The COVID-19 pandemic presented unique challenges to global healthcare. Face-to-face outpatient care was dramatically reduced. This study implemented a remote consultation service via a mobile app (Pexip) to monitor patients with major CHD.

Study design was quasi-experimental and prospective.

Remote consultations were carried out at a tertiary paediatric cardiology centre in Northern Ireland.

Children with major CHD aged 0–16years in Northern Ireland.

The intervention was a Pexip-enabled remote consultation.

Primary outcome measures included the number of attendances to hospital both initiated and avoided via remote consultation. Remote consultations were conducted by doctor and/or cardiac specialist nurse or by specialist nurse alone (52% vs. 48%).

In the study, 32 patients enrolled; three were non-responders and a further two excluded. There were 201 remote consultations delivered (mean = 7.4). There were 12 admissions to hospital resulting from the remote consultation; the commonest indication was abnormal oxygen saturations (42%). There were 38 hospital attendances avoided, predominantly related to infant feeding and medication advice (both 42%).

A significant number of unnecessary hospital attendances were avoided (n = 38). Remote consultation technology proved a user-friendly and valuable adjunct to the provision of ongoing specialist patient care in challenging circumstances. There was a reduction in parental anxiety, and both parents and clinicians found this initiative beneficial to patient care. There was prompt identification of unwell children on remote consultations.

To assess whether isolated very low QRS voltage of ≤0.3 mV in the frontal leads might be a marker for diagnosing paediatric vasovagal syncope and the risk of recurrence.

We included 118 children with vasovagal syncope, comprising 70 males and 48 females in our retrospective analysis. All patients underwent head-up tilt test and supine 12-lead electrocardiography. Furthermore, the QRS voltage was measured from each one of the 12 leads on basal electrocardiography. Patients were followed up for 6–24 months (average, 16 months).

Eighty-six patients (73%) patients displayed isolated very low QRS voltage in frontal leads. Furthermore, the patients were classified into two groups based on the presence or absence of isolated very low QRS voltage. Enhanced syncopic spells over the past 6 months, and the positive rates of head-up tilt test were observed in patients having isolated very low QRS voltage in the frontal leads than those without isolated very low QRS voltage (p < 0.05). The single factor and time-to-event analyses also showed an increased syncope recurrence rate in patients with isolated very low QRS voltage in frontal leads when compared with those without isolated very low QRS voltage (p < 0.01).

Isolated very low QRS voltage in frontal leads is correlated with the positive response of the head-up tilt test in children who experience syncope and its recurrence post-treatment. Hence, isolated very low QRS voltage in frontal leads might become a novel diagnostic indicator and a risk factor for syncope recurrence in children with vasovagal syncope.

The COVID pandemic has had deleterious effects on the mental health of the global population. Parents of children with CHD were particularly vulnerable to negative mental health outcomes such as depression, anxiety, and perceived stress. A better understanding of the CHD parent experiences, needs, and concerns while navigating the healthcare system during a pandemic is needed.

Online survey responses from 71 parents of young children with CHD representing families across the United States of America and Canada were analysed. Qualitative data were collected one year into the COVID pandemic. Thematic analysis was used to examine responses to the open-ended question “What would you like healthcare professionals (doctors, nurses) to know about your experience of being a parent with a child with CHD during the COVID-19 pandemic?.”

Two major themes with subthemes and an umbrella theme emerged from the parents’ responses (1) Pandemic Parenting: The Emotional Toll of Hospital Visitation Restrictions, Dealing with Social Distancing, Feeling Isolated, Decision Making in Uncertainty, and Playing it Safe versus Returning to Normal and (2) Unmet Expectations of Care: Needing Information, Wanting Empathy, Requesting Respect, Questioning Care Quality, and the umbrella theme of: Our Lives were Turned Upside Down.

CHD parents describe a negative impact of healthcare-related challenges during the COVID pandemic. These findings may offer insight to how healthcare professionals can better support the mental health and care burden of CHD parents during future pandemics.

We sought to examine the relative importance of surgical lesion complexity versus the presence of genetic/syndromic/extracardiac anomalies (GSAs) in determining survival, morbidity or need for reinterventions following repair for aortic arch hypoplasia.

A single-centre, retrospective cohort study of infants undergoing biventricular aortic arch repair via sternotomy from 2010 to 2021 was conducted. Survival analysis was performed using Kaplan–Meier methods, with additional Bayesian survival modelling for subgroups. Composite morbidity comprised respiratory, renal, neurologic, or sepsis-related complications.

