Impact of cardiopulmonary bypass on cerebrovascular autoregulation assessed by ultrafast ultrasound imaging.

Newborns with congenital heart disease undergoing cardiac surgery are at risk of neurodevelopmental impairment with limited understanding of the impact of intra-operative cardiopulmonary bypass (CPB), deep hypothermia and selective cerebral perfusion on the brain. We hypothesized that a novel ultrasound technique, ultrafast power Doppler (UPD), can assess variations of cerebral blood volume (CBV) in neonates undergoing cardiac surgery requiring CPB. UPD was performed before, during and after surgery in newborns with hypoplastic left heart syndrome undergoing a Norwood operation. We found that global CBV was not significantly different between patients and controls (P = 0.98) and between pre- and post-surgery (P = 0.62). UPD was able to monitor changes in CBV throughout surgery, revealing regional differences in CBV during hypothermia during which CBV correlated with CPB flow rate (R2  = 0.52, P = 0.021). Brain injury on post-operative magnetic resonance imaging was observed in patients with higher maximum variation in CBV. Our findings suggest that UPD can quantify global and regional brain perfusion variation during neonatal cardiac surgery with this first intra-operative application demonstrating an association between CBV and CPB flow rate, suggesting loss of autoregulation. Therefore, the measurement of CBV by UPD could enable optimization of cerebral perfusion during cardiac surgery in neonates. KEY POINTS: The impact of cardiopulmonary bypass (CPB) on the neonatal brain undergoing cardiac surgery is poorly understood. Ultrafast power Doppler (UPD) quantifies cerebral blood volume (CBV), a surrogate of brain perfusion. CBV varies throughout CPB surgery and is associated with variation of the bypass pump flow rate during deep hypothermia. Association between CBV and bypass pump flow rate suggests loss of cerebrovascular autoregulatory processes. Quantitative monitoring of cerebral perfusion by UPD could provide a direct parameter to optimize CPB flow rate.

Training on Congenital 3D Cardiac Models - Will Models Improve Surgical Performance?

Technical skill development in congenital heart surgery (CHS) is challenging due to numerous factors which potentially limit the hands-on operative exposure in surgical training. These challenges have stimulated the growth of simulation-based training through the development of 3D-printed models, providing hands-on surgical training (HOST). From its inception in 2015, the models used in the HOST program have constantly improved, and now include valvar/subvalvar apparatus and better materials that mimic real tissue. Evidence shows that deliberate, regular simulation practice can improve a surgeon's technical skills across the spectrum of CHS. Furthermore, surgical trainees who undergo simulation training are able to translate this improved performance into the operative environment with improved patient outcomes. Despite evidence to support the incorporation of simulation methods into congenital training, its widespread adoption into training curricula remains low. This is due to numerous factors including funding, lack of dedicated time or proctorship and access to models-all of which can be overcome with the newer generation of models and committed trainers. Training programs should consider incorporating simulation-methods as a routine component of congenital training programs.

Medium-term Outcome of Prenatally Diagnosed Hypoplastic Left-Heart Syndrome and Impact of a Restrictive Atrial Septum Diagnosed in-utero.

