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[Aims] Patients with adult congenital heart disease (ACHD) are a potentially vulnerable patient cohort in case of COVID-19. Some cardiac defects may be associated with a poor COVID-19 outcome. Risk estimation in ACHD is currently based on expert opinion. The aim of this study was to collect clinical outcome data and to identify risk factors for a complicated course of COVID-19 in patients with ACHD., [Methods] Twenty-five ACHD centres in nine European countries participated in the study. Consecutive patients with ACHD diagnosed with COVID-19 presenting to one of the participating centres between 27 March and 6 June 2020 were included. A complicated disease course was defined as hospitalisation for COVID-19 requiring non-invasive or invasive ventilation and/or inotropic support, or a fatal outcome., [Results] Of 105 patients with a mean age of 38±13 years (58% women), 13 had a complicated disease course, of whom 5 died. In univariable analysis, age (OR 1.3, 95% CI 1.1 to 1.7, per 5 years), ≥2 comorbidities (OR 7.1, 95% CI 2.1 to 24.5), body mass index of >25 kg/m2 (OR 7.2, 95% CI 1.9 to 28.3) and cyanotic heart disease (OR 13.2, 95% CI 2.5 to 68.4) were associated with a complicated disease course. In a multivariable logistic regression model, cyanotic heart disease was the most important predictor (OR 60.0, 95% CI 7.6 to 474.0)., [Conclusions] Among patients with ACHD, general risk factors (age, obesity and multiple comorbidities) are associated with an increased risk of complicated COVID-19 course. Congenital cardiac defects at particularly high risk were cyanotic lesions, including unrepaired cyanotic defects or Eisenmenger syndrome., The European Collaboration for Prospective Outcome Research in Congenital Heart Disease is funded by internal grants without support from the pharmaceutical industry.

Embaràs i malaltia cardíaca; Resultats de l’embaràs; Transposició de les grans artèries Embarazo y enfermedad cardíaca; Resultados del embarazo; Transposición de las grandes arterias Pregnancy and cardiac disease; Pregnancy outcomes, Transposition of the great arteries Background In the past 3 decades, the arterial switch procedure has replaced the atrial switch procedure as treatment of choice for transposition of the great arteries. Although survival is superior after the arterial switch procedure, data on pregnancy outcomes are scarce and transposition of the great arteries after arterial switch is not yet included in the modified World Health Organization classification of maternal cardiovascular risk. Methods and Results The ROPAC (Registry of Pregnancy and Cardiac disease) is an international prospective registry of pregnant women with cardiac disease, part of the European Society of Cardiology EURObservational Research Programme. Pregnancy outcomes in all women after an arterial switch procedure for transposition of the great arteries are described. The primary end point was a major adverse cardiovascular event, defined as combined end point of maternal death, supraventricular or ventricular arrhythmias requiring treatment, heart failure, aortic dissection, endocarditis, ischemic coronary events, and thromboembolic events. Altogether, 41 pregnant women (mean age, 26.7±3.9 years) were included, and there was no maternal mortality. A major adverse cardiovascular event occurred in 2 women (4.9%): heart failure in one (2.4%) and ventricular tachycardia in another (2.4%). One woman experienced fetal loss, whereas no neonatal mortality was observed. Conclusions Women after an arterial switch procedure for transposition of the great arteries tolerate pregnancy well, with a favorable maternal and fetal outcome. During counseling, most women should be reassured that the risk of pregnancy is low. Classification as modified World Health Organization risk class II seems appropriate. Funding from “Zabawas Foundation” and “De Hoop Foundation” in addition to the support from EURObservational Research Programme (EORP) is greatly acknowledged. Since the start of EORP, the following companies have supported the program: Abbott Vascular Int (2011–2021), Amgen Cardiovascular (2009–2018), AstraZeneca (2014–2021), Bayer AG (2009–2018), Boehringer Ingelheim (2009–2019), Boston Scientific (2009–2012), The Bristol Myers Squibb and Pfizer Alliance (2011–2019), Daiichi Sankyo Europe GmbH (2011–2020), The Alliance Daiichi Sankyo Europe GmbH and Eli Lilly and Company (2014–2017), Edwards (2016–2019), Gedeon Richter Plc (2014–2016), Menarini Int Op (2009–2012), MSD‐Merck & Co (2011–2014), Novartis Pharma AG (2014–2020), ResMed (2014–2016), Sanofi (2009–2011), SERVIER (2009–2021), and Vifor (2019–2022).

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.

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

Background/objective: Constant pain causes suffering and affects performance of activities of daily living (ADL). In clients with chronic musculoskeletal pain, we wanted to determine (i) the relationship between the perceived burden of suffering (measured with the Pictorial Representation of Illness and Self Measure (PRISM)) and the observed quality of ADL task performance (measured with the Assessment of Motor and Process Skills (AMPS)); and (ii) the change in these assessments before and after a 12-week pain programme. Methods: In this cross-sectional cohort study, we retrospectively collected data from participants in a Swiss pain management programme. We calculated the relationship, correlations and effect sizes for the PRISM and AMPS using non-parametric tests. We set the level of significance at α = 0.05. Results: Out of 138 clients, 74 participated. We found no significant correlations between the PRISM and AMPS (p = 0.55–0.36), except for the PRISM and AMPS process ability measure after the pain management programme (p = 0.023). Pre-post-correlations of the AMPS and PRISM were significant, with medium to strong effect sizes (−0.48–0.66). Conclusion: Participation in this pain programme improved both, the PRISM and AMPS scores. The lack of correlation between these assessments in clients with chronic musculoskeletal pain, however, strongly argues for a thorough clinical assessment.

