Mechanical support as a bridge to cardiac transplantation in children is an accepted treatment. With improved devices and increasing experience, the length of time that children can be supported has ...increased. Donor organs remain scarce and there is significant associated morbidity.
Retrospective review of all children offered mechanical support as a bridge to heart transplant over 10 years in one of the two UK pediatric heart transplant centers. Outcomes during the years 1998 to 2002 were compared with outcomes during the years 2003 to 2007.
Forty children in 41 separate patient episodes received mechanical support as a bridge to transplantation or, in 1 case, to recovery. Survival to transplant or recovery was achieved in 29 of 41 (71%); 26 of 40 children (63%) survived to hospital discharge. Devices used were extracorporeal membrane oxygenation (ECMO), the Medos HIAA, the Berlin Heart (from November 2005) and the Levitronix ventricular assist device (VAD) from 2007. All 3 children supported with the Levitronix survived to transplant (median duration of support 10 days). Ten of 13 children (77%) supported by the Berlin Heart survived to transplant or recovery (median duration of support 44 days). Four of 7 (57%) children supported using the Medos device survived to transplant (median duration of support 7 days). Neurologic events were the most common cause of death in both eras (1998 to 2002 and 2003 to 2008).
Waiting times to pediatric cardiac transplant in the UK have increased. The Berlin Heart allows children to be bridged to transplant over long periods. Neurologic morbidity remains as a major concern.
Mechanical cardiac assistance for infants and children may be accomplished using extracorporeal membrane oxygenation or ventricular assist device support, and are now well established as a bridge to ...cardiac transplantation or recovery in biventricular hearts, usually in the setting of low cardiac output states due to cardiomyopathy or acute myocarditis. Ventricular assist device support remains less well described in the setting of single ventricle physiology. We report the case of a 3-year-old girl who developed severe right ventricular failure 2 years after cavopulmonary shunt after an initial Stage I Norwood operation for hypoplastic left heart syndrome. She was successfully supported to cardiac transplantation using a single chamber Berlin Heart EXCOR ventricular assist device using right ventricular apex and aortic cannulation and is now well at home 10 months after transplant.
Direct immunofluorescence (DIF) testing is a useful adjunct for the diagnosis of immune-mediated oral vesiculobullous diseases, helping to identify separate, histologically similar, but ...prognostically different, conditions. It is unknown how often biopsy of these lesions yields positive DIF results.
A total of 270 consecutive archival cases submitted to a reference laboratory in Buffalo, New York, over a 2-year span were examined. These specimens were submitted to establish or rule out a diagnosis of a DIF-positive oral vesiculobullous disease. Demographic, clinical, and diagnostic information, based on conventional microscopic and DIF analysis, was tabulated. To assess the contribution of DIF to successful diagnosis, three pathologists examined the hematoxylin and eosin-stained slides of the known DIF-positive specimens without knowledge of the DIF results.
Approximately 48% of the specimens demonstrated positive DIF findings and consisted of pemphigus vulgaris, mucous membrane pemphigoid, lichen planus, linear immunoglobulin A disease, and chronic ulcerative stomatitis. The remaining specimens had negative DIF findings and consisted of numerous non-specific inflammatory conditions. Of particular interest were several cases of epithelial dysplasia and squamous cell carcinoma. Of the DIF-positive cases, only pemphigus vulgaris could be diagnosed reliably by conventional microscopy alone.
Approximately half of biopsies of oral conditions that clinically resembled typically DIF-positive vesiculobullous diseases did not yield positive findings on DIF testing. Instead, a wide range of oral diseases can mimic these lesions clinically. With the exception of pemphigus vulgaris, DIF is essential for establishing a definitive diagnosis for known DIF-positive diseases.
An 8-month-old boy with acute myocarditis was mechanically supported with the Berlin Heart EXCOR biventricular assist device. Signs of myocardial recovery were not apparent until 40 days from ...presentation and ventricular function continued to improve with device removal possible after 120 days of support. He had normal cardiac function 3 months after device removal. Recovery from myocarditis in infants can take much longer than previously recognized and an extended wait for recovery is possible with the Berlin Heart.
The prognosis of acute heart failure is such that many children are considered for transplantation. Recovery of severe heart failure in a proportion of patients diagnosed with either dilated ...cardiomyopathy or myocarditis is well recognized, and this complicates the assessment for transplantation. There is little data on the time scale of recovery of heart function in children.
To describe the time course over which echocardiographic improvement of systolic function occurred in a cohort of children who presented in acute heart failure, without structural or metabolic abnormality.
Children with a first presentation of acute severe heart failure between 1990 and 2005. Time from presentation to the echocardiogram before left ventricular fractional shortening (FS) improved to 20% and 30% (complete recovery) was recorded.
