The cardiovascular adaptations associated with structured exercise training in Fontan patients remain unknown. We hypothesised that short-term training causes cardiac remodelling and parallel improvement in maximal exercise capacity (VO2 max) in these patients.

Five patients, median age 19.5 (17.6–21.3) years, with a history of Fontan operation meeting inclusion/exclusion criteria, participated in a 3-month training programme designed to improve endurance. Magnetic resonance images for assessment of cardiac function, fibrosis, cardiac output, and liver elastography to assess stiffness were obtained at baseline and after training. Maximal exercise capacity (VO2 max) and cardiac output Qc (effective pulmonary blood flow) at rest and during exercise were measured (C2H2 rebreathing) at the same interval. VO2 max increased from median (IQR) 27.2 (26–28.7) to 29.6 (28.5–32.2) ml/min/kg (p = 0.04). There was an improvement in cardiac output (Qc) during maximal exercise testing from median (IQR) 10.3 (10.1–12.3) to 12.3 (10.9–14.9) l/min, but this change was variable (p = 0.14). Improvement in VO2 max correlated with an increase in ventricular mass (r = 0.95, p = 0.01), and improvement in Quality-of-life inventory (PedsQL) Cardiac scale scores for patient-reported symptoms (r = 0.90, p = 0.03) and cognitive problems (r = 0.89, p = 0.04). The correlation between VO2 max and Qc showed a positive trend but was not significant (r = 0.8, p = 0.08). No adverse cardiac or liver adaptations were noted.

Short-term training improved exercise capacity in this Fontan pilot without any adverse cardiac or liver adaptations. These results warrant further study in a larger population and over a longer duration of time.

NCT03263312, Unique Protocol ID: STU 122016-037; Registration Date: 18 January, 2017.

Up to 90% of adults with untreated atrial septal defect will be symptomatic by 4th decade, and 30-49% will develop heart failure. 8–10% of these patients have pulmonary arterial hypertension with a female predominance regardless of age. We aimed to demonstrate that fenestrated closure can be safely performed in patients with decompensated heart failure and atrial septal defect-associated pulmonary arterial hypertension with improved outcome.

Transcatheter fenestrated atrial septal defect closures (Occlutech GmbH, Jena, Germany) were performed on a compassionate-use basis in 5 consecutive adult patients with atrial septal defect-associated pulmonary arterial hypertension and severe heart failure with prohibitive surgical mortality risks. Change in systemic oxygen saturation, 6-minute walk test, NYHA class, echocardiographic and haemodynamic parameters were used as parameters of outcome.

All patients were female, mean age 48.8 ± 13.5 years, followed up for a median of 29 months (max 64 months). Significant improvements observed in the 6-minute walk test, and oxygen saturation comparing day 0 time point to all other follow-up time points data (B = 1.32, SE = 0.28, t (22.7) = -4.77, p = 0.0001); and in the haemodynamic data (including pulmonary vascular resistance and pulmonary pressure) (B = –0.60, SE = 0.22, t (40.2) = 2.74, p = .009). All patients showed improved right ventricular size and function along with NYHA class. There were no procedure-related complications.

Fenestrated atrial septal defect closure is feasible in adults with decompensated heart failure and atrial septal defect-associated pulmonary arterial hypertension. It results in sustained haemodynamic and functional improvement

To characterise transesophageal echocardiography practice patterns among paediatric cardiac surgical centres in the United States and Canada.

A 42-question survey was sent to 80 echocardiography laboratory directors at paediatric cardiology centres with surgical programmes in the United States and Canada. Question domains included transesophageal echocardiography centre characteristics, performance and reporting, equipment use, trainee participation, and quality assurance.

