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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]

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]

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]

Heart failure is an important cause of morbidity and mortality in adults with congenital heart disease (ACHD). Sacubitril/valsartan is an established treatment for heart failure with reduced ejection fraction due to acquired cardiovascular disease. Data in adults with complex congenital heart disease (CHD) is lacking. Retrospective study of ACHD patients with CHD of moderate/severe complexity and heart failure under treatment with sacubitril/valsartan. Clinical data was retrieved from medical records. Altogether, 23 patients (mean age 41.2 ± 11.9 years, female 17.4%) were included. A systemic right ventricle was present in 12 pat. (52.2%), a single ventricle physiology in 4 (17.4%), and a systemic left ventricle in 7 (30.4%). During a median follow-up of 221 days [IQR 79–430], systemic ventricular function (p = 0.88) and functional status according to New York Heart Association class (p = 0.38) did not improve. While NT-proBNP levels did not change significantly under treatment (2561 ± 2042 ng/l vs. 1938 ± 1524 ng/l, p = 0.20), creatinine levels increased (1.14 ± 0.52 mg/dl vs. 1.35 ± 0.74 mg/dl, p = 0.002). Systolic (110 ± 15 mm Hg vs. 103 ± 14 mm Hg, p = 0.02) and diastolic blood pressures (68 ± 10 mm Hg vs. 61 ± 12 mm Hg, p = 0.01) were reduced under therapy. Five patients discontinued therapy, four of these due to side effects. In this small group of complex ACHD patients with heart failure, treatment with sacubitril/valsartan did not improve systemic ventricular function or functional status. Renal function needs close surveillance. • The number of adults with congenital heart disease (ACHD) is increasing. • Heart failure is an important cause of morbidity and mortality in ACHD patients. • Sacubitril/valsartan did not improve heart failure symptoms in ACHD patients. • 17% of patients discontinued the therapy due to side effects. [ABSTRACT FROM AUTHOR]

Abstract In the summer of 2016, delegates from the German Society of Cardiology (DGK), the German Respiratory Society (DGP), and the German Society of Pediatric Cardiology (DGPK) met in Cologne, Germany, to define consensus-based practice recommendations for the management of patients with pulmonary hypertension (PH). These recommendations were built on the 2015 European Pulmonary Hypertension guidelines, aiming at their practical implementation, considering country-specific issues, and including new evidence, where available. To this end, a number of working groups was initiated, one of which was specifically dedicated to the definition, clinical classification and initial diagnosis of PH. While the European guidelines provide a detailed clinical classification and a structured approach for diagnostic testing, their application in routine care may be challenging, particularly given the changing phenotype of PH patients who are nowadays often elderly and may present with multiple potential causes of PH, as well as comorbid conditions. Specifically, the working group addresses the thoroughness of diagnostic testing, and the roles of echocardiography, exercise testing, and genetic testing in diagnosing PH. Furthermore, challenges in the diagnostic work-up of patients with various causes of PH including "PAH with comorbidities", CTEPH and coexisting conditions are highlighted, and a modified diagnostic algorithm is provided. The detailed results and recommendations of the working group on definition, clinical classification and initial diagnosis of PH, which were last updated in the spring of 2018, are summarized in this article. [ABSTRACT FROM AUTHOR]

Abstract In the summer of 2016, delegates from the German Respiratory Society (DGP), the German Society of Cardiology (DGK) and the German Society of Pediatric Cardiology (DGPK) met in Cologne, Germany, to define consensus-based practice recommendations for the management of patients with pulmonary hypertension (PH). These recommendations were built on the 2015 European Pulmonary Hypertension guidelines, aiming at their practical implementation, considering country-specific issues, and including new evidence, where available. To this end, a number of working groups was initiated, one of which was specifically dedicated to PH in adults associated with congenital heart disease (CHD). As such patients are often complex and require special attention, and the general PAH treatment algorithm in the ESC/ERS guidelines appears too unspecific for CHD, the working group proposes an analogous algorithm for the management of PH-CHD which takes the special features of this patient group into consideration, and includes general measures, supportive therapy, targeted PAH drug therapy as well as interventional and surgical procedures. The detailed results and recommendations of the working group on PH in adults with CHD, which were last updated in the spring of 2018, are summarized in this article. [ABSTRACT FROM AUTHOR]

