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Am «Spitze Stei» oberhalb von Kandersteg im Berner Oberland drohen grössere Felsabbrüche mit Volumen von 100 000 bis einigen Millionen Kubikmetern. Die instabile Bergflanke besteht aus mehreren Rutschkörpern. Eine Ursache der aktuellen Krise dürfte die Permafrostdegradation sein, welche die Wasserdurchlässigkeit im Fels erhöht, wodurch der Wassereintrag in die instabile Bergflanke steigt. Die Sicherheit der Bevölkerung wird durch eine Kombination von organisatorischen, baulichen und raumplanerischen Massnahmen erreicht. Eine grosse Herausforderung für die verantwortlichen Stellen des Kantons und der Gemeinde sind die ständige Bereitschaft und die Vorbereitung auf ein Ereignis, das unter Umständen noch Jahre auf sich warten lässt.

Background Adults with congenital heart disease (CHD) have been considered potentially high risk for novel coronavirus disease-19 (COVID-19) mortality or other complications. Objectives This study sought to define the impact of COVID-19 in adults with CHD and to identify risk factors associated with adverse outcomes. Methods Adults (age 18 years or older) with CHD and with confirmed or clinically suspected COVID-19 were included from CHD centers worldwide. Data collection included anatomic diagnosis and subsequent interventions, comorbidities, medications, echocardiographic findings, presenting symptoms, course of illness, and outcomes. Predictors of death or severe infection were determined. Results From 58 adult CHD centers, the study included 1,044 infected patients (age: 35.1 ± 13.0 years; range 18 to 86 years; 51% women), 87% of whom had laboratory-confirmed coronavirus infection. The cohort included 118 (11%) patients with single ventricle and/or Fontan physiology, 87 (8%) patients with cyanosis, and 73 (7%) patients with pulmonary hypertension. There were 24 COVID-related deaths (case/fatality: 2.3%; 95% confidence interval: 1.4% to 3.2%). Factors associated with death included male sex, diabetes, cyanosis, pulmonary hypertension, renal insufficiency, and previous hospital admission for heart failure. Worse physiological stage was associated with mortality (p = 0.001), whereas anatomic complexity or defect group were not. Conclusions COVID-19 mortality in adults with CHD is commensurate with the general population. The most vulnerable patients are those with worse physiological stage, such as cyanosis and pulmonary hypertension, whereas anatomic complexity does not appear to predict infection severity., Central Illustration

ObjectivePregnancy in women with aortic coarctation (CoA) has an estimated moderately increased risk (mWHO II–III) of adverse cardiovascular, obstetric or fetal events, but prospective data to validate this risk classification are scarce. We examined pregnancy outcomes and identified associations with adverse outcomes.MethodsPregnancies in women with CoA were selected from the worldwide prospective Registry of Pregnancy and Cardiac Disease (ROPAC, n=303 out of 5739), part of the European Society of Cardiology EURObservational Research Programme. The frequency of and associations with major adverse cardiac events (MACE) and hypertensive disorders (pregnancy-induced hypertension, (pre-)eclampsia or haemolysis, elevated liver enzymes and low platelets syndrome) were analysed.ResultsOf 303 pregnancies (mean age 30 years, pregnancy duration 39 weeks), 9.6% involved unrepaired CoA and 27.1% were in women with pre-existing hypertension. No maternal deaths or aortic dissections occurred. MACE occurred in 13 pregnancies (4.3%), of which 10 cases were of heart failure (3.3%). Univariable associations with MACE included prepregnancy clinical signs of heart failure (OR 31.8, 95% CI 6.8 to 147.7), left ventricular ejection fraction 1 (OR 11.4, 95% CI 3.6 to 36.3) and cardiac medication use (OR 4.9, 95% CI 1.3 to 18.3). Hypertensive disorders of pregnancy occurred in 16 (5.3%), cardiac medication use being their only predictor (OR 3.2, 95% CI 1.1 to 9.6). Premature births were 9.1%, caesarean section was performed in 49.7% of pregnancies. Of 4 neonatal deaths, 3 were after spontaneous extreme preterm birth.ConclusionsThe ROPAC data show low MACE and hypertensive disorder rates during pregnancy in women with CoA, suggesting pregnancy to be more safe and better tolerated than previously appreciated.