Fifty of the 80 centres (62.5%) responded to the survey. Most settings were academic (86.0%) with 42.0% of centres performing > 350 surgical cases/year. The median number of transesophageal echocardiograms performed/cardiologist/year was 50 (26, 73). Pre-operative transesophageal echocardiography was performed in most surgical cases by 91.7% of centres. Transesophageal echocardiography was always performed by most centres following Norwood, Glenn, and Fontan procedures and by < 10% of centres following coarctation repair. Many centres with a written guideline allowed transesophageal echocardiography transducer use at weights below manufacturer recommendations (50.0 and 61.1% for neonatal and paediatric transducers, respectively). Most centres (36/37, 97.3%) with categorical fellowships had rotations which included transesophageal echocardiography participation. Large surgical centres (>350 cases/year) had higher median number of transesophageal echocardiograms/cardiologist/year (75.5 [53, 86] versus 35 [20, 52], p < 0.001) and more frequently used anaesthesia for diagnostic transesophageal echocardiography ≥ 67% of time (100.0 versus 62.1%, p = 0.001).

There is significant variability in transesophageal echocardiography practice patterns and training requirements among paediatric cardiology centres in the United States and Canada. Findings may help inform programmatic decisions regarding transesophageal echocardiography expectations, performance and reporting, equipment use, trainee involvement, and quality assurance.

Emergent resuscitation of postoperative paediatric cardiac surgical patients requires specialised skills and multidisciplinary teamwork. Bedside resternotomy is a rare but life-saving procedure and few studies focus on ways to prepare providers and improve performance. We created a multidisciplinary educational intervention that addressed teamwork and technical skills. We aimed to evaluate the efficiency of the intervention to decrease time to perform critical tasks and improve caregiver comfort.

A simulation-based, in situ resternotomy educational intervention was implemented. Pre-intervention data were collected. Educational aids were used weekly during day and night nursing huddles over a three-month period. All ICU charge nurses had separate educational sessions with study personnel and were required to demonstrate competency in all the critical tasks. Post-intervention simulations were performed after intervention and at 6 months and post-intervention surveys were performed.

A total of 186 providers participated in the intervention. There was a decrease in time to obtain defibrillator, setup resternotomy equipment and internal defibrillator paddles and deliver sedation and fluid (all p < 0.05). Time to escort family from the room and obtain blood was significantly decreased after intervention (p < 0.05). There was no difference in time to first dose of epinephrine, defibrillator pads on the patient, or time to call the cardiovascular surgeon or blood bank. Providers reported increased comfort in identifying equipment needed for resternotomy (p < 0.01) and setting up the internal defibrillator paddles (p < 0.01).

Implementation of a novel educational intervention increased provider comfort and decreased time to perform critical tasks in an emergent resternotomy scenario.

Perioperative immunisation administration surrounding congenital heart surgery is controversial. Delayed immunisation administration results in children being at risk of vaccine-preventable illnesses and is associated with failure to complete immunisation schedules. Among children with CHD, many of whom are medically fragile, vaccine-preventable illnesses can be devastating. Limited research shows perioperative immunisation may be safe and effective.

We surveyed Pediatric Acute Care Cardiology Collaborative member centres and explored perioperative immunisation practices. We analysed responses using descriptive statistics.

Complete responses were submitted by 35/46 (76%) centres. Immunisations were deferred for any period prior to surgery by 23 (66%) centres and after surgery by 31 (89%) centres. Among those who deferred post-operative immunisation, 20 (65%) required deferral only for patients whose operations required cardiopulmonary bypass. Duration of deferral in the pre- and post-operative periods was variable. Many centres included exceptions to their policy for specific vaccine-preventable illnesses. Almost all (34, 97%) centres administer routine childhood immunisation to patients who remain admitted for prolonged periods.

Most centres defer routine childhood immunisation for some period before and after congenital heart surgery. Centre specific practices vary. Immunisation deferral confers risk to patients and may not be warranted in this population. Further research would be necessary to understand the immunologic impact of these practices.

Thromboembolism is a complication in paediatric patients with CHD requiring cardiac surgery. Previous research has focused on post-operative thromboembolism. This study aimed to describe thromboembolism frequency before or after cardiac surgery in children with CHD.