The prone position is frequently used for breast irradiation in an effort to minimize dose to normal tissue and reduce skin toxicities. Immobilization required for prone breast irradiation can cause collision issues with the linear accelerator, disrupting treatment and negatively affecting the patient experience. The purpose of this retrospective study was to determine if an isocenter location guideline could be developed to prevent collisions with the prone breast immobilization device and gantry head, while still creating a clinically acceptable treatment plan. Clearance isocenter guidelines were established by measuring clearance between the Civco Horizon breast board and Varian linear accelerator. Fourteen patients with known clearance issues at a single institution were selected for this study and re-planned using clearance isocenter guidelines. Collision plans were compared to clearance plans created within the established clearance threshold through the institutions breast treatment guidelines based on arm II of the Radiation Therapy Oncology Group (RTOG) 1005 recommendations. Researchers in this study demonstrated clinical relevance by establishing that a clearance isocenter location guideline can be developed to prevent collisions with the prone breast immobilization and gantry head, while still creating a clinically acceptable treatment plan.

Musculoskeletal disorders (MSDs) account for almost 70 million physician office visits per year in the United States and are the most common workplace injuries. These are conditions involving the nerves, tendons, muscles, and supporting structures of the body. Previous studies have concluded that computer users are at high risk of developing work-related musculoskeletal disorders (WRMSDs). As computer users, medical dosimetrists are at risk of developing WRMSDs, yet there is a lack of information regarding the incidence of WRMSDs among medical dosimetrists. The purpose of this study was to determine the incidence of WRMSDs and variables of workstation ergonomics that contribute to the increased risk of WRMSDs in medical dosimetrists. A Qualtrics survey was created to support the 3 research questions guiding this study. The survey was distributed to 2,646 full members of the American Association of Medical Dosimetrists (AAMD), which included only certified medical dosimetrists (CMDs), via email. The distribution of email surveys sent through the AAMD email distribution list resulted in 988 emails opened, for a contact rate of 37% (988/2646). One hundred sixty-four responses were recorded yielding a completion rate of 17% (164/988). Fifty-five percent (90/163) of participants responded that they have experienced WRMSDs. Forty-four percent (289/652) of responses indicated WRMSDs have a slight or moderate interference on work. Sixty-two percent (94/152) of participants felt that their workstations were not ergonomically designed; even greater 68% (104/153) did not feel their workstations were designed for their individually needs. Of those respondents 64% (98/152) would like to see further adaptations made to their workspaces.

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.

ZusammenfassungZielsetzung Public Reporting (PR) von Qualitätsdaten gehört zu modernen Gesundheitssystemen. Jedoch zeigen aktuelle Reviews, dass der Nutzen begrenzt ist. Mit dem Nationalen Verein für Qualitätsentwicklung in Spitälern und Kliniken (ANQ) hat man auch in der Schweiz ein Messprogramm etabliert. Er misst in den stationären Sektoren unter anderem die Patientenzufriedenheit. Insbesondere bei den Leistungserbringern interessieren die Verhaltensstrategien auf solche Initiativen, da man idealerweise von einer Übernahme ins interne Qualitätsmanagement ausgeht.Methodik Semistrukturierte Interviewstudie von Seniormanagern in Deutschschweizer Rehabilitationskliniken unter Berücksichtigung des Neo-Institutionalismus.Ergebnisse Seniormanager wenden eine Fülle von Verhaltensstrategien an, wobei zustimmende und ablehnende Strategien gleichzeitig vorkommen.Schlussfolgerungen Externe Qualitätssicherungssysteme, wie der ANQ, führen nicht zwangsläufig zur Berücksichtigung für die interne Qualitätsarbeit. Vor allem lokale Faktoren scheinen erfolgskritisch für PR.

A pregnant patient with shortness of breath and arterial oxygen desaturation is presented. The primary and tentative initial diagnosis was pulmonary embolism. Her desaturation and dyspnea were aggravated in the upright compared with the supine position. The minimal response to supplemental oxygen suggested right-to-left shunting, which was confirmed by echocardiography. Shunting was minimal in the supine and maximal in the upright position, leading to the diagnosis of platypnea-orthodeoxia syndrome. By two weeks postpartum the patient's symptoms had resolved and shunting was undetectable.

