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Background and aims: The objective of this study was to assess the associations of birth weight with cardiac structure and function in adults with dextro-transposition of the great arteries (D-TGA) who underwent the arterial switch operation (ASO). Methods and results: Thirty-nine ASO patients (age 24.4 ± 3.3 years) were included during routine clinical follow-up from July 2019 to December 2021. All patients underwent cardiopulmonary exercise testing and cardiac magnetic resonance imaging at rest and during exercise. Early-life characteristics, including birth weight, were extracted from electronic medical health records. Linear regression analysis showed that lower birth weight was associated with smaller left ventricular (LV) and right ventricular (RV) end-diastolic volume index (LV: −14.5 mL/m2 [95 % confidence interval, CI: −26.5 to −2.5] per 1-kg decrease in birth weight, p = 0.04; RV: −11.2 mL/m2 [-20.7 to −1.7] per 1-kg decrease in birth weight, p = 0.03). Lower birth weight was associated with greater LV and RV ejection fraction at rest (LV: +8.5 % [+4.4 to +12.5] per 1-kg decrease in birth weight, p

Background A risk model has been proposed to provide a patient individualized estimation of risk for major clinical events (heart failure events, ventricular arrhythmia, all‐cause mortality) in patients with transposition of the great arteries and atrial switch surgery. We aimed to externally validate the model. Methods and Results A retrospective, multicentric, longitudinal cohort of 417 patients with transposition of the great arteries (median age, 24 years at baseline [interquartile range, 18–30]; 63% men) independent of the model development and internal validation cohort was studied. The performance of the prediction model in predicting risk at 5 years was assessed, and additional predictors of major clinical events were evaluated separately in our cohort. Twenty‐five patients (5.9%) met the major clinical events end point within 5 years. Model validation showed good discrimination between high and low 5‐year risk patients (Harrell C index of 0.73 [95% CI, 0.65–0.81]) but tended to overestimate this risk (calibration slope of 0.20 [95% CI, 0.03–0.36]). In our population, the strongest independent predictors of major clinical events were a history of heart failure and at least mild impairment of the subpulmonary left ventricle function. Conclusions We reported the first external validation of a major clinical events risk model in a large cohort of adults with transposition of the great arteries. The model allows for distinguishing patients at low risk from those at intermediate to high risk. Previous episode of heart failure and subpulmonary left ventricle dysfunction appear to be key markers in the prognosis of patients. Further optimizing risk models are needed to individualize risk predictions in patients with transposition of the great arteries.

Summary: Background: Dysregulation of the gut microbiome has been implicated in Parkinson's disease (PD). This study aimed to evaluate the clinical effects and safety of a single faecal microbiota transplantation (FMT) in patients with early-stage PD. Methods: The GUT-PARFECT trial, a single-centre randomised, double-blind, placebo-controlled trial was conducted at Ghent University Hospital between December 01, 2020 and December 12, 2022. Participants (aged 50–65 years, Hoehn and Yahr stage 2) were randomly assigned to receive nasojejunal FMT with either healthy donor stool or their own stool. Computer-generated randomisation was done in a 1:1 ratio through permutated-block scheduling. Treatment allocation was concealed for participants and investigators. The primary outcome measure at 12 months was the change in the Movement Disorders Society-Unified Parkinson's Disease Rating Scale (MDS-UPDRS) motor score obtained during off-medication evaluations. Intention-to-treat analysis was performed using a mixed model for repeated measures analysis. This completed trial is registered on ClinicalTrials.gov (NCT03808389). Findings: Between December 2020 and December 2021, FMT procedures were conducted on 46 patients with PD: 22 in the healthy donor group and 24 in the placebo group. Clinical evaluations were performed at baseline, 3, 6, and 12 months post-FMT. Full data analysis was possible for 21 participants in the healthy donor group and 22 in the placebo group. After 12 months, the MDS-UPDRS motor score significantly improved by a mean of 5.8 points (95% CI −11.4 to −0.2) in the healthy donor group and by 2.7 points (−8.3 to 2.9) in the placebo group (p = 0.0235). Adverse events were limited to temporary abdominal discomfort. Interpretation: Our findings suggested a single FMT induced mild, but long-lasting beneficial effects on motor symptoms in patients with early-stage PD. These findings highlight the potential of modulating the gut microbiome as a therapeutic approach and warrant a further exploration of FMT in larger cohorts of patients with PD in various disease stages. Funding: Flemish PD patient organizations (VPL and Parkili), Research Foundation Flanders (FWO), Biocodex Microbiota Foundation.

