Extubation failure after neonatal cardiac surgery is associated with increased intensive care unit length of stay, morbidity, and mortality. We performed a quality improvement project to create and implement a peri-extubation bundle, including extubation readiness testing, spontaneous breathing trial, and high-risk criteria identification, using best practices at high-performing centers to decrease neonatal and infant extubation failure by 20% from a baseline of 15.7% to 12.6% over a 2-year period.

Utilising the transparency of the Pediatric Cardiac Critical Care Consortium database, five centres were identified as high performers, having better-than-expected neonatal extubation success rates with the balancing metric of as-expected or better-than-expected mechanical ventilation duration. Structured interviews were conducted with cardiac intensive care unit physician leadership at the identified centers to determine centre-specific extubation practices. Data from those interviews underwent qualitative content analysis which was used to develop a peri-extubation bundle. The bundle was implemented at a single-centre 17-bed cardiac intensive care unit. Extubation failure, defined as reintubation within 48 hours of extubation for anything other than a procedure, ventilator days and bundle compliance was tracked.

There was a 41.4% decrease in extubation failure following bundle implementation (12 failures of 76 extubations pre-implantation; 6 failures of 65 extubations post-implementation). Bundle compliance was 95.4%. There was no difference in ventilator days (p = 0.079) between groups.

Implementation of a peri-extubation bundle created from best practices at high-performing centres reduced extubation failure by 41.4% in neonates and infants undergoing congenital heart surgery.

Our aim was to investigate all children admitted to paediatric intensive care units (ICU) in the Republic of Ireland between January 2020 and August 2022 with an admitting diagnosis of acute COVID-19 infection or paediatric inflammatory multi-system syndrome, temporally associated with SARS-CoV-2 (PIMS-TS) or associated illness. The patients were identified to catalogue the severity of illness, analyse cardiovascular manifestations of their disease, and short-term outcomes.

This is a retrospective multi-centre observational study.

127 children were admitted to paediatric ICU in Ireland with a COVID-19- related illness between January 2020 and August 2022. 87 (68.5%) of patients had acute COVID-19 infection, 39 (30.7%) had PIMS-TS and 1 (0.8%) patient had post-COVID vaccine-related myocarditis. Ventilatory support was required for 47/87 (54%) in the COVID-19 group comparative to 9/39 (23%) of patients with PIMS-TS. Inotropic support was required for 13/87 (14.9%) children with COVID-19 and 29/39 (74.3%) with PIMS-TS. Evidence of any cardiac disease on ECHO was identified in 23/38 (60.5%) of the PIMS-TS cohort comparative to only 5/36 (13.9%) of patients with COVID-19. 38/39 (97.4%) of patients with PIMS-TS-related cardiac disease and 100% with COVID-19 had a normal echo at the time of discharge from hospital. Overall survival of patients was 100%.

The burden of cardiac disease in children requiring paediatric ICU care for COVID-19-related disease was high in the acute phase; however, all children survived, and all cardiac investigations had normalised by short-term follow-up.

To determine the short- and medium-term cardiac outcomes in children admitted with multisystem inflammatory syndrome in children at a tertiary care centre in Pakistan.

Children fulfilling the criteria for multisystem inflammatory syndrome and admitted to the hospital between April 2020 and March 2022 were enrolled in this prospective longitudinal cohort study. From admission to discharge, laboratory and cardiac parameters were recorded for all patients, who were subsequently followed up in clinics at various intervals. Data analysis was conducted using STATA version 15.0.

A total of 51 children were included, with viral myocarditis (41.2%) and toxic shock syndrome (33.3%) being the most common phenotypes. The cardiovascular system was most commonly affected in 27 children (53%) with laboratory evidence of inflammation and myocardial injury with median and interquartile levels of ferritin 1169 (534-1704), C-reactive protein 83 (24-175), lactate dehydrogenase 468 (365-1270), N-terminal pro-B-type natriuretic peptide 8,656 (2,538-31,166), and troponin 0.16 (0.02-2.0).

On admission, decreased left ventricular ejection fraction was observed in 58.8% of patients and impaired global longitudinal strain in 33.3%. At discharge, left ventricular ejection fraction had normalised in 83% of patients. Pericardial effusion resolved in all patients, and valvulitis resolved in 86% by 12 months. Paediatric ICU admission was required in 42 (82%) of patients with an overall mortality of 12% (n = 6).