We performed a single-centre retrospective study from October 2020 to June 2023 (inclusive). Patients were eligible for inclusion if they were <21 years of age and underwent cardiac surgery. Outcomes of interest included the occurrence and characteristics of thromboembolism in the 12 months before and after surgery, antithrombotic therapies, recurrent thromboembolism, and clinically significant bleeding.

Among 260 patients included, 35 (13.5%) developed an index thromboembolism. Twelve (34.3%) patients had an index thromboembolism <12 months before surgery and 23 (65.7%) had an index thromboembolism <12 months after surgery, including 8 (22.9%) patients who had thromboembolism during both exposure periods. The median interquartile range (IQR) time of thromboembolism relative to cardiac surgery was –26 (–4 to –140) days and 15 (4 to 41) days, respectively. Seven (20%) patients had arterial, 18 (51.4%) venous, and 3 (8.6%) had both arterial and venous thromboembolism. Median (IQR) antithrombotic therapy duration was 49 (24–84) days. Nine (25.7%) patients developed recurrent thromboembolism and five (14.3%) patients experienced clinically significant bleeding.

The risk of thromboembolism and recurrence is high both before and after cardiac surgery among paediatric patients with CHD. Prospective multi-centre studies should seek to identify risk factors for preoperative and postoperative thromboembolism to inform the design of future risk-stratified thromboembolism prevention trials in children with CHD.

It is often assumed, that adult patients with CHD (ACHD) have impairments regarding their cognitive function (CF) and health-related quality of life. In particular, it seems reasonable to assume that cyanosis may have a potential impact on CF as well as surgical or drug treatment into adulthood. This study assesses neuromental health aspects such as CF and health-related quality of life in ACHD patients.

Seventy-eight ACHD patients (female n = 39 (50%); 34.1 ± 12.9 years; cyanotic CHD n = 49 (62.8%) with a cyanosis duration of 159.8 ± 196.2 month) who underwent open heart surgery as first intervention were asked to participate during routinely follow-up in 2018. Wechsler Intelligence Scale IV was used for CF and the Short Form 36 Health Survey to assess health-related quality of life.

Intelligence quotient measures showed significant differences comparing never cyanotic and with a cyanotic phase in verbal comprehension (p = 0.013). There was no association of CF with cyanosis duration, number of surgery or catheter, CHD severity, and time of first surgery. The group of early surgery showed significantly better results in physical function (p = 0.040) of health-related quality of life, and in comparison with their assigned reference, both groups showed significantly reduced results in all domains except in bodily pain and mental health. Full-Scale intelligence quotient correlates with physical function of health-related quality of life.

The results show normal CF in ACHD. Health-related quality of life was weak in comparison with the reference. There is a need to improve the well-being of our ACHD with structured programmes, including physical activity programmes. This growing ACHD population should be focused in order of their needs, medical ones on one hand and on the other hand psychosocial matters.

Controversial data exist about the impact of Down syndrome on outcomes after surgical repair of atrioventricular septal defect.

(A) assess trends and outcomes of atrioventricular septal defect with and without Down syndrome and (B) determine risk factors associated with adverse outcomes after atrioventricular septal defect repair.

We queried The National Inpatient Sample using International Classification of Disease codes for patients with atrioventricular septal defect < 1 year of age from 2000 to 2018. Patients’ characteristics, co-morbidities, mortality, and healthcare utilisation were evaluated by comparing those with versus without Down syndrome.

