Novel Staged Device Occlusion of Portosystemic Shunt With Atrial Flow Regulator in IVC Stent Platform.

We present the cases of 2 children diagnosed with extrahepatic portosystemic shunts, a very rare vascular anomaly, on investigation of cardiac symptomatology. Poorly developed portal venous systems necessitated staged shunt occlusion. This was achieved using atrial flow regulator devices positioned in an inferior vena cava stent platform performed in the cardiac catheterization laboratory.

The Effects of Aspirin dose in Children with Congenital and Acquired Heart Disease. Results from the Paediatric Study of Aspirin Efficacy using Diagnostic and Monitoring Tools (PAED-M).

The optimal dose of aspirin required in children with congenital and acquired heart disease is not known. The primary aim of this prospective observational study was to evaluate the effects of aspirin dose on platelet inhibition. The secondary aim was to determine the prevalence and clinical predictors of aspirin non-responsiveness. Measurements were by Thromboelastography with Platelet Mapping (TEGPM) only in children less than 2 years (y) of age with particular emphasis on the parameter known as maximum amplitude with arachidonic acid (MAAA) and using both TEGPM, and light transmission aggregometry (LTA) in children greater than 2 y. We prospectively studied 101 patients with congenital and acquired cardiac disease who were receiving empirical doses of aspirin for a minimum of 4 weeks but no other antiplatelet agents. Patients were stratified according to dose concentration and age. There was a trend toward lower age in patients with no response or semi-response to aspirin. All patients were considered responsive to aspirin in the higher-dose quartile (Q4) with a median dose of 4.72 (4.18-6.05) mg/kg/day suggesting that patients in this age group may require 5 mg/kg/day as an empirical dose. In children > 2 y, there was no significant difference in inhibition found in patients dosed at higher doses in Q3 versus Q4 suggesting that patients in this cohort are responsive with 3 mg/kg/day dose. The current practices may lead to reduced platelet inhibition in some children due to under-dosing or overdosing in others. In conclusion, younger children require higher doses of aspirin. Laboratory assessment is warranted in this population to mitigate against under and overdosing.

Twinning International Pediatric Cardiology Fellowship Programs: A Transformative Educational Experience for Trainees with Potential for Global Adoption.

Over the last decade, having endured the COVID-19 pandemic, education and training in pediatric cardiology have undergone a profound disruptive transformation. Trainees experience considerable stress achieving all the competencies required to become a competent pediatric cardiologist. Often the quality of the training experienced by trainees, the approach to patients, and potential institutional preference in management strategy is heavily influenced by the center in which they train. We developed an online live twin program of education between Texas Children's Hospital, Houston, Texas and Children's Health at Crumlin Dublin Ireland in 2019. We explored using grounded theory whether a regular scheduled shared teaching program improved fellow education and training between both centers. Trainees were surveyed to evaluate the benefits and disadvantages of such a twin program. The majority (93%) found the sessions helpful from an educational standpoint with many trainees reporting it to be a transformative experience. Three important learning themes emerged: practice variation between centers, managing uncertainty in clinical practice and cognitive overload. This pedagogical model could be replicated across multiple international pediatric cardiology units and facilitate "collaborative learning" among centers across the globe. Furthermore, this novel educational model could also be adopted by other medical specialties.

Aspirin Responsiveness in a Cohort of Pediatric Patients with Right Ventricle to Pulmonary Artery Conduits and Transcatheter Valve Replacement Systems.

The aim of this study was to determine the rate of aspirin responsiveness in a cohort of pediatric patients with in situ xenograft valved right ventricle to pulmonary artery (RV-PA) conduits and/or transcatheter valve replacements (TVR). Aspirin is routinely prescribed to these patients. Optimizing anti-platelet therapy could promote valve longevity and reduce the risk of infective endocarditis in this at-risk group. This was a prospective, observational study. Patients were recruited from both ward and outpatient settings. Patients were eligible if under 18 years and taking aspirin. Non-response to aspirin was defined as > 20% platelet aggregation using light transmission platelet aggregometry (LTA) and < 50% platelet inhibition by thromboelastography with platelet mapping (TEGPM). Participants were invited to provide a confirmatory sample in cases of aspirin resistance and dose adjustments were made. Thirty patients participated. Median age was 9 years (2 months to 18 years). The majority (93%) had complex right ventricular outflow tract pathology. 13 (43%) had an RV-PA conduit and 24 (80%) had a TVR, with valve situated in conduit in 7 (23%) cases. Rate of aspirin non-response on initial testing was 23% (n = 7/30) with median LTA 74.55% (60-76%) and TEG 13.25% (0-44%) in non-responders. Non-responders were more likely to be under 1 year. Two patients required dose increases and one patient non-adherence to dose was identified. Four patients on repeat testing were responsive to aspirin by laboratory tests. The rate of aspirin non-response on laboratory testing in this cohort of patients was 23% and resulted in therapeutic intervention in 10%.

Outcomes and characteristics in term infants with necrotising enterocolitis and CHD.