OBJECTIVE: Surgical outcome data differs from overall outcomes of prenatally diagnosed fetuses with hypoplastic left heart syndrome (HLHS). Our aim was to describe outcome of prenatally diagnosed fetuses with this anomaly. METHODS: Retrospective review of prenatally diagnosed classical HLHS at a tertiary hospital over a 13-year period, estimated due dates 01/08/2006 to 31/12/2019. HLHS-variants and ventricular disproportion were excluded. RESULTS: 203 fetuses were identified with outcome information available for 201. There were extra-cardiac abnormalities in 8% (16/203), with genetic variants in 14% of those tested (17/122). There were 55 (27%) terminations of pregnancy, 5 (2%) intrauterine deaths and 10 (5%) babies had prenatally planned compassionate care. There was intention to treat (ITT) in the remaining 131/201(65%). Of these, there were 8 neonatal deaths before intervention, two patients had surgery in other centers. Of the other 121 patients, Norwood procedure performed in 113 (93%), initial hybrid in 7 (6%), and 1 had palliative coarctation stenting. Survival for the ITT group from birth at 6-months, 1-year and 5-years was 70%, 65%, 62% respectively. Altogether of the initial 201 prenatally diagnosed fetuses, 80 patients (40%) are currently alive. A restrictive atrial septum (RAS) is an important sub-category associated with death, HR 2.61, 95%CI 1.34-5.05, p = 0.005, with only 5/29 patients still alive. CONCLUSION: Medium-term outcomes of prenatally diagnosed HLHS have improved however it should be noted that almost 40% do not get to surgical palliation, which is vital to those doing fetal counselling. There remains significant mortality particularly in fetuses with in-utero diagnosed RAS.

Appearance Orientation and Dating Anxiety in Emerging Adults: Considering the Roles of Appearance-Based Rejection Sensitivity, Social Physique Anxiety, and Self-Compassion.

Emerging research has suggested that appearance-related factors, such as greater appearance orientation, are associated with dating anxiety in emerging adults, but much more could be done to understand mechanistic pathways and potential buffers. Here, we tested a moderated mediation model in which appearance-based rejection sensitivity and social physique anxiety were explored as mediators, and self-compassion was explored as a moderator, of the relationship between appearance orientation and dating anxiety. A total of 501 heterosexual emerging adults (248 women, 253 men) from the UK completed instruments measuring the aforementioned constructs. Relationships among all variables were largely similar across women and men, with only the association between social physique anxiety and appearance-based rejection sensitivity being significantly stronger in women. Mediation analysis in the total sample indicated that both social physique anxiety and appearance-based rejection anxiety were significant mediators. Additionally, we confirmed a serial mediation involving appearance orientation → appearance-based rejection sensitivity → social physique anxiety → dating anxiety. Conversely, self-compassion did not moderate the effects of either social physique anxiety or appearance-based rejection sensitivity on dating anxiety, although greater self-compassion was moderately associated lower dating anxiety. We suggest ways in which existing interventions aimed at reducing dating anxiety could be combined with body image interventions to reduce dating anxiety in heterosocial contexts.

Congenital Heart Surgery Skill Training Using Simulation Models: Not an Option but a Necessity.

Congenital heart surgery (CHS) is technically demanding, and its training is extremely complex and challenging. Training of the surgeon's technical skills has relied on a preceptorship format in which the trainees are gradually exposed to patients in the operating room under the close tutelage of senior staff surgeons. Training in the operating room is an inefficient process and the concept of a learning curve is no longer acceptable in terms of patient outcomes. The benefits of surgical simulation in training of congenital heart surgeons are well known and appreciated. However, adequate surgical simulation models and equipment for training have been scarce until the recent development of three-dimensionally (3D) printed models. Using comprehensive 3D printing and silicone-molding techniques, realistic simulation training models for most congenital heart surgical procedures have been produced. Newly developed silicone-molded models allow efficient CHS training in a stress-free environment with instantaneous feedback from the proctors and avoids risk to patients. The time has arrived when all congenital heart surgeons should consider surgical simulation training before progressing to real-life operating in a similar fashion to the aviation industry where all pilots are required to complete simulation training before flying a real aircraft. It is argued here that simulation training is not an option anymore but should be a mandatory component of CHS training.

How and When Should Tetralogy of Fallot be Palliated Prior to Complete Repair?

