Pulmonary blood flow in children with univentricular heart and unilateral diaphragmatic paralysis.

OBJECTIVES: Spontaneous breathing has an important effect on pulmonary arterial blood flow in patients with Glenn/Fontan circulation. Unilateral diaphragmatic paralysis (DP) is a frequent complication after heart surgery in congenital heart disease. The aim of this study was to investigate the influence of unilateral DP on blood flow distribution in the pulmonary arteries with Glenn/Fontan circulation. METHODS: Magnetic resonance phase-contrast imaging was used to evaluate stroke volume index (SVI) in the left and right pulmonary arteries in patients with Glenn/Fontan circulation with unilateral DP. Data for 18 patients with univentricular heart and unilateral DP were analysed, 8 in the Glenn stage and 10 in the Fontan stage. Ten patients had right-sided DP, and 8 had left-sided DP. A diaphragmatic plication was performed in 7 patients. The control group consisted of 36 patients with Glenn (n = 16)/Fontan (n = 20) circulation without DP. RESULTS: In both left- and right-sided DP, the SVI to the ipsilateral side was significantly lower than in controls [2.81 (1.45-4.50) ml/m2 left vs 11.97 (7.36-16.37) ml/m2 in controls, P < 0.0002; 8.2 (4.49-12.64) ml/m2 with right vs 12.64 (9.66-16.61) ml/m2 in controls; P = 0.0284]. The SVI to the contralateral side showed a slight but non-significant increase in the presence of unilateral DP. CONCLUSIONS: Unilateral DP in patients with Glenn/Fontan circulation has a negative impact on pulmonary arterial SVI on the side of the paralysis.

[Episodic bleeding events due to arteriovenous malformation (AVM) in the region of the distal ileum in a young male patient]. / Episodenhafte Blutungsereignisse bei arteriovenöser Malformation (AVM) im Bereich des distalen Ileums beim jungen Patienten.

We report the case of a 32-year-old male patient who presented with episodic, self-limiting gastrointestinal bleeding events. After both esophagogastroduodenoscopy (EGD) and colonoscopy remained unremarkable, capsule endoscopy revealed an unexplained mucosal lesion that presented as an ulcerated process on spiral enteroscopy. Appropriate enteroscopic ink marking was followed by surgical partial resection of the distal ileum, with histopathology revealing evidence of an arteriovenous malformation (AVM). This case emphasizes the importance of deep enteroscopy both in the diagnosis and to facilitate therapeutic resection in rare gastrointestinal bleeding events affecting young people.

Dynamic behaviour of platinum and copper dopants in gold nanoclusters supported on ceria catalysts.

Understanding the behaviour of active catalyst sites at the atomic level is crucial for optimizing catalytic performance. Here, the evolution of Pt and Cu dopants in Au25 clusters on CeO2 supports is investigated in the water-gas shift (WGS) reaction, using operando XAFS and DRIFTS. Different behaviour is observed for the Cu and Pt dopants during the pretreatment and reaction. The Cu migrates and builds clusters on the support, whereas the Pt creates single-atom active sites on the surface of the cluster, leading to better performance. Doping with both metals induces strong interactions and pretreatment and reaction conditions lead to the growth of the Au clusters, thereby affecting their catalytic behaviour. This highlights importance of understanding the behaviour of atoms at different stages of catalyst evolution. These insights into the atomic dynamics at the different stages are crucial for the precise optimisation of catalysts, which ultimately enables improved catalytic performance.

The impact of the COVID-19 pandemic on speech and language therapy services in Ireland: A mixed-methods survey at two time points during the pandemic.