ZusammenfassungDie Patientengruppe der Erwachsenen mit angeborenen Herzfehlern (EMAH) ist mittlerweile bereits größer als die der Kinder mit angeborenen Herzfehlern. EMAH-Patienten weisen auch nach Reparaturoperationen oft komplexe pathophysiologische und anatomische Verhältnisse auf. Bei Komplikationen kann es sehr rasch zu Notfallsituationen auch bei ansonsten asymptomatischen oder nur wenig symptomatischen Patienten kommen. Gemessen an der Gesamtzahl der Patienten, die von Notärzten und in den Notaufnahmen versorgt werden, sind EMAH-Notfallsituationen nach wie vor sehr selten. Diese Übersicht soll die Notfallbetreuung von EMAH-Patienten erleichtern. Für ca. zwei Drittel aller Notfälle sind Rhythmusstörungen und akute Herzinsuffizienz verantwortlich. Rhythmusstörungen müssen in der Regel zügig terminiert werden, weil sie unbehandelt rasch zur kardialen Dekompensation führen können. Bei Scheitern der medikamentösen Therapie oder hämodynamischer Instabilität müssen EMAH-Patienten mit tachykarden Rhythmusstörungen rasch elektrisch kardiovertiert werden. Symptomatische Bradykardien können eine rasche Schrittmacherversorgung erforderlich machen. Aufgrund der komplexen Anatomie kann das Einschwemmen eines transvenösen Interim-Schrittmachers bei einzelnen Vitien unmöglich sein. Die akute kardiale Dekompensation bei EMAH ist oft durch ein akutes Rechtsherzversagen verursacht. Weitere relativ häufige Aufnahmegründe sind Infektionen, Synkopen, Thromboembolien und Aortendissektion. Der Herzpass der Patienten informiert über das vorliegende Vitium. Die umgehende Kontaktaufnahme mit dem behandelnden EMAH-Zentrum wird dringend empfohlen.

Background The arterial switch operation (ASO) for repair of transposition of the great arteries (TGA) requires transection of the great arterial trunks and re-implantation of the coronary arteries into the neoaortic root resulting in cardiac sympathetic denervation which may affect myocardial blood flow (MBF) regulation. The aims of the present study were to evaluate sympathetic (re-)innervation in young adults after ASO and its impact on MBF. Methods Twelve patients (age 22.5 ± 2.6 years) after ASO for TGA in the neonatal period and ten healthy controls (age 22.0 ± 1.7 years) were included. Positron emission tomography (PET) was used for measuring cardiac sympathetic innervation with [11C]meta-hydroxyephedrine (mHED) and MBF with [15O]H2O PET at rest, during adenosine stimulation, and during sympathetic stimulation with cold pressor test. Cold pressor-induced MBF response capacity was calculated as maximal global MBF over peak rate-pressure product multiplied by 10′000. Results Global [11C]mHED uptake was significantly lower in patients compared to controls (7.0 ± 2.3 versus 11.8 ± 2.1%/min, p Conclusions With only partial sympathetic re-innervation of the coronary arteries, maximal dilator capacity of the coronary microvasculature and cold pressor-induced MBF response capacity remain substantially impaired in young adults after ASO compared to healthy controls.

Background: Long-term outcomes in adults with prior arterial switch operation (ASO) have not yet been well defined. The aim of this study is to elucidate incidence and predictors of adverse cardiac outcomes in a prospectively followed cohort of adults after their ASO. Methods: The comprehensive long-term follow up of adults with ASO is a project within the European collaboration for prospective outcome research in congenital heart disease (EPOCH). It is designed as a prospective, international multicenter cohort study. Consecutive patients (age ≥ 16 years) with prior ASO will be included at 11 European tertiary care centers. Participants will be followed according to a standardized protocol following international recommendations, including standardized protocols for imaging and for exercise testing. Results: Main outcome measures are all-cause and cardiac-related mortality, rate of cardiac re-intervention, neo-aortic dissection, myocardial infarction, stroke, infective endocarditis, sustained atrial and ventricular arrhythmias, new-onset or worsening pulmonary hypertension or heart failure. Secondary endpoints are frequency and progression of right ventricular outflow stenosis, neo-aortic root dilatation, neo-aortic valve regurgitation and ventricular dysfunction. The impact of demographic, anatomic (e.g., coronary artery anato-my) and functional variables on the above-mentioned outcomes, as well as quality of life and incidence of pregnancy related complications will also be assessed. Conclusion: The prospective, international, multicenter EPOCH-ASO study will provide a better understanding of adverse outcomes and their predictors in adults after ASO. The results of the EPOCH-ASO study may help to optimize future care of this novel patient cohort in adult cardiology.

Our aim was to assess changes of right ventricular end-diastolic volumes (RVEDVi) and right ventricular ejection fraction (RVEF) in asymptomatic adults with repaired tetralogy of Fallot, with native right ventricular outflow tract and severe pulmonary regurgitation by serial cardiac magnetic resonance imaging (CMR). The study included 23 asymptomatic adults who underwent ���3 CMR studies (total of 88 CMR studies). We compared changes in RVEDVi and RVEF between first and last study (median follow-up: 8.8��years, interquartile range: 6.3 to 13.1 years) and between all study pairs. Variability of measurements between study pairs (65 consecutive and 139 nonconsecutive CMR study pairs) were assessed using Bland-Altman analysis and intraclass correlation coefficients. On average, there were no significant changes of RVEDVi or RVEF over the study period (change in RVEDVi: +0.4 �� 17.8��ml/m2, change in RVEF: -1.0 �� 5.5%). Assessment of variability of measurements between study pairs demonstrated no systematic change in RVEDVi and RVEF between study pairs with limits of agreement within the range of previously published studies (RVEDVi -29.1 to +27.2��ml/m2; RVEF -11.5% to 10.2%). High intraclass correlation coefficients for RVEDVi (0.943, 95% CI 0.906 to 0.965, p