Twenty-seven children (11 male) were identified, and all had an initial FS <15%. Twenty-one patients required intravenous inotropes and three patients required extracorporeal membrane oxygenation. Seven patients had been on the active transplant list for a mean duration of 155 days. Four patients had probable viral myocarditis. Mean age at presentation was 15.7 (range, 0.1-72) months. Mean time to an FS of 20% was 3.6 (0.2-18) months and to 30% was 8.9 (0.7-24) months. Complete recovery occurred within 6, 9, 12, 18, and 24 months of presentation in 44%, 55%, 66%, and 96%, respectively. There was no correlation between age of presentation and length of time to recovery.
Complete recovery of left ventricular systolic function is often delayed to more than 1 year from presentation. This may have major implications for timing of transplantation in an era where prolonged mechanical cardiac support is feasible even in infants.
ObjectivesTo report the numbers of consultant congenital cardiac surgeons and cardiologists who have joined and left UK practice over the last 10 years and explore the reasons for ...leaving.MethodsRetrospective observational questionnaire study completed between 11 June 2019 and 1 July 2020 by UK level 1 congenital cardiac centres of 10-year consultant staff movement and reasons suggested for leaving UK practice.ResultsAt survey completion there were 218 (202 whole time equivalent (WTE)) consultant cardiologists and surgeons working within level 1 centres made up of 39 (38 WTE) surgeons, 137 (128.5 WTE) paediatric cardiologists, 42 (35.5 WTE) adult congenital heart disease (ACHD) cardiologists. 161 (74%) consultants joined in the last 10 years of whom 103 (64%) were UK trained. There were 91 leavers giving a staff turnover rate 42% (surgeons 56%, paediatric cardiologists 42%, ACHD cardiologists 29%). Of those, leaving 43% moved to work abroad (surgeons 55%, paediatric cardiologists 40%, ACHD cardiologists 67%). Among the 65 reported reasons for leaving 16 were financial, 9 for work life balance, 6 to working conditions within the National Health Service (NHS) and 12 related to the profession in the UK including six specifically highlighting the national review process.ConclusionsThere has been a high turnover rate of consultant staff within UK congenital cardiac services over the last 10 years with almost half of those leaving moving to work overseas. Financial reasons and pressures relating to working in the NHS or the specialty in the UK were commonly reported themes for leaving. This has major implications for future planning and staff retention within this specialised service.
The thymus provides a nurturing environment for the differentiation and selection of T cells, a process orchestrated by their interaction with multiple thymic cell types. We used single-cell RNA ...sequencing to create a cell census of the human thymus across the life span and to reconstruct T cell differentiation trajectories and T cell receptor (TCR) recombination kinetics. Using this approach, we identified and located in situ CD8αα
T cell populations, thymic fibroblast subtypes, and activated dendritic cell states. In addition, we reveal a bias in TCR recombination and selection, which is attributed to genomic position and the kinetics of lineage commitment. Taken together, our data provide a comprehensive atlas of the human thymus across the life span with new insights into human T cell development.
Left atrial appendage closure is a non-pharmacological alternative for stroke prevention in high-risk patients with non-valvular atrial fibrillation. The objective of the multicentre EWOLUTION ...registry was to obtain clinical data on procedural success and complications, and long-term patient outcomes, including bleeding and incidence of stroke/transient ischaemic attack (TIA). Here, we report on the peri-procedural outcomes of up to 30 days.
Baseline/implant data are available for 1021 subjects. Subjects in the study were at high risk of stroke (average CHADS2 score: 2.8 ± 1.3, CHA2DS2-VASc: 4.5 ± 1.6) and moderate-to-high risk of bleeding (average HAS-BLED score: 2.3 ± 1.2). Almost half of the subjects (45.4%) had a history of TIA, ischaemic stroke, or haemorrhagic stroke; 62% of patients were deemed unsuitable for novel oral anticoagulant by their physician. The device was successfully deployed in 98.5% of patients with no flow or minimal residual flow achieved in 99.3% of implanted patients. Twenty-eight subjects experienced 31 serious adverse events (SAEs) within 1 day of the procedure. The overall 30-day mortality rate was 0.7%. The most common SAE occurring within 30 days of the procedure was major bleeding requiring transfusion. Incidence of SAEs within 30 days was significantly lower for subjects deemed to be ineligible for oral anticoagulation therapy (OAT) compared with those eligible for OAT (6.5 vs. 10.2%, P = 0.042).
Left atrial appendage closure with the WATCHMAN device has a high success rate in complete LAAC with low peri-procedural risk, even in a population with a higher risk of stroke and bleeding, and multiple co-morbidities. Improvement in implantation techniques has led to a reduction of peri-procedural complications previously limiting the net clinical benefit of the procedure.
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