BACKGROUND: During the COVID-19 pandemic, Ireland implemented a series of stringent public health measures, including lockdowns and suspension of non-urgent clinical services. AIMS: To investigate the impact of the COVID-19 pandemic on the demand for and delivery of speech and language therapy (SLT) services in Ireland in 2020. METHODS & PROCEDURES: Two iterations of a cross-sectional, mixed-methods online survey were distributed to speech and language therapists (SLTs) and SLT students in Ireland in the spring and autumn of 2020 using a combination of purposive and snowball sampling. The spring survey yielded 407 responses (including 14 from SLT students), while 197 respondents took part in the autumn (13 students). Survey analysis focused on questions related to the impact of the COVID-19 pandemic on delivery and demand for SLT services (student responses were excluded from analysis owing to low response rate). The largest group in respect of experience were senior SLTs (58% in both surveys). The work settings most strongly represented were HSE primary care (34.4%) and disability services (26.5%) in the spring, and HSE primary care (39.1%), acute hospitals (22.8%) and disability services (20.8%) in the autumn. We used descriptive statistics, including distribution analysis, to analyse the quantitative data. Free text data were interrogated through a variant of a conventional qualitative content analysis. OUTCOMES & RESULTS: In the spring, cessation of face-to-face services featured prominently (reported by 65.6% versus 14.2% in the autumn), across SLTs' work settings, except acute hospitals. Lower demand was reported by 42.5% in the spring, while in the autumn, 48.7% indicated that demand was higher. SLTs experienced large-scale redeployment (spring: 45.9%, autumn: 38.4%), with HSE primary care SLTs redeployed most (spring: 71.7%; autumn: 62.3%). The need to suddenly pivot to telehealth was a significant challenge in terms of training, technology and logistics. New ways of working emerged and gradually, telehealth became more embedded. SLTs also had to adapt to working with evolving public health measures, such as space restrictions and personal protective equipment (PPE) requirements. Across the two survey iterations, SLTs reported tensions between demands and capacity: while referrals and demand initially decreased in the spring, this led to increased backlog and longer waiting lists, ongoing and increasing pressure on clinicians and services, and negatively impacted clients and families. CONCLUSIONS & IMPLICATIONS: The COVID-19 pandemic had a significant negative impact on SLT services in Ireland. Going forward, the SLT profession and its services will require sustained support to mitigate long-term negative consequences, such as increased waiting lists. WHAT THIS PAPER ADDS: What is already known on this subject The negative impact of the COVID-19 pandemic on SLT services has been examined in several countries. Ireland imposed more stringent pandemic-management measures than many other countries, and it was therefore warranted to investigate how SLT services in the country were affected. What this study adds to the existing knowledge Face-to-face SLT services effectively ceased in most non-urgent contexts in spring 2020. This coincided with large-scale redeployment of SLTs to non-SLT contexts. By autumn 2020, demand had increased again, but not all services had recommenced, and redeployment was still a factor. Although SLTs adapted to the ongoing changes imposed by the pandemic, they voiced concern about increasing backlogs and longer waiting lists, ongoing and increasing pressure on both SLTs and services, and negative impacts on clients and families. What are the actual and clinical implications of this work? The COVID-19 pandemic had a significant negative impact on SLT services in Ireland. Going forward, the SLT profession and its services will require sustained support to mitigate long-term negative consequences, such as increased waiting lists.

Implications of pediatric extracorporeal cardiopulmonary resuscitation simulation for intensive care team confidence and coordination: A pilot study.

INTRODUCTION: Extracorporeal cardiopulmonary resuscitation (ECPR) is associated with improved outcomes in select populations, however, crisis resource management (CRM) in this setting is logistically challenging. This study evaluates the impact of ECPR simulation on self-perceived confidence and collaboration of intensive care unit team members. METHODS: This is a prospective observational study analyzing data obtained between July 2018-December 2019. This study focused on non-surgical members of critical care team consisting of pediatric intensivists, resident physicians, registered nurses, respiratory therapists. Participants were expected to perform cardiopulmonary resuscitation (CPR) during the ECPR event, participate in code-team responsibilities and provide ancillary support during cannulation. Pre- and post-simulation surveys employed the Likert scale (1 = not at all confident, 5 = highly confident) to assess self-perceived scores in specified clinical competencies. RESULTS: Twenty-nine providers participated in the simulation; 38% had prior ECPR experience. Compared to mean pre-study Likert scores (2.4, 2.4, 2.5), post-simulation scores increased (4.2, 4.4, 4.3) when self-evaluating: confidence in assessing patients needing ECPR, confidence in participating in ECPR workflow and confidence in performing high-quality CPR, respectively. Post-simulation values of >3 were reported by 100% of participants in all domains (p < .0001). All participants indicated the clinical scenario and procedural environment to be realistic and appropriately reflective of situational stress. Additionally, 100% of participants reported the simulation to improve perceived team communication and teamwork skills. CONCLUSION: This study demonstrated preliminary feasibility of pediatric ECPR simulation in enhancing independent provider confidence and team communication. This self-perceived improvement may establish a foundation for cohesive CRM, in preparation for a real life ECPR encounter.