ABSTRACTAs small and stable high-affinity antigen binders, VHHs boast attractive characteristics both for therapeutic use in various disease indications, and as versatile reagents in research and diagnostics. To further increase the versatility of VHHs, we explored the VHH scaffold in a structure-guided approach to select regions where the introduction of an N-glycosylation N-X-T sequon and its associated glycan should not interfere with protein folding or epitope recognition. We expressed variants of such glycoengineered VHHs in the Pichia pastoris GlycoSwitchM5 strain, allowing us to pinpoint preferred sites at which Man5GlcNAc2-glycans can be introduced at high site occupancy without affecting antigen binding. A VHH carrying predominantly a Man5GlcNAc2 N-glycan at one of these preferred sites showed highly efficient, glycan-dependent uptake by Mf4/4 macrophages in vitro and by alveolar lung macrophages in vivo, illustrating one potential application of glyco-engineered VHHs: a glycan-based targeting approach for lung macrophage endolysosomal system delivery. The set of optimal artificial VHH N-glycosylation sites identified in this study can serve as a blueprint for targeted glyco-engineering of other VHHs, enabling site-specific functionalization through the rapidly expanding toolbox of synthetic glycobiology.

Purpose: Several studies have demonstrated increased auditory thresholds in patients with Parkinson's disease (PD) based on subjective tonal audiometry. However, the pathophysiological mechanisms underlying auditory dysfunction in PD remain elusive. The primary aim of this study was to investigate cochlear and olivocochlear function in PD using objective measurements and to assess the effect of dopaminergic medication on auditory function. Method: Eighteen patients with PD and 18 gender- and age-matched healthy controls (HCs) were included. Patients with PD participated in medication on and off conditions. Linear mixed models were used to determine the effect of PD on tonal audiometry, transient evoked and distortion product otoacoustic emissions (OAEs), and efferent suppression (ES). Results: Tonal audiometry revealed normal auditory thresholds in patients with PD for their age across all frequencies. OAE signal amplitudes demonstrated a significant interaction effect between group (PD vs. HC) and frequency, indicating decreased OAEs at low frequencies and increased OAEs at high frequencies in patients with PD. No significant differences were found between patients with PD and HCs regarding ES. In addition, no significant effect of medication status was found on auditory measurements in patients with PD. Conclusions: Altered OAEs support the hypothesis of cochlear alterations in PD. No evidence was found for the involvement of the medial olivocochlear system. Altogether, OAEs may provide an objective early indicator of auditory alterations in PD and should complement subjective tonal audiometry when assessing and monitoring auditory function in PD.

IntroductionEngaging in meaningful activities contributes to health and wellbeing. Research identifies meaningfulness by analysing retrospective and subjective data such as personal experiences in activities. Objectively measuring meaningful activities by registering the brain (fNIRS, EEG, PET, fMRI) remains poorly investigated.MethodsA systematic review using PubMed, Web of Science, CINAHL, and Cochrane Library.FindingsThirty-one studies investigating the correlations between daily activities in adults, their degree of meaningfulness for the participant, and the brain areas involved, were identified. The activities could be classified according to the degree of meaningfulness, using the attributes of meaningfulness described in the literature. Eleven study activities contained all attributes, which means that these can be assumed to be meaningful for the participant. Brain areas involved in these activities were generally related to emotional and affective processing, motivation, and reward.ConclusionAlthough it is demonstrated that neural correlates of meaningful activities can be measured objectively by neurophysiological registration techniques, “meaning” as such has not yet been investigated explicitly. Further neurophysiological research for objective monitoring of meaningful activities is recommended.