Of 83 included infants, n = 13/83 (15.7%) had complex repairs; 27/83 (32.5%) were GSA+. Operative mortality was significantly higher in GSA+ versus GSA− patients (18.5% vs. 1.8%; p = 0.01), though not for complex versus non-complex repairs. Overall 10-year Kaplan–Meier survival was 86.7%. Bayesian modelling suggested equivalent post-discharge attrition in non-complex/GSA+ and complex/GSA− patients, with the poorest outcomes in complex/GSA+ patients; non-complex/GSA− patients had 100% survival. GSA+ patients exhibited higher composite morbidity (44.4% vs. 7.1% in GSA− p < 0.001), with their mode of death seemingly related to a high incidence of respiratory and neurological morbidity, notably in Dandy–Walker syndrome. The 10-year freedom from arch reinterventions was 87.7%; neither complexity, GSA status, nor post-repair peak arch velocity predicted the need for arch reinterventions.

Whilst anatomic complexity may have been somewhat neutralised as a risk factor for operative mortality, in contrast to GSA+ status, there is further post-discharge attrition attributable to complexity or GSA+ status, with additive risk effects. Morbidity directly related to certain syndromes underlies some of this risk. Non-anatomic substrates represent a persistent limitation to outcomes of surgical aortic arch repair in infants.

Risk stratification is recommended for patients with ventricular pre-excitation, particularly when sports eligibility is required. Few studies have examined the changes in the electrophysiological properties of the accessory pathway during growth. This study investigates the evolution of electrophysiological properties of the ventricular pre-excitation in young athletes referred for sports eligibility.

Between January 2011 and July 2022, 44 paediatric patients (32 males; mean age, 10 ± 2.42) with ventricular pre-excitation underwent an electrophysiological study, both at rest and during adrenergic stress at two different times (T0 and T1) within a minimal interval of 2 years. Transcatheter ablation was not performed between the two electrophysiological studies. Electrophysiological data were collected and compared.

Electrophysiological study under basal conditions showed a significant decrease in the anterograde accessory pathway effective refractory period and 1:1 conduction over the accessory pathway from T0 to T1. The shortest pre-excited R-R interval during atrial fibrillation did not significantly change at the basal condition; however, it decreased during the stress test. Furthermore, six patients (13.6%) changed the risk profile of their accessory pathway: two “high-risk” patients at T0 became “low-risk” and four “low-risk” patients became “high-risk” at T1. Atrioventricular re-entry tachycardia inducibility did not differ significantly between the two electrophysiological studies.

This study highlights the importance of repeating electrophysiological study (transesophageal or intracardiac) in paediatric athletes with ventricular pre-excitation because significant and clinically relevant changes in the conduction and refractoriness of accessory pathway can occur. This could influence risk stratification for sports eligibility and the correct indication and timing for accessory pathway ablation.

Paediatric patients with heart failure requiring ventricular assist devices are at heightened risk of neurologic injury and psychosocial adjustment challenges, resulting in a need for neurodevelopmental and psychosocial support following device placement. Through a descriptive survey developed in collaboration by the Advanced Cardiac Therapies Improving Outcomes Network and the Cardiac Neurodevelopmental Outcome Collaborative, the present study aimed to characterise current neurodevelopmental and psychosocial care practices for paediatric patients with ventricular assist devices.

Members of both learning networks developed a 25-item electronic survey assessing neurodevelopmental and psychosocial care practices specific to paediatric ventricular assist device patients. The survey was sent to Advanced Cardiac Therapies Improving Outcomes Network site primary investigators and co-primary investigators via email.

Of the 63 eligible sites contacted, responses were received from 24 unique North and South American cardiology centres. Access to neurodevelopmental providers, referral practices, and family neurodevelopmental education varied across sites. Inpatient neurodevelopmental care consults were available at many centres, as were inpatient family support services. Over half of heart centres had outpatient neurodevelopmental testing and individual psychotherapy services available to patients with ventricular assist devices, though few centres had outpatient group psychotherapy (12.5%) or parent support groups (16.7%) available. Barriers to inpatient and outpatient neurodevelopmental care included limited access to neurodevelopmental providers and parent/provider focus on the child’s medical status.