Consonant articulation acoustics and intelligibility in Swedish speakers with Parkinson's disease: a pilot study.

Imprecise consonant articulation is common in speakers with Parkinson's disease and can affect intelligibility. The research on the relationship between acoustic speech measures and intelligibility in Parkinson's disease is limited, and most of the research has been conducted on English. This pilot study investigated aspects of consonant articulation acoustics in eleven Swedish speakers with Parkinson's disease and six neurologically healthy persons. The focus of the study was on consonant cluster production, articulatory motion rate and variation, and voice onset time, and how these acoustic features correlate with speech intelligibility. Among the measures in the present study, typicality ratings of heterorganic consonant clusters /spr/ and /skr/ had the strongest correlations with intelligibility. Measures based on syllable repetition, such as repetition rate and voice onset time, showed varying results with weak to moderate correlations with intelligibility. One conclusion is that some acoustic measures may be more sensitive than others to the impact of the underlying sensory-motor impairment and dysarthria on speech production and intelligibility in speakers with Parkinson's disease. Some aspects of articulation appear to be equally demanding in terms of acoustic realisation for elderly healthy speakers and for speakers with Parkinson's disease, such as sequential motion rate measures. Clinically, this would imply that for the purpose of detecting signs of disordered speech motor control, choosing measures with less variation among older speakers without articulation impairment would lead to more robust results.

Conversations in dementia with Lewy bodies: Resources and barriers in communication.

BACKGROUND: In dementia with Lewy bodies (DLB), limitations in linguistic and cognitive abilities may lead to difficulties in participating in conversations. The conversational outcome is also dependent on how the conversation partner adjusts to potential communicative challenges. AIMS: This study explored resources and barriers in communication in DLB. METHODS & PROCEDURES: Linguistic and cognitive function was explored through standard clinical testing. The dyad's perception of function in daily life was explored through semi-structured interviews analysed with content analysis. Interactional patterns and participation in casual conversation was analysed with conversation analysis. OUTCOME & RESULTS: The results show how the husband diagnosed with DLB performed with high scores across most cognitive and linguistic test tasks. The interview data, however, revealed how both he and his wife experienced significant challenges regarding, for example, conversational tempo, as well as negative feelings relating to adjusting to these conversational changes. The interactional data from the casual conversation revealed, among other patterns, how the wife engaged in most of the storytelling in the conversation. The husband contributed details when his wife asked for help, or he acknowledged a faulty or missing detail in his wife's storyline. Thus, they both oriented to the husband's competence in monitoring and keeping track of the conversational content, despite challenges in taking the floor. CONCLUSIONS & IMPLICATIONS: A holistic picture of communication in DLB necessitates the use of different evaluation approaches. Both monological (e.g., test tasks revealing cognitive and linguistic resources) and dialogical information sources (e.g., observations of conversations revealing adjustments in conversations), as well as the perceptions of those engaging in everyday conversations (i.e., people with DLB and their conversation partner(s)), need to be evaluated when assessing resources and barriers in communication. WHAT THIS PAPER ADDS: What is already known on the subject It is well-known that dementia with Lewy bodies (DLB) affects language and cognition. In conversations, persons with DLB experience difficulties in turn-taking, topic initiation, entering conversations and keeping up with the conversational tempo. What this study adds This study sheds light on conversations in one dyad where the husband has been diagnosed with DLB. The results from three different information sources (testing of language and cognition, interviews and a video-recorded conversation) reveal patterns of resources and barriers that at first appear to contradict each other. However, the contradictions can be resolved when these discrepancies are examined in light of the differences in task structure, in terms of, for example, predetermined topics and how turn-taking is managed. What are the potential or actual clinical implications of this work? In order to gather a holistic picture of a person's conversational abilities, clinicians need to include information from both monological tasks (e.g., linguistic testing) as well as dialogical tasks (e.g., video recordings from conversation). The results also need to be evaluated in light of all conversation partners' perspectives on function in daily life. Furthermore, it is important to consider the nature of assessment tasks (particularly their interactional structure) when interpreting assessment results.

Conceptualizations of aging and cognitive competence: Evaluations and accounts after cognitive testing.