Introduction Alcohol Use Disorders (AUDs) are highly prevalent psychiatric disorders with often a poor treatment outcome in terms of high dropout rates and relapses. A vulnerability to disinhibition or a lack of self-regulation/low Effortful Control (EC) seems to be a core risk factor associated with both the initiation and continuation of AUDs. EC is the regulative dimension of temperament that involves attentional control, inhibitory control and activation control, and reflects self-regulation abilities that develop later in life parallel with the maturation of the prefrontal cortex. Objectives In this study we want to investigate whether EC, operationalised in terms of self-report and in terms of behavioral measures, can predict relapse. When a low EC indeed turns out to be a significant contributor to relapse, treatment interventions aiming at strengthening EC could result in better treatment outcomes and less relapse in AUD patients. Methods The sample consisted of 75 adult patients with AUD (68.9% males, mean age 47,4y) admitted at a specialized, inpatient treatment unit for addiction. To assess the regulative temperament dimension, we used the Effortful Control Scale (ECS) from the Adult Temperament Questionnaire Short-Form), a self-report questionnaire as well as five behavioral/neuropsychological tasks using the Cambridge Neuropsychological Test Automated Battery: Cambridge Gambling Task (CGT), Stop-Signal Task (SST), Intra-Extradimensional Set Shift (IED), Spatial Working Memory (SWM) and Spatial Span (SSP). Results We performed binary logistic regression analyses with EC/CANTAB measures as predictors and relapse/no relapse (during treatment and after 3 months follow up) as dependent variable. According to these analyses, the self-report measure of EC nor the behavioral tasks CGT, SST, SWM and SSP (CANTAB) were able to significantly predict relapse neither during treatment nor after 3 months follow-up. Only the IED (outcome measure stages completed) was significant in predicting relapse (p

Purpose: Alterations in primary auditory functioning have been reported in patients with Parkinson's disease (PD). Despite the current findings, the pathophysiological mechanisms underlying these alterations remain unclear, and the effect of dopaminergic medication on auditory functioning in PD has been explored insufficiently. Therefore, this study aimed to systematically investigate primary auditory functioning in patients with PD by using both subjective and objective audiological measurements. Method: In this case-control study, 25 patients with PD and 25 age-, gender-, and education-matched healthy controls underwent an audiological test battery consisting of tonal audiometry, short increment sensitivity index, otoacoustic emissions (OAEs), and speech audiometry. Patients with PD were tested in the on- and off-medication states. Results: Increased OAE amplitudes were found when patients with PD were tested without dopaminergic medication. In addition, speech audiometry in silence and multitalker babble noise demonstrated higher phoneme scores for patients with PD in the off-medication condition. The results showed no differences in auditory functioning between patients with PD in the on-medication condition and healthy controls. No effect of disease stage or motor score was evident. Conclusions: This study provides evidence for a top-down involvement in auditory processing in PD at both central and peripheral levels. Most important, the increase in OAE amplitude in the off-medication condition in PD is hypothesized to be linked to a dysfunction of the olivocochlear efferent system, which is known to have an inhibitory effect on outer hair cell functioning. Future studies may clarify whether OAEs may facilitate an early diagnosis of PD.

Background and aim: Temperament and personality traits are important factors underlying the vulnerability for both initiation and continuation of addictive behaviors. In SUDs there is a high comorbidity with mood and anxiety disorders and personality disorders. As there is a growing interest in the possible transdiagnostic role of Effortful Control in both SUDs as in their comorbid disorders the current study therefore investigates the influence of reactive (Behavioral Inhibition System and Behavioral Activation System, BIS/BAS) and regulative (Effortful Control, EC) temperament and their interaction in relation to clinical symptomatology and PDs in adult inpatients with SUD. Methods: The EC, BIS/BAS, SCL-90-R and ADP-IV questionnaires were administered to 841 patients (age between 17 y and 71 y) with a SUD admitted at the treatment unit for addiction of a psychiatric hospital. Results: Hierarchical regression analyses showed that both, clinical symptomatology and PDs were related to low levels of effortful control (EC). Furthermore, cluster C PDs were related to high levels of BIS and low levels of EC; whereas cluster B PDs were related to low levels of EC and high levels of BAS for the narcissistic and histrionic PD. An interaction effect (BIS*EC) was found for the Schizotypal PD, all other interactions (BIS*EC and BAS*EC) were not significant. Discussion: We found that low EC is involved in all clinical symptomatology and PDs in a sample of inpatients with SUDs. Therapeutic interventions aiming at strengthening effortful control can possibly result in better treatment outcomes for both the addiction as the comorbid psychopathology