Objectives Supine lumbar spine examinations underestimate body weight effects on neuroforaminal size. Therefore, our purpose was to evaluate size changes of the lumbar neuroforamina using supine and upright 3D tomography and to initially assess image quality compared with computed tomography (CT). Methods The lumbar spines were prospectively scanned in 48 patients in upright (3D tomographic twin robotic X-ray) and supine (30 with 3D tomography, 18 with CT) position. Cross-sectional area (CSA), cranio-caudal (CC), and ventro-dorsal (VD) diameters of foramina were measured by two readers and additionally graded in relation to the intervertebral disc height. Visibility of bone/soft tissue structures and image quality were assessed independently on a 5-point Likert scale for the 18 patients scanned with both modalities. Descriptive statistics, Wilcoxon’s signed-rank test (p < 0.05), and interreader reliability were calculated. Results Neuroforaminal size significantly decreased at all levels for both readers from the supine (normal intervertebral disc height; CSA 1.25 ± 0.32 cm2; CC 1.84 ± 0.24 cm2; VD 0.88 ± 0.16 cm2) to upright position (CSA 1.12 ± 0.34 cm2; CC 1.78 ± 0.24 cm2; VD 0.83 ± 0.16 cm2; each p < 0.001). Decrease in intervertebral disc height correlated with decrease in foraminal size (supine: CSA 0.88 ± 0.34 cm2; CC 1.39 ± 0.33 cm2; VD 0.87 ± 0.26 cm2; upright: CSA 0.83 ± 0.37 cm2, p = 0.010; CC 1.32 ± 0.33 cm2, p = 0.015; VD 0.80 ± 0.21 cm2, p = 0.021). Interreader reliability for area was fair to excellent (0.51–0.89) with a wide range for cranio-caudal (0.32–0.74) and ventro-dorsal (0.03–0.70) distances. Image quality was superior for CT compared with that for 3D tomography (p < 0.001; κ, CT = 0.66–0.92/3D tomography = 0.51–1.00). Conclusions The size of the lumbar foramina is smaller in the upright weight-bearing position compared with that in the supine position. Image quality, especially nerve root delineation, is inferior using 3D tomography compared to CT. Key Points • Weight-bearing examination demonstrates a decrease of the neuroforaminal size. • Patients with higher decrease in intervertebral disc showed a narrower foraminal size. • Image quality is superior with CT compared to 3D tomographic twin robotic X-ray at the lumbar spine.

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.

Zusammenfassung. Schmerzen beeinträchtigen den Alltag von Betroffenen massiv. Ist ein Nerv verletzt, sind die Schmerzen zudem oft komplexer, da sie neben der Einschränkung der Sensibilität und / oder der Motorik auch die Trophik des betroffenen Hautareals massgeblich verändern können. Schmerz zu objektivieren ist schwierig. Dennoch gibt es einige Möglichkeiten, diesen in der Handtherapie zu erfassen. Eine umfassende Dokumentation von Schmerzen, speziell den neurogen verursachten, und deren Auswirkungen ist deshalb eine unabdingbare Voraussetzung, um die Therapie individuell gestalten zu können. Guidelines empfehlen einen frühen Therapiestart, ein multimodales Vorgehen sowie eine Mischung aus medikamentösen und nicht medikamentösen Massnahmen. Empfehlungen sind aufgrund eines vielfach komplexen Verlaufs individuell anzupassen. Daher bilden eine offene und transparente Kommunikation, eine stabile therapeutische Beziehung sowie die aktive Mitarbeit der Betroffenen neben dem fachlichen Wissen eine Grundvoraussetzung für eine erfolgreiche Therapie. In diesem Artikel werden die häufigsten handtherapeutischen Assessments bei neurogenen Schmerzen vorgestellt, wie sie zum Beispiel bei einem Complex Regional Pain Syndrome angewendet werden. Anhand eines Patientenbeispiels wird die klinische Untersuchung einer Allodynie genauer beschrieben. Mögliche Therapieziele und -inhalte werden aufgezeigt. Trotz aller eingeleiteter Massnahmen bleibt die Behandlung von Schmerzen eine Herausforderung, welche nur im interdisziplinären Setting und mit Hilfe der Betroffenen angegangen werden kann.

Surgical intervention for severe lymphedema is reserved for failure of conservative management. The reductive approach aims to remove fibrosclerotic tissue acquired from prolonged lymphatic stasis. One such reductive approach is the modified Charles procedure, direct circumferential excision down to fascia followed by split-thickness skin graft application. The 1-year outcomes in our patients suggest that a multidisciplinary approach to the modified Charles procedure is an effective management option for severe lymphedema refractory to conservative therapy.

Purpose: The purpose of this qualitative study was to explore how stroke patients with unilateral spatial neglect experience; (a) performance in activities of daily living; (b) alterations in bodily perceptions; and (c) personal hopes and expectations, looking at the period between stroke onset and discharge from inpatient rehabilitation. Materials and methods: We conducted individual semi-structured interviews with 7 (5 men, 2 women, mean age 69 years) consecutively sampled participants. All interviews were transcribed verbatim and analyzed using thematic analysis. Findings: Participants' experiences were captured in three over-arching themes: "unawareness of neglect," "emergent awareness for neglect-related difficulties," and "comparing the new life with the old one." Findings showed that participants progressed from initial unawareness to emergent awareness for their neglect-related difficulties over the course of rehabilitation. Comparing their current life situation with the one before their stroke triggered feelings of uncertainty and regret, with associated decreased pleasure in meaningful activities. Conclusions: This study informs health professionals regarding personal experiences of orientation in and reorganization of life of stroke patients with unilateral spatial neglect. The findings highlight that being aware of neglect-related deficits is a prerequisite for using coping strategies and incorporating them in daily life. Possible therapeutic strategies that fit the current stage of recovery and level of awareness are discussed.

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.