AIM: To investigate how conceptualizations of cognition and aging are displayed through evaluations and accounts after cognitive testing with elders who do not have dementia. METHOD: 14 persons >65 years of age without clinical memory loss were tested with the MMSE and interviewed about their experiences. The audio-recordings were transcribed according to Conversation Analytic conventions. Examples of evaluations and accounts related to cognitive competence were collected, categorized, and analyzed by topic. RESULTS: The results show how the interviewer and participant being tested evaluate and account for face threats related to the cognitive and communicative competence of the person being tested. Examples of evaluations include downplaying one's own competence, while accounts include attributing difficulties with test tasks to personal circumstances, such as participants' lived experiences or to expected (as opposed to pathological) cognitive change in aging. CONCLUSIONS: Inviting participants to share their experiences of cognitive testing can reveal valuable information on how they conceptualize cognition and aging, as well as the testing process itself. Reflections may also reveal potential dementia worry. Evaluations and accounts are closely related to face-saving acts.

Influence of 24 h Simulated Altitude on Red Blood Cell Deformability and Hematological Parameters in Patients with Fontan Circulation.

Patients with Fontan circulation are particularly dependent on low pulmonary vascular resistance because their lungs are passively perfused. Hypoxia drives pulmonary vasoconstriction; thus, red blood cell (RBC) deformability and stability of hematological parameters might be of particular importance, because alterations during hypoxia might further influence circulation. This study aimed to measure respective parameters in patients with Fontan circulation exposed to normobaric hypoxia. A total of 18 patients with Fontan circulation (16 to 38 years) were exposed to normobaric hypoxia (15.2% ambient oxygen). Blood samples were taken in normoxia, after 24 h in hypoxia, and 60 min after return to normoxia. Blood count, RBC age distribution, EPO, RBC deformability, marker of RBC nitric oxide, oxidative state, and RBC ATP were measured. Hypoxia increased oxidative stress in RBC, but without affecting RBC deformability. RBC age distribution remained unaffected, although EPO concentrations increased, followed by a rise in reticulocyte count at an already high hematocrit. NO metabolism was not affected by hypoxia. Modest normobaric hypoxia for 24 h did not impair RBC deformability in patients with Fontan circulation; however, the oxidative system seemed to be stressed. Given the high baseline Hct in these patients, hypoxia-induced erythropoiesis could adversely affect rheology with more prolonged hypoxia exposure.

Evaluation of the Roche point of care system for determination of NT-proBNP in urine samples.

INTRODUCTION: The diagnostic potential of urine NT-pro-BNP has been studied recently and shows great promise, especially for children. Bedside serum-NT-proBNP determination with point of care testing (POCT) is established in adults. This study investigated the analytical capabilities of NT-proBNP POCT measurements for urine-samples. Furthermore, the stability of this biomarker in urine samples at room-temperature was explored. METHODS: 104 urine samples were analysed, partly with double and triple determinations and compared to the established Elecsys system. To investigate the stability of NT-proBNP in urine, five samples were stored at room temperature and analysed 12-hourly over 96 h. RESULTS: 34 % of samples lay outside the POCT range (60 pg/ml-9000 pg/ml). The 69 values within the range were correlated with those measured by Elecsys (correlation coefficient of 0.92; p < 0.001). Linear regression yields an r2 of 0.84. The stability of urine NT-proBNP was shown with correlation coefficients between 0.97 and 1 (p < 0.001). CONCLUSION: Analysing NT-ProBNP in urine with cobas h232 POC system is feasible, but the detection range needs to be adjusted. As urine NT-proBNP proved stable for at least 96 h even postal shipping would be possible. Adjusting this system's measuring range could help introduce a new, non-invasive, pain-free parameter.

Adequate exercise response at artificial altitude in Fontan patients.