Paediatric patients with ventricular assist devices often have access to neurodevelopmental providers in the inpatient setting, though supports vary by centre. Strengthening family neurodevelopmental education, referral processes, and family-centred psychosocial services may improve current neurodevelopmental/psychosocial care for paediatric ventricular assist device patients.

This research seeks to ascertain the prevalence and determinants of mirror-image dextrocardia in fetuses

With December 2022 as the reference point, we compiled colleted data on pregnant women who carried fetuses with mirror-image dextrocardia in Xi’an, Shaanxi Province: September–October 2022, November 2022, and December 2022–January 2023. An online questionnaire was distributed to 209 pregnant across China who had contracted COVID-19. The case group comprised women whose final menstrual cycle occurred in November 2022 and who had a fetus with mirror-image dextrocardia. Women with a November 2022 final menstrual period and a fetus without this condition made up the control group. To identify the risk factors associated with fetal mirror-image dextrocardia, both univariate and multivariate logistic regression analyses were employed.

A significant difference was noted in the gestational age at COVID-19 infection women with a September to October 2022 and December 2022 to January 2023 final menstrual period who did not bear a fetus with mirror-image dextrocardia, and those with a November 2022 final menstrual period whose fetus exhibited this condition. The univariate and multivariate analyses conducted on pregnant women with a final menstrual period in November 2022 who had contracted COVID-19 revealed significant differences in the presence and duration of fever between those bearing fetuses with mirror-image dextrocardia and those without (P = 0.000).

The findings suggest two critical factors to the increased prevalence of fetal mirror-image dextrocardia: 1) the infection timing which occurs between the 4th and 6th week of pregnancy and 2) the presence of fever and its prolonged duration.

Alternate electrocardiogram acquisition with fewer leads lacks systematic evaluation in children. This study aims to determine if electrocardiograms with fewer leads maintain diagnostic accuracy in paediatrics.

This is a single-centre review of 200 randomly selected standard 12-lead electrocardiograms from our hospital database (2017–2020) for patients aged 2 weeks to 21 years. An overlay technique generated 8-lead (limb + V1/V6) and 6-lead (limb only) variations of the 12-lead tracings, resulting in a total of 600 electrocardiograms, which were then interpreted by two independent paediatric electrophysiologists.

In total, 18% (35/200) of the baseline electrocardiograms were abnormal. Intervals were measured in lead II for all electrocardiograms. Comparing 12-lead to 6- and 8-lead electrocardiograms, there was almost perfect agreement for specific rhythm identification (97.5–100%, κ 0.85-1). The 8-lead showed substantial agreement with 12-lead electrocardiograms when identifying specific electrocardiogram patterns (97.5–100%, κ 0.66–1). A similar degree of agreement was not demonstrated with the 6-lead variant. Utilising the 12-lead electrocardiogram as the gold standard, sensitivity and specificity of the 8- and 6-lead electrocardiogram were > 89% for specific rhythm identification. Specificity for specific pattern recognition was > 99% while sensitivity was < 90% for certain variables for both 6- and 8-lead electrocardiogram, likely due to smaller sample size and fewer abnormal electrocardiograms. There was high percent reader agreement (92.5–100%).

8-lead electrocardiograms provide comparable diagnostic accuracy to 12-lead electrocardiograms for children. This information holds potential for future technological advancements in electrocardiogram acquisition tailored specifically for paediatrics. Additional studies are required to further refine conventional electrocardiogram acquisition.

Giant coronary artery aneurysms and myocardial fibrosis after Kawasaki disease may lead to devastating cardiovascular outcomes. We characterised the vascular and myocardial outcomes in five selected Kawasaki disease patients with a history of giant coronary artery aneurysms that completely regressed.

Five patients were selected who had giant coronary artery aneurysm in early childhood that regressed when studied 12–33 years after Kawasaki disease onset. Coronary arteries were imaged by coronary CT angiography, and coronary artery calcium volume scores were determined. We used endocardial strain measurements from CT imaging to assess myocardial regional wall function. Calprotectin and galectin-3 (gal-3) as biomarkers of inflammation and myocardial fibrosis were measured by enzyme-linked immunosorbent assay.

The five selected patients with regressed giant coronary artery aneurysms had calcium scores of zero, normal levels of calprotectin and gal-3, and normal appearance of the coronary arteries by coronary computed tomography angiography. CT strain demonstrated normal peak systolic and diastolic strain patterns in four of five patients. In one patient with a myocardial infarction at the time of Kawasaki disease diagnosis at the age of 10 months, CT strain showed altered global longitudinal strain, reduced segmental peak strain, and reduced diastolic relaxation patterns in multiple left ventricle segments.

