as V̇ E/V̇ CO2 slope was more enhanced in ES (PS: 45.7 (37.6;52.9) vs. ES: 54.6 (43.4;68.7); p =0.005). Oxygen saturation at rest was correlated to peak V̇ O2 (r =0.436; p =0.001) and V̇ E/V̇ CO2 slope (r =−0.388; p =0.003). Self estimated quality of life was poor, with worse results in physical and psychosocial domains in ES group. Conclusions: Despite similar cyanosis, patients with ES show less exercise performance, more ventilation–perfusion-mismatch and a worse quality of life compared to complex cyanotic congenital heart disease patients with PS. Moreover, oxygen saturation at rest predicts exercise capacity and ventilatory efficiency in this cohort. [Copyright &y& Elsevier]

Patients with Fontan circulation have no subpulmonary ventricle and a passive pulmonary perfusion. Considerable percentage of the pulmonary blood flow is driven by pressure shift due to respiration. Impairments in respiratory musculature strength are associated with a reduced exercise capacity. This study investigated the effect of a daily six months inspiratory muscle training (IMT) on exercise and lung capacity in adult Fontan patients. After a lung function and cardiopulmonary exercise test (CPET), 42 Fontan patients (50% female; 30.5 ± 8.1 years) were randomized into either an intervention group (IG), or a control group (CG). The IG performed a telephone-supervised, daily IMT of three sets with 10–30 repetitions for six months. After six months of IMT, the IG did not improve in any exercise and lung capacity parameter compared to CG. VO 2 peak (ΔVO 2 peak: IG: 0.05 [−1.53; 1.33] ml/kg/min vs. CG: −0.50 [−1.20; 0.78] ml/kg/min; p =.784) and FVC (ΔFVC: IG: 0.07 [−0.16; 0.22] l vs. CG:−0.05 [−0.24; 0.18] l; p =.377) remained unchanged, while FEV1 trended to improve (ΔFEV 1 : IG: 0.05 [−0.07; 0.13] l vs. CG: −0.10 [−0.19; 0.03] l; p =.082). Only oxygen saturation at rest improved significantly (ΔSpO 2 : IG: 1.50 [−0.25; 3.00] % vs. CG: −0.50 [−1.75; 0.75] %; p =.017). A daily six months IMT did not improve exercise and lung capacity and lung volumes in Fontan patients. • Fontan patients show reduced forced vital capacity. • Fontan patients show reduced forced expiratory volume in the first second. • Inspiratory muscle training did not improve exercise capacity in Fontan patients. • Inspiratory muscle training did not improve lung capacity in Fontan patients. • Inspiratory muscle training improved oxygen saturation in Fontan patients. [ABSTRACT FROM AUTHOR]

Objective The purpose of this study was to assess the longitudinal follow-up of exercise performance in patients with native Ebstein anomaly and patients who had received previous tricuspid valve surgery. Patients and methods We identified 59 patients with Ebstein anomaly who had performed at least two cardiopulmonary exercise tests within a minimum of 6 months. 22 patients (15 female, 29.0 ± 13.4 years) with native conditions, 37 patients (21 female, 32.1 ± 15.3 years) had undergone surgery of their tricuspid valve before baseline testing. Results In the non-operated group peak workload (2.58 ± 0.7 to 2.38 ± 0.7 W/kg, p = .019) declined during a mean follow-up of 4.6 ± 2.6 years, whereas peak workload remained stable in the surgical group (2.02 ± 0.6 to 2.06 ± 0.7 W/kg, p = .229) throughout the follow-up of 3.4 ± 2.3 years. In the 18 patients who had their surgery less than one year prior to the first CPET the exercise performance even increased during the follow-up. Peak blood pressure as a marker of cardiac adaption to exercise increased in the follow-up examination only in the surgical group (159 ± 23 vs. 171 ± 30 mm Hg, p = .007). In the non-operated group blood pressure remained unchanged at follow-up (161 ± 26 vs. 166 ± 29, p = .358). Conclusion Exercise performance deteriorates in non-operated patients with Ebstein anomaly in contrast to patients after tricuspid valve surgery where exercise performance remains stable. These facts promote a surgical intervention latest at the time when exercise performance decreases. [ABSTRACT FROM AUTHOR]

Objective Health-related quality of life questionnaires are frequently used to involve patients' impressions and feelings in the outcome evaluation. In patients with congenital heart disease (CHD) methodological heterogeneities, assessment in different age and diagnostic groups led to controversial findings. This study aims to give a comprehensive answer to the health-related quality of life in patients with CHD. Patients and methods From July 2001 to June 2013, 2360 patients (1058 female, 28.6±11.2-years, range 14-75-years) with various kinds of CHD underwent a quality of life assessment with the SF-36 questionnaire and underwent a cardiopulmonary exercise test as part of their routine follow-up. Results Physical component summary score (PCS) develops from 96.1 %predicted in patients younger than 20-years, to 96.2 %predicted in patients aged 20 to 30-years, 92.3 %predicted in patients aged 30 to 40-years, and 92.6 %predicted in patients 40-years or older (r=-.114; p<.001). The decline was more prominent in the mental component summary score (MCS) declining from 104.1 %predicted in patients younger than 20years, to 103.4 %predicted, 99.9 %predicted, and 97.5 %predicted (r=-.132; p<.001). Exercise capacity was impaired with 80.1±23.0 %predicted and also declined slowly with age (r=-.084; p<.001). Conclusions Health-related quality of life in patients with CHD is progressively reduced in older age-groups. The decline is small, but more prominent in the mental components. This should be considered in the interpretation of studies, and strategies have to be developed to reduce this effect in future. [ABSTRACT FROM AUTHOR]