Purpose: For Fontan-palliated patients, altitude exposure is still a part of discussion since the extent of hypoxic pulmonary vasoconstriction potentially resulting in decreasing cardiac output (Qc), especially during physical exercise, is still unclear. We investigated the effects of normobaric hypoxia (15.2% O2) simulating 2,500 m above sea level on cardiopulmonary and metabolic parameters and the benefit of daily physical activity (PA) on hypoxic exercise capacity. Methods: A total of 21 Fontan patients (14-31 years) and 20 healthy controls performed cardiopulmonary exercise tests on a bicycle ergometer in normoxia and hypoxia until subjective exhaustion, measuring capillary lactate (cLa) every 2 min. In between, participants underwent an activity tracking over 5 days with a triaxial accelerometer. Results: Hypoxic exercise was well tolerated by Fontan patients, and no adverse clinical events were observed. Fontan patients showed reduced physical capacity under both conditions compared to controls (63% normoxia, 62% hypoxia), but the relative impairment due to hypoxia was similar for both (≈10%). Up to workloads of 2 W/kg oxygen uptake ( V . O2) and heart rate (HR) developed similarly in patients and controls. cLa increased faster in relation to workload in Fontan patients, but remained significantly lower at peak workload (normoxia 3.88 ± 1.19 mmol/l vs. 7.05 ± 2.1 mmol/l; hypoxia 4.01 ± 1.12 mmol/l vs. 7.56 ± 1.82 mmol/l). Qc was diminished but could be increased similar to controls. Fontan patients with higher PA levels showed a higher V . O2peak in hypoxia. Conclusion: Exercise during short-time artificial altitude exposure seems to be safe for young Fontan patients. Further studies are needed to validate longer exposure under real conditions. V . O2, HR, and Qc might not be a limiting factor for exercise until workloads of 2 W/kg. Higher daily PA levels might improve physical capacity under altitude conditions.

Anger and aspirations: Linguistic analysis of identity after traumatic brain injury.

It has been well established that positive identity construction or reconstruction is important for positive rehabilitation outcomes after Traumatic Brain Injury (TBI). Such identities are constructed and communicated through interactional exchanges. In this study, the tools of systemic functional linguistics are used to investigate how language was used to create and establish identities for three participants with a severe TBI. Four conversations related to each participant with TBI were analysed. These included a casual conversation between a stranger and the participant, as well as ethnographic interviews with each participant, their closest family member, and the staff member that worked most closely with each at the residential facility. Topic analysis revealed that topics of anger and of aspirations were commonly discussed by participants and their caregivers, and a detailed analysis of identity construction within these exchanges is presented. The data highlights how lack of independence and autonomy is associated with anger, and how aspirations and goal setting may facilitate positive outcomes. In conclusion, language analysis methods can provide detailed information about how individuals with TBI communicate and negotiate identities. Such identities may not always match those projected by their caregivers. This research highlights implications for language interventions that promote identity development.

Exome sequencing in individuals with cardiovascular laterality defects identifies potential candidate genes.

The birth prevalence of laterality defects is about 1.1/10,000 comprising different phenotypes ranging from situs inversus totalis to heterotaxy, mostly associated with complex congenital heart defects (CHD) and situs abnormalities such as intestinal malrotation, biliary atresia, asplenia, or polysplenia. A proportion of laterality defects arise in the context of primary ciliary dyskinesia (PCD) accompanied by respiratory symptoms or infertility. In this study, exome sequencing (ES) was performed in 14 case-parent trios/quattros with clinical exclusion of PCD prior to analysis. Moreover, all cases and parents underwent detailed clinical phenotyping including physical examination, echocardiography by a skilled paediatric cardiologist and abdominal ultrasound examinations not to miss mildly affected individuals. Subsequent survey of the exome data comprised filtering for monoallelic de novo, rare biallelic, and X-linked recessive variants. In two families, rare variants of uncertain significance (VUS) in PKD1L1 and ZIC3 were identified. Both genes have been associated with laterality defects. In two of the remaining families, biallelic variants in LMBRD1 and DNAH17, respectively, were prioritized. In another family, an ultra-rare de novo variant in WDR47 was found. Extensive exome survey of 2,109 single exomes of individuals with situs inversus totalis, heterotaxy, or isolated CHD identified two individuals with novel monoallelic variants in WDR47, but no further individuals with biallelic variants in DNAH17 or LMBRD1. Overall, ES of 14 case-parent trios/quattros with cardiovascular laterality defects identified rare VUS in two families in known disease-associated genes PKD1L1 and ZIC3 and suggests DNAH17, LMBRD1, and WDR47 as potential genes involved in laterality defects.

Performance of urinary NT-proBNP in ambulatory settings.

