ABO-incompatible (ABOi) heart transplantation (HT) has increased organ availability for infants with end-stage heart failure. Despite increasing adoption of ABOi listing for HT, data remain limited regarding pre- and post-HT immunologic profiles to guide listing practices and post-HT follow-up. Thus, the purpose of this study was to evaluate post-HT outcomes at a single center employing inclusive ABOi listing irrespective of pre-HT isohemagglutinin titers. All HT recipients listed at less than 24months of age at our institution from 2010-2020 were included. Pre- and post-operative variables were compared for ABOi and ABO-compatible (ABOc) recipients. Separate iso-IgG and iso-IgM titers were monitored pre- and post-HT. Primary outcomes were compared between ABOi versus ABOc groups at mid-term follow-up. 51 HTs were performed on 50 patients from 2010-2020 (ABOi, N = 13; ABOc, N = 38). Six ABOi recipients received intra-operative plasma exchange for elevated titers (greater than 1:8 for IgG or IgM or reverse type greater than 2 +). Treated rejection, DSA, CAV, primary graft failure, need for re-HT, and survival were comparable between ABOi and ABOc groups at mid-term follow-up. An inclusive approach to ABOi HT listing for infants less than 24months of age results in comparable post-transplant rejection-free survival, CAV, and prevalence of DSA at mid-term follow-up. These data define a potential role for specific IgM and IgG testing to promote understanding of risk stratification in pediatric ABOi listing, and support an inclusive strategy irrespective of high pre-HT titers to expand the number of available donor hearts for infants and older children awaiting HT.

Noninvasive functional assessment of systemic right ventricles (RV) in hypoplastic left heart syndrome (HLHS) is challenging. This study aimed to compare pediatric cardiologists' current noninvasive imaging practices to those reported in 2013. A web-based survey was distributed to pediatric cardiologists via various listservs which queried timing of echocardiograms during HLHS palliative stages and measurements of RV function. Demographics of 156 participants who completed the 2023 survey were similar to survey participants in 2013 (n = 222). Respondents were mostly male (62%), echocardiographers (48%), in university-based practice (67%) in North America (95%). Echocardiograms were predominantly obtained monthly during interstage I (41%), every 6months during interstage II (56%), and every year post-Fontan (68%), which is consistent from 2013. Routine cardiac magnetic resonance imaging (cMRI) significantly increased in interstage II (8.2%, 17%) and post-Fontan (24%, 56%) populations, respectively. Qualitative assessment (41%), ejection fraction (EF) by 3D (20%), fractional area change (16%), and RV strain/strain rate (13%) were preferred methods for systolic assessment, whereas a plurality of respondents (41%) did not believe RV diastolic measurements were valid. The largest gap between currently obtained and desired measurements existed for EF by 3D (46.5% points) and RV strain/strain rate (44.5% points). No differences existed between imagers compared to non-imagers. Variability in evaluating HLHS patients continues among pediatric cardiologists compared to 10years ago. Qualitative assessment remains the primary RV systolic functional evaluation. Providers do not rely on quantitative RV diastolic function assessments in HLHS patients. Use of cMRI is increasing for RV functional analysis.

Influenza is associated with adverse outcomes in children, although modification by additional medical conditions is not well-documented. We aimed to compare outcomes in children with versus without congenital heart defects (CHDs) who were hospitalized for influenza. We retrospectively evaluated patients 1-18y hospitalized for influenza in the Pediatric Health Information (PHIS) databasefrom 2004to2019. Outcomes were compared by CHD presence and then by CHD severity (minor biventricular, major biventricular, and single ventricle disease) using log-binomial regression adjusted for propensity scores accounting for ageat admission, sex, and history of asthma. Outcomes included inpatient mortality, intensive care unit (ICU) admission, mechanical ventilation, and length of stay (LOS) > 12days. To evaluate foreffect modification by genetic diagnoses, analyses were repeated stratified by CHD and genetic diagnosis. Among 55,161 children hospitalized for influenza, 2369 (4.3%) had CHDs, including 963 with minor biventricular, 938 with major biventricular, and 468 with single ventricle CHDs. Adjusting for propensity scores, children with CHDs had higher mortality (4.1% versus 0.9%) compared to those without CHDs (risk ratio [RR] 2.5, 95% confidence interval [CI] 1.9-3.4). Children with CHDs were at higher risk of mechanical ventilation (RR 1.6, 95% CI 1.6-1.7), ICU admission (RR 1.9, 95% CI 1.8-2.1), and LOS > 12days (RR 2.2, 95% CI 2.0-2.3). Compared to those with neither CHD nor genetic condition, children with both had significantly higher risk of all outcomes, with the largest difference for LOS > 12days (RR 2.3, 95% CI 2.0-2.7). Children with CHDs hospitalized for influenza are particularly susceptible to adverse outcomes compared to those without CHDs. Future studies are needed to corroborate findings in light of influenza vaccination.

