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.

To evaluate the acceptability and safety of educational videos utilising visual storytelling to provide information about the cardiac ICU and post-operative care to parents. Videos were designed to educate, further encourage parents to engage in their child’s cardiac care, and address common sources of distress.

Two educational videos and survey were sent to 29 families of children previously admitted to the cardiac ICU (April 2020–March 2021). Views regarding information quality, quantity, format, and relevance were assessed, as were parents’ emotional responses. Quantitative thresholds for safety and acceptability were set a priori. An inductive approach to content analysis was applied to identify themes in qualitative data.

Sixteen parents participated (response rate: 55%). All acceptability and safety thresholds were met; 92% of parents rated the videos as helpful and 85% were “very” or “extremely likely” to recommend them to other families of children with CHD. No participants reported significant distress after viewing the videos. Expressions of parental engagement with their child’s care team were common (92%). In qualitative responses, parents perceived the videos as potentially helpful in reducing distress if viewed prior to cardiac ICU admission.

Visual storytelling to orient parents to the cardiac ICU and address common stressors was found to be safe and acceptable when tested with parents of children previously admitted to the cardiac ICU. Further prospective studies are needed to test intervention effects when videos are viewed before or during cardiac ICU admission, especially for mitigating anxiety and traumatic stress associated with admission.

The main objective measure to assess the health of the Fontan circulation is the pressure measurement of the superior vena cava or pulmonary arteries. We reviewed the literature for benefits of measuring resting pressure in the Fontan circuit and explored whether dynamic measurement by volume loading or exercise has the potential to refine this diagnostic tool.

PubMed was searched for articles showing a relationship between resting post-operative central venous pressure or pulmonary artery pressure and Fontan failure. Relationships between post-operative central venous pressure or pulmonary artery pressure and volume loading changes, such as during exercise or volume loading during cardiac catheterization, were also queried.

A total of 44 articles mentioned relationships between resting central venous pressure or pulmonary artery pressure and Fontan failure. Only 26 included an analysis between the variables and only seven of those articles found pressure to be predictive of Fontan failure. Ten articles examined the relationship between exercise or volume loading and outcomes and demonstrated a large individual variation of pressures under these dynamic conditions.

Based on current literature, there is not a lot of strong evidence to show that elevated resting central venous pressure or pulmonary artery pressure is predictive of Fontan failure. Some individuals experience dramatic increases in central venous pressure or pulmonary artery pressure under increased loading conditions with exercise or bolus fluid infusion, while others experience increases closer to that of a healthy control population. Further studies are needed to examine whether more dynamic and continuous monitoring of systemic venous pressures might better predict outcomes in patients with Fontan circulation.

Pulmonary atresia with intact ventricular septum and critical pulmonary stenosis usually have to undergo treatment in the neonatal period. Compared to traditional surgical intervention, catheter-based cardiac interventions may achieve similar or superior outcomes for neonates with pulmonary atresia with intact ventricular septum and critical pulmonary stenosis. However, there is limited literature on anaesthesia techniques, challenges, and risks associated with cardiac catheterisation in this population.

This article retrospectively analysed the clinical data of pulmonary atresia with intact ventricular septum and critical pulmonary stenosis neonates who were treated with interventional cardiac catheterisation in our hospital from January 2015 to October 2022. Clinical outcomes considered were haemodynamic or pulse oxygen saturation instability, vasoactive requirements, prolonged intubation (>24 h postoperatively), and cardiovascular adverse events.

A total of 63 patients met the inclusion criteria. All patients survived the intervention. Among the patients with critical pulmonary stenosis, 40 successfully received percutaneous balloon pulmonary valvuloplasty, while three patients received ductal stenting due to moderate right ventricular dysplasia at the same time. For patients with pulmonary atresia with intact ventricular septum, 17 of the 23 patients successfully underwent percutaneous pulmonary valve perforation and percutaneous balloon pulmonary valvuloplasty. Of these, five patients underwent ductal stenting due to unstable pulmonary blood flow. Three patients only underwent ductal stenting. In addition, three patients received hybrid therapy.

There are various clinical techniques and risk challenges in the interventional cardiac catheterisation of neonatal pulmonary atresia with intact ventricular septum and critical pulmonary stenosis. However, by mastering the physiological and pathophysiological characteristics of the disease, adequately preparing for the perioperative period, and predicting the procedure process and potential complications, anaesthesia and surgical risks can be effectively managed.

To quantify the proportion of referrals sent to Crumlin Cardiology Department for cardiac screening prior to commencement or modifying attention deficit hyperactivity disorder medication and assess the number detected with a clinically significant abnormality.

A prospective audit was performed over a 6-month period, from November 2021 to April 2022 inclusive. Referrals sent via outpatient department triage letters, electrocardiogram dept. email, and walk-in electrocardiogram service were screened for those pertaining to commencing or modifying medication for children with attention deficit hyperactivity disorder. Each referral was coded against National Institute for Health and Care Excellence guidelines to determine the degree of clinical details given. Reported abnormalities, recommended management, and correspondence were recorded.