The controversy regarding the best or ideal surgical management of Tetralogy of Fallot (ToF) stems from the recognition of there being a spectrum of morphology and associated lesions, each of which require a different approach to achieve the three goals of minimizing mortality preserving right ventricular function long-term and minimizing reinterventions. A one-size-fits-all approach to ToF needs to be replaced by a considered and personalized approach in order to yield the best outcomes possible for individual patients. The great majority of patients with ToF undergo primary complete repair between age 3-9 months with excellent outcomes. However, the greatest challenge is the severely cyanotic neonates where primary repair is still associated with high mortality and reintervention rates. Risk factors are low weight and small/poorly developed pulmonary vasculature. High-risk neonates have better outcomes with palliation-but mortality is still high. Palliative interventions in the catherization lab are showing better outcomes than traditional BT shunt and the RVOT stent is emerging as potential game-changer. Primary neonatal repair is still recommended if weight >3 kg and Nakata >100 mm2/m2. However, neonates with low weight, small pulmonary arteries or multiple comorbidities (including ToF/AVSD and anomalous LAD) may do better with a staged approach, There is good argument for RVOT stenting as a bridge to complete repair due to its stable circulation without diastolic run off and volume loading of the circulation, and its potential to allow branch PA growth.

A cluster of children with facial nerve palsy in a high prevalence area for COVID-19.

BACKGROUND: COVID-19 is a disease of varying presentation and neurological sequelae of the disease are being studied. Following a cluster of paediatric facial nerve palsy (FNP) cases in an area of South Wales with a high prevalence of COVID-19, we conducted an opportunistic study to determine whether there has been an increase of incidence of FNP and if there is an association between the FNP and COVID-19 in children. METHODS: We performed a retrospective review of the incidence of FNP between 2015 and 2020 across two hospitals within the health board. The incidence was compared with that in 2020 including a cluster of six children in 14 weeks, presenting to Royal Glamorgan Hospital between June and October. RESULTS: There were 48 cases of children with FNP across both hospital within the study years. Seven (7) cases in 2020. The incidence was not statistically different in comparison to other years. Five out of six of these children in 2020 had antibody testing for COVID-19. All serology testing (100%) returned negative for SARS-CoV- 2 antibodies. CONCLUSIONS: In high prevalence area for COVID-19, cases of children with FNP have not shown a commensurate increase. we have found no causal link between COVID-19 and FNP in children. While this is a small study, larger cohort studies are needed to support this finding. As new strains of COVID-19 are being reported in UK, South Africa and Brazil, physicians need to continue to be vigilant for consistent pattern of signs and symptoms, especially in children.

Parents' Experiences of Transition From Hospital to Home After Their Infant's First-Stage Cardiac Surgery: Psychological, Physical, Physiological, and Financial Survival.

BACKGROUND: The intersurgical stage is a critical time for fragile infants with complex congenital heart disease, but little is known about the impact on parents. OBJECTIVE: The aim of this study was to explore parents' experiences of the transition from hospital to home with their infant after stage 1 cardiac surgery for complex congenital heart disease. METHOD: This is a prospective, longitudinal, mixed methods feasibility study using semistructured interviews and self-report instruments at 4 time points: before discharge (baseline), 2 weeks post discharge, 8 weeks post discharge, and after stage 2 surgery. Qualitative data were analyzed thematically; and quantitative data, descriptively. RESULTS: Sixteen parents of 12 infants participated. All parents described signs of acute stress disorder; 4 parents described symptoms of posttraumatic stress disorder before discharge. Parents' fear and uncertainty about going home were multifaceted, underpinned by exposure to numerous traumatic events. By 8 weeks post discharge, parents' feelings and emotions were positive, relieved, and relaxed. Mean generalized anxiety and depression scores were higher before discharge; most individual anxiety and depression scores decreased over time. Physiological survival included self-care needs, such as eating and sleeping properly. Physical survival included preparation of the home environment and home alterations adapting to their infant's equipment needs. Financial survival was a burden, particularly for those unable to return to work. CONCLUSION: Patterns of experience in surviving the transition included psychological, physical, physiological, and financial factors. Authors of further longitudinal research could test the effectiveness of psychological preparation interventions, while encouraging early consideration of the other factors influencing parents' care of their infant after discharge from hospital.

Clinical course of porokeratosis ptychotropica over 7 years in an otherwise healthy child.