Scoring systems used to predict morbidity in children with Kawasaki disease (KD) have been developed and validated in Asian populations. The objective of this study was to assess their utility in predicting the development of coronary artery dilation in children with KD in North America. This was a secondary analysis of a National Institutes of Health / National Heart, Lung, and Blood Institute (NIH/NHLBI) Pediatric Heart Network public use dataset from a multicenter, randomized controlled trial of pulse steroid therapy in KD in a North American cohort. The primary outcome of interest was development of coronary artery dilation. The Harada, Kobayashi, Egami, and Sano scoring systems, originally developed to predict risk of intravenous immunoglobulin (IVIG) resistance in Kawasaki patients in Japan, were applied to this cohort. Subjects were kept in the analysis only if there were complete data for every element of each scoring system-Harada (n = 132), Kobayashi (n = 88), Egami (n = 139), and Sano (n = 82). Patients classified as high-risk by the Harada score were more likely to have significant coronary artery dilation (p = 0.042), were more likely to require IVIG retreatment (p = 0.002), and were more likely to require hospital readmission (p < 0.001). The Egami, Kobayashi, and Sano scores were not predictive for any measured outcome. The Harada score can be useful in identifying KD patients at risk for developing coronary artery dilation and IVIG resistance. The Harada score has demonstrated higher sensitivity but lower specificity, making it a valuable screening tool that may benefit from supplementary diagnostic methods.

To compare the efficacy and safety of bedside ultrasound-guided and fluoroscopy-guided transvenous cardiac temporary pacing in the treatment of bradyarrhythmia in children. Children treated by temporary intravenous cardiac pacing from January 2016 to June 2023 in Hunan Provincial Children's Hospital were enrolled, and the characteristics and data of the cases were summarized. Patients were divided into bedside ultrasound-guided group (ultrasound group) and fluoroscopy-guided group (fluoroscopy group) according to the implantation guidance methods. The efficacy, safety, and incidence of complications in children were compared, and follow-up analysis was carried out. A total of 30 children were enrolled, including 18 males and 12 females, with a median age of 5.5 (2.9, 10.0) years and a median weight of 18.7 (12.7, 32.7) kg. The most common primary diseases were fulminant myocarditis (13/30 cases) and congenital high-grade AVB (10/30 cases). Among them, the proportion of congenital high AVB in the fluoroscopy group was significantly higher than that in the ultrasound group, and the difference was statistically significant (p = 0.007). The implantation process was successful in all 30 children. From the time of pacing decision to implantation, the median time of ultrasound group was 56 (30, 60) min and that of fluoroscopy group was 154 (78,180) min, with a statistically significant difference (P < 0.001). A total of 5 cases developed complications. There was no statistically significant difference between the two groups (P > 0.05). Compared with traditional fluoroscopic temporary pacing, bedside ultrasound-guided temporary pacing technology can effectively shorten the operation time and reduce the occurrence of complications and has become a better choice for children's emergency and critical care treatment. The right internal jugular vein is preferred for intravenous implantation.

In this review we provide a brief description of recently published articles addressing topics relevant to pediatric cardiologists. Our hope is to provide a summary of the latest articles published recently in other journals in our field. The articles address: 1- The use of AI in fetal echocardiography, 2- The role of Apixaban in thromboembolism prevention in pediatric congenital heart disease, 3- Cardiovascular events in childhood cancer survivors, and lastly 4- the new consensus statement on cardiac catheterization for pediatrics and adults with congenital heart disease.

The cone operation has revolutionized care for patients with Ebstein anomaly; however, acute post-operative right ventricular dysfunction (RVD) is common in this patient population. A single-center, retrospective review of 28 patients with Ebstein anomaly who underwent cardiac MRI (CMR) prior to cone reconstruction of the tricuspid valve was conducted. Measurements of atrial and ventricular size/function were assessed. Post-operative RVD was defined as the presence of moderate or severe systolic dysfunction on discharge echo. A two-tail t test was employed to compare the two groups. The average age at operation was 21.4years (range 1.6-57.8) and 14 (50%) had RVD at discharge. Patients with post-operative RVD had significantly larger pre-operative right atrial (RA) maximum volume (p = 0.016) and RA minimum volume (p = 0.030). Patients with RVD had smaller pre-operative left atrial (LA) minimum volume (p = 0.012). Larger pre-operative right ventricular (RV) end-systolic volume (p = 0.046), lower RV ejection fraction (0.029), and smaller left ventricular (LV) end-diastolic volume (p = 0.049) were significantly associated with post-operative RVD. Post-operative RVD was associated with longer milrinone duration (p = 0.009) and higher maximum milrinone dose (p = 0.005) but was not associated with intensive care or hospital length of stay (p = 0.19 and 0.67, respectively). Increased RA and RV dilation and decreased LA and LV volumes are associated with the development of post-operative RVD following cone operation for Ebstein anomaly. Post-operative RVD affects milrinone dose and duration but is not associated with increased length of stay.