Patients with a systemic right ventricle (sRV) have a high risk of developing heart failure (HF), with heart transplantation as the only long-term treatment option. Unfortunately, more than half of sRV patients have pulmonary hypertension (PH) which may impact the decision to opt for heart-only transplantation. Moreover, an increased scarcity of donor hearts prolongs time to transplantation, increasing the probability of dying on the waiting list. Hence, for selected sRV patients, a ventricular assist device (VAD) could provide a solution to bridge the time to transplantation. This case series aimed at evaluating all patients with a sRV who received a VAD at our institution, with a focus on technical aspects of VAD implantation and invasive hemodynamics prior to VAD implantation and after heart transplantation.In total 5 patients with a sRV who received a VAD at our institution were included. All five were male with a median age of 38 years (19–49) at the time of VAD implantation. All patients were subsequently successfully transplanted. Pulmonary artery (PA) pressures were significantly elevated prior to VAD implantation and decreased significantly after VAD implantation and subsequent heart transplantation. With this case series we highlight the ability of a VAD to support the failing sRV, as well as to reduce PA pressures, making patients eligible for heart-only transplantation.

There is limited research examining stability and change in attachment security in middle childhood. The current study addresses this gap using data from a 3-year longitudinal study. Specifically, we examined stability and change in secure base script knowledge during middle childhood using a sample of 157 children (Wave 1 mean age [M[subscript age]] = 10.91, standard deviation [SD] = 0.87) assessed at 1-year intervals across 4 waves. Secure base script knowledge was moderately stable over time, as script scores were significantly correlated between each wave. We also investigated the impact of life stress on change in secure base script knowledge within individuals across waves. The results demonstrated that daily hassles (minor and frequently occurring stressful life events) but not major (more severe and infrequent) stressful life events predicted change in script knowledge. Implications for attachment-based interventions and, more broadly, the stability of attachment security are discussed.

Abstract Background Paroxysmal dyskinesias (PDs) are a group of central nervous system diseases characterized by episodes of abnormal involuntary hyperkinetic movement without altered consciousness that increasingly have been recognized in dogs. Objectives To present the phenotypical characterization, treatment, and outcome of a PD observed in Maltese dogs. Animals Client‐owned Maltese dogs (n = 19) with presumed diagnosis of PD. Methods Data were collected retrospectively from medical records (2014‐2019), and supporting information was added prospectively by using a questionnaire directed to the owners of the affected dogs. Results The episodes were characterized mainly by sudden dystonia of ≥1 limbs and generalized body tremors with preserved consciousness. The mean age of clinical onset was 5.4 years. Episode frequency varied widely both among and within individuals. Median episode duration was 4.5 minutes. Most episodes were stress‐ or exercise‐induced. Acetazolamide was administered to 6 dogs, and 4 dogs experienced a decrease in episode frequency. In 7 dogs that received a gluten‐free diet, 6 dogs became episode‐free. In 4 dogs, the episodes stopped spontaneously and in 2 dogs no medication or specific diet was given and the episodes continued at the same frequency. Conclusions and Clinical Importance Given the breed predisposition and regional distribution of the disease, additional research should focus on elucidating the underlying genetic cause doing so might advance both our understanding of the pathophysiology and treatment of this disease, not only in dogs, but also in humans. Regardless of the treatment protocol selected, prognosis appears fair to good.

