This study aimed to explore relationships between parental stress, coping, and outcomes for parents of infants with CHD, via observational approach reflecting domains of the Parental Stress and Resilience in CHD (PSRCHD) model.

Fifty-five parents of 45 infants with CHD completed questionnaires with measures of parental stress, Problem-Focused Coping (PFC), Emotion-Focused Coping (EFC), Avoidant Coping (AC), mental health (symptoms of anxiety and symptoms of depression), post-traumatic growth (PTG) and quality of life (QoL). Demographic and infant clinical data were obtained.

Parental stress showed significant small to medium positive correlations with MH and PTG, but no significant correlations with QoL. EFC and AC showed significant small to medium positive correlations with MH, and medium negative correlations with parental QoL. EFC and PFC had significant small to medium correlations with PTG. PFC and AC had significant small to medium correlations with infant QoL. Hierarchical multiple regression analyses indicated that parental symptoms of anxiety, PTG, parental QoL, infant QoL were significantly predicted by models comprising of parental stress, coping styles, and clinical controls (adjusted R2 = 13.0–47.9%, p range < 0.001–.048), with results for parental symptoms of depression falling marginally above significance (adjusted R2 = 12.3%, p = .056).

Parental stress, coping styles, and length of hospital stay are related to psychological outcomes in parents of infants with CHD. Future research may use the PSRCHD framework to assess mechanisms underlying CHD parents’ stress and coping experiences and investigate longitudinal relationships between parental factors and parent and child outcomes.

Venovenous collaterals are abnormal connections between the systemic and pulmonary venous systems. They are commonly seen in the Fontan circulation and may lead to significant hypoxaemia. Transcatheter closure of venovenous collaterals is a potential but controversial treatment as the long-term benefits and outcomes are not well understood.

This retrospective cohort study utilised data from the Australian and New Zealand Fontan Registry. Patients who underwent transcatheter venovenous collateral occlusion for hypoxemia from the year 2000 onwards were included. Atriopulmonary and Kawashima-type Fontan circulations were excluded to reflect a more contemporary Fontan cohort.

Nineteen patients (age 19.3 ± 7.8 years, 53% female) underwent transcatheter venovenous collateral occlusion. Compared to baseline, mean oxygen saturation was improved at latest follow-up (90.5% vs 87.0%; p = 0.003). Nine patients achieved a clinically significant response (defined as an increase of at least 5% to 90% or greater), and this was associated with lower baseline Fontan pressures (12.9 v 15.6 mmHg; p = 0.02). No heart failure hospitalisations, arrhythmia, transplant referrals, or mortality were observed during the median follow-up period of 4 years. Two patients experienced thromboembolic events and five patients underwent re-intervention.

Transcatheter occlusion of venovenous collaterals in Fontan patients with chronic hypoxaemia resulted in a modest increase in oxygenation over a median follow-up of 4 years and longer-term prognosis did not appear to be adversely affected. Lower Fontan pressures at baseline were associated with a greater improvement in oxygenation.

The Duke Activity Status Index is used to assess an individual patient’s perception of their fitness abilities. It has been validated and shown to predict actual fitness in adults but has been studied less in the paediatric population, specifically those with heart disease. This study aims to assess if the Duke Activity Status Index is associated with measured markers of physical fitness in adolescents and young adults with heart disease.

This retrospective single-centre cohort study includes patients who completed a minimum of 12 weeks of cardiac rehabilitation between 2016 and 2022. Cardiac rehabilitation outcomes included physical, performance, and psychosocial measures. A comparison between serial testing was performed using a paired t-test. Univariable and multivariable analyses for Duke Activity Status Index were performed. Data are reported as median [interquartile range].

Of the 118 participants (20 years-old [13.9–22.5], 53% male), 33 (28%) completed at least 12 weeks of cardiac rehabilitation. Median peak oxygen consumption was 60.1% predicted [49–72.8%], and Duke Activity Status Index was 32.6 [21.5–48.8]. On Pearson’s correlation assessing the Duke Activity Status Index, there were significant associations with % predicted peak oxygen consumption (r = 0.49, p < 0.0001), 6-minute walk distance (r = 0.45, p < 0.0001), Duke Activity Status Index metabolic equivalents (r = 0.45, p < 0.0001), and dominant hand grip (r = 0.48, p < 0.0001). In multivariable analysis, the % predicted peak oxygen consumption (r = 0.40, p = 0.005) and dominant hand grip (r = 0.37, p = 0.005) remained statistically significant.