In total, 2,318,706 patients with CHD were examined; of them, 61,101 (2.6%) had atrioventricular septal defect. The incidence of hospitalisation in infants with atrioventricular septal defect ranged from 4.5 to 7.5% of all infants hospitalised with CHD per year. A total of 33,453 (54.7%) patients were associated with Down syndrome. Double outlet right ventricle, coarctation of the aorta, and tetralogy of Fallot were the most commonly associated with CHD in 6.9, 5.7, and 4.3% of patients, respectively. Overall atrioventricular septal defect mortality was 6.3%. Multivariate analysis revealed that prematurity, low birth weight, pulmonary hypertension, and heart block were associated with mortality. Down syndrome was associated with a higher incidence of pulmonary hypertension (4.3 versus 2.8%, p < 0.001), less arrhythmia (6.6 versus 11.2%, p < 0.001), shorter duration for mechanical ventilation, shorter hospital stay, and less perioperative mortality (2.4 versus 11.1%, p < 0.001).

Trends in atrioventricular septal defect hospitalisation had been stable over time. Perioperative mortality in atrioventricular septal defect was associated with prematurity, low birth weight, pulmonary hypertension, heart block, acute kidney injury, and septicaemia. Down syndrome was present in more than half of atrioventricular septal defect patients and was associated with a higher incidence of pulmonary hypertension but less arrhythmia, lower mortality, shorter hospital stay, and less resource utilisation.

Persistent pulmonary hypertension of the newborn is a life-threatening condition that affects about 1–2 per 1,000 live births worldwide. Bosentan is an oral dual endothelin receptor antagonist that may have a beneficial effect on persistent pulmonary hypertension of the newborn by reducing pulmonary vascular resistance and improving oxygenation. However, its role in persistent pulmonary hypertension of the newborn remains unclear.

To systematically evaluate the efficacy and safety of bosentan as an adjuvant therapy for persistent pulmonary hypertension of the newborn in newborns.

We searched six English and two Chinese databases from their inception to 1 January 2023 following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. We included randomised controlled trials and retrospective studies that compared bosentan with placebo or other drugs for persistent pulmonary hypertension of the newborn in newborns. We performed a meta-analysis using random-effects models and assessed the risk of bias and heterogeneity in the included studies.

We included 10 studies with a total of 550 participants. Bosentan significantly reduced the treatment failure rate (relative risk = 0.25, P < 0.001), pulmonary artery pressure (mean difference = −11.79, P < 0.001), and length of hospital stay (mean difference = −1.04, P = 0.003), and increased the partial pressure of oxygen (mean difference = 10.02, P < 0.001) and blood oxygen saturation (SpO2) (mean difference = 8.24, P < 0.001) compared with a placebo or other drugs. The occurrence of adverse reactions was not significantly different between bosentan and a placebo or other drugs.

Bosentan is effective in the treatment of persistent pulmonary hypertension of the newborn but adverse reactions such as abnormal liver function should be observed when using it.

Left ventricular diastolic dysfunction is a recognised sequela following transplantation in paediatric heart transplant patients. Traditional echocardiographic indices do not correlate well with left ventricular filling pressure immediately after transplantation. This study aimed to assess whether these indices have any long-term correlation after transplantation in paediatric patients.

A retrospective chart review of 41 patients who had a heart transplant before the age of 24 years was performed. The median time since the transplantation was 11 years. Data obtained from surveillance cardiac catheterisation and echocardiographic examination were reviewed. Traditional echocardiographic indices of diastolic function were compared with the pulmonary capillary wedge pressure and left ventricular end-diastolic pressure obtained from cardiac catheterisation.

The median age at transplant was 12.1 years, and the median time since transplant was 11 years. Eighteen patients (43%) had a history of at least one rejection episode and 12 patients (29%) had a history of cardiac allograft vasculopathy. There was no correlation between mitral inflow E velocity, mitral E/A ratio, tissue Doppler velocities, mitral E/e’ (mitral inflow E velocity to mitral annular velocity), and elevated pulmonary capillary wedge pressure or elevated left ventricular end-diastolic pressure. There was no correlation between mitral valve deceleration time or isovolumetric relaxation time with elevated pulmonary capillary wedge pressure or elevated left ventricular end-diastolic pressure.