BACKGROUND: At the beginning of the COVID-19 pandemic, professionals in charge of particularly vulnerable populations, such as adult congenital heart disease (ACHD) patients, were confronted with difficult decision-making. We aimed to assess changes in risk stratification and outcomes of ACHD patients suffering from COVID-19 between March 2020 and April 2021. METHODS AND RESULTS: Risk stratification among ACHD experts (before and after the first outcome data were available) was assessed by means of questionnaires. In addition, COVID-19 cases and the corresponding patient characteristics were recorded among participating centres. Predictors for the outcome of interest (complicated disease course) were assessed by means of multivariable logistic regression models calculated with cluster-robust standard errors. When assessing the importance of general and ACHD specific risk factors for a complicated disease course, their overall importance and the corresponding risk perception among ACHD experts decreased over time. Overall, 638 patients (n = 168 during the first wave and n = 470 during the subsequent waves) were included (median age 34 years, 52% women). Main independent predictors for a complicated disease course were male sex, increasing age, a BMI >25 kg/m2, having ≥2 comorbidities, suffering from a cyanotic heart disease or having suffered COVID-19 in the first wave vs. subsequent waves. CONCLUSIONS: Apart from cyanotic heart disease, general risk factors for poor outcome in case of COVID-19 reported in the general population are equally important among ACHD patients. Risk perception among ACHD experts decreased during the course of the pandemic. ispartof: Int J Cardiol Congenit Heart Dis vol:11 pages:100428- ispartof: location:Netherlands status: published

Background:Infective endocarditis is a major threat after prosthetic pulmonary valve replacement. Early diagnosis may improve outcomes.Methods:A structured patient education programme for prevention and early diagnosis of infective endocarditis was developed at our institution since 2016. Time delay between onset of symptoms of prosthetic pulmonary valve endocarditis and its diagnosis (defined as initiation of appropriate high-dose intravenous antibiotic treatment) was compared for patients presenting before (cohort 1) and after (cohort 2) initiation of the patient education programme.Results:Between 2008–2019, 26 patients (median age 24.9, range: 16.8–62.0 years, 73% male) were diagnosed with prosthetic pulmonary valve endocarditis, 13 patients (cohort 1) before (1.7 cases/year) and 13 patients (cohort 2) after June 2016 (3.7 cases/year). There were no differences in baseline characteristics or clinical presentation between the study cohorts. Overall, the median delay between onset of symptoms and diagnosis of infective endocarditis was 6 days (range: 0–133 days) with a significantly longer delay among patients in cohort 1, compared to cohort 2 (25 days, range: 5–133 days versus 3 days, range: 0–13 days, p < 0.0001). A delay of >7 days was documented in 11/13 patients (85%) in cohort 1 as compared to 1/13 (8%) in cohort 2 (p < 0.001). Need for urgent valve replacement or permanent deterioration of prosthetic valve function was higher in cohort 1, compared to cohort 2 (11/13, 85% versus 5/13, 39%; p = 0.041).Conclusions:Prosthetic pulmonary valve endocarditis is increasingly recognised. A structured patient education programme may improve early diagnosis and clinical outcomes.

This study aimed to evaluate age at the first onset of cardiac complications and variation of frequency of complications between different congenital heart defects.The analysis included participants of the Swiss Adult Congenital Heart Disease Registry (SACHER). For this study, cardiac complications up to the time of inclusion in SACHER were analysed. Complications included atrial fibrillation, atrial flutter, supraventricular tachycardia, ventricular tachycardia, complete heart block, heart failure, stroke, endocarditis, myocardial infarction, and pulmonary hypertension. Incidence rates (IR; incidence rate per 1000 patient-years) for different age categories and diagnosis groups were analysed.Of 2731 patients (55% male, mean age 34 ± 14 years, 92,349 patient-years), a total of 767 (28%) had experienced at least 1 cardiac complication. The majority of complications (550; 72%) occurred in adulthood (18 years). Apart from perioperative stroke (IR: 1.77 in age group ≤ 4 years) and complete heart block (IR: 2.36 in age group ≤ 4 years), IR were much lower in childhood (IR1 for all complications between 5 and 17 years). Incidence of cardiac complications increased during adult life with highest IR for atrial fibrillation and atrial flutter in the age group ≥ 50 years (IR: 17.6 and 9.7, respectively). There were important variations of the distribution of complications among different diagnosis groups.Cardiac complications are frequent in congenital heart disease. Apart from perioperative stroke and complete heart block, IR are low in childhood but the incidence increases during adult life. These data underscore the need of lifelong follow-up and may help for better allocation of resources maintaining follow-up.