Duke Activity Status Index is associated with measures of physical fitness in paediatric and young adults with heart disease who complete a cardiac rehabilitation program.

The cause of most CHD is unknown and considered complex, implicating genetic and environmental factors in disease causation. The Kids Heart BioBank was established in 2003 to accelerate genetic investigations into CHD.

Recruitment includes patients undergoing interventions for CHD at The Children’s Hospital at Westmead. Informed consent is obtained from parents/guardians, and blood is collected at the time of cardiac intervention from which DNA is extracted and stored. Associated detailed clinical information and a family history are stored in the purpose-designed database.

To date, the Kids Heart BioBank contains biospecimens and associated clinical information from over 4,900 patients with CHD and their families. Two-thirds (64.1%) of probands have been included in research studies with 28.9% of participants who underwent genomic sequencing receiving a molecular diagnosis with direct clinical utility. The value of this resource to patients and families is highlighted by the high consent rate (94.6%) and the low withdrawal of consent rate (0.4%). The Kids Heart BioBank has supported many large national and international collaborations and contributed significantly to CHD research.

The Kids Heart BioBank is an invaluable resource and, together with other similar resources, the resulting research has paved the way for clinical genetic testing options for CHD patients, previously not possible. With research in the field moving away from diagnosing monogenic disease, the Kids Heart BioBank is ideally placed to support the next chapter of research efforts into complex disease mechanisms, requiring large patient cohorts with detailed phenotypic information.

Many paediatric studies report that patients must be established on aspirin therapy for a minimum of 5 days to achieve adequate response. This is not always practical especially in critical settings. Prospective identification of patients that are unresponsive to aspirin sooner could potentially prevent thrombotic events.

The aim of this study was to investigate prospectively if the first dose of aspirin is effective in decreasing platelet aggregation, and thromboxane formation and if this can be measured after 2 hours in paediatric cardiology patients. A secondary aim was to identify a cut-off for a novel marker of aspirin responsiveness the maximum amplitude with arachidonic acid, which could potentially dramatically reduce the blood volume required. Third, we aimed to prospectively identify potentially non-responsive patients by spiking a sample of their blood ex vivo with aspirin.

The majority (92.3%) of patients were responsive, when measured 2 hours post first dose of aspirin. Non-response or inadequate response (7.7%) can also be identified at 2 hours after taking the first dose of aspirin. Additionally, we have shown a novel way to reduce blood sample volume requirements by measurement of the maximum amplitude with arachidonic acid as a marker of response, particularly for monitoring.

These findings of rapid efficacy in the majority of patients offer assurance in a sound, practical way to attending clinicians, patients, and families.

Complications following the Fontan procedure include prolonged pleural drainage and readmission for effusions. To address these complications, a post-Fontan management pathway was implemented with primary goals of reducing chest tube duration/reinsertion rates and decreasing hospital length of stay and readmissions.

Fontan patients were identified by retrospective chart review (2017–2019) to obtain baseline data for chest tube duration/reinsertion rates, hospital length of stay, and readmission rates for effusion. A post-Fontan management pathway was implemented (2020–2021) utilising post-operative vasopressin, nasal cannula oxygen until chest tube removal, and discharge regimen of three times daily diuretics, sildenafil, and afterload reducing medications. Patients were followed to evaluate primary outcomes.

The pre- and post-pathway groups were similar in single ventricle morphology, demographics, and pre-operative haemodynamics. Forty-three and 36 patients were included in the pre- and post-pathway cohorts, respectively. There were statistically significant reductions in chest tube duration (8 vs. 5 days, p ≤ 0.001), chest tube output on post-operative day 4 (20.4 vs. 9.9 mL/kg/day, p = 0.003), and hospital readmission rates for effusion (13[30%] vs. 3[8%], p = 0.02) compared to baseline. There was an absolute reduction in hospital length of stay (11 vs. 9.5 days, p = 0.052). When combining average cost savings for the Fontan hospitalisations, readmissions for effusion, and cardiac catheterisations within 6 months of Fontan completion, there was a $325,144 total cost savings for 36 patients following pathway implementation.