Our findings suggest that traditional echocardiographic indices of diastolic function do not correlate well with elevated invasive pulmonary capillary wedge pressure or elevated left ventricular end-diastolic pressure in paediatric heart transplant patients’ long-term post-transplantation.

Infection with Sars-CoV-2 is known to cause cardiac injury and coronary artery changes in moderate to severe acute COVID-19 and post-acute multisystem inflammatory syndrome in children (MIS-C). However, little is known about the potential for cardiac involvement, in particular coronary artery dilation, in asymptomatic or mild cases of COVID-19.

A retrospective review of children ≤ 18 years of age with a history of asymptomatic or mild COVID-19 disease who underwent echocardiography after Sars-CoV-2 infection is conducted. Patients were excluded if they had been hospitalised for COVID-19/MIS-C or had a history of cardiac disease that could affect coronary artery dimension. Coronary artery dilation was defined as the Boston Z-score greater than 2.0.

One hundred and fifty-seven patients met inclusion criteria with a mean age of 9.4 years (+/– 5.4 years). Eighty-four (54%) patients were identified as having COVID-19 through positive antibody testing. All patients underwent electrocardiogram and echocardiogram as part of their cardiology evaluation. One hundred and thirty-five (86%) patients had a normal evaluation or only a minor variant on electrocardiogram, while 22 patients had abnormalities on echocardiogram, 4 of which demonstrated coronary artery dilation based on the Boston Z-score.

Much of the literature for post-infectious screening and follow-up focuses on patients with a history of moderate to severe COVID-19 disease, emphasising the need for surveillance for the potential development of myocarditis. In this study, 4 out of 157 (2.5%) children with a history of asymptomatic or mild COVID-19 disease without MIS-C were found to have some degree of coronary artery dilation. The significance of this finding currently remains unknown.

We performed a single-centre retrospective study comparing the accuracy of non-invasive elastography with liver biopsy in accurate assessment of Fontan-associated liver disease.

Fontan patients who underwent combined assessment with a percutaneous liver biopsy and non-invasive elastography between January 2015 and December 2023 at our Children’s hospital were included. Liver biopsies were classified using the Congestive Hepatic Fibrosis Score as early Fontan-associated liver disease (scores 1, 2) and advanced Fontan-associated liver disease (score 3/bridging fibrosis and score 4/cirrhosis). Elastography values were categorised as advanced Fontan-associated liver disease for liver elasticity >2.1 m/s by ultrasound and liver stiffness >5 KPa on magnetic resonance elastography.

We included 130 patients (116 children, 89%, mean age at biopsy: 14.6 years ± 3.6) who underwent liver biopsy at a mean duration of 11.1 years (±0.3) following Fontan surgery. Advanced Fontan-associated liver disease was noted in 41 (31.5%) patients with 13 (10%) showing frank cirrhosis. Pre-biopsy ultrasound showed advanced liver fibrosis in 18/125 (14%), with low sensitivity (23%), high specificity (90%), and low accuracy (68%, k = 0.1) in diagnosing advanced Fontan-associated liver disease. Similarly, pre-biopsy magnetic resonance elastography showed advanced fibrosis in 23/86 (27%) of patients, with low sensitivity (30%), fair specificity (75%), and low accuracy (63%, k = 0.1). Interestingly, advanced Fontan-associated liver disease was missed by ultrasound in 29% and by magnetic resonance elastography in 25% of patients. Advanced Fontan-associated liver disease was associated with lower platelet count (p = 0.02) and higher Gamma-glutamyl Transferase levels (p = 0.02).

Advanced hepatic fibrosis is common among paediatric Fontan patients. Non-invasive elastography may overestimate and underestimate the degree of liver fibrosis, and therefore, liver biopsy may be required for confirming disease severity.

Atrioventricular valve regurgitation in patients with univentricular heart is a well-known risk factor for adverse outcomes and atrioventricular valve repair remains a particular surgical challenge.

