Access to healthcare for children with CHDs in middle and low-income countries: a systematic literature review.

CHD includes a wide range of cardiac disorders present at birth. If appropriate care is delivered in time, the prognosis is relatively good. However, in many parts of the world, access to healthcare continues to be a problem for these patients, particularly in low- and middle-income countries.

Thromboelastography-guided Intraoperative Platelet Transfusion in Pediatric Heart Surgery.

Background: Postoperative bleeding is associated with significant resource use and is an important contributor to other major adverse events in pediatric patients undergoing complex cardiac surgical procedures. Thromboelastography (TEG; TEG 6S, Haemonetics) can guide perioperative blood product transfusions to reduce the risk of postoperative bleeding. This study validated the use of a previously developed TEG 6S maximum amplitude (TEG-MA)-based platelet transfusion calculator used during cardiac surgical procedures to minimize the risk of postoperative bleeding.

Perioperative Hyper-coagulation and Thrombosis: Cost Analysis After Congenital Heart Surgery.

Thrombosis, a major adverse event of congenital heart surgery, has been associated with poor outcomes. We hypothesized that in CHD patients undergoing cardiac surgery, increased perioperative use of pro-coagulant products may be associated with postoperative thrombosis in the setting of hyperfibrinogenemia, leading to greater hospital and blood product costs. Single-center retrospective study.

Barriers to healthcare access for children with congenital heart disease in eight Latin American countries.

Background: Mortality from congenital heart disease has decreased considerably in the last two decades due to improvements in overall health care. However, there are barriers to access to healthcare in Latin America for this population, which could be related to factors such as healthcare system, policies, resources, geographic, cultural, educational, and psychological factors. Understanding the barriers to access to care is of paramount importance for the design and implementation of policies and facilitate the provision of care.

Comparison of Thromboelastography Devices TEG6S Point of Care Device vs. TEG5000 in Pediatric Patients Undergoing Cardiac Surgery.

Thromboelastography (TEG) can predict bleeding in pediatric patients undergoing cardiac surgery. We hypothesized that results obtained from TEG5000 correlate with the new point-of-care TEG6S system and that TEG6S rewarming maximum amplitude (MA) is associated with surrogate endpoints for perioperative bleeding in pediatric patients who underwent complex cardiac surgery. We describe a retrospective study of pediatric (≤18 years) patients who underwent complex cardiac surgery on cardiopulmonary bypass.

Implications of Transfusion in Adults with Congenital Heart Disease Undergoing Cardiac Surgery.

The number of adults with congenital heart disease (CHD) requiring cardiovascular (CV) surgery is increasing rapidly in today's era. We hypothesized that exposure to perioperative blood products is associated with worse outcomes in adults. All adults (≥ 18 years old) undergoing CV surgery with Cardio-Pulmonary Bypass (CPB) between 2015 and 2020 were reviewed retrospectively.

Contribution of ADAMTS13-independent VWF regulation in sickle cell disease.

Background: Von Willebrand factor (VWF) is elevated in sickle cell disease (SCD) and contributes to vaso-occlusion through its thrombogenic properties. VWF is regulated by ADAMTS13, a plasma protease that cleaves VWF into less bioactive multimers. Independent investigations have shown VWF to be elevated in SCD, whereas measurements of ADAMTS13 have been variable.

Synonymous ADAMTS13 variants impact molecular characteristics and contribute to variability in active protein abundance.

The effects of synonymous single nucleotide variants (sSNVs) are often neglected because they do not alter protein primary structure. Nevertheless, there is growing evidence that synonymous variations may affect messenger RNA (mRNA) expression and protein conformation and activity, which may lead to protein deficiency and disease manifestations. Because there are >21 million possible sSNVs affecting the human genome, it is not feasible to experimentally validate the effect of each sSNV.

Clinical implications of acute shunt thrombosis in paediatric patients with systemic-to-pulmonary shunt re-interventions.

Purpose: Systemic-to-pulmonary shunts are used as a source of pulmonary blood flow in palliated Congenital Heart Disease in neonates and young infants. Shunt thrombosis, often requiring shunt interventions during index hospitalisation, is associated with poor outcomes. We hypothesised that extensive use of perioperative pro-coagulant products may be associated with shunt thrombosis.

Definitive Closure of the Patent Ductus Arteriosus in Preterm Infants and Subsequent Short-Term Respiratory Outcomes.

A persistent patent ductus arteriosus (PDA) can have significant clinical consequences in preterm infants, depending on the degree of left-to-right shunting, its impact on cardiac performance, and associated perinatal risk factors that can mitigate or exacerbate the shunt. Although the best management strategy remains contentious, PDAs that have contraindications to, or have failed medical management have historically undergone surgical ligation. Recently smaller occluder devices and delivery systems have allowed for minimally invasive closure in the catheterization laboratory even in extremely premature infants.

Plasma and Platelet Transfusions Strategies in Neonates and Children Undergoing Cardiac Surgery With Cardiopulmonary Bypass or Neonates and Children Supported by Extracorporeal Membrane Oxygenation: From the Transfusion and Anemia EXpertise Initiative-Control/Avoidance of Bleeding.

Objectives: To present the recommendations and consensus statements with supporting literature for plasma and platelet transfusions in critically ill neonates and children undergoing cardiac surgery with cardiopulmonary bypass or supported by extracorporeal membrane oxygenation from the Transfusion and Anemia EXpertise Initiative-Control/Avoidance of Bleeding.

Design: Systematic review and consensus conference of international, multidisciplinary experts in platelet and plasma transfusion management of critically ill children.