[Objective] Adults with congenital heart disease (ACHD) may be at a higher risk of a fatal outcome in case of COVID-19. Current risk stratification among these patients relies on personal experience and extrapolation from patients with acquired heart disease. We aimed to provide an expert view on risk stratification while awaiting results from observational studies., [Methods] This study was an initiative of the EPOCH (European Collaboration for Prospective Outcome Research in Congenital Heart disease). Among nine European countries (Austria, Belgium, Denmark, France, Germany, Italy, the Netherlands, Spain and Switzerland), 24 experts from 23 tertiary ACHD centres participated in the survey. ACHD experts were asked to identify ACHD-specific COVID-19 risk factors from a list of potential outcome predictors and to estimate the risk of adverse COVID-19 outcomes in seven commonly seen patient scenarios., [Results] 82% of participants did not consider all ACHD patients at risk of COVID-19 related complications. There was a consensus on pulmonary arterial hypertension, Fontan physiology and cyanotic heart disease as risk factors for adverse outcomes. Among different ACHD scenarios, a patient with Eisenmenger syndrome was considered to be at the highest risk. There was a marked variability in risk estimation among the other potential outcome predictors and ACHD scenarios., [Conclusions] Pulmonary arterial hypertension, Fontan palliation and cyanotic heart disease were widely considered as risk factors for poor outcome in COVID-19. However, there was a marked disparity in risk estimation for other clinical scenarios. We are in urgent need of outcome studies in ACHD suffering from COVID-19., EPOCH-ASO is funded by internal grants without support from the pharmaceutical industry.

OBJECTIVES The aim of this study was to evaluate the effects of the coronavirus 2019 pandemic on elective and acute thoracic aortic surgery in the Netherlands. METHODS The Netherlands Heart Registration database was used to compare the volume of elective and acute surgery on the thoracic aorta in 2019 and 2020, starting from week 11 in both years. A sub-analysis was done to assess the impact of the pandemic on high-volume and low-volume aortic centres. RESULTS During the pandemic, the number of elective thoracic aortic operations declined by 18% [incidence rate ratio (IRR) 0.82 [0.73–0.91]; P < 0.01]. The decline in volume of elective surgery was significant in both high-volume (IRR 0.82 [0.71–0.94]; P < 0.01) and low-volume aortic centres (IRR 0.81 [0.68–0.98]; P = 0.03). The overall number of acute aortic operations during the pandemic remained similar to that in 2019 (505 vs 499; P = 0.85), but an increased share of these operations occurred at high-volume centres. The number of acute operations performed in high-volume centres increased by 20% (IRR 1.20 [1.01–1.42]; P = 0.04), while the number of acute operations performed in low-volume centres decreased by 17% (IRR 0.83 [0.69–1.00]; P = 0.04). CONCLUSIONS The coronavirus 2019 pandemic led to a significant decrease in elective thoracic aortic surgery but did not cause a change in the volume of acute thoracic aortic surgery in the Netherlands. Moreover, the pandemic led to a centralization of care for acute thoracic aortic surgery., The coronavirus disease 19 (COVID-19) pandemic has impacted the delivery of health care around the world.

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.

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 …

Background There is growing evidence that equine papillomavirus type 2 (EcPV2) infection is causally associated with the development of equine genital squamous cell carcinomas (SCCs). Early stages of disease present clinically as plaques or wart-like lesions which can gradually progress to tumoural lesions. Histologically these lesions are inconsistently described as benign hyperplasia, papilloma, penile intraepithelial neoplasia (PIN), carcinoma in situ (CIS) or SCC. Guidelines for histological classification of early SCC precursor lesions are not precisely defined, leading to potential misdiagnosis. The aim of this study was to identify histologic criteria and diagnostic markers allowing for a more accurate diagnosis of EcPV2-associated equine penile lesions. Results A total of 61 archived equine penile lesions were histologically re-assessed and classified as benign hyperplasia, papilloma, CIS or SCC. From these, 19 representative lesions and adjacent normal skin were comparatively analysed for the presence of EcPV2 DNA and transcripts using PCR and RNA in situ hybridisation (RISH). All lesional samples were positive by EcPV2 PCR and RISH, while adjacent normal skin was negative. RISH analysis yielded signal distribution patterns that allowed distinction of early (hyperplasia, papilloma) from late stage lesions (CIS, SCC). Subsequently, the 19 lesions were further assessed for expression of p53, Ki67, MCM7 and MMP1 by immunohistochemistry (IHC). All four proteins were expressed in both normal and lesional tissue. However, p53 expression was up-regulated in basal keratinocyte layers of papillomas, CIS and SCCs, as well as in upper keratinocyte layers of CIS and SCCs. MCM7 expression was only up-regulated in upper proliferating keratinocyte layers of papillomas, CIS and SCCs. Conclusion This study proposes combining a refined histological protocol for analysis of equine penile lesions with PCR- and/or RISH based EcPV2-screening and p53/MCM7 IHC to more accurately determine the type of lesion. This may help to guide the choice of optimum treatment strategy, especially at early stages of disease.

Umbilical preservation can be challenging, particularly in complex cases involving simultaneous ventral hernia repair and abdominoplasty. Although the umbilicus serves no functional purpose, removal of the umbilicus can draw unwanted attention to the abdominal area and can cause emotional distress to patients. There are several well documented options for umbilical reconstruction. We present a new umbilical reconstruction technique relevant for such cases. This neoumbilicoplasty allows for preservation of the original umbilicus with relocation and reconstruction using local flaps. The technique is relatively simple and the time needed is minimal. The result is a natural, well positioned umbilicus using the principles of spare part surgery.

We sought to investigate whether robotic pancreatoduodenectomy (RPD) mitigates adverse outcomes in patients with high-risk morphometric features compared to the open approach (OPD).Morphometric parameters for RPD and OPDs were measured by two blinded radiologists. The morphometric parameter best correlating with adverse outcomes was identified and used in multivariable models to evaluate the impact of surgical approach (open vs. robotic) on outcomes of patients with high-risk morphometric features.Of 282 PDs available for morphometric analysis, 134 (47.5%) underwent RPD. Average Psoas Density demonstrated the most frequent association with adverse outcomes, with correlations to prolonged LOS (ρ= -0.154, p=0.01), severe complications (ρ= -0.159, p=0.007), readmission (ρ= -0.16, p=0.007), and discharge to home (ρ= 0.2, p0.001). On multivariable analysis of patients with high-risk morphometric features (defined as APD ≤ 50This study confirms the association between morphometrics and outcomes following PD, and suggests that the robotic approach may be associated with improved outcomes in PD patients with high-risk morphometric features.