Background: The objective of this prospective study was to assess the efficacy of exercise training as add-on to medical therapy in patients with congenital heart disease associated pulmonary arterial hypertension (CHD–APAH). Methods: Patients with invasively confirmed CHD–APAH received in-hospital exercise training for 3weeks and continued at home. Efficacy parameters were evaluated at baseline, after 3 and 15weeks. Medical treatment remained unchanged. Worsening events and survival rate were assessed in a follow-up period of 21±14months. Results: Twenty consecutive CHD–APAH patients (16 female, 4 male, mean pulmonary arterial pressure 60±23mmHg) were included. Patients significantly improved the mean distance walked in 6min compared to baseline by 63±47m after 3weeks (p<0.001) and by 67±59m after 15weeks (p=0.001). Quality of life-score (p=0.05), peak oxygen consumption (p=0.002) and maximal workload (p=0.003) improved significantly by exercise training after 15weeks. The 1- and 2‐year survival rates were 100%, the transplantation-free survival rate was 100% after 1year and 93% after 2years. Conclusion: Exercise training as add-on to medical therapy may be effective in patients with CHD–APAH and improved work capacity, quality of life and further prognostic relevant parameters. It was associated with an excellent long-term survival. Further randomized controlled studies are needed to confirm these results. [ABSTRACT FROM AUTHOR]

Abstract: Background: Clinical, hemodynamic and functional effects of tricuspid valve surgery in patients with Ebstein''s anomaly are not well understood. Methods: Sixteen patients (median age of 27.7years) were examined before and eight months after surgery by means of echocardiography, cardiovascular magnetic resonance (CMR) and cardiopulmonary exercise testing. Results: Peak work load (1.87 to 2.0W/kg; p=0.026), maximum oxygen uptake (21 to 22ml/kg/min; p=0.034) as well as cardiac output (2.7 to 2.9l/min/m2; p=0.035) increased postoperatively. The reduction of tricuspid regurgitation led to a higher pulmonary stroke volume (29 to 42ml/m2, p=0.005) and augmented the left ventricular (LV) volume (55 to 63ml/min/m2; p=0.001) with a trend to better ejection fraction (61 to 64%; p=0.083). Right ventricular (RV) volume index (124 to 108ml/m2; p=0.034) and ejection fraction (50 to 42%; p=0.036) decreased on CMR. Echocardiographic measurements of RV function also decreased (tricuspid annular plane systolic excursion 2.3 to 1.7; p=0.002; isovolumic acceleration 0.98 to 0.65; p=0.004; and 2-d longitudinal global strain −19.3 to −16.25; p=0.006). Conclusion: Tricuspid valve surgery improves exercise capacity in patients with Ebstein''s anomaly. The reduction of tricuspid regurgitation decreases the volume of the right ventricle and increases pulmonary antegrade flow. As a result LV volume and cardiac output increase. This hemodynamic benefit occurs despite the preload dependent reduction in RV volume and ejection fraction. [Copyright &y& Elsevier]

Abstract: Background: In the end of the last century arterial switch operation (ASO) replaced atrial redirection according to Senning or Mustard as the surgical technique for infants with transposition of the great arteries (TGA). Better survival with the new technique has been shown. However, exercise performance and quality of life have not yet been compared directly. Methods and results: All consecutive patients aged sixteen years or older that had undergone ASO for TGA at our institution and had performed a cardiopulmonary exercise test as part of their routine follow-up were included into the study. Those twenty-eight patients (20 male, median age 18.8years) were matched for age and gender with twenty-eight patients (20 male, median age 18.9years) who underwent atrial redirection in the same surgical period at our institution. Peak oxygen uptake was higher in patients after ASO (median 38.1 [quartiles 30.3; 44.5] ml/kg/min vs. 29.8 [23.5; 33.9] ml/kg/min; p<.0001) representing 92.0% predicted and 66.1% predicted (p<.0001), respectively. In addition, patients after ASO presented a better ventilatory efficiency ( slope, p=.029), ventilatory threshold (p=.006), peak O2 pulse (p=.0001), and oxygen saturation (p=.016), as well as a superior blood pressure (p=.046) response to exercise. Self-estimated quality of life was rather good in both groups but with better results in the physical functioning and general health domains in patients after ASO. Conclusions: Adolescents and young adults with TGA still have an improved exercise performance and quality of life in the long-term follow-up after ASO than those patients after atrial redirection. [Copyright &y& Elsevier]

Abstract: A modified Fontan operation (Fontan Björk [Bjork VO, Olin CL, Bjarke BB, Thoren CA. Right atrial–right ventricular anastomosis for correction of tricuspid atresia. J Thorac Cardiovasc Surg 1979;77:452--8]) for absent right atrio-ventricular connection was done by using a homograft between the right atrium and the right ventricle in some patients. We present the case of a 31-year-old patient, 21 years after a modified Fontan operation with severe homograft dysfunction. A percutaneous “tricuspid valve” implantation was performed with a Melody™ valve resulting in a good functional result. [Copyright &y& Elsevier]