These patients illustrate that regression of giant aneurysms after Kawasaki disease is possible with no detectable calcium, normal biomarkers of inflammation and fibrosis, and normal myocardial function. Individuals with regressed giant coronary artery aneurysm still require longitudinal surveillance to assess the durability of this favourable outcome.

This study aims to evaluate the clinical characteristics and outcomes of children diagnosed with sinus node dysfunction.

This was a retrospective review of patients diagnosed with sinus node dysfunction in two tertiary paediatric cardiology centres in Turkey from January 2011 to June 2022.

In all, 77 patients (50, 64.9% males) were included, with a mean age of 8.2 ± 6.3 years and a mean weight of 28.2 ± 18.8 kg. While age-incompatible bradycardia and pauses were the most common rhythm disturbances, syncope, presyncope, and dizziness (n:33, 43%) were the most frequent initial symptoms. Structural heart disease was present in 58 (75.3%) of the 77 patients, 47 (61%) of whom were congenital. The most commonly associated CHDs were transposition of the great arteries (n:8), atrial septal defect (n:7), and atrioventricular septal defect (n:5). Seven of them also had left atrial isomerism. The remaining 19 patients were isolated. Four patients had SCN5A mutation (two of them were siblings) and two of them had Emery–Dreifuss muscular dystrophy.

Although sinus node dysfunction is rare in children, it has been diagnosed with increasing frequency with structural heart disease, especially in patients who have undergone corrective cardiac surgery related to atrial tissue. Since sinus node dysfunction can occur at any time postoperatively, these patients should be kept under constant control. If symptomatic sinus node dysfunction is confirmed, permanent pacing is an effective therapeutic modality.

Racial disparities in healthcare have been well documented in the United States. We hypothesise that there will be a racial variance in different clinical variables in single-ventricle patients through stages of palliation.

Retrospective single-centre study stratified all single-ventricle patients who reached stage 2 palliation by race: Black and White. Other races were excluded. Demographic and clinical characteristics were compared, alongside follow-up survival data. Primary outcomes were progression to Fontan and overall survival.

Among 526 patients, 325 (61.8%) were White, and 201 (38.2%) were Black. Median age at stage 2 palliation was 150 days for White and 165 for Black patients (p = 0.005), with similar weights. Black patients exhibited higher median cardiopulmonary bypass times (87 vs. 74 minutes, p = 0.001) and a greater frequency of genetic syndromes (30.1% vs. 22.1%, p = 0.044). No significant differences were observed in outcomes between groups from stage 2 to stage 3, pre-stage 3 cardiac catheterisation variables, or perioperative outcomes. Multivariable regression analysis identified hypoplastic pulmonary arteries as the risk factor for mortality after stage 2. Survival analysis showed no difference in survival by race; however, occurrence of combined cardiovascular event was significantly higher in Black race.

Significant racial disparities exist among single-ventricle patients regarding the timing of stage 2 palliation, cardiopulmonary bypass duration, and frequency of genetic syndromes. Black race was a risk factor for sub-optimal long-term outcome, although perioperative mortality was similar. These race-related factors warrant further studies to improve our understanding of the impact of race on outcomes.

Lipid levels in paediatric patients with anomalous aortic origin of a coronary artery (AAOCA) have not previously been explored. Patients with CHD have an increased risk of atherosclerotic cardiovascular disease later in life compared to the general population. We aim to characterise the lipid profiles in paediatric patients with AAOCA and explore its relation to diagnosis, race/ethnicity, and exercise.

Single institution retrospective cohort of 180 AAOCA paediatric patients (median age 13.7 years interquartile range 9.7–15.6, 66% male). Total cholesterol, HDL, LDL, triglycerides, total cholesterol to HDL ratio, and non-HDL cholesterol were evaluated across race/ethnicity, sex, type of AAOCA, documented ischaemia on imaging, exercise level, and surgery status. Normality of the data distribution for each lipid parameter was evaluated using Kolmogorov–Smirnov testing. Accordingly, Mann–Whitney U and t-tests were used to compare variables. The proportion of abnormal lipid levels by sex and race/ethnicity was calculated.