BACKGROUND: CHD is a significant risk factor for the development of necrotising enterocolitis. Existing literature does not differentiate between term and preterm populations. Long-term outcomes of these patients are not well understood. The aim was to investigate the baseline characteristics and outcomes of term normal birth weight infants with CHD who developed necrotising enterocolitis. METHODS: A retrospective review was performed of infants from a single tertiary centre with CHD who developed necrotising enterocolitis of Bell's Stage 1-3, over a ten-year period. Inclusion criteria was those born greater than 36 weeks' gestation and birth weight over 2500g. Exclusion criteria included congenital gastro-intestinal abnormalities. Sub-group analysis was performed using Fisher's exact test. RESULTS: Twenty-five patients were identified, with a median gestational age of 38 weeks. Patients with univentricular physiology accounted for 32% (n = 8) and 52% of patients (n = 13) had a duct-dependent lesion. Atrioventricular septal defect was the most common cardiac diagnosis (n = 6, 24%). Patients with trisomy 21 accounted for 20% of cases. Mortality within 30 days of necrotising enterocolitis was 20%. Long-term mortality was 40%, which increased with increasing Bell's Stage. In total, 36% (n = 9) required surgical management of necrotising enterocolitis, the rate of which was significantly higher in trisomy 21 cases (p < 0.05). CONCLUSION: Not previously described in term infants is the high rate of trisomy 21 and atrioventricular septal defect. This may reflect higher baseline incidence in our population. Infants with trisomy 21 were more likely to develop surgical necrotising enterocolitis. Mortality at long-term follow-up was high in patients with Bell's Stage 2-3.

Towards a greater understanding of reduced response to aspirin in children with congenital heart disease post-cardiac surgery using immature platelet fraction.

OBJECTIVE: A high platelet turnover rate may produce a population of platelets that confers an inadequate response to aspirin. We aimed to investigate the relationship between residual platelet aggregation and platelet turnover in paediatric cardiology patients on aspirin monotherapy by evaluating the fraction of immature platelets as a marker for turnover and secondly to test the predictive value of the immature platelet fraction (IPF) to classify patients as responsive or non-responsive to aspirin. METHODS: Sixty patients divided into two age categories (≤90 days, >90 days of age) were included in this prospective observational study. Patients were then stratified into tertiles using their IPF level. Platelet studies included thromboelastography with platelet mapping (TEGPM). RESULTS: The overall incidence of 'inadequate response to aspirin' was 38 % in our patient cohort recently post-cardiac surgery a consequence that warrants further study. The frequency of inadequate response to aspirin was higher in the upper tertile of IPF when compared to the lower tertile, (88 %) versus (4 %) respectively (p < 0.05). The 'cut off' for IPF was determined to be 3.9 % with a sensitivity of 95.7 %, and a specificity of 92.9 % (area under the curve of 0.955 [CI 0.896-1.014, p < 0.05]). CONCLUSION: This study demonstrates that inadequate response to aspirin occurs in approximately 38 % of patients undergoing specific high-risk congenital cardiac procedures using the dosing practice of a national centre. This study supports the hypothesis that an elevated platelet turnover may result in aspirin being less effective in patients who are recently post cardiac surgery. These data are of direct translational relevance.

Staff competence in caring for LGBTQ+ patients in the paediatric emergency department.

OBJECTIVE: This study aimed to assess the competency of paediatric emergency department (PED) multidisciplinary staff in caring for LGBTQ+ (lesbian, gay, bisexual, transgender, queer/questioning, + inclusive of all identities) adolescents. DESIGN: This was an observational study within which participants were required to complete the LGBT-Development of Clinical Skills Scale self-assessment tool of clinical competence. SETTING: It was conducted across three PEDs and one urgent care centre pertaining to the Children's Health Ireland healthcare group. PARTICIPANTS: Doctors, nurses and healthcare workers were eligible to participate. EXCLUSION CRITERIA: non-front facing staff; prior completion of an eLearning module intended to serve as a future educational intervention. MAIN OUTCOME MEASURES: Participants were assessed on: (1) attitudinal awareness towards LGBTQ+ individuals; (2) knowledge of LGBTQ+ health issues and (3) clinical preparedness in caring for LGBTQ+ patients. Each domain is scored out of a maximum of 7 points. RESULTS: 71 eligible participants completed the study. 40/71 (56%) were doctors, and 31/71 (44%) were nurses. The mean score for attitudinal awareness was 6.54/7 (SD 0.59), indicating overall positive attitudes. The mean score for knowledge was lower (5.34/7, SD 1.03) and lowest for clinical preparedness (3.39/7, SD 0.94). Participants were less confident in caring for transgender than LGB patients and scored very low when asked if they had received adequate training in caring for transgender young people (2.11/7). CONCLUSIONS: This study demonstrates positive attitudes towards LGBTQ+ patients among PED staff. However, there was a gap in knowledge and clinical preparedness. Increased training in caring for LGBTQ+ young people is necessary.

Necrotizing Enterocolitis in Children with Congenital Heart Disease: A Literature Review.

Infants with congenital heart disease (CHD) are at an increased risk of developing necrotising enterocolitis (NEC), a serious inflammatory intestinal condition classically associated with prematurity. CHD not only increases the risk of NEC in preterm infants but is one of the most commonly implicated risk factors in term infants. Existing knowledge on the topic is limited largely to retrospective studies. This review acts to consolidate existing knowledge on the topic in terms of disease incidence, pathophysiology, risk factors, outcomes and the complex relationship between NEC and enteral feeds. Potential preventative strategies, novel biomarkers for NEC in this population, and the role of the intestinal microbiome are all explored. Numerous challenges exist in the study of this complex multifactorial disease which arise from the heterogeneity of the affected population and its relative scarcity. Nevertheless, its high related morbidity and mortality warrant renewed interest in identifying those infants most at risk and implementing strategies to reduce the incidence of NEC in infants with CHD.