Background Maternal congenital heart disease (CHD) is the most common cardiac condition to complicate pregnancy and women now become pregnant even with complex CHD. The Registry Of Pregnancy And Cardiac disease (ROPAC) examines the relationship between maternal heart disease and pregnancy outcome. Methods The ESC EORP ROPAC is a worldwide prospective registry of pregnancies in women with structural heart disease (n=5739, recruiting between 2007-2018), including CHD. Maternal and fetal outcomes were examined in all women with CHD. Multivariable regression was used to identify associations with a composite endpoint of maternal mortality and/or heart failure. Results In CHD pregnancies (n=3295, mean age 29 years), maternal mortality was 0.3% and heart failure occurred in 6.6%. Preterm births (16%) and Caesarean section (46.3%) were higher than global averages, but otherwise cardiac, obstetric and fetal outcomes were good. The composite endpoint was highest in complex CHD: Eisenmenger syndrome (58.1%), congenitally corrected transposition of the great arteries (12.8%), Fontan circulation (11.2%), double outlet right ventricle (11.1%). Pre-pregnancy signs of heart failure (OR 10.6, 95% CI 7.1-16), multiple gestation (4.6, 2-10.8), pulmonary hypertension (2.5, 1.5-4), estimated LVEF Conclusion Overall pregnancy outcomes for women with CHD are good but women with complex CHD are at increased risk of complications. Pre-pregnancy assessment can identify women at increased risk of an adverse outcome and should be used to counsel women appropriately.

The National Association for Quality Development in Hospitals and Clinics (ANQ) has conducted patient satisfaction measurements in the inpatient sector in Switzerland since 2009. Specifically designed for this measurement, an instrument consisting of five questions was evaluated on an 11-point rating scale. Nevertheless, the instrument showed substantial ceiling effects, which did not allow for hospital discrimination. Therefore, ANQ initiated a revision testing different scales in a pilot study. The results showed that a 5-point verbal scale displayed good psychometric properties. Compared to the 7- or 11-point scales, the 5-point verbal scale exhibited reduced ceiling effects, which was more appropriate to compare hospitals. For the national public reporting of hospitals and clinics, risk adjustment by age and self-reported health status was recommended, which was not the case for gender, principal diagnosis, type of admission and insurance status.

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.

Importance: Therapeutic inertia (TI) is the failure to escalate therapy when treatment goals are unmet and is associated with low tolerance to uncertainty and aversion to ambiguity in physician decision-making. Limited information is available on how physicians handle therapeutic decision-making in the context of uncertainty. Objective: To evaluate whether an educational intervention decreases TI by reducing autonomic arousal response (pupil dilation), a proxy measure of how physicians respond to uncertainty during treatment decisions. Design, setting, and participants: In this randomized clinical trial, 34 neurologists with expertise in multiple sclerosis (MS) practicing at 15 outpatient MS clinics in academic and community institutions from across Canada were enrolled. Participants were randomly assigned to receive an educational intervention that facilitates treatment decisions (active group) or to receive no exposure to the intervention (usual care [control group]) from December 2017 to March 2018. Participants listened to 20 audio-recorded simulated case scenarios as pupil responses were assessed by eye trackers. Autonomic arousal was assessed as pupil dilation in periods in which critical information was provided (first period [T1]: clinical data, second period [T2]: neurologic status, and third period [T3]: magnetic resonance imaging data). Data were analyzed from September 2018 to March 2020. Interventions: The traffic light system (TLS)–based educational intervention vs usual care (unexposed). The TLS (use of established associations between traffic light colors and actions to stop or proceed) assists participants in identifying factors associated with worse prognosis in MS care, thereby facilitating the treatment decision-making process by use of established associations between red, green, and yellow colors and risk levels, and actions (treatment decisions). Main outcomes and measures: Pupil assessment was the primary autonomic outcome. To test the treatment effect of the educational intervention (TLS), difference-in-differences models (also called untreated control group design with pretest and posttest) were used. Results: Of 38 eligible participants, 34 (89.4%) neurologists completed the study. The mean (SD) age was 44.6 (11.6) years; 38.3% were female and 20 (58.8%) were MS specialists. Therapeutic inertia was present in 50.0% (17 of 34) of all participants and was associated with greater pupil dilation. For every additional SD of pupil dilation, the odds of TI increased by 51% for T1 (odds ratio, 1.51; 95% CI, 1.12-2.03), by 31% for T2 (odds ratio, 1.31; 95% CI, 1.08-1.59), and by 49% for T3 (odds ratio, 1.49; 95% CI, 1.13-1.97). The intervention significantly reduced TI (risk reduction, 31.5%; 95% CI, 16.1%-47.0%). Autonomic arousal responses mediated 29.0% of the effect of the educational intervention on TI. Conclusions and relevance: In this randomized clinical trial, the TLS intervention decreased TI as measured by pupil dilation, which suggests that individual autonomic arousal is an indicator of how physicians handle uncertainty when making live therapeutic decisions. Pupil response, a biomarker of TI, may eventually be useful in medical education. Trial registration: ClinicalTrials.gov Identifier: NCT03134794