Total cholesterol was elevated in 29%, (51/177) of patients, HDL 37% (64/174), triglycerides 44% (72/165), LDL 16% (28/170), total cholesterol-HDL ratio 29%, (48/163), and non-HDL cholesterol 28% (47/165). Across subgroups categorised on the basis of surgery status, exercise level, AAOCA type, and sex, the mean and median levels for individual lipid parameters were normal. By race/ethnicity, Hispanic patients had significantly higher triglyceride (median 99, interquartile range 71–136.5, p = <0.001) and total cholesterol to HDL ratios (median 3.2, interquartile range 2.7–4.5, p = 0.014) versus non-Hispanic White and Black patients. Two-thirds of patients exercise recreationally.

Hispanic patients have significantly elevated triglycerides and total cholesterol to HDL ratios compared to others. Longitudinal follow-up evaluating differences in long-term lipid status in patients with AAOCA and risk for cardiovascular events is warranted.

Early recognition of cardiac involvement and prediction of disease prognosis are essential for the management of inflammatory diseases such as multisystem inflammatory syndrome. This study aimed to investigate the role of Pentraxin-3 levels in identifying cardiac involvement and evaluating disease severity in patients with multisystem inflammatory syndrome.

The study included 56 multisystem inflammatory syndrome patients and 26 healthy children as a control group. The multisystem inflammatory syndrome group was divided into those with cardiac involvement (n = 34) and those without (n = 22), as well as those with clinically mild-moderate (n = 30) and severe (n = 26) multisystem inflammatory syndrome. Blood samples for measurement of Pentraxin-3 levels were obtained from all patients before treatment and from the healthy controls.

In the patient group, the mean age was 8.2 ± 4 years (range: 2–17 years), and the male-to-female ratio was 1.8. In the control group, these values were 9.5 ± 3.7 years (range: 2–16 years) and 1.9, respectively (p > 0.05). Plasma Pentraxin-3 levels were significantly higher in multisystem inflammatory syndrome patients compared to controls (7.1 ± 5 ng/mL vs. 2.9 ± 2.1 ng/mL, p = 0.001). Patients with cardiac involvement had a significantly higher median Pentraxin-3 level than those without (5.8 ng/mL vs. 4.1 ng/mL, p = 0.004). Severe disease was also associated with a higher median Pentraxin-3 level compared to mild-moderate disease (6.1 ng/mL vs. 4.4 ng/mL, p = 0.001). Pentraxin-3 level was negatively correlated with left ventricular ejection fraction and positively correlated with B-type natriuretic peptide, troponin.

Elevated Pentraxin-3 levels in multisystem inflammatory syndrome patients may help predict the clinical course of the disease and cardiac involvement. However, larger-scale prospective studies are needed to further elucidate this.

The microRNA-200 family plays a key role in inflammatory and vascular processes, making it a relevant target for Kawasaki disease, a vasculitis with coronary complications. This study aimed to evaluate the diagnostic potential of urinary exosomal microRNA-200 family members in Kawasaki disease patients.

Urine samples from 15 Kawasaki disease patients and 15 healthy controls underwent total exosome isolation and high-throughput sequencing. Differential expression of microRNA-200 family members was validated using quantitative real-time polymerase chain reaction. Diagnostic potential was assessed via receiver operating characteristic analysis, and correlations with clinical parameters were evaluated using Spearman correlation.

High-throughput sequencing identified upregulation of microRNA-429, microRNA-200b-3p/5p, microRNA-141-3p, microRNA-200a-3p/5p, and microRNA-200c-3p in Kawasaki disease patients. We confirmed significant upregulation of microRNA-200a-3p/5p, microRNA-200b-3p/5p, and microRNA-429, with receiver operating characteristic analysis showing high diagnostic potential for these microRNAs (area under the curves of 0.844, 0.791, 0.942, 0.842, and 0.898, respectively) and a combined analysis yielding a perfect area under the curve of 1.000. MicroRNA-141 and microRNA-200c-3p/5p, however, showed no significant diagnostic value. MicroRNA-200a-3p and microRNA-200a-5p were positively correlated with white blood cells, platelet counts, and C-reactive protein, while microRNA-200b-3p and microRNA-429 were positively correlated with white blood cells, platelet counts, erythrocyte sedimentation rate, and C-reactive protein. microRNA-200b-5p showed moderate correlations with platelet counts and erythrocyte sedimentation rate.