Porokeratosis ptychotropica is an unusual variant of porokeratosis characterized by papules and plaques located on the buttocks and gluteal cleft and showing multiple coronoid lamellae on histology. In this case report, we present the longitudinal clinical course of porokeratosis ptychotropica in a pediatric patient with individual red-brown hyperkeratotic lesions that enlarged and became confluent prior to surgical intervention. We also discuss the etiology of porokeratosis ptychotropica and review current as well as future treatment options for the disease.

Predicting the Future: Tetralogy of Fallot Will Be Primarily Treated with Catheter Based Intervention Within Two Decades. Surgeon's Perspective.

Interventional cardiology has made extraordinary advances over recent years, but most are still limited to addressing single intracardiac or valvular lesions. This debate considers whether complete interventional repair of more complex congenital defects might become achievable. Tetralogy of Fallot (ToF) is probably the first candidate where complete interventional repair might be achieved-given that various components of the defect have already been successfully addressed-albeit as either a palliative intervention (RVOT stenting) or to address the sequelae of standard surgery (percutaneous PVR). This article considers the challenges that would need to be overcome in terms of the morphology of the condition, the age limitations, and the necessary technological advancements that would be required-while setting these against the benchmark of current surgical outcomes and the parallel progress that is being developed in surgical correction. While complete interventional repair of ToF may still be beyond current techniques, a hybrid approach between surgeons and intentional cardiologists can strive to create a life-long paradigm of care that minimizes the need for surgery and focuses on the maintenance of a healthy right ventricle, such that patients born with ToF can achieve normal life expectancy.

A tool for routine monitoring and feedback of morbidities following paediatric cardiac surgery.

Short-term survival after paediatric cardiac surgery has improved significantly over the past 20 years and increasing attention is being given to measuring and reducing incidence of morbidities following surgery. How to best use routinely collected data to share morbidity information constitutes a challenge for clinical teams interested in analysing their outcomes for quality improvement. We aimed to develop a tool facilitating this process in the context of monitoring morbidities following paediatric cardiac surgery, as part of a prospective multi-centre research study in the United Kingdom.We developed a prototype software tool to analyse and present data about morbidities associated with cardiac surgery in children. We used an iterative process, involving engagement with potential users, tool design and implementation, and feedback collection. Graphical data displays were based on the use of icons and graphs designed in collaboration with clinicians.Our tool enables automatic creation of graphical summaries, displayed as a Microsoft PowerPoint presentation, from a spreadsheet containing patient-level data about specified cardiac surgery morbidities. Data summaries include numbers/percentages of cases with morbidities reported, co-occurrences of different morbidities, and time series of each complication over a time window.Our work was characterised by a very high level of interaction with potential users of the tool, enabling us to promptly account for feedback and suggestions from clinicians and data managers. The United Kingdom centres involved in the project received the tool positively, and several expressed their interest in using it as part of their routine practice.

Mental health literacy of maternal and paternal postnatal (postpartum) depression in British adults.

Background: Postnatal depression affects between 6 and 13% of new parents, but only a small proportion of individuals who meet diagnostic criteria receive optimal treatment. One reason for this is poor mental health literacy of postnatal depression.Aims: Studies have examined mental health literacy of maternal postnatal depression, but there are no similar studies of paternal postnatal depression, which we sought to rectify.Methods: A sample of 406 British adults was presented with vignettes describing cases of either maternal or paternal postnatal depression. Based on the vignettes, participants were asked to report if they thought anything was wrong with the targets and, if so, to describe what they thought was wrong. Participants also rated the targets on a range of attitudinal dimensions.Results: Participants were more likely to indicate that something was wrong when the target was female (97.0%) compared to male (75.9%). Of those who believed something was wrong, 90.1% of participants correctly described the female target as experiencing postnatal depression, but only 46.3% did so for the male target. Participants also held more positive attitudes toward the female target than the male target.Conclusions: There is a gender binary in symptom recognition of postnatal depression, which highlights the need for greater awareness of paternal postnatal depression.