Pediatric pericarditis presents challenges in its management, necessitating effective therapeutic interventions. Colchicine, known for its efficacy in adults, requires further investigation for its application and safety in pediatric cohorts. A systematic search across renowned databases identified relevant literature on colchicine use in pediatric pericarditis. Twenty-nine articles underwent rigorous screening, with 18 studies meeting inclusion criteria. Data extraction, quality assessment, and synthesis were conducted meticulously. Included studies comprised case reports, case series, and retrospective cohort studies. Colchicine demonstrated efficacy in reducing recurrence rates and symptom burden, with doses ranging from 0.25mg/day to 2mg/day. Adverse events were minimal, predominantly gastrointestinal. Notably, nausea was the most common side effect reported. The safety profile of colchicine was favorable, with rare instances of hepatic and hematologic toxicity. Colchicine emerges as a promising therapeutic option for pediatric pericarditis, demonstrating efficacy in reducing recurrence rates and alleviating symptoms. Its favorable safety profile suggests potential as a preferred long-term therapy. However, further research, including randomized controlled trials, is warranted to confirm its efficacy and safety and explore potential combination therapies.

Patent Ductus Arteriosus (PDA) stenting is an alternative to Modified Blalock-Taussig-Thomas Shunt (BT) to increase pulmonary blood flow in ductal-dependent pulmonary circulation. The objective of this study is to compare the immediate Intensive Care Unit (ICU) outcomes of PDA stent versus BT shunt in infants with ductal-dependent pulmonary circulation. This is a single center, retrospective study in infants less than 6months with ductal-dependent pulmonary circulation palliated with either PDA stenting or BT shunt from January 2015 till December 2023. Data were reviewed from pediatric ICU database. Demographics details, ICU parameters, mortality and morbidity were compared. The propensity score with inverse probability weighting was used to control the effect of covariates. A total of 302 patients with PDA stenting and 100 patients with BT shunt were included. Majority of the patients had intervention at first month of life. 126 (41.7%) patient underwent PDA stenting while 36 (36%) had BTS. The PDA stenting group has shorter ICU stay, 3 (2.0,6.0) days versus 8 (4.8,13.0) days (p < 0.001), shorter length of hospital stay, 9 (6.8,15.0) days versus 16 (11.0,22.0) days (p < 0.001), shorter ventilation days, 2 (1.0,4.0) days versus 3 (2.0,7.0) days (p < 0.001) and lower mortality, 7 (2.3%) versus 16 (16.0%) (p < 0.001). At 3months follow-up, the survival was higher in the PDA stenting group (p < 0.001). Conclusion: PDA stenting in infants with ductal-dependent pulmonary blood flow has a more favorable intensive care outcomes with shorter ICU and hospital stay. PDA stenting has a better survival outcome.

To describe critically ill neonates with premature closure of the ductus arteriosus (DA) and assess the frequency as well as predictive factors for extracorporeal membrane oxygenation (ECMO) support in the latter. This was a monocentric retrospective observational study conducted in the NICU of a French academic medical center between 01/01/2013 and 01/01/2023. All neonates diagnosed with premature closure of the DA were included. Exclusion criteria were associated congenital heart disease amenable to urgent surgeryor contraindication for ECMO. Eleven neonates with complete premature closure of the DA were included. Births were full-term with a weight of 3.60kg [3.16-3.89]. Only one case (9%) was diagnosed antenatally. Premature closure of the DA was idiopathic in seven neonates (64%) and associatedto maternal exposure to non-steroidal anti-inflammatory drugs (NSAIDs) in three (27%). All newborns had pulmonary hypertension (PH) and right ventricular hypertrophy. Three neonates (27%) were supported on veno-arterial ECMO and appeared to have more pathological adaptation to extra-uterine life, greater need for vasopressor and/or inotropic support, and higher frequency of bi-ventricular failure (100%) or morphological anomaly of the tricuspid valve (67%). No patient died but more than half had non-cardiological sequelae. Veno-arterial ECMO support is not uncommon in critically ill neonates with premature DA closure and appeared to be associated with more pathological adaptation to extra-uterine life, greater need for vasoactive-inotropic support and higher frequency of bi-ventricular failure or morphological anomaly of the tricuspid valve. These parameters can help clinicians to identify neonates likely to require such an assistance.