We reviewed all surgical atrioventricular valve procedures in patients with univentricular heart and two separate atrioventricular valves who underwent surgical palliation. Endpoints of the study were reoperation-free survival and cumulative incidence of reoperation.

Between 1994 and 2021, 202 patients with univentricular heart and two separate atrioventricular valve morphology underwent surgical palliation, with 15.8% (32/202) requiring atrioventricular valve surgery. Primary diagnoses were double inlet left ventricle (n = 14, 43.8%), double outlet right ventricle (n = 7, 21.9%), and congenitally corrected transposition of the great arteries (n = 7, 21.9%). Median weight at valve surgery was 10.6 kg (interquartile range, 7.9–18.9). Isolated left or right atrioventricular valve surgery was required in nine (28.1%) and 22 patients (68.8%), respectively. Concomitant left and right atrioventricular valve surgery was performed in one patient (3.1%). Closure of the left valve was conducted in four patients (12.5%) and closure of the right valve in three (9.4%). Operative and late mortality were 3.1% and 9.7%, respectively. Reoperation-free survival and cumulative incidence of reoperation at 10 years after surgery were 62.3% (standard error of the mean: 6.9) and 30.9% (standard error of the mean: 9.6), respectively.

In patients with univentricular heart and two separate atrioventricular valves, surgical intervention on these valves is required in a minority of patients and is associated with low mortality but high incidence of reoperation.

Packed red blood cell transfusions occur frequently after congenital heart surgery to augment haemodynamics, with limited understanding of efficacy. The goal of this study was to analyse the hemodynamic response to packed red blood cell transfusions in a single cohort, as “proof-of-concept” utilising high-frequency data capture of real-time telemetry monitoring.

Retrospective review of patients after the arterial switch operation receiving packed red blood cell transfusions from 15 July 2020 to 15 July 2021. Hemodynamic parameters were collected from a high-frequency data capture system (SickbayTM) continuously recording vital signs from bedside monitors and analysed in 5-minute intervals up to 6 hours before, 4 hours during, and 6 hours after packed red blood cell transfusions—up to 57,600 vital signs per packed red blood cell transfusions. Variables related to oxygen balance included blood gas co-oximetry, lactate levels, near-infrared spectroscopy, and ventilator settings. Analgesic, sedative, and vasoactive infusions were also collected.

Six patients, at 8.5[IQR:5-22] days old and weighing 3.1[IQR:2.8-3.2]kg, received transfusions following the arterial switch operation. There were 10 packed red blood cell transfusions administered with a median dose of 10[IQR:10-15]mL/kg over 169[IQR:110-190]min; at median post-operative hour 36[IQR:10-40]. Significant increases in systolic and mean arterial blood pressures by 5-12.5% at 3 hours after packed red blood cell transfusions were observed, while renal near-infrared spectroscopy increased by 6.2% post-transfusion. No significant changes in ventilation, vasoactive support, or laboratory values related to oxygen balance were observed.

Packed red blood cell transfusions given after the arterial switch operation increased arterial blood pressure by 5-12.5% for 3 hours and renal near-infrared spectroscopy by 6.2%. High-frequency data capture systems can be leveraged to provide novel insights into the hemodynamic response to commonly used therapies such as packed red blood cell transfusions after paediatric cardiac surgery.

Rastelli surgery is used for the correction of several CHDs. Although late-onset cardiac arrhythmias have emerged as a major complication after corrective surgeries, there is a paucity of data on arrhythmias after Rastelli surgery.

This retrospective cohort study was conducted on patients who had undergone Rastelli surgery and have been followed at the adult CHD clinic at our hospital.