Match performance in a reference futsal team during an international tournament - implications for talent development in soccer.

Anecdotal evidence suggests that futsal can aid talent development for 11 a-side soccer through the potential for transfer of the game's skills and constraints. However, additional scientific research is necessary to enhance understanding of the technical and physical demands of futsal and the potential returns for soccer player development. Accordingly, this study examined selected characteristics of physical (internal and external loads) and technical performance in international futsal match-play in order to study the potential of these for skill transfer and aiding soccer player development. Performance was investigated in futsal players (n = 16, 25.74±4.71 years) belonging to a national team during an international tournament. Pre-tournament fitness testing determined maximum heart rate (MHR) via the Yo-Yo IR1 test (194.6±11.1 beats min-1) to aid interpretation of internal load via heart rate measures during match-play. External load (accelerations and deceleration events) was measured using an inertial movement unit. Finally, post-tournament analysis of selected technical events was performed. Results reported a mean heart rate value during 'court time' of 164.7±22.3 beats min-1, which as a percentage of participants' MHR was 87.7%±4.4%, and a mean peak MHR of 98.3±2.5%. Results showed 2.16±0.25 accelerating (>1.5 m·s-2) and 2.78±0.13 decelerating (>1.5 m·s-2) events per player per minute. Team statistics included 647 passing, ~51 dribbling and 78 set play events per team per game. Match analyses showed that 77.3% of ball receptions were completed with the sole of the foot. Assessment of two-footedness showed 80.1±16.7% of individual possessions used the dominant foot to receive and 84.1±10.7% to pass the ball thereby displaying strong foot dominance. These results have quantified characteristics of elite futsal match-play that are no doubt intrinsically related to the environmental and task constraints of the sport. In addition to enhancing understanding of futsal, there are potential learning returns and implications for skills transfer and the development of soccer players.

Left Ventricular Re-training: Feasibility and Effectiveness-What Are the Limits?

The concept of 're-training' the morphologic left ventricle (mLV) is based on the crude principle of applying a fixed afterload by means of pulmonary artery banding. The complex physiological, molecular and structural responses to banding are poorly understood, and complicated by the fact that re-training is undertaken in a variety of different morphological settings and age-groups. This article reviews the evidence for re-training in different situations with particular focus on the age at banding and on the best ways to assess suitability for subsequent repair. Particular importance is placed on the role of re-training in congenitally corrected transposition as this is the commonest current indication - looking at better ways to train the mLV, the role of combined pressure and volume loading, and more sophisticated ways of assessing the adequacy of training. Current evidence suggests that age at banding has a fundamental impact on ability to re-train and long-term mLV function with the best results being achieved in infancy and concerns at any attempt beyond 2 years of age.

Approaches to Pulmonary Atresia With Major Aortopulmonary Collateral Arteries.

Pulmonary atresia with major aortopulmonary collateral arteries (MAPCAs) is one of the most challenging surgical conditions to manage-not only because of the technical complexity of the surgery but also in terms of defining the anatomy of the pulmonary vasculature, the timing of surgery, and decision making on staged vs complete repair. The importance of early definition of pulmonary blood supply is paramount, establishing which areas of the lung are supplied by MAPCAs alone and which have dual supply with the native system (noting that 20% of patients have absent intrapericardial native vessels). Early unifocalization (3-6 months) is ideal, with closure of the ventricular septal defect (VSD) performed if 15 or more out of 20 lung segments can be recruited. Leaving the ventricular septal defect open with a limiting right ventricle-pulmonary artery conduit can be a useful interim or even definitive circulation in patients with borderline vasculature. Rehabilitation of small native vessels with central shunts can be very effective, but best outcomes are achieved by a combination of unifocalization of MAPCAs together with the native vessels (if present). A variety of reconstructive techniques are necessary to be able to effect these complex repairs with careful choice of materials. Ideally, surgery can be completed through sternotomy alone, but separate thoracotomies may be necessary to control and access some MAPCAs.