BACKGROUND: NT-proBNP (N-terminal prohormone of brain natriuretic peptide) has been established as a useful biomarker in plasma for children with congenital heart disease (CHD). Plasma values were shown to correlate well with urinary values. We designed a study to investigate the general utility of urinary NT-proBNP in children with and without CHD in an ambulatory setting. MATERIAL AND METHODS: 202 children (mean age 93 months (1-225 months)) were included in the analysis. We investigated the performance of urinary NT-proBNP values determined from spot urine as a diagnostic tool for different forms of congenital heart disease. RESULTS: Urinary NT-proBNP is a good diagnostic tool for children with congenital heart disease (ROC area under the curve 0.807). Combining these values with the Ross-classification further improves the diagnostic power (ROC area under the curve 0.831) Analysis also showed significant differences between Lg10 urinary NT-proBNP values of healthy controls and those of children after corrective surgery. Furthermore, children who have completed the stages of Fontan palliation showed higher values than age matched controls. CONCLUSIONS: Urinary NT-proBNP can be used in an ambulatory setting to discriminate between relevant and nonrelevant CHD and might be valuable as a follow up parameter for children after biventricular repair or univentricular palliation. Age dependant urinary NT-proBNP normal values for children could be an easy-to-use tool for general practitioners as well as specialised clinics.

Picture Description in the Assessment of Connected Speech Intelligibility in Parkinson's Disease: A Pilot Study.

INTRODUCTION: Assessment of intelligibility in dysarthria tends to rely on oral reading of sentences or words. However, self-generated utterances are closer to a client's natural speech. This study investigated how transcription of utterances elicited by picture description can be used in the assessment of intelligibility in speakers with Parkinson's disease. METHODS: Speech samples from eleven speakers with Parkinson's disease and six neurologically healthy persons were audio-recorded. Forty-two naive listeners completed transcriptions of self-generated sentences from a picture description task and orally read sentences from the Swedish Test of Intelligibility, as well as scaled ratings of narrative speech samples. RESULTS: Intelligibility was higher in orally read than self-generated sentences and higher for content words than for the whole sentence in self-generated sentences for most of the speakers, although these within-group differences were not statistically significant at the group level. Adding contextual leads for the listeners increased intelligibility in self-generated utterances significantly but with individual variation. Although correlations between the intelligibility measures were at least moderate or strong, there was a considerable inter- and intra-speaker variability in intelligibility scores between tasks for the speakers with Parkinson's disease, indicating individual variation of factors that impact intelligibility. Intelligibility scores from neurologically healthy speakers were generally high across tasks with no significant differences between the conditions. CONCLUSION: Within-speaker variability supports literature recommendations to use multiple methods and tasks when assessing intelligibility. The inclusion of transcription of self-generated utterances elicited by picture description to the intelligibility assessment has the potential to provide additional information to assessment methods based on oral reading of pre-scripted sentences and to inform the planning of interventions.

Swedish Clinical Professionals' Perspectives on Evaluating Cognitive and Communicative Function in Dementia.

Objectives: This study investigated Swedish clinical professionals' experiences of diagnostic pathways in dementia, focusing on the assessment of cognitive and communicative abilities.Methods: Interdisciplinary teams in Memory Clinics, General Practitioners in Primary Health Care, and Speech Language Pathologists were interviewed. The transcripts were analyzed using qualitative Content Analysis.Results: The study sheds light upon the perceived barriers and facilitators of good practice, e.g. time and clinical collaborations. Perspectives among professionals vary as to how informal and formal information and procedures are to be integrated and weighted. External factors (e.g. physical proximity of professions) have considerable influence on information availability, transmission, and diagnostic processes. Communication impairment does not emerge as a clinical priority.Conclusions: Published clinical guidelines notwithstanding, there is in practice no "gold standard" regarding diagnostic processes. Reorganization of services that impact feasibility of cross-disciplinary contact may negatively impact diagnostics.Clinical implications: Interprofessional collaboration is impacted by many factors, e.g. physical proximity and availability of specific professions. In order to optimize collaboration in dementia diagnosis, communication channels between professions need to be optimized. Additionally, making clinical impressions and "gut-feelings" explicit could contribute valuable information to the diagnostic process.

The latest development of the DELAD project for sharing corpora of speech disorders.