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)

Heart failure is a significant and growing cause of disease in the developed world with substantial cost and morbidity associated with it. This review focuses on methamphetamine-associated cardiomyopathy (MACM) as an increasingly recognized cause of heart failure. MACM is increasingly recognized worldwide. Prevalence of MACM in heart failure hospital admissions has been increasing in the western USA. Risk factors for development of MACM include male sex, hypertension, and alcohol use disorder. MACM is associated with severe left ventricular dysfunction, greater heart failure symptom burden, and younger age of presentation than non-ischemic controls. Improvement of ejection fraction has been seen with guideline-directed therapy and methamphetamine cessation. EF improvement is correlated with lack of evidence of chronicity on transthoracic echocardiography and extent of fibrosis on endomyocardial biopsy. Limited studies show white and Māori patients are most often affected, though race/ethnicity has not been shown to be an independent risk factor in the development of MACM. MACM represents a subset of heart failure that affects predominantly young men, and tends to present with severely reduced ejection fraction and significant symptom burden. There is evidence that ejection fraction can be improved in patients without extensive cardiac fibrosis or evidence of cardiomyopathy chronicity on imaging in the setting of abstinence and medical therapy. In this context, methamphetamine use is a modifiable risk factor in the development of heart failure that would likely benefit from early intervention, though larger longitudinal studies are needed.

Introduction: Focal therapy of prostate cancer aims to destroy known cancer while preserving uninvolved prostatic tissue with the goal of preserving genitourinary function.1,2 Previous methods of f...

Status quo (SQ) bias is defined as patient´s tendency to continue taking a previously selected but inferior therapeutic option.To assess the presence of SQ bias and its associated factors in patients with relapsing-remitting multiple sclerosis (RRMS).A multicenter, non-interventional study involving 211 patients with RRMS was conducted. Participants answered questions regarding risk preferences and management of simulated MS case-scenarios. The SymptoMScreen (SMSS) questionnaire was used to assess the perception of severity from the patients´ perspective. SQ bias was defined as patients' preference to maintain the current treatment despite evidence of disease activity. Mixed linear models adjusting for clustering assessed the association of candidate predictors with the outcome of interest.The mean age (SD) was 39.1 (9.5) years and 70.6% were women. SQ bias was observed in 74.4% (n=161) participants. Univariate analysis showed that SMSS score was associated with SQ bias (OR 1.04; 95% CI 1.01-1.07). Mixed linear regression models suggest that for every point increase in SMSS, there was a 4% increase in the likelihood of SQ bias (β 0.04; 95%CI 0.015-0.06; p0.002). Among the different symptomatic dimensions included in the SMSS, only vision impairment (β 0.32; 95%CI 0.05-0.50) and depression (β 0.29; 95%CI 0.006-0.58) remained associated with SQ bias in the multivariate analysis. There was no association between participants' risk preferences and SQ bias.Unwillingness to pursue treatments that are more effective is a common phenomenon affecting over 7 out of 10 patients with RRMS. This phenomenon appears to be driven by patients' negative self-perception of their clinical status.

Background: Gastroesophageal reflux disease (GERD) is one of the most important obesity-related comorbidity, with prevalence >50% in obese population. Roux-en-Y gastric bypass (RYGB) is considered the gold standard for metabolic surgery in obese patients with GERD, but in a subgroup of patients this pathological GERD may be not really controlled after this technique. Aims of this article are to discuss surgical and endoscopic options to manage refractory GERD after RYGB. Materials and Methods: We realized a literature review using the PubMed database and searching articles published before December 2019 about GERD after RYGB. Results: We found six studies, four case reports, and two retrospective studies about surgical and endoscopic options to treat this subgroup of patients. Discussion: Pharmacological therapy and life style optimization are the first line of treatment. For resistant GERD, new surgical and endoscopic strategies are proposed in the past years to manage this subgroup of patients related to anatomic limitation of RYGB. Conclusion: More studies are needed to compare surgical and endoscopic solutions. The choice of treatment depends on local resources and skills, and if necessary refer the patient to a specialist center.

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

One aspect of goal-directed behavior, which is known to be impaired in patients with schizophrenia (SZ), is balancing between exploiting a familiar choice with known reward value and exploring a lesser known, but potentially more rewarding option. Despite its relevance to several symptom domains of SZ, this has received little attention in SZ research. In addition, while there is increasing evidence that SZ is associated with chronic low-grade inflammation, few studies have investigated how this relates to specific behaviors, such as balancing exploration and exploitation. We therefore assessed behaviors underlying the exploration–exploitation trade-off using a three-armed bandit task in 45 patients with SZ and 19 healthy controls (HC). This task allowed us to dissociate goal-unrelated (random) from goal-related (directed) exploration and correlate them with psychopathological symptoms. Moreover, we assessed a broad range of inflammatory proteins in the blood and related them to bandit task behavior. We found that, compared to HC, patients with SZ showed reduced task performance. This impairment was due to a shift from exploitation to random exploration, which was associated with symptoms of disorganization. Relative to HC, patients with SZ showed a pro-inflammatory blood profile. Furthermore, high-sensitivity C-reactive protein (hsCRP) positively correlated with random exploration, but not with directed exploration or exploitation. In conclusion, we show that low-grade inflammation in patients with SZ is associated with random exploration, which can be considered a behavioral marker for disorganization. hsCRP may constitute a marker for severity of, and a potential treatment target for maladaptive exploratory behaviors., npj Schizophrenia, 7 (1), ISSN:2334-265X

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.