Implementation of a post-Fontan management pathway resulted in significant reductions in chest tube duration and output, and readmission rates for effusion in the perioperative period.

The minimum diameter of the patent ductus arteriosus measured in the lateral angiographic view is usually used to determine the device size. Sometimes the device can be easily removed from the patent ductus arteriosus, even if it appears to be the optimum size.

From 2016 to 2021, 29 patients who underwent contrast-enhanced CT prior to patent ductus arteriosus closure included. Morphological evaluation of the narrowest part of the patent ductus arteriosus was performed on contrast-enhanced CT. We also examined whether there were differences in morphology depended on Krichenko classification, age, and the diameter of the narrowest portion of the patent ductus arteriosus.

At the time of treatment, the median age was 4.8 (range, 1–52) months, and the median weight was 5.0 (2.5–12.7) kg. The median minimum vertical diameter of patent ductus arteriosus was 2.9 (1.6–6.6) mm. The narrowest patent ductus arteriosus part in the contrast CT imaging showed horizontal-to-vertical diameter ratios in the range of 1.0–1.7, with no case where the vertical diameter was larger than the horizontal diameter. The median horizontal-to-vertical diameter ratio by Krichenko type was: A, 1.22; C, 1.29; E, 1.62(p = 0.017). When classifying the patients into a group aged under six months (n = 21) and a group aged six months or older (n = 8), the respective median horizontal-to-vertical diameter ratio was 1.34 and 1.15 (p = 0.027). The vertical patent ductus arteriosus diameter was not correlated with the elliptical shape.

Most patent ductus arteriosus cases have a horizontally oriented elliptical shape in this study. This characteristic showed high reproducibility and is important information that angiography cannot evaluate.

It is known established that the cardiac effects of COVID-19 infection are associated with poor prognosis and high mortality rates in infected patients. The aim of this study was to evaluate the cardiac effects of COVID-19 infection in paediatric patients and identify the correlations between clinical and laboratory data and the degree of cardiac involvement.

A retrospective data analysis was conducted on 64 paediatric patients at Gazi University Department of Pediatrics who were treated as inpatients with a diagnosis of COVID-19. Patients were classified as “COVID-19-related cardiac involvement cases” if their electrocardiogram and echocardiogram results indicated a pathology and/or if their laboratory data indicated increased cardiac enzymes. All patients were divided into subgroups based on whether they had cardiac involvement and whether they were diagnosed with multisystem inflammatory syndrome in children.

In comparison to patients who did not have cardiac involvement, those with cardiac involvement had significantly higher levels of hs-Troponin T, Pro-BNP, and D-dimer. Patients with multisystem inflammatory syndrome in children had significantly longer PR intervals than those without multisystem inflammatory syndrome in children (p = 0.0001). Patients with multisystem inflammatory syndrome in children had a significantly higher rate of pathological valve insufficiencies (68.1%) than those without multisystem inflammatory syndrome in children (14.2%) (p = 0.001).

In our study, the strongest predictive biomarker of cardiac involvement in paediatric patients with COVID-19 infection was determined to be hs-Troponin T. It was observed that pathologic electrocardiogram changes could reflect cardiac involvement in the absence of any other signs. Patients with multisystem inflammatory syndrome in children exhibited significantly greater rates of pathologic echocardiogram findings and myocardial dysfunction than those without multisystem inflammatory syndrome in children. In all patients, pathologic electrocardiogram and echocardiogram findings were found to be strongly associated with the severity of inflammation.

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.

With the rise of online references, podcasts, webinars, self-test tools, and social media, it is worthwhile to understand whether textbooks continue to provide value in medical education, and to assess the capacity they serve during fellowship training.

A prospective mixed-methods study based on surveys that were disseminated to seven paediatric cardiology fellowship programmes around the world. Participants were asked to read an assigned chapter of Anderson’s Pediatric Cardiology 4th Edition textbook, followed by the completion of the survey. Open-ended questions included theming and grouping responses as appropriate.