Corpora of speech of individuals with communication disorders (CSD) are invaluable resources for education and research, but they are costly and hard to build and difficult to share for various reasons. DELAD, which means 'shared' in Swedish, is a project initiated by Professors Nicole Müller and Martin Ball in 2015 that aims to address this issue by establishing a platform for researchers to share datasets of speech disorders with interested audiences. To date four workshops have been held, where selected participants, covering various expertise including researchers in clinical phonetics and linguistics, speech and language therapy, infrastructure specialists, and ethics and legal specialists, participated to discuss relevant issues in setting up such an archive. Positive and steady progress has been made since 2015, including refurbishing the DELAD website (http://delad.net/) with information and application forms for researchers to join and share their datasets and linking with the CLARIN K-Centre for Atypical Communication Expertise (https://ace.ruhosting.nl/) where CSD can be hosted and accessed through the CLARIN B-Centres, The Language Archive (https://tla.mpi.nl/tools/tla-tools/) and TalkBank (https://talkbank.org/). The latest workshop, which was funded by CLARIN (Common Language Resources and Technology Infrastructure) was held as an online event in January 2021 on topics including Data Protection Impact Assessments, reviewing changes in ethics perspectives in academia on sharing CSD, and voice conversion as a mean to pseudonomise speech. This paper reports the latest progress of DELAD and discusses the directions for further advance of the initiative, with information on how researchers can contribute to the repository.

Systemic ventricular function in Fontan patients at rest and after exercise at altitude.

Objective: Physical activity at high altitude is expected to pose risks for patients with Fontan circulation and to impair systemic ventricular function. This study aims to determine the effect of high-altitude hypoxia on ventricular function in Fontan patients at rest and after exercise. We hypothesize that systemic ventricular function deteriorates under hypoxic conditions in Fontan patients. Methods: In this prospective study, 21 Fontan patients (NYHA class I-II) and 21 age-, gender- and body mass index-matched healthy controls were enrolled (median age 17.9 and 16.9 years). Transthoracic echocardiography was performed at rest, after peak (PE) and after continuous exercise (CE) in normoxia and hypoxia at simulated altitude (2,500 m above sea level). The effect of hypoxia on echocardiographic parameters was quantified by linear mixed-effects models and the difference between normoxia and hypoxia (Δ= hypoxia-normoxia). Results: At rest, cardiac output (CO) estimated by outflow tract velocity time integral × heart rate and annular plane systolic excursion (APSE) were lower in hypoxia compared to normoxia in Fontan patients (CO: Δ = -12.0%, n.s.; APSE: Δ = -9.6%, p < 0.001), an increase was observed in controls (CO: Δ = 8.5%, n.s.; APSE: Δ = 2.5%, n.s.). Other parameters of systolic and diastolic function did not show relevant changes. After exercise under hypoxic conditions, Fontan patients did not show relevant deterioration of systolic function compared to normoxia. Late, active diastolic filling reflected by A-wave velocity remained unchanged in Fontan patients, but increased in controls. Under hypoxic conditions, CO and workload were higher after CE than PE in Fontan patients (CO: PE Δ = 1,530 vs. CE 1630), whereas controls showed higher work load and CO estimates after PE than CE as expected (CO: PE Δ = 2,302 vs. CE 2149). Conclusion: Fontan patients clinically tolerated short-term altitude exposure up to two hours and exercise and showed no consistent deterioration of systolic systemic ventricular function, but parameters of myocardial contractility, heart rate and cardiac output did not increase as observed in controls. This is likely to be multifactorial and may include intrinsic cardiac dysfunction as well as preload inadequacy and the lack of augmented atrial contraction. CE may be better tolerated than PE.

Evaluation of a Paper-Based Checklist versus an Electronic Handover Tool Based on the Situation Background Assessment Recommendation (SBAR) Concept in Patients after Surgery for Congenital Heart Disease.

(1) Background: we compare a new SBAR based electronic handover tool versus a paper-based checklist for handover in a pediatric intensive care unit (PICU). (2) Methods: this is a randomized, observational study of 40 electronic vs. 40 paper checklist handovers after pediatric cardiac surgery, with a 48 items checklist for comparison of reporting frequencies and notification of disturbances and noise. PICU staff satisfaction was evaluated by a 12-item questionnaire. (3) Results: in 14 out of 40 cases, there were problems with data processing (incomplete or no data processing). Some item groups (e.g., hemodynamics) were consistently reported at higher frequencies than other groups. Items not specifically asked for did not get reported. Some items, automatically processed in the SBAR handover page, did not get reported. Many handovers suffered a noisy and distracting atmosphere. There was no difference in staff satisfaction between the two handover approaches. Nurses were highly unsatisfied with the general approach by which the handover was performed. (4) Conclusions: human error appears to be a main factor for unreliable data processing. Software is still too complicated, and multitasking is a stressful and error prone event. Handover is a complex task with many factors required for a successful completion.