A total of 55 patients (36.4% female, age 22.2 ± 6.4 years) were followed for a median period of 24.2 (20.6–31.0) years. Tachyarrhythmias occurred in 21 (38.4 %) patients (n = 15 for atrial tachycardia, 5 for ventricular tachycardia, and 1 for both atrial and ventricular tachycardia). Older age at surgery was significantly associated with the risk of tachyarrhythmias (P = 0.022). Bradyarrhythmia occurred in 12 (21.8%) patients and consisted of perioperative AV block (n = 5), late AV block (n = 1), and sinus node dysfunction (n = 6). Nine (16.4%) patients underwent catheter ablation. The mechanisms of atrial arrhythmias include cavotricuspid isthmus-dependent and surgical scar-dependent intra-atrial reentrant tachycardias. Among the three patients who underwent ablation for ventricular tachycardia, all circuits were dependent on the scar at the base of the right ventricle to pulmonary artery conduit. Median survival free from any event (arrhythmia, death, or heart failure) was 31.6 (28.1–35.1) years after Rastelli surgery.

The prevalence of arrhythmias late after Rastelli surgery is substantial and increases in the second decade after surgery. Older age at surgery is associated with a higher prevalence of arrhythmias.

Complexity stratification for CHD is an integral part of clinical research due to its heterogenous clinical presentation and outcomes. To support our ongoing research efforts into CHD requiring disease severity stratifications, a simplified CHD severity classification system was developed and verified, with potential utility for clinical researchers without specialist CHD knowledge or access to clinical/medical records.

A two-tiered analysis approach was undertaken. First-tier analysis included the audit of a comprehensive system based on: i) timing of intervention, ii) cardiac morphology, and iii) cardiovascular physiology using real patient data (n = 30), across 10 common CHD lesions. Second-tier analysis allowed for a simplified version of the classification system using morphology as a stand-alone predictor. Twelve clinicians of varying specialities involved in CHD care ranked 10 common lesions from least to most severe based on typical presentation and clinical course.

First-tier analysis identified that cardiac morphology was the principal driver of complexity. Second-tier analysis largely confirmed the ranking and classification of the lesions into the broad CHD severity groups, although some variation was noted, specifically among non-cardiac specialists. This simplified version of the classicisation system, with morphology as a stand-alone predictor of severity, allowed for effective stratification for the purposes of analysis.

The findings presented here support this comprehensive and simple CHD severity classification system with broad utility in CHD research, particularly among clinicians and researchers with limited knowledge of CHD. The model may be applied to produce locally relevant research tools.

Lack of sexual orientation and gender identity (SOGI) data creates barriers for lesbian, gay, bisexual, transgender, and queer (LGBTQ+) people in health care. Barriers to SOGI data collection include physician misperception that patients do not want to answer these questions and discomfort asking SOGI questions. This study aimed to assess patient comfort towards SOGI questions across five quaternary care adult congenital heart disease (ACHD) centres.

A survey administered to ACHD patients (≥18 years) asked (1) two-step gender identity and birth sex, (2) acceptance of SOGI data, and (3) the importance for ACHD physicians to know SOGI data. Chi-square tests were used to analyse differences among demographic groups and logistic regression modelled agreement with statement of patient disclosure of SOGI improving patient–physician communication.

Among 322 ACHD patients, 82% identified as heterosexual and 16% identified as LGBTQ+, across the age ranges 18–29 years (39.4%), 30–49 years (47.8%), 50–64 years (8.7%), and > 65 years (4.0%). Respondents (90.4%) felt comfortable answering SOGI questions. Respondents with bachelor’s/higher education were more likely to “agree” that disclosure of SOGI improves patient–physician communication compared to those with less than bachelor’s education (OR = 2.45; 95% CI 1.41, 4.25; p = .0015).

These findings suggest that in this largely heterosexual population, SOGI data collection is unlikely to cause patient discomfort. Respondents with higher education were twice as likely to agree that SOGI disclosure improves patient–physician communication. The inclusion of SOGI data in future studies will provide larger samples of underrepresented minorities (e.g. LGBTQ+ population), thereby reducing healthcare disparities within the field of cardiovascular research.

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.