Background: The purpose of this study was to investigate whether patients with adult congenital heart disease (ACHD) benefit from exercise-based cardiac rehabilitation (CR) short- and long-term with regard to improvement of cardiorespiratory fitness. Methods: Cardiopulmonary exercise tests (CPET) completed by ACHD patients between January 2000 and October 2019 were analysed retrospectively. Linear mixed models were performed for peak oxygen consumption (VO2) with patients as random effect and age, sex, disease classification, preceding surgery (≤3 months) and preceding CR (≤4 weeks for short term and >4 weeks for long term) as fixed effects. Results: 1056 CPETs of 311 ACHD patients with simple (7), moderate (188) or great (116) complexity heart defects were analysed. The 59 patients who completed a CR (median age 27 yrs, 38% females) increased peak VO2 from before to after CR by a median of 2.7 (IQR –0.6 to 5.5) ml/kg/min. However, in the multivariate mixed model, peak VO2 was non-significantly increased short-term after CR (β 0.8, 95%CI –0.7 to 2.4), not maintained long-term after CR (β 0.0, 95%CI –1.7 to 1.6) but significantly reduced after surgery (β –5.1, 95%CI –7.1 to –3.1). The 20 CR patients after surgery increased their peak VO2 by 6.2 (IQR 3.6–9.5) ml/kg/min, while the 39 CR patients without preceding surgery increased it by 0.9 (IQR –1.5 to 3.1) ml/kg/min. Conclusions: The increase in peak VO2 with CR was mainly due to recovery from surgical intervention. The small independent benefit from CR was not maintained long-term, highlighting the potential to improve current CR concepts in ACHD populations.

BACKGROUND Patients with systemic right ventricle (sRV), including transposition of great arteries (TGA) after atrial switch procedure and congenitally corrected transposition of great arteries (ccTGA), may require anticoagulation for thromboembolism (TE) prevention. In the absence of data on non-vitamin K antagonist oral anticoagulants (NOACs), vitamin K antagonists (VKAs) remain the agent of choice. We investigated the safety, efficacy and feasibility of NOACs treatment in adults with sRV in a worldwide study. METHODS This is an international multicentre prospective study, using data from the NOTE registry on adults with sRV taking NOACs between 2014 and 2019. The primary endpoints were TE and major bleeding (MB). The secondary endpoint was minor bleeding. RESULTS A total of 76 patients (42.5��������10.0���years, 76% male) with sRV (74% TGA, 26% ccTGA) on NOACs were included in the study. During a median follow-up of 2.5���years (IQR1.5-3.9), TE events occurred in 3 patients (4%), while no MB episodes were reported. Minor bleeding occurred in 9 patients (12%). NOAC treatment cessation rate was 1.4% (95%CI:0.3-4%) during the first year of follow-up. All the patients with TE events had a CHA2DS2-VASc score���������2 and impaired sRV systolic function at baseline. The total incidence of major events during follow-up was significantly lower compared to historical use of VKAs or aspirin before study inclusion (1.4% (95%CI:0.29-4%) vs 6,9% (95%CI:2.5-15.2%); p���=���.01). CONCLUSIONS In this prospective study, NOACs appear to be well-tolerated, with excellent efficacy and safety at mid-term in patients with sRV.

Introduction Partial anomalous pulmonary venous connection (PAPVC) is a rare congenital heart disease, characterized by one or some but not all pulmonary veins anomalously connected to the right atrium or a systemic vein. PAPVC is either an isolated shunt lesion or associated with an atrial septal defect (ASD). This study compares the arrhythmic outcome of patients treated surgically with those clinically monitored. Method Clinical, surgical, imaging and invasive data of PAPVC patients were retrospectively reviewed from 7 centers from the Swiss Adult Congenital HEart disease Registry (SACHER) and one French center. Results A total of 168 patients with PAPVC were identified. Most (77%) patients underwent surgery, while the remaining (23%) ones were treated conservatively with clinical monitoring. The operated group (OG) had a significantly higher number of associated ASD (N=106, 82%) (p Age did not differ at latest follow-up (table). Holter recordings revealed a higher prevalence of arrhythmia in the OG (p=0.031), mainly of supraventricular tachyarrhythmias (table). The occurrence of ventricular non-sustained tachycardia and of bradyarrhythmia did not statistically differ between groups. Patients in the OG required more often medical treatment for arrhythmias: 12 (9%) needed electrophysiological study in the OG and none in the NOG (p=0.057). The amount of patients requiring a pacemaker implantation in the OG (11%) was significantly higher than that of NOG (0%) (p=0.039). Conclusion Patients after PAPVC repair present with a significant higher burden of arrhythmia than conservatively treated patients, either due to a larger shunt pre-operatively and/or as a late complication of the corrective surgery itself. Funding Acknowledgement Type of funding source: Private grant(s) and/or Sponsorship. Main funding source(s): Unrestricted grant from Actelion, Switzerland