Background: Until today, there is no satisfying explanation for why one language may recover worse than another in differential bilingual aphasia. One potential explanation that has been largely unexplored is that differential aphasia is the consequence of a loss of language control rather than a loss of linguistic representations. Language control is part of a general control mechanism that also manages non-linguistic cognitive control. If this system is impaired, patients with differential aphasia could still show bilingual language activation, but they may be unable to manage activation in non-target languages, so that performance in another language is hindered. Aims: To investigate whether a loss of cognitive control, rather than the loss of word representations in a particular language, might underlie differential aphasia symptoms. Methods & Procedures: We compared the performance of seven bilinguals with differential and eight bilinguals with parallel aphasia with 19 control bilinguals in a lexical decision and a flanker task to assess bilingual language co-activation and non-linguistic control respectively. Outcomes & Results: We found similar cognate effects in the three groups, indicating similar lexical processing across groups. Additionally, we found a larger non-linguistic control congruency effect only for the patients with differential aphasia. Conclusions & Implications: The present data indicate preserved language co-activation for patients with parallel as well as differential aphasia. Furthermore, the results suggest a general cognitive control dysfunction, specifically for differential aphasia. Taken together, the results of the current study provide further support for the hypothesis of impaired cognitive control abilities in patients with differential aphasia, which has both theoretical and practical implications.

Research on auditory processing in Parkinson's disease (PD) has recently made substantial progress. At present, evidence has been found for altered auditory processing in the clinical stage of PD. The auditory alterations in PD have been demonstrated with low-cost and non-invasive assessments that are already used in routine clinical practice. Since auditory alterations have been reported early in disease progression, it would be highly relevant to investigate whether auditory markers could be provided in the prodromal stage of PD. In addition, auditory alterations in early stage PD might be modulated by dopaminergic medication. Therefore, the aim of this review is (1) to summarize the literature on auditory processing in PD with a specific focus on the early disease stages, (2) to give future perspectives on which audiological and electrophysiological measurements could be useful in the prodromal stage of PD and (3) to assess the effect of dopaminergic medication on potential auditory markers in the prodromal stage of PD.

L’objectif de cette étude est de dégager d’éventuelles différences entre l’acquisition de la morphologie verbale à l’écrit en français langue étrangère d’une part et celle à l’oral d’autre part. Nous nous sommes plus spécifiquement intéressés aux trajets que parcourent des apprenants néerlandophones dans les deux types de production, avec une attention particulière pour leur maîtrise de la finitude verbale et de l’accord sujetverbe. L’hypothèse de l’importance du type de production s’est confirmée : les deux aspects mentionnés précédemment sont mieux intégrés à l’écrit qu’à l’oral. Pour ce qui est de la morphologie silencieuse, son influence varie d’après le nombre : elle se manifeste au singulier mais n’affecte pas la 3ème personne du pluriel dans notre corpus.

Neurodegeneration with brain iron accumulation encompasses a heterogeneous group of rare neurodegenerative disorders that are characterized by iron accumulation in the brain. Severe generalized dystonia is frequently a prominent symptom and can be very disabling, causing gait impairment, difficulty with speech and swallowing, pain and respiratory distress. Several case reports and one case series have been published concerning therapeutic outcome of pallidal deep brain stimulation in dystonia caused by neurodegeneration with brain iron degeneration, reporting mostly favourable outcomes. However, with case studies, there may be a reporting bias towards favourable outcome. Thus, we undertook this multi-centre retrospective study to gather worldwide experiences with bilateral pallidal deep brain stimulation in patients with neurodegeneration with brain iron accumulation. A total of 16 centres contributed 23 patients with confirmed neurodegeneration with brain iron accumulation and bilateral pallidal deep brain stimulation. Patient details including gender, age at onset, age at operation, genetic status, magnetic resonance imaging status, history and clinical findings were requested. Data on severity of dystonia (Burke Fahn Marsden Dystonia Rating Scale-Motor Scale, Barry Albright Dystonia Scale), disability (Burke Fahn Marsden Dystonia Rating Scale-Disability Scale), quality of life (subjective global rating from 1 to 10 obtained retrospectively from patient and caregiver) as well as data on supportive therapy, concurrent pharmacotherapy, stimulation settings, adverse events and side effects were collected. Data were collected once preoperatively and at 2-6 and 9-15 months postoperatively. The primary outcome measure was change in severity of dystonia. The mean improvement in severity of dystonia was 28.5% at 2-6 months and 25.7% at 9-15 months. At 9-15 months postoperatively, 66.7% of patients showed an improvement of 20% or more in severity of dystonia, and 31.3% showed an improvement of 20% or more in disability. Global quality of life ratings showed a median improvement of 83.3% at 9-15 months. Severity of dystonia preoperatively and disease duration predicted improvement in severity of dystonia at 2-6 months; this failed to reach significance at 9-15 months. The study confirms that dystonia in neurodegeneration with brain iron accumulation improves with bilateral pallidal deep brain stimulation, although this improvement is not as great as the benefit reported in patients with primary generalized dystonias or some other secondary dystonias. The patients with more severe dystonia seem to benefit more. A well-controlled, multi-centre prospective study is necessary to enable evidence-based therapeutic decisions and better predict therapeutic outcomes.