Objective The commercially available C-Port distal anastomotic device (Food and Drug Administration cleared in 2007) is an automated miniature vascular stapler that performs the coronary anastomosis. This prospective multicenter registry sought to evaluate midterm patency using this device compared with hand-sewn grafts. Methods Patients receiving at least one C-Port anastomosis during coronary artery bypass grafting surgery were enrolled at eight sites. Of the 117 patients enrolled, 78 patients (67%) with 104 C-Port vein grafts completed the study to patency assessment via computed tomography angiography. Clinical follow-up and index graft patency (Gated 64-slice computed tomography scan) were performed at least 12 months postoperatively. The primary efficacy endpoint was patency compared with the peer-reviewed results from the PRoject of Ex-vivo Vein graft ENgineering via Transfection IV (PREVENT IV) trial. Results The patient population was consistent with the PREVENT IV placebo cohort. The mortality at 12 months was 0.85% (1/117). The major cardiac morbidity rate was 3.4% (4/117). The C-Port vein graft occlusion rate was 16.3% (17/104) compared with 26.6% (597/2242) in the PREVENT IV trial ( P = 0.011). Within this study, C-Port graft occlusion rates were not significantly different from the hand-sewn grafts ( P = 0.821). Conclusions The C-Port device is safe and effective in creating the distal anastomosis with equivalent patency rates to hand-sewn grafts at 12 months. When compared with hand-sewn anastomoses from a recent large prospective trial, the C-Port device demonstrated a statistically significant reduction in midterm graft occlusion. Further studies are required to evaluate its effect in less invasive coronary surgery.

Background Although rare, pseudoaneurysms (PA) can develop following Le Fort I osteotomy and lead to life-threatening hemorrhage. However, the typical presentation of a PA following a Le Fort I osteotomy is not well characterized. Evidence-based guidelines are not currently available for evaluation of PA following Le Fort I osteotomy. Methods A case report is presented of a 27-year-old man who underwent Le Fort I advancement and subsequently developed a bleeding PA. A comprehensive search of journal articles was performed using the MEDLINE/PubMed database between 1964 and April 2016. Keywords and phrases used were "(osteotomy OR craniofacial OR orthognathic) AND (pseudoaneurysm OR aneurysm OR epistaxis)." Preferred Reporting Items for Systematic Reviews and Meta- Analyses (PRISMA) guidelines were followed. Results The literature search yielded 13 reports of 18 patients. All 18 patients underwent Le Fort I osteotomy and subsequently developed a delayed postoperative bleeding PA. All studies were level IV or V evidence. Twenty-eight percent (N = 5) of the cases documented "excessive" intraoperative bleeding or more than 500 mL of estimated blood loss. The average time for the first bleeding episode and time until final bleed was 17.3 ± 14.3 days (range: 3-62 days) and 27.8 ± 21.2 days following surgery (range: 6-77 days), respectively. Sixty-seven percent (N = 12) had multiple episodes of bleeding. The duration between the bleeding events averaged 10.6 (± 7.9 days) with a range of 1 to 35 days. Bleeding PA's were treated with image-guided embolization (15/18 patients; 83.3%) or ligation or clamping (3/18 patients; 16.7%). Conclusion In the setting of recurrent and/or delayed postoperative epistaxis following Le Fort I osteotomy, surgeons should maintain a high clinical suspicion for PA. Ruptured PA's are often preceded by multiple episodes of progressively worsening epistaxis. Based on the presented case report and pooled data from the literature, angiography is recommended to evaluate for PA in the setting of recurrent epistaxis following Le Fort I osteotomy, especially within the first 4 weeks following surgery.

BACKGROUND Magnetic resonance imaging (MRI) accuracy after neoadjuvant systemic therapy (NST) for breast cancer varies according to hormone receptor (HR), human epidermal growth factor receptor type-2 (HER2) subtype and Ki-67 proliferation index. Whether MRI accuracy varies by genomic signatures is unknown. We examined the accuracy of MRI in the NEONAB trial (Clinicaltrials.gov #: NCT01830244). AIM To examine the accuracy of MRI to predict pathological response to neoadjuvant therapy for breast cancer in the NEONAB trial. METHODS Patients with stages II-III breast cancer received sequential epirubicin, cyclophosphamide and nab-paclitaxel and trastuzumab if they were HER2+. Sensitivity, specificity, positive predictive value (PPV) and negative predictive value (NPV) were calculated to assess the utility of preoperative MRI to predict pathological complete response (pCR). Bland-Altman plots were used to assess agreement between MRI and pathological assessment of residual disease. RESULTS MRI correctly predicted pCR in 64.1% of the cohort. Sensitivity and specificity were 52% and 78%, respectively; PPV 73% and NPV 58%. MRI predicted pCR most accurately in HER2-positive patients; sensitivity 58%, specificity 100%, PPV 100% and NPV 38%. MRI had higher PPV and NPV in tumours with Ki-67 ≥ 15% than tumours with Ki-67 < 15%, 75% versus 50% and 57.5% versus 50%, respectively. In this study, MRI underestimated residual tumour size by 1.65 mm (limits of agreement: 43.07-39.77 mm). CONCLUSIONS MRI appears more accurate for predicting pCR in HER2+ disease than other subtypes and in cancers with Ki-67 ≥ 15% compared to those with Ki-67 < 15%. Accuracy of MRI in our HR+, RS ≥ 25 cohort is comparable to previous reports of unselected HR+ disease. MRI post-NST should be interpreted in conjunction with HER2 status and Ki-67 index of the primary.