Introduction Total anomalous pulmonary venous connection is a rare cyanotic congenital heart disease, where all pulmonary veins aberrantly connect to a systemic vein or the right atrium. The only curative treatment is surgery allowing the patients to reach adulthood. This study describes the long-term outcome of these individuals focusing on arrhythmias. Methods Clinical, surgical, imaging and invasive data were retrospectively reviewed from 7 centers participating in the Swiss Adult Congenital Heart disease Registry (SACHER) and one French center. Results A total of 57 patients were identified and analyzed 22±8 years after surgery. At last follow-up, 21% of patients presented cardiac symptoms, mainly palpitations. No patient had pulmonary hypertension or a relevant valvulopathy. Echocardiography revealed in 5 (8.8%) patients a dilated right ventricle (RV) and in 3 (5.3%) patients a diminished RV systolic function. Exercise capacity was normal in most patients (table). Cardiac magnetic resonance imaging found in 2 (4%) had a residual shunt with an mean Qp:Qs of 1.25, due to a single anomalously connected pulmonary vein. Holter recordings revealed arrhythmias in 23% of patient. Ten (17.5%) had atrial fibrillation, flutter or tachycardia. Three (5%) patients presented ventricular arrhythmias: two patients showed non-sustained ventricular tachycardia and one patient complex ventricular extrasystoles. Four patients (7%) were on antiarrhythmic medication for supraventricular arrhythmias. Three patients (5%) underwent an electrophysiological study with a mean time since surgery of 20 years. Three (5%) patients underwent pacemaker implantation within 3 weeks to 36 months after surgical correction, which were removed in 2 patients after 7 years. Age and the presence of a valvulopathy at follow-up predicted tachyarrhythmia on binomial logistic regression analysis (p Conclusions In adult survivors after TAPVC repair, supraventricular but also ventricular arrhythmias are frequently observed which appears to be related to age and valvular lesions at follow-up. This study underlies the importance of long-term follow-up as some of the patients currently without arrhythmia will probably develop rhythm disorders in the future. Funding Acknowledgement Type of funding source: Private grant(s) and/or Sponsorship. Main funding source(s): Unrestricted grant by Actelion SA, Switzerland

Background Pregnancy in women with repaired aortic coarctation (CoA) has a moderately increased risk (mWHO II-III) of an adverse cardiovascular, obstetric or fetal event, but prospective data to validate this estimated risk is scarce. Purpose We examined maternal and fetal outcomes in pregnant women with unrepaired and repaired CoA to identify predictors of adverse outcomes. Methods Pregnancies in women with CoA were selected from the worldwide prospective Registry of Pregnancy and Cardiac Disease (ROPAC, n=5739). The frequency and predictors of major adverse cardiac events (MACE, defined as maternal cardiac death, heart failure, atrial fibrillation/flutter, ventricular tachyarrhythmia, endocarditis, thromboembolic events, aortic dissection and acute coronary syndrome), and hypertensive complications (defined as pregnancy-induced hypertension, (pre)eclampsia or HELLP syndrome) were analyzed. Results Of 303 pregnancies in women with CoA (median age 30 years, median pregnancy duration 39 weeks), 10% were in women with unrepaired CoA and 27% in women with pre-existing hypertension. There were no maternal deaths. There were 4 neonatal deaths of which 3 occurred after a spontaneous extreme preterm birth. MACE occurred in 4.3% of pregnancies, predominantly involving heart failure (3.3%). MACE rate was similar in unrepaired vs repaired CoA (3.4% vs 4.4%, p=0.814). Predictors of MACE included pre-pregnancy clinical signs of heart failure (OR 31.8, 95% CI 6.8–147.7), LVEF 1 (OR 11.4, 95% CI 3.6–36.3), cardiac medication use (OR 4.9, 95% CI 1.3–18.3) and living in an emerging country (OR 4.88, 95% CI 1.58–15.07). Hypertensive complications occurred in 6.3%, more often in the subgroup with pre-existing hypertension (11% vs 5%, p=0.040). Pre-existing hypertension was the only predictor (OR 2.6, 95% CI 1.01–6.6). Caesarean section was performed in 50% of the total cohort. Conclusions Pregnancies in women with CoA are safe, well tolerated and MACE and hypertensive complication rates are low. These findings support mWHO risk score reevaluation to mWHO II for women with CoA without cardiac impairment. Funding Acknowledgement Type of funding source: Foundation. Main funding source(s): ESC EURObservational Research Programme (EORP)

Introduction Partial anomalous pulmonary venous connection (PAPVC) is a rare congenital heart disease, which is characterized by one or some but not all pulmonary veins anomalously connected to the right atrium or a systemic vein. PAPVC is either an isolated shunt lesion or associated with an atrial septal defect (ASD). The only curative treatment is surgery, however the indication for surgery can be challenging. This study compares the outcome of patients treated surgically with those clinically monitored. Method Clinical, surgical, imaging and invasive data were retrospectively reviewed from 7 centers from the Swiss Adult Congenital HEart Registry (SACHER) and a French center. Results A total of 168 patients with partial anomalous pulmonary venous connection were identified. The majority (77%) of patients underwent surgery and the remaining (23%) were treated conservatively with clinical monitoring. The operated group (OG) had a significantly higher proportion of associated ASD (N=106, 82%) (p Latest follow-up was 12 years after surgery. Mean age was 40±17 years. Patients in the non-operated group (NOG) were significantly more dyspneic than the OG (p=0.002). However, the need for medical treatment did not differ between groups: 58% of the NOG and 48% in the OG (p=0.203). Right ventricular (RV) ejection fraction did not differ between groups despite a significantly larger RV end-diastolic volume and a higher Qp:Qs on cardiac magnetic resonance (CMR) in the NOG (table). On echocardiography, the NOG showed a significantly better right ventricular longitudinal function and a higher systolic pulmonary artery pressure than the OG (table). The prevalence of significant valvulopathies did not differ between groups (table). Both groups had normal exercise capacity and with no differences between groups (table). Eighteen (14%) OG patients required a re-intervention either for residual shunt and/or stenosis of the pulmonary veins, superior and inferior vena cava. Conclusion PAPVC patients after surgical correction, show a favorable outcome in terms of imaging parameters and exercise capacity, however, a significant number presents with symptoms. PAPVC patients treated conservatively with small left to right shunting, have similar outcome justifying a conservative approach. Funding Acknowledgement Type of funding source: Private grant(s) and/or Sponsorship. Main funding source(s): Unrestricted grant from Actelion, Switzerland