Our study finds high hospital mortality for multisystem inflammatory syndrome in children compared to 1-2% from previous studies. Yet, in Pakistan, surviving children with multisystem inflammatory syndrome show favourable short- to medium-term cardiac outcomes

Multisystem inflammatory syndrome in children is a rare, post-infectious complication of SARS-CoV-2 infection in children. We aimed to assess the long-term sequelae, particularly cardiac, in a large, diverse population.

We performed a retrospective cohort study of all children (aged 0–20 years, n = 304) admitted to a tertiary care centre with a diagnosis of multisystem inflammatory syndrome in children from March 1, 2020 to August 31, 2021 and had at least one follow-up visit through December 31, 2021. Data were collected at hospitalisation, 2 weeks, 6 weeks, 3 months, and 1 year after diagnosis, where applicable. Cardiovascular outcomes included left ventricular ejection fraction, presence or absence of pericardial effusion, coronary artery abnormalities, and abnormal electrocardiogram findings.

Population was median age 9 years (IQR 5–12), 62.2% male, 61.8% African American (AA), and 15.8% Hispanic. Hospitalisation findings included abnormal echocardiogram 57.2%, mean worst recorded left ventricular ejection fraction 52.4% ± 12.4%, non-trivial pericardial effusion 13.4%, coronary artery abnormalities 10.6%, and abnormal ECG 19.6%. During follow-up, abnormal echocardiogram significantly decreased to 6.0% at 2 weeks and 4.7% at 6 weeks. Mean left ventricular ejection fraction significantly increased to 65.4% ± 5.6% at 2 weeks and stabilised. Pericardial effusion significantly decreased to 3.2% at 2 weeks and stabilised. Coronary artery abnormalities significantly decreased to 2.0% and abnormal electrocardiograms significantly decreased to 6.4% at 2 weeks and stabilised.

Children with multisystem inflammatory syndrome in children have significant echocardiographic abnormalities during the acute presentation, but these findings typically improve within weeks. However, a small subset of patients may have persistent coronary abnormalities.

Long hospital stays for neonates following cardiac surgery can be detrimental to short- and long-term outcomes. Furthermore, it can impact resource allocation within heart centres' daily operations. We aimed to explore multiple clinical variables and complications that can influence and predict the post-operative hospital length of stay.

We conducted a retrospective observational review of the full-term neonates (<30 days old) who had cardiac surgery in a tertiary paediatric cardiac surgery centre – assessment of multiple clinical variables and their association with post-operative hospital length of stay.

A total of 273 neonates were screened with a mortality rate of 8%. The survivors (number = 251) were analysed; 83% had at least one complication. The median post-operative hospital length of stay was 19.5 days (interquartile range 10.5, 31.6 days). The median post-operative hospital length of stay was significantly different among patients with complications (21.5 days, 10.5, 34.6 days) versus the no-complication group (14 days, 9.6, 19.5 days), p < 0.01. Among the non-modifiable variables, gastrostomy, tracheostomy, syndromes, and single ventricle physiology are significantly associated with longer post-operative hospital length of stay. Among the modifiable variables, deep vein thrombosis and cardiac arrest were associated with extended post-operative hospital length of stay.

Complications following cardiac surgery can be associated with longer hospital stay. Some complications are modifiable. Deep vein thrombosis and cardiac arrest are among the complications that were associated with longer hospital stay and offer a direct opportunity for prevention which may be reflected in better outcomes and shorter hospital stay.

CHD is an important phenotypic feature of chromosome 22q11.2 copy number variants. Biventricular repair is usually possible, however there are rare reports of patients with chromosome 22q copy number variants and functional single ventricle cardiac disease.

This is a single centre retrospective review of patients with chromosome 22q copy number variants who underwent staged single ventricle reconstructive surgery between 1 July, 1984 and 31 December, 2020.

Seventeen patients met inclusion criteria. The most common diagnosis was hypoplastic left heart syndrome (n = 8) and vascular anomalies were present in 13 patients. A microdeletion of the chromosome 22 A-D low-copy repeat was present in 13 patients, and the remaining had a duplication. About half of the patients had documented craniofacial abnormalities and/or hypocalcaemia, and developmental delay was very common. Fifteen patients had a Norwood operation, 10 patients had a superior cavopulmonary anastomosis, and 7 patients had a Fontan. Two patients had cardiac transplantation after Fontan. Overall survival is 64% at 1 year, and 58% at 5 and 10 years. Most deaths occurred following Norwood operation (n = 5).

