T cells develop from circulating precursor cells, which enter the thymus and migrate through specialized subcompartments that support their maturation and selection. In humans, this process starts in early fetal development and is highly active until thymic involution in adolescence. To map the microanatomical underpinnings of this process in pre- and early postnatal stages, we established a quantitative morphological framework for the thymus-the Cortico-Medullary Axis-and used it to perform a spatially resolved analysis.
View Article and Find Full Text PDFWorld J Pediatr Congenit Heart Surg
September 2024
Pulmonary autograft failure remains a cause of reoperation following the Ross procedure. The aim of this study is to describe our evolving approach to autograft reoperations. Retrospective study of all patients who underwent a pulmonary autograft reoperation following a Ross procedure between June 1997 and July 2022.
View Article and Find Full Text PDFT cells develop from circulating precursors, which enter the thymus and migrate throughout specialised sub-compartments to support maturation and selection. This process starts already in early fetal development and is highly active until the involution of the thymus in adolescence. To map the micro-anatomical underpinnings of this process in pre- vs.
View Article and Find Full Text PDFAims: Patients with systemic right ventricles are at high risk of sudden cardiac death. Arrhythmia is a significant risk factor. Routine Holter monitoring is opportunistic with poor adherence.
View Article and Find Full Text PDFBackground: COVID-19 has caused significant worldwide morbidity and mortality. Congenital heart disease (CHD) is likely to increase vulnerability and understanding the predictors of adverse outcomes is key to optimising care.
Objective: Ascertain the impact of COVID-19 on people with CHD and define risk factors for adverse outcomes.
Together, heart failure and arrhythmia represent the most important cardiovascular sources of morbidity and mortality among adults with congenital heart disease (ACHDs). Although traditionally conceptualized as operating within 2 distinct clinical silos, these scenarios frequently coexist within the same individual; consequently the mechanistic, therapeutic, and prognostic overlap between them demands increased recognition. In fact, given the near ubiquity of heart failure and arrhythmia among ACHDs, there is perhaps no other arena within cardiology where this critical intersection is more frequently observed.
View Article and Find Full Text PDFObjectives: Contemporarily modified Ross procedure continues to deliver excellent outcomes and remains part of the treatment strategy for aortic valve disease in the young adult population. The aim of this study was to assess whether Ross procedure carried out as a second or subsequent intervention for aortic valve disease carries similar risk and long-term benefit, when compared to Ross procedure for the first-time aortic valve replacement.
Methods: A total of 158 patients aged 16-60 years from a single congenital cardiac centre between 1997 and 2020 were included.
Aims: Post-infarction ventricular septal defect (PIVSD) is a mechanical complication of acute myocardial infarction (AMI) with a poor prognosis. Surgical repair is the mainstay of treatment, although percutaneous closure is increasingly undertaken.
Methods And Resuts: Patients treated with surgical or percutaneous repair of PIVSD (2010-2021) were identified at 16 UK centres.
Background: A 9-year-old boy with Berlin Heart biventricular assist device for diagnosis of dilated cardiomyopathy developed progressive reduction in left ventricular assist device(VAD) emptying and evidence of low cardiac output despite alterations to the device settings. Computed tomography revealed that the Dacron graft attaching the systemic cannula to the ascending aorta was stenosed.
Method & Result: A minimally invasive approach with novel circuit modification was used to achieve antegrade stenting of the stenosed graft.
Semin Fetal Neonatal Med
August 2022
Pulmonary hypertension, conventionally defined by absolute pulmonary artery pressure, is the result of a range of diagnoses that can result in clinical problems in neonatal practice. Causes include persistent pulmonary hypertension of the newborn, congenital heart disease, and left heart dysfunction, as well as the normally high pulmonary artery resistance in neonates. Elucidating the cause of pulmonary hypertension is vital to guide appropriate management.
View Article and Find Full Text PDFWorld J Pediatr Congenit Heart Surg
May 2022
Hypoplastic left heart syndrome (HLHS) with either intact atrial septum (IS) or highly restrictive interatrial communication (HRIC) is associated with poor survival. Immediate postpartum access to cardiac therapy and timely left atrial decompression (LAD) are paramount to a successful outcome. We describe herein our evolving approach to LAD and report interstage and longer-term results.
View Article and Find Full Text PDFPurpose: There are marked gender differences in all etiologies of advanced heart failure. We sought to determine whether there is evidence of gender-specific decision making for transplant assessments, and how gender effects outcomes.
Methods: Retrospective analysis of adult heart transplant assessments at a single UK center between April 2015 and March 2020.
Background: To compare short and long outcomes between early (≤ 28 days) (EL) and late ligation (LL) groups. To explore factors predicting early extubation (≤ 7 days) after patent ductus arteriosus (PDA) ligation.
Methods: We conducted a single center, retrospective cohort study of preterm infants < 32 weeks who underwent surgical ligation over a 10-year period (2009-2019).
Objectives: Ventricular assist device (VAD) for systemic right ventricular (RV) failure patients post-atrial switch, for transposition of the great arteries (TGA), and those with congenitally corrected TGA has proven useful to reduce transpulmonary gradient and bridge-to-transplantation. The purpose of this study is to describe our experience of VAD in systemic RV failure and our move towards concomitant tricuspid valve replacement (TVR).
Methods: This is a single-centre retrospective study of consecutive adult patients receiving HeartWare VAD for systemic RV failure between 2010 and 2019.
Objectives: To 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.
Methods: Retrospective 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.
Results: At 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.
Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu
October 2021
Patients with ccTGA or d-TGA managed via atrial switch (Mustard or Senning operations) have biventricular circulations with a sub-aortic right ventricle (2V-RV). Other than in a tiny percentage of ccTGA patients, premature heart failure (HF) is common, driven by chronic RV dilatation and dysfunction and/or tricuspid regurgitation. These patients are different from the general HF population in that they are younger, more heterogeneous, are predisposed to pulmonary hypertension and present unique and complex surgical challenges.
View Article and Find Full Text PDFWorld J Pediatr Congenit Heart Surg
July 2020
Poor ventricular assist device filling is often seen in patients supported with the Berlin Heart Excor. Caval stenosis is an uncommon complication following the bicaval approach to orthotopic heart transplantation. We report the case of a five-year-old female patient post heart transplantation whose poor right ventricular assist device filling immediately resolved following management of caval stenosis.
View Article and Find Full Text PDFThe 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.
View Article and Find Full Text PDFThere is mounting recognition that some of the most urgent problems of adult congenital heart disease (ACHD) are the prevention, diagnosis, and management of heart failure (HF). Recent expert consensus and position statements not only emphasize a specific and pressing need to tackle HF in ACHD (ACHD-HF) but also highlight the difficulty of doing so given a current sparsity of data. Some of the challenges will be addressed by this review.
View Article and Find Full Text PDFMalignant ascites in prostatic acinar adenocarcinoma is very rare. We present an 84-year-old man with a rare malignant ascites due to prostatic adenocarcinoma demonstrating hepatoid differentiation by immunohistochemistry. The patient was diagnosed with the malignant ascites due to metastatic prostatic adenocarcinoma.
View Article and Find Full Text PDFObjective: There are no multi-centre data on the outcomes of transplant for adult congenital heart disease (ACHD) outside of North America. The literature has identified a number of concerns for this population such as increased wait-list and early post-operative mortality. We investigated outcomes in a national cohort to see if these problems are replicated outside of America.
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