AIMS OF THE STUDY Most patients with reduced exercise capacity and acquired or congenital structural heart disease also have a reduced respiratory muscle strength. The aim of this pilot study was to investigate whether choir singing in combination with respiratory muscle training positively influences respiratory muscle strength, exercise capacity and quality of life in this population. METHODS In this single-centre, randomised and open-label interventional study we compared respiratory muscle strength, exercise capacity and quality of life in patients with acquired or congenital structural heart disease who received either standard of care and a 12-week intervention (weekly choir rehearsal and daily breathing exercises) or standard of care alone. The primary endpoint was the difference in change in maximum inspiratory pressure (∆MIP%predicted). Secondary endpoints included the difference in change in maximum expiratory pressure (∆MEP%predicted), exercise capacity quantified as maximal oxygen uptake during exercise (∆MVO2%predicted) and quality of life quantified by the Minnesota living with heart failure questionnaire (∆MLHFQ score). RESULTS Overall 24 patients (mean age 65, standard deviation [SD] 19 years, 46% male) were randomised after exclusion. ∆MIP%predicted was significantly higher in the intervention group (∆MIP%predicted +14, SD 21% vs −14, SD 23%; p = 0.008) and quality of life improved significantly (∆MLHFQ score −5, SD 6 vs 3, SD 5; p = 0.006) after 12 weeks. ∆MEP%predicted and ∆MVO2%predicted did not differ between both groups (∆MEP%predicted −3, SD 26% vs −3, SD 16%; p = 1.0 and ∆MVO2%predicted 18, SD 12% vs 10, SD 15%; p = 0.2). CONCLUSIONS Choir singing in combination with respiratory muscle training improved respiratory muscle strength and quality of life in patients with structural heart disease and may therefore be valuable supplements in cardiac rehabilitation. (Clinical trial registration number: NCT03297918)

Learning objectives Simple shunt lesions are among the most common forms of congenital heart disease.1 Depending on location and size of the shunts, haemodynamic effects and presentation vary considerably. For example, while haemodynamically relevant ventricular septal defects (VSDs) nowadays typically are managed during childhood, atrial septal defects (ASDs) often escape diagnosis in childhood and many patients are diagnosed in adult life.2 Therefore, general cardiologists are frequently confronted with diagnosis and management of interatrial communications. This article focuses on anatomy, pathophysiology, natural history and common complications of simple shunt lesions; discusses imaging techniques and invasive procedures to diagnose and manage ASDs and VSDs and provides a practical algorithm on how to manage patients with ASDs with and without pulmonary hypertension. ### Atrial septal defects #### ​Anatomy of ASDs ASD are defined as direct communications on the atrial level. The ostium secundum ASD (figure 1A1) is located within the fossa ovalis but may extend in any direction. In contrast to a patent foramen ovale (PFO), which is a flap-valve of tissue formed by the septum primum and septum secundum, an ostium secundum ASD is characterised by a true tissue defect of variable size. Occasionally, there may be multiple defects or a combination of an ostium secundum ASD in addition to a PFO with or without an associated atrial septal aneurysm. Figure 1 Anatomy and haemodynamic consequences of simple shunt lesions. Panels (1A and 1B) anatomy of secundum atrial septal defects and haemodynamic consequences of relevant left-to-right shunting in interatrial communications. Panels (2A and 2B) anatomy of muscular ventricular septal defects and haemodynamic consequences of relevant left-to-right shunting. Panels (3A and 3B) anatomy of patent ductus arteriosus and the …

Survival prospects in adults with congenital heart disease (CHD), although improved in recent decades, still remain below expectations for the general population. Patients and their loved ones benefit from preparation for both unexpected and predictable deaths, sometimes preceded by a prolonged period of declining health. Hence, advance care planning (ACP) is an integral part of comprehensive care for adults with CHD. This position paper summarizes evidence regarding benefits of and patients' preferences for ACP and provides practical advice regarding the implementation of ACP processes within clinical adult CHD practice. We suggest that ACP be delivered as a structured process across different stages, with content dependent upon the anticipated disease progression. We acknowledge potential barriers to initiate ACP discussions and emphasize the importance of a sensitive and situation-specific communication style. Conclusions presented in this article reflect agreed expert opinions and include both patient and provider perspectives. ispartof: EUROPEAN HEART JOURNAL vol:41 issue:43 pages:4200-4210 ispartof: location:England status: published