Setting: Not applicable.

Phenotyping respiratory decompensation following definitive closure of the patent ductus arteriosus in preterm infants.

Objective: To identify risk factors associated with high-frequency ventilation (HFV) following definitive closure of the patent ductus arteriosus (PDA).

Methods: We performed a retrospective study of premature infants (<37 weeks) who were mechanically ventilated before and after surgical or transcatheter PDA closure. Primary outcome was HFV requirement within 24 h of procedure.

In Silico Evaluation of Cyclophilin Inhibitors as Potential Treatment for SARS-CoV-2.

Background: The advent of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) provoked researchers to propose multiple antiviral strategies to improve patients' outcomes. Studies provide evidence that cyclosporine A (CsA) decreases SARS-CoV-2 replication in vitro and decreases mortality rates of coronavirus disease 2019 (COVID-19) patients. CsA binds cyclophilins, which isomerize prolines, affecting viral protein activity.

Thromboelastography During Rewarming for Management of Pediatric Cardiac Surgery Patients.

Background: Thromboelastography (TEG) predicts bleeding in pediatric patients undergoing cardiac surgical procedure. We hypothesize that TEG indicators at rewarming correlate with postprotamine values and that rewarming TEG is associated with surrogate end points for postoperative bleeding in pediatric patients undergoing complex cardiac surgical procedure.

Methods: In a retrospective study of 703 pediatric (≤18 years) patients undergoing complex cardiac surgical procedures, TEG results obtained during rewarming and after protamine administration were compared using linear regression.

Coagulopathy and Thrombosis as a Result of Severe COVID-19 Infection: A Microvascular Focus.

Coronavirus disease of 2019 (COVID-19) is the clinical manifestation of the respiratory infection caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). While primarily recognized as a respiratory disease, it is clear that COVID-19 is systemic illness impacting multiple organ systems. One defining clinical feature of COVID-19 has been the high incidence of thrombotic events.

In silico features of ADAMTS13 contributing to plasmatic ADAMTS13 levels in neonates with congenital heart disease.

Introduction: Risk factors contributing to heightened thrombosis in pediatric congenital heart disease (CHD) patients are not fully understood. Among the neonatal CHD population, those presenting with single ventricular physiology are at the highest risk for perioperative thrombosis. The von Willebrand factor and ADAMTS13 interactions have emerged as causative risk factors for pediatric stroke and could contribute to heightened thrombosis in CHD neonates.

TissueCoCoPUTs: Novel Human Tissue-Specific Codon and Codon-Pair Usage Tables Based on Differential Tissue Gene Expression.

Protein expression in multicellular organisms varies widely across tissues. Codon usage in the transcriptome of each tissue is derived from genomic codon usage and the relative expression level of each gene. We created a comprehensive computational resource that houses tissue-specific codon, codon-pair, and dinucleotide usage data for 51 Homo sapiens tissues (TissueCoCoPUTs: https://hive.

Magnetic Resonance-Based Diagnostics for Bleeding Assessment in Neonatal Cardiac Surgery.

Purpose: Infants undergoing a cardiac operation are at high risk for postsurgical bleeding. To date, there are no highly predictive models for postsurgical bleeding in this population. This study's objective was to assess the predictive ability of T2 magnetic resonance (T2MR).

Results of a phase 1 multicentre investigation of dexmedetomidine bolus and infusion in corrective infant cardiac surgery.

Background: Dexmedetomidine (DEX) is increasingly used intraoperatively in infants undergoing cardiac surgery. This phase 1 multicentre study sought to: (i) determine the safety of DEX for cardiac surgery with cardiopulmonary bypass; (ii) determine the pharmacokinetics (PK) of DEX; (iii) create a PK model and dosing for steady-state DEX plasma levels; and (iv) validate the PK model and dosing.

Methods: We included 122 neonates and infants (0-180 days) with D-transposition of the great arteries, ventricular septal defect, or tetralogy of Fallot.

Translational and transcriptional responses in human primary hepatocytes under hypoxia.

The liver is the primary source of a large number of plasma proteins and plays a critical role in multiple biological processes. Inadequate oxygen supply characterizing various clinical settings such as liver transplantation exposes the liver to hypoxic conditions. Studies assessing hypoxia-induced global translational changes in liver are lacking.

von Willebrand factor/ADAMTS-13 interactions at birth: implications for thrombosis in the neonatal period.

von Willebrand factor (VWF) and its cleaving protease ADAMTS-13 (A Disintegrin and Metalloproteinase with Thrombospondin type 1 motif, member 13) are essential components to hemostasis. These plasma proteins have also been implicated in a number of disease states, including those affecting children. The best described abnormality is the congenital form of thrombotic thrombocytopenic purpura (TTP) resulting from germline mutations in the ADAMTS-13 gene.

A red cell preservation strategy reduces postoperative transfusions in pediatric heart surgery patients.

Background: Blood transfusion has well-documented adverse effects. As part of a blood conservation initiative at our center, we began routine use of cell saver for all congenital heart surgery performed on cardiopulmonary bypass since 2014.

Aims: This study aimed to compare transfusion rates prior to, and in the first and second year after this initiative.

Preoperative Thromboelastographic Profile of Patients with Congenital Heart Disease: Association of Hypercoagulability and Decreased Heparin Response.

Objective: To describe the demographic and thromboelastographic characteristics of patients with congenital heart disease presenting with decreased heparin response before cardiac surgery.

Design: Retrospective, observational study.

Setting: Single institution, tertiary, academic, university hospital.