Abstract Background In this paper, we describe the clinical and neuropathological findings of nine members of the Belgian progranulin gene (GRN) founder family. In this family, the loss-of-function mutation IVS1 + 5G > C was identified in 2006. In 2007, a clinical description of the mutation carriers was published that revealed the clinical heterogeneity among IVS1 + 5G > C carriers. We report our comparison of our data with the published clinical and neuropathological characteristics of other GRN mutations as well as other frontotemporal lobar degeneration (FTLD) syndromes, and we present a review of the literature. Methods For each case, standardized sampling and staining were performed to identify proteinopathies, cerebrovascular disease, and hippocampal sclerosis. Results The neuropathological substrate in the studied family was compatible in all cases with transactive response DNA-binding protein (TDP) proteinopathy type A, as expected. Additionally, most of the cases presented also with primary age-related tauopathy (PART) or mild Alzheimer’s disease (AD) neuropathological changes, and one case had extensive Lewy body pathology. An additional finding was the presence of cerebral small vessel changes in every patient in this family. Conclusions Our data show not only that the IVS1 + 5G > C mutation has an exclusive association with FTLD-TDP type A proteinopathy but also that other proteinopathies can occur and should be looked for. Because the penetrance rate of the clinical phenotype of carriers of GRN mutations is age-dependent, further research is required to investigate the role of co-occurring age-related pathologies such as AD, PART, and cerebral small vessel disease.

Purpose: This study investigated the possible relationship between hypokinetic speech production and speech intensity perception in patients with Parkinson's disease (PD). Method: Participants included 14 patients with idiopathic PD and 14 matched healthy controls (HCs) with normal hearing and cognition. First, speech production was objectified through a standardized speech intelligibility assessment, acoustic analysis, and speech intensity measurements. Second, an overall estimation task and an intensity estimation task were addressed to evaluate overall speech perception and speech intensity perception, respectively. Finally, correlation analysis was performed between the speech characteristics of the overall estimation task and the corresponding acoustic analysis. The interaction between speech production and speech intensity perception was investigated by an intensity imitation task. Results: Acoustic analysis and speech intensity measurements demonstrated significant differences in speech production between patients with PD and the HCs. A different pattern in the auditory perception of speech and speech intensity was found in the PD group. Conclusions: Auditory perceptual deficits may influence speech production in patients with PD. The present results suggest a disturbed auditory perception related to an automatic monitoring deficit in PD.

This study tested whether children's more anxious and avoidant attachment is linked to decreased support-seeking behavior toward their mother during stress in middle childhood, and whether children's decreased support-seeking behavior enhances the impact of experiencing life events on the increase of depressive symptoms 18 months later. Ninety-eight 8- to 12-year-old children filled out questionnaires assessing their level of anxious and avoidant attachment and depressive symptoms. Children's support-seeking behavior was observed through measuring the time children waited before calling for their mother's help while carrying out a stressful task. Results supported the hypothesis that more anxiously or avoidantly attached children waited longer before seeking maternal support. Moreover, waiting longer was related to increased depressive symptoms at follow-up in children who reported more experienced life events.