Urinary exosomal microRNA-200 family members, especially microRNA-200a-3p/5p, microRNA-200b-3p/5p, and microRNA-429, demonstrate strong diagnostic potential for Kawasaki disease, correlating with key inflammatory markers. MicroRNA-141 and microRNA-200c did not demonstrate significant diagnostic utility.

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.

To assess the prevalence of obesity and investigate any changes in body mass index in children with CHD compared to age-matched healthy controls, in Southwestern Ontario.

The body mass index z-scores of 1259 children (aged 2–18) with CHD were compared with 2037 healthy controls. The body mass index z-scores of children who presented to our paediatric cardiology outpatient clinic from 2018 to 2021 were compared with previously collected data from 2008 to 2010. A longitudinal analysis of patients with data in both cohorts was also completed.

In total, 21.4% of patients with CHD and 26.6% of healthy controls were found to be overweight or obese (p < 0.001). The 2018–2021 cohort of CHD patients and controls had significantly higher body mass index z-scores compared to the 2008–2010 cohort (p < 0.001). Longitudinal analysis showed that body mass index z-scores significantly increased over time for CHD patients with data in both cohorts (2018–2021: M = 0.59, SD = 1.26; 2008–2010: M = −0.04, SD = 1.05; p < 0.001).

The prevalence of obesity in all children, irrespective of CHD, is rising. The coexistence of obesity and CHD may pose additional cardiovascular risks and complications.

Several organizations including the Environmental Protection Agency, World Health Organization and American Academy of Pediatrics recommend that hospital sound levels not exceed 45 decibels. Yet, several studies across multiple age groups have observed higher than recommended levels in the intensive care setting. Elevated sound levels in hospitals have been associated with disturbances in sleep, patient discomfort, delayed recovery, and delirium.

We measured sound levels in a pediatric cardiac intensive care unit and collected vital signs data, sedation dosing and delirium scores. During a 5-week study period, sound levels for 68 patients in 22 private and 4 semi-private rooms were monitored.

Sound levels were consistently above stated recommendations with an average daytime level of 50.6 decibels (maximum, 76.9 decibels) and an average nighttime level of 49.5 decibels (maximum, 69.6 decibels). An increase in average and maximum sound levels increased the probability of sedation administration the following hour (p-value < 0.001 and 0.01, respectively) and was predictive of an increase in heart rate and blood pressure (p-value < 0.001).

Sound levels in the CICU were consistently higher than recommended. An increase in heart rate, blood pressure and sedation utilization may suggest a stress response to persistent and sudden loud sounds. Given known negative impacts of excessive noise on stress, sleep, and brain development, as well as the similar adverse effects from the related use of sedative medications, reducing excessive and sudden noise may provide an opportunity to improve short- and long-term hemodynamic and neurodevelopmental outcomes in the pediatric cardiac intensive care unit.

Pulmonary atresia with intact ventricular septum is a rare congenital cardiac lesion with significant anatomical heterogeneity. Surgical planning of borderline cases remains challenging and is primarily based on echocardiography. The aim was to identify echocardiographic parameters that correlate with surgical outcome and to develop a discriminatory calculator.

Retrospective review of all pulmonary atresia with intact ventricular septum cases at a statewide tertiary paediatric cardiac centre was performed between 2004 and 2020. Demographic, clinical, and echocardiographic data were collected. Logistic regression was used to develop a discriminatory tool for prediction of biventricular repair.

Forty patients were included. Overall mortality was 27.5% (n = 11) and confined to patients managed as univentricular (11 vs 0, p = 0.027). Patients who underwent univentricular palliation were more likely to have an associated coronary artery abnormality (17 vs 3, p = 0.001). Fifteen surviving patients (51.7%) achieved biventricular circulation while 14 (48.3%) required one-and-a-half or univentricular palliation. Nineteen patients (47.5%) underwent percutaneous pulmonary valve perforation. No patients without tricuspid regurgitation achieved biventricular repair. The combination of tricuspid valve/mitral valve annulus dimension ratio and right ventricle/left ventricle length ratio identified biventricular management with a sensitivity of 93% and specificity of 96%. An online calculator has been made available.

Pulmonary atresia with intact ventricular septum is a challenging condition with significant early and interstage morbidity and mortality risk. Patient outcomes were comparable to internationally reported data. Right ventricle/left ventricle length and tricuspid valve/mitral valve annulus dimension ratios identified a biventricular pathway with a high level of sensitivity and specificity. Absent tricuspid regurgitation was associated with a univentricular outcome.