Background: Current guidelines consider vitamin K antagonists (VKA) the oral anticoagulant agents of choice in adults with atrial arrhythmias (AA) and moderate or complex forms of congenital heart disease, significant valvular lesions, or bioprosthetic valves, pending safety data on non-VKA oral anticoagulants (NOACs). Therefore, the international NOTE registry was initiated to assess safety, change in adherence and quality of life (QoL) associated with NOACs in adults with congenital heart disease (ACHD). Methods: An international multicenter prospective study of NOACs in ACHD was established. Follow-up occurred at 6 months and yearly thereafter. Primary endpoints were thromboembolism and major bleeding. Secondary endpoints included minor bleeding, change in therapy adherence (≥80% medication refill rate, ≥6 out of 8 on Morisky-8 questionnaire) and QoL (SF-36 questionnaire). Results: In total, 530 ACHD patients (mean age 47 SD 15 years; 55% male) with predominantly moderate or complex defects (85%), significant valvular lesions (46%) and/or bioprosthetic valves (11%) using NOACs (rivaroxaban 43%; apixaban 39%; dabigatran 12%; edoxaban 7%) were enrolled. The most common indication was AA (91%). Over a median follow-up of 1.0 [IQR 0.0–2.0] year, thromboembolic event rate was 1.0% [95%CI 0.4–2.0] (n = 6) per year, with 1.1% [95%CI 0.5–2.2] (n = 7) annualized rate of major bleeding and 6.3% [95%CI 4.5–8.5] (n = 37) annualized rate of minor bleeding. Adherence was sufficient during 2 years follow-up in 80–93% of patients. At 1-year follow-up, among the subset of previous VKA-users who completed the survey (n = 33), QoL improved in 6 out of 8 domains (p ≪ 0.05). Conclusions: Initial results from our worldwide prospective study suggest that NOACs are safe and may be effective for thromboembolic prevention in adults with heterogeneous forms of congenital heart disease., SCOPUS: ar.j, info:eu-repo/semantics/published

The Eiger hanging glacier is located in the west face of the Eiger in the Bernese Alps (Switzerland). Large ice avalanches, especially if they trigger secondary snow avalanches, endanger parts of the Jungfrau ski area and the Jungfraujoch railway. The latter leads to the Jungfraujoch, one of the top tourist destinations in Europe. The formation of a crevasse immediately behind the front of the hanging glacier was detected in autumn 2015, indicating an impending icefall with a maximum ice volume of 80,000 m3. Consequently, a hazard analysis was performed for four different scenarios with varying ice volumes and snow conditions. The analysis showed that a 100 m high rocky ridge situated in the main flow direction of the avalanches plays a crucial role due to its braking and deflecting effect. Closure plans were prepared for the four scenarios investigated. The railway station is especially endangered in the extreme scenario assuming an ice volume of 80,000 m3 and unstable snow conditions. We therefore recommended to trigger avalanches below the hanging glacier artificially after snowfall events and to install an early warning and alarm system to minimize the closure times for the railway and ski area., Cold Regions Science and Technology, 144, ISSN:0165-232X, ISSN:1872-7441

Interrupted aortic arch (IAA) associated with right-sided aorta descendens is a rare congenital malformation. We present a case of a female patient who was diagnosed with type B IAA and received primary corrective surgery as a neonate. A bovine umbilical vascular graft was used to establish aortic arch continuity. Thirty-nine years later the same patient presents with graft-dilatation and a distal aneurysm requiring a surgery. This case shows a late complication of a biological xenograft in term of long-term material performance. Moreover, it highlights the necessity for regular follow-ups in order to detect and treat late onset complications respectively.

A rapidly growing novel cohort of adults with complex congenital heart disease is evolving, many of them at high risk for cardiovascular complications and premature death as young adults. Although there is progress with medical and surgical treatment, caregivers will be inevitably confronted with an increasing number of patients dying under their care. Given their young age, these patients are at high risk of receiving (overly) aggressive or even futile treatments before their demise.

Trisomy 21 (T21) is associated in 40-45% of cases with heart defects, most commonly shunt lesions. These defects, if not repaired, can lead to irreversible shunt-induced pulmonary hypertension (i.e. Eisenmenger syndrome [ES]). In ES patients, intracardiac repair is no longer possible, but selective pulmonary vasodilators may increase exercise capacity and improve prognosis. This study aimed to estimate the prevalence of cardiac defects and ES in adult T21 patients and to assess the impact of T21 on treatment modalities and outcome in ES patients. A questionnaire was sent to 6906 Swiss physicians inviting them to indicate the number of adults with T21 under their care (survey report). We also analyzed all adults with ES (with and without T21) included in the Swiss Adult Congenital HEart disease Registry (SACHER) and studied the impact of T21 on the use of selective pulmonary vasodilators and survival. In the survey, 348 physicians cared for 695 adult T21 patients. Overall, 24% of T21 survey patients were known to have a cardiac defect, one in four with a defect had developed ES and 13% of those with ES were on specific pulmonary vasodilators. In SACHER, ES was present in 2% of adults with congenital heart disease and selective pulmonary vasodilators were used in 68% of ES patients with T21. In SACHER, survival during follow-up was worse with higher nt-proBNP levels (hazard ratio [HR] = 1.15 per 1000 units, 95% confidence interval [CI] = 1.02-1.29) and lower left ventricular ejection fraction (HR = 1.07 per percent decrease, 95% CI = 1.01-1.13). Age at inclusion and T21 did not affect survival. The prevalence of cardiac defects in adults with T21 in Switzerland is half the prevalence in children. T21 is over-represented among adults with ES. Raised awareness of the therapeutic options for T21 patients with ES is warranted.