The development of new therapeutic agents for the treatment of Duchenne muscular dystrophy has put a focus on defining outcome measures most sensitive to capture treatment effects. This cross-sectional analysis investigates the relation between validated clinical assessments such as the 6-minute walk test, motor function measure and quantitative muscle MRI of thigh muscles in ambulant Duchenne muscular dystrophy patients, aged 6.5 to 10.8 years (mean 8.2, SD 1.1). Quantitative muscle MRI included the mean fat fraction using a 2-point Dixon technique, and transverse relaxation time (T2) measurements. All clinical assessments were highly significantly inter-correlated with p

ObjectiveCardiac disease is the leading cause of indirect maternal mortality. The aim of this study was to analyse to what extent socioeconomic factors influence the outcome of pregnancy in women with heart disease.MethodsThe Registry of Pregnancy and Cardiac disease is a global prospective registry. For this analysis, countries that enrolled ≥10 patients were included. A combined cardiac endpoint included maternal cardiac death, arrhythmia requiring treatment, heart failure, thromboembolic event, aortic dissection, endocarditis, acute coronary syndrome, hospitalisation for cardiac reason or intervention. Associations between patient characteristics, country characteristics (income inequality expressed as Gini coefficient, health expenditure, schooling, gross domestic product, birth rate and hospital beds) and cardiac endpoints were checked in a three-level model (patient–centre–country).ResultsA total of 30 countries enrolled 2924 patients from 89 centres. At least one endpoint occurred in 645 women (22.1%). Maternal age, New York Heart Association classification and modified WHO risk classification were associated with the combined endpoint and explained 37% of variance in outcome. Gini coefficient and country-specific birth rate explained an additional 4%. There were large differences between the individual countries, but the need for multilevel modelling to account for these differences disappeared after adjustment for patient characteristics, Gini and country-specific birth rate.ConclusionWhile there are definite interregional differences in pregnancy outcome in women with cardiac disease, these differences seem to be mainly driven by individual patient characteristics. Adjustment for country characteristics refined the results to a limited extent, but maternal condition seems to be the main determinant of outcome.

Background: Cardiac disease is 1 of the major causes of maternal mortality. We studied pregnancy outcomes in women with rheumatic mitral valve disease. Methods: The Registry of Pregnancy and Cardiac Disease is an international prospective registry, and consecutive pregnant women with cardiac disease were included. Pregnancy outcomes in all women with rheumatic mitral valve disease and no prepregnancy valve replacement is described in the present study (n=390). A maternal cardiac event was defined as cardiac death, arrhythmia requiring treatment, heart failure, thromboembolic event, aortic dissection, endocarditis, acute coronary syndrome, and hospitalization for other cardiac reasons or cardiac intervention. Associations between patient characteristics and cardiac outcomes were checked in a 3-level model (patient–center–country). Results: Most patients came from emerging countries (75%). Mitral stenosis (MS) with or without mitral regurgitation (MR) was present in 273 women, isolated MR in 117. The degree of MS was mild in 20.9%, moderate in 39.2%, severe in 19.8%, and severity not classified in the remainder. Maternal death during pregnancy occurred in 1 patient with severe MS. Hospital admission occurred in 23.1% of the women with MS, and the main reason was heart failure (mild MS 15.8%, moderate 23.4%, severe 48.1%; P 1 was an independent predictor of maternal cardiac events. Follow-up at 6 months postpartum was available for 53%, and 3 more patients died (1 with severe MS, 1 with moderate MS, 1 with moderate to severe MR). Conclusions: Although mortality was only 1.9% during pregnancy, ≈50% of the patients with severe rheumatic MS and 23% of those with significant MR developed heart failure during pregnancy. Prepregnancy counseling and considering mitral valve interventions in selected patients are important to prevent these complications.

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.

Background In recent years, non-vitamin K antagonist (VKA) oral anticoagulants (NOACs) have been increasingly prescribed to adults with congenital heart disease (CHD) and atrial arrhythmias without good evidence for either safety or efficacy. To address this gap, we initiated an ongoing prospective global registry (NOTE: non-vitamin K antagonist oral anticoagulants for thromboembolic prevention in patients with congenital heart disease). Using the NOTE registry data, the present study aimed to evaluate the occurrence of any adverse events during the initiation phase (first 30 days) of NOACs in adults with CHD and atrial arrhythmias. Methods and Results For this prospective observational study, 99 adults with CHD and atrial arrhythmias (median age 49 years [IQR 38-61], 53% male) who initiated NOACs at or after the inclusion point were analysed. Thromboembolic events, major bleeding and other minor adverse events were assessed after the first 30 days since the initiation of NOACs. In 54 patients transitioning from VKA to NOACs, 8 minor adverse events (5 minor bleeding; 3 side-effects; 1 drop-out due to minor bleeding) occurred within 30 days after the transition. No adverse events were reported in 46 VKA-naive patients within 30 days after the initiation of NOACs. Conclusions Initiation of NOACs and transition from VKA to NOACs seem to be safe during the first month, without major adverse events and with only limited minor side effects in adults with CHD and atrial arrhythmias. This global ongoing prospective registry enables precise collection of important clinical information in real-world adults with CHD, managed with NOACs. Electronic supplementary material The online version of this article (doi:10.1007/s10557-017-6745-y) contains supplementary material, which is available to authorized users.