Associations Between Positive Body Image, Sexual Liberalism, and Unconventional Sexual Practices in U.S. Adults.

While studies have documented robust relationships between body image and sexual health outcomes, few studies have looked beyond sexual functioning in women. Here, we hypothesized that more positive body image would be associated with greater sexual liberalism and more positive attitudes toward unconventional sexual practices. An online sample of 151 women and 164 men from the U.S. completed measures of sexual liberalism, attitudes toward unconventional sexual practices, and indices of positive body image (i.e., body appreciation, body acceptance by others, body image flexibility, and body pride), and provided their demographic details. Regression analyses indicated that, once the effects of sexual orientation, relationship status, age, and body mass index had been accounted for, higher body appreciation was significantly associated with greater sexual liberalism in women and men. Furthermore, higher body appreciation and body image flexibility were significantly associated with more positive attitudes toward unconventional sexual practices in women and men. These results may have implications for scholars working from a sex-positive perspective, particularly in terms of understanding the role body image plays in sexual attitudes and behaviors.

RUNX1 is essential for mesenchymal stem cell proliferation and myofibroblast differentiation.

Myofibroblasts are a key cell type in wound repair, cardiovascular disease, and fibrosis and in the tumor-promoting microenvironment. The high accumulation of myofibroblasts in reactive stroma is predictive of the rate of cancer progression in many different tumors, yet the cell types of origin and the mechanisms that regulate proliferation and differentiation are unknown. We report here, for the first time to our knowledge, the characterization of normal human prostate-derived mesenchymal stem cells (MSCs) and the TGF-ß1-regulated pathways that modulate MSC proliferation and myofibroblast differentiation. Human prostate MSCs combined with prostate cancer cells expressing TGF-ß1 resulted in commitment to myofibroblasts. TGF-ß1-regulated runt-related transcription factor 1 (RUNX1) was required for cell cycle progression and proliferation of progenitors. RUNX1 also inhibited, yet did not block, differentiation. Knockdown of RUNX1 in prostate or bone marrow-derived MSCs resulted in cell cycle arrest, attenuated proliferation, and constitutive differentiation to myofibroblasts. These data show that RUNX1 is a key transcription factor for MSC proliferation and cell fate commitment in myofibroblast differentiation. This work also shows that the normal human prostate gland contains tissue-derived MSCs that exhibit multilineage differentiation similar to bone marrow-derived MSCs. Targeting RUNX1 pathways may represent a therapeutic approach to affect myofibroblast proliferation and biology in multiple disease states.

Parents' preparedness for their infants' discharge following first-stage cardiac surgery: development of a parental early warning tool.

UNLABELLED: Aim The aim of this study was to explore parental preparedness for discharge and their experiences of going home with their infant after the first-stage surgery for a functionally univentricular heart. BACKGROUND: Technological advances worldwide have improved outcomes for infants with a functionally univentricular heart over the last 3 decades; however, concern remains regarding mortality in the period between the first and second stages of surgery. The implementation of home monitoring programmes for this group of infants has improved this initial inter-stage survival; however, little is known about parents' experiences of going home, their preparedness for discharge, and parents' recognition of deterioration in their fragile infant. METHOD: This study was conducted in 2011-2013; eight sets of parents were consulted in the research planning stage in September, 2011, and 22 parents with children aged 0-2 years responded to an online survey during November, 2012-March, 2013. Description of categorical data and deductive thematic analysis of the open-ended questions were undertaken. RESULTS: Not all parents were taught signs of deterioration or given written information specific to their baby. The following three themes emerged from the qualitative data: mixed emotions about going home, knowledge and preparedness, and support systems. CONCLUSIONS: Parents are not adequately prepared for discharge and are not well equipped to recognise deterioration in their child. There is a role for greater parental education through development of an early warning tool to address the gap in parents' understanding of signs of deterioration, enabling appropriate contact and earlier management by clinicians.