This document has been written by paediatric and adult cardiologists as well as radiologists with expertise in the field of congenital heart disease imaging from the five university hospitals in Switzerland.

OBJECTIVE High altitude-related hypoxia induces pulmonary vasoconstriction. In Fontan patients without a contractile subpulmonary ventricle, an increase in pulmonary artery pressure is expected to decrease circulatory output and reduce exercise capacity. This study investigates the direct effects of short-term high altitude exposure on pulmonary blood flow (PBF) and exercise capacity in Fontan patients. METHODS 16 adult Fontan patients (mean age 28±7 years, 56% female) and 14 matched controls underwent cardiopulmonary exercise testing with measurement of PBF with a gas rebreathing system at 540 m (low altitude) and at 3454 m (high altitude) within 12 weeks. RESULTS PBF at rest and at exercise was higher in controls than in Fontan patients, both at low and high altitude. PBF increased twofold in Fontan patients and 2.8-fold in the control group during submaximal exercise, with no significant difference between low and high altitude (p=0.290). A reduction in peak oxygen uptake at high compared with low altitude was observed in Fontan patients (22.8±5.1 and 20.5±3.8 mL/min/kg, p

Imaging of the heart anatomy plays an important role, especially in catheter ablation for the treatment of arrhythmias in adults with congenital heart disease (ACHD). We present a comprehensive overview of the current state-of-the-art modalities available to plan and guide catheter ablation in an ACHD patient. In addition to the clinical assessment of the computed tomography and the integration of 3D reconstructions into the electroanatomical mapping system, 3D printing and virtual reality assessment showed its value in preprocedural planning of the intervention.

BACKGROUND In adults with congenital heart disease (CHD) and atrial arrhythmias, recommendations for thromboprophylaxis are vague and evidence is lacking. We aimed to identify factors that influence decision-making in daily practice. METHODS From the Swiss Adult Congenital HEart disease Registry (SACHER) we identified 241 patients with either atrial fibrillation (Afib) or atrial flutter/intraatrial reentrant tachycardia (Aflut/IART). The mode of anticoagulation was reviewed. Logistic regression models were used to assess factors that were associated with oral anticoagulation therapy. RESULTS Compared with patients with Aflut/IART, patients with Afib were older (51 ± 16.1 vs 37 ± 16 years, P < .001) and had a higher CHA DS -VASc (P < .001) and HAS-BLED scores (P = .005). Patients with Afib were more likely on oral anticoagulation than patients with Aflut/IART (67% vs 43%, P < .001). In a multivariate logistic regression model, age [odds ratio (OR) 1.03 per year, 95%CI (1.01-1.05), P = .019], atrial fibrillation [OR 2.75, 95%CI (1.30-5.08), P = .007], non-paroxysmal atrial arrhythmias [OR 5.33, 95%CI (2.21-12.85)], CHA DS -VASc-Score >1 [OR 2.93, 95%CI (1.87-4.61), P < .001], and Fontan palliation [OR 17.5, 95%CI (5.57-54.97), P < .001] were independently associated with oral anticoagulation treatment, whereas a HAS-BLED score >1 was associated with absence of thromboprophylaxis [OR 0.32, 95%CI (0.17-0.60), P < .001]. CONCLUSIONS In this multicenter study, age, type, and duration of atrial arrhythmias, CHA DS -VASc and HAS-BLED scores as well as a Fontan palliation had an impact on the use of thromboprophylaxis in adult CHD patients with atrial arrhythmias. In daily practice, anticoagulation strategies differ between patients with Afib and those with Aflut/IART. Prospective observational studies are necessary to clarify whether this attitude is justified.

Aims Renal failure is a major challenge in treating heart failure (HF) patients. HF medication may deteriorate renal function, but the impact thereof on outcome is unknown. We investigated the effects of HF medication on worsening renal function (WRF) and the relationship to outcome. Methods and results This post-hoc analysis of TIME-CHF (NT-proBNP-guided vs. symptom-guided management in chronic HF) included patients with LVEF ≤45% and ≥1 follow-up visit (n = 462). WRF III was defined as a rise in serum creatinine ≥0.5 mg/dL (i.e. 44.2 µmol/L) at any time during the first 6 months. Four classes of medication were considered: loop diuretics, beta-blockers, renin–angiotensin system (RAS)-blockers, and spironolactone. Functional principal component analysis of daily doses was used to comprehend medication over time. All-cause mortality after 18 months was the primary outcome. Interactions between WRF, medication, and outcome were tested. Patients with WRF III received on average higher loop diuretic doses (P = 0.0002) and more spironolactone (P = 0.02), whereas beta-blockers (P = 0.69) did not differ and lower doses of RAS-blockers were given (P = 0.09). There were significant interactions between WRF III, medicationn and outcome. Thus, WRF III was associated with poor prognosis if high loop diuretic doses were given (P = 0.001), but not with low doses (P = 0.29). The opposite was found for spironolactone (poor prognosis in the case of WRF III with no spironolactone, P