CHD necessitating single ventricle reconstruction associated with chromosome 22q copy number variants is not common, but typically occurs as a variant of hypoplastic left heart syndrome with the usual cytogenetic microdeletion. The most common neonatal surgical intervention performed is the Norwood, where most of the mortality burden occurs. Associated anomalies and medical issues may cause additional morbidity after cardiac surgery, but survival is similar to infants with other types of single ventricle disease.

The Pediatric Acute Care Cardiology Collaborative (PAC3) was established to improve acute care cardiology outcomes through the development of an accurate and well-validated clinical registry. We report the validation results of the initial PAC3 registry audits and describe a novel regional audit format developed to accommodate a rapidly expanding membership facilitate collaborative learning and allow for necessary modification due to the COVID-19 pandemic.

Six hospitals were audited using a regional audit format and three hospitals were subsequently audited virtually. Critical and challenging-to-collect data elements were audited among at least 40 randomly selected cases. Discrepancies were categorised as either major or minor depending on their relative importance to patient outcomes and clinical care. Results were tabulated and reported.

We audited 386 encounters and 27,086 individual data fields across 9 hospitals. The aggregate overall accuracy rate was 99.27% and the aggregate major discrepancy rate was 0.51%. The overall accuracy rate ranged from 98.77% to 99.59%, and the major discrepancy rate ranged from 0.26% to 0.88% across the cohort. No appreciable difference was seen between audit formats. Both the regional and virtual audit methods were viewed favourably by participants.

A low data discrepancy rate was found demonstrating that the PAC3 registry is a highly accurate data source for use in quality improvement, benchmarking, and research. Regional audits and virtual audits were both successfully implemented.

Paramedics received training in point-of-care ultrasound (POCUS) to assess for cardiac contractility during management of medical out-of-hospital cardiac arrest (OHCA). The primary outcome was the percentage of adequate POCUS video acquisition and accurate video interpretation during OHCA resuscitations. Secondary outcomes included POCUS impact on patient management and resuscitation protocol adherence.

A prospective, observational cohort study of paramedics was performed following a four-hour training session, which included a didactic lecture and hands-on POCUS instruction. The Prehospital Echocardiogram in Cardiac Arrest (PECA) protocol was developed and integrated into the resuscitation algorithm for medical non-shockable OHCA. The ultrasound (US) images were reviewed by a single POCUS expert investigator to determine the adequacy of the POCUS video acquisition and accuracy of the video interpretation. Change in patient management and resuscitation protocol adherence data, including end-tidal carbon dioxide (EtCO2) monitoring following advanced airway placement, adrenaline administration, and compression pauses under ten seconds, were queried from the prehospital electronic health record (EHR).

Captured images were deemed adequate in 42/49 (85.7%) scans and paramedic interpretation of sonography was accurate in 43/49 (87.7%) scans. The POCUS results altered patient management in 14/49 (28.6%) cases. Paramedics adhered to EtCO2 monitoring in 36/36 (100.0%) patients with an advanced airway, adrenaline administration for 38/38 (100.0%) patients, and compression pauses under ten seconds for 36/38 (94.7%) patients.

Paramedics were able to accurately obtain and interpret cardiac POCUS videos during medical OHCA while adhering to a resuscitation protocol. These findings suggest that POCUS can be effectively integrated into paramedic protocols for medical OHCA.

The Pediatric Cardiac Critical Care Consortium (PC4) is a multi-institutional quality improvement registry focused on the care delivered in the cardiac ICU for patients with CHD and acquired heart disease. To assess data quality, a rigorous procedure of data auditing has been in place since the inception of the consortium.

This report describes the data auditing process and quantifies the audit results for the initial 39 audits that took place after the transition from version one to version two of the registry’s database.

In total, 2219 total encounters were audited for an average of 57 encounters per site. The overall data accuracy rate across all sites was 99.4%, with a major discrepancy rate of 0.52%. A passing score is based on an overall accuracy of >97% (achieved by all sites) and a major discrepancy rate of <1.5% (achieved by 38 of 39 sites, with 35 of 39 sites having a major discrepancy rate of <1%). Fields with the highest discrepancy rates included arrhythmia type, cardiac arrest count, and current surgical status.

The extensive PC4 auditing process, including initial and routinely scheduled follow-up audits of every participating site, demonstrates an extremely high level of accuracy across a broad array of audited fields and supports the continued use of consortium data to identify best practices in paediatric cardiac critical care.