The Flemish Child Welfare System (CWS) is in great need of a shared empirically informed clinical strategy for working with depressed adolescents and their families. Many evidence-based practices (EBP) exist, but little is known as to whether they can be successfully imported in the CWS. Therefore, the current study explores the implementation of a particular EBP, Attachment-Based Family Therapy (ABFT), in home-based services of the Flemish CWS in Belgium. Specifically, the study focused on (1) the acceptability of ABFT by counselors and whether negative attitudes about EBP can be changed ('n' = 73 counselors), and (2) the feasibility of implementing ABFT ('n' = 43 adolescents, 11–17 years old, 72% female) by exploring initial effectiveness. The results suggest that (1) initial negative attitudes of counselors towards ABFT were significantly more positive after attending training and discussions about ABFT, and that (2) ABFT could be used by counselors to successfully reduce adolescent depressive symptoms. Future research should include a control group to draw stronger causal conclusions. Strengths and limitations of the study’s design and implications for further dissemination are discussed.

Background Reduced ventricular function and decreased exercise capacity are widespread in adults with complete transposition of the great arteries after atrial switch (TGA‐Mustard/Senning) and congenitally corrected TGA (ccTGA). Advanced imaging techniques may help to better phenotype these patients and evaluate exercise cardiac response. Methods and Results Thirty‐three adults with a systemic right ventricle (70% TGA‐Mustard/Senning, 37±9 years of age, 24% female, 94% New York Heart Association class I‐II) underwent echocardiogram, cardiopulmonary exercise testing, and cardiovascular magnetic resonance imaging at rest and during a 4‐stage free‐breathing bicycle test. They were compared with 12 healthy controls (39±10 years of age, 25% female, all New York Heart Association class I). TGA‐Mustard/Senning patients had a higher global circumferential strain (−15.8±3.6 versus −11.2±5.2%, P=0.008) when compared with ccTGA, whereas global longitudinal strain and systemic right ventricle contractility during exercise were similar in both groups. Septal extracellular volume (ECV) in ccTGA was significantly higher than in TGA‐Mustard/Senning (30.2±2.0 versus 27.1±2.7%, P=0.005). During exercise, TGA‐Mustard/Senning had a fall in end‐diastolic volume and stroke volume (11% and 8%, respectively; both P≤0.002), whereas ccTGA could increase their stroke volume in the same way as healthy controls. Because of a greater heart rate reserve in TGA‐Mustard/Senning (P for interaction=0.010), cardiac index and peak oxygen uptake were similar between both patient groups. Conclusions Caution should be exercised when evaluating pooled analyses of systemic right ventricle patients, given the differences in myocardial contraction pattern, septal extracellular volume, and the exercise response of TGA‐Mustard/Senning versus ccTGA patients. Longitudinal follow‐up will determine whether abnormal exercise cardiac response is a marker of earlier failure.

Patients with Parkinson's disease (PD) display a variety of impairments in motor and non-motor language processes; speech is decreased on motor aspects such as amplitude, prosody and speed and on linguistic aspects including grammar and fluency. Here we investigated whether verbal monitoring is impaired and what the relative contributions of the internal and external monitoring route are on verbal monitoring in patients with PD relative to controls. Furthermore, the data were used to investigate whether internal monitoring performance could be predicted by internal speech perception tasks, as perception based monitoring theories assume. Performance of 18 patients with Parkinson's disease was measured on two cognitive performance tasks and a battery of 11 linguistic tasks, including tasks that measured performance on internal and external monitoring. Results were compared with those of 16 age-matched healthy controls. PD patients and controls generally performed similarly on the linguistic and monitoring measures. However, we observed qualitative differences in the effects of noise masking on monitoring and disfluencies and in the extent to which the linguistic tasks predicted monitoring behavior. We suggest that the patients differ from healthy subjects in their recruitment of monitoring channels.