The survey was completed by 36 participants. When asked about the content, organisation, and utility of the chapter, responses were generally positive, at greater than 89%. The chapters, overall, were rated relatively easy to read, scoring at 6.91, with standard deviations plus or minus 1.72, on a scale from 1 to 10, with higher values meaning better results. When asked to rank their preferences in where they obtain educational content, textbooks were ranked the second highest, with in-person teaching ranking first. Several themes were identified including the limitations of the use of textbook use, their value, and ways to enhance learning from their reading. There was also a near-unanimous desire for more time to self-learn and read during fellowship.

Textbooks are still highly valued by trainees. Many opportunities exist, nonetheless, to improve how they can be organised to deliver information optimally. Future efforts should look towards making them more accessible, and to include more resources for asynchronous learning.

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.

Children with prolonged hospital admissions for CHD often develop delirium. Antipsychotic medications (APMs) have been used to treat delirium but are known to prolong the QTc duration. There is concern for prolongation of the QTc interval in cardiac patients who may be more vulnerable to electrocardiogram (ECG) changes and may have postoperative QTc prolongation already. The goal of this study was to determine the effect of APM on QTc duration in postoperative paediatric cardiac patients and determine the effect of quetiapine and risperidone in treating delirium and QTc prolongation.

Retrospective study, July 1, 2017–May 31, 2022.

Tertiary children’s hospital.

Included were patients admitted to the paediatric cardiac ICU at Children’s Healthcare of Atlanta.

None.

ECGs, delirium scores, and drug information were collected. Delirium was defined as Cornell Assessment of Pediatric Delirium (CAPD) score >9. Mixed effect models were performed to evaluate the effect of surgery on QTc change and the effect of antipsychotics on QTc and CAPD changes. There were 139 children, 55% male and 67% surgical admissions. Median age was 5.9 months. Mean QTc increased after cardiac surgery by 18 ms (p = 0.014, 95% CI 3.65–32.4). There was no significant change in QTc after antipsychotic administration (p = 0.064). The mean CAPD score decreased (12.5–7.2; p < 0.001). Quetiapine had the most improvement in delirium, and risperidone had the least improvement (77.8%, n = 14; 37.8%, n = 34, respectively; p = 0.002).

The QTc interval did not have a statistically significant change after the administration of antipsychotics, while there was improvement in the CAPD score. APMs may be administered safely without significant prolongation of the QTc and are an effective treatment for delirium.

The aim of this study was to review our institution’s experience with truncus arteriosus from prenatal diagnosis to clinical outcome.

and results: We conducted a single-centre retrospective cohort study for the years 2005–2020. Truncus arteriosus antenatal echocardiographic diagnostic accuracy within our institution was 92.3%. After antenatal diagnosis, five parents (31%) decided to terminate the pregnancy. After inclusion from referring hospitals, 16 patients were offered surgery and were available for follow-up. Right ventricle-to-pulmonary artery continuity was preferably established without the use of a valve (direct connection), which was possible in 14 patients (88%). There was no early or late mortality. Reinterventions were performed in half of the patients at latest follow-up (median follow-up of 5.4 years). At a median age of 5.5 years, 13 out of 14 patients were still without right ventricle-to-pulmonary artery valve, which was well tolerated without signs of right heart failure. The right ventricle demonstrated preserved systolic function as expressed by tricuspid annular plane systolic excursion z-score (−1.4 ± 1.7) and fractional area change (44 ± 12%). The dimensions and function of the left ventricle were normal at latest follow-up (ejection fraction 64.4 ± 6.2%, fractional shortening 34.3 ± 4.3%).

This study demonstrates good prenatal diagnostic accuracy of truncus arteriosus. There was no mortality and favourable clinical outcomes at mid-term follow-up, with little interventions on the right ventricle-to-pulmonary artery connection and no right ventricle deterioration. This supports the notion that current perspectives of patients with truncus arteriosus are good, in contrast to the poor historic outcome series. This insight can be used in counselling and surgical decision-making.