Ninety-one referrals were received during the 6-month audit period. More than half lacked a clinical indication for referral (53/91, 58.2%), with fewer than one third (26/91, 28.5%) meeting National Institute for Health and Care Excellence criteria for referral for cardiology. Eighty (80/91) referrals had clinical outcomes available for review (missing outpatient department information and age outside of service range accounted for eleven referrals with unavailable clinical outcomes). Of the eighty clinically reviewed referrals, seventy-two (72/80, 90%) were reported as normal with no cardiology follow up required. Eight referrals (8/80, 10%) were reviewed in the Cardiology Outpatient Department prior to commencement or modifying attention deficit hyperactivity disorder medication. Of these, only one (1/80 1%) had a clinically significant abnormality which was a potential contraindication to attention deficit hyperactivity disorder medication use, and this referral was appropriate as per National Institute for Health and Care Excellence guidelines.

Routine screening prior to attention deficit hyperactivity disorder medication prescription in the absence of clinical indications (as per National Institute for Health and Care Excellence) contributed to delays in medication initiation among young people with attention deficit hyperactivity disorder. Unnecessary referrals have resource implications for cardiology clinical team. Improved adherence to National Institute for Health and Care Excellence guidelines would provide benefits for patients and clinicians.

Interstage monitoring programs for single ventricle disease have been developed to reduce morbidity and mortality. There is increased use of telemedicine and mobile application monitoring. It is unknown if there are disparities in use based on patient socio-demographic factors.

We conducted a retrospective cohort study of patients enrolled in the single ventricle monitoring program and KidsHeart application at a single centre from 4/21/2021 to 12/31/2023. We investigated the association of socio-demographic factors with telemedicine usage, mobile application enrollment and usage. We assessed resource utilisation and weight changes by program era.

There were 94 children in the cohort. Patients with Norwood and ductal stent had higher mean telemedicine visits per month (1.8 visits, p = 0.004), without differences based on socio-demographic factors. There were differences in application enrollment with more Black patients enrolled compared to White patients (p = 0.016). There were less Hispanic patients enrolled than Non-Hispanic patients (p = 0.034). There were no Spaish speaking patient’s enrolled (p = 0.0015). There were no patients with maternal education of less than high school enrolled and all those with maternal education of advanced degree were enrolled (p = 0.0016). There was decreased mobile application use in those from neighbourhoods in the lowest income quartile. There were decreased emergency department visits with mobile application monitoring. Mean weight-for-age z-scores had increased from start to completion of the program in all eras.

Differences were seen in mobile application enrollment and usage based on socio-demographic factors. Further work is needed to ensure that all patients have access to mobile application usage.

Double-chambered right ventricle is a rare and progressive condition that is characterised by obstruction of the right ventricular tract. Double-chambered right ventricle is usually associated with ventricular septal defect. Early surgical intervention is recommended in patients with these defects. Based on this background, the present study aimed to review early and midterm outcomes of primary repair after double-chambered right ventricle.

Between January 2014 and June 2021, 64 patients with a mean age of 13.42 ± 12.31 years underwent surgical repair for double-chambered right ventricle. The clinical outcomes of these patients were reviewed and assessed retrospectively.

An associated ventricular septal defect was present in all the recruited patients; 48 (75%) patients of sub-arterial type, 15 (23.4%) of perimembranous, and 1 (1.6%) patient of muscular type. The patients were followed up for a mean period of 46.73 ± 27.37 months. During their follow-up, a significant decrease in the mean pressure gradient from 62.33 ± 5.52 mmHg preoperatively to 15.73 ± 2.94 mmHg postoperatively was observed (p < 0.001). Notably, there were no hospital deaths.

The development of double-chambered right ventricle in association with ventricular septal defect results in an increased pressure gradient within the right ventricle. The defect needs correction in a timely manner. In our experience, the surgical correction of double-chambered right ventricle is safe and shows excellent early and mid-term results.

CHD is associated with considerable burden of care. Up to one-third of babies born with CHD require surgery or intervention during the first year of life with an associated increased risk of surgical site infection. Pediatric wound care is informed largely by adult data, with no national or international guidelines available.

To examine pediatric cardiac surgical wound care practices reported by healthcare professionals Australia and New Zealand-wide.

A bi-national cross-sectional survey exploring pre-, intra- and post-operative wound practices was distributed using Exponential Non-Discriminative Snowball Sampling. Data were subject to descriptive analysis using SPSS Statistics 22.0.

Sixty-eight surveys representing all Australian and New Zealand pediatric cardiac surgical services were analyzed. Most respondents were experienced nurses. Pre-operative care varied greatly in practice and pharmaceutical agents used. Little consistency was reported for intra- and post-operative wound care. Nursing and medical staff shared responsibility for wound care. Wound photography was widely used, but only uploaded to electronic medical records by some.

These results highlight that wound care management is largely informed at an institutional level. The many practices reported are likely to reflect a range of factors including cardiac condition complexity, surgery, prematurity, and the presence of scar tissue. The importance of a research and training program, which is multimodal, available, and reflective, is highlighted.

These findings call for the establishment of a nurse-led program of research and education. The resultant suite of cardiac wound strategies could offer an effective and consistent pathway forward.

Children with CHD develop heart failure due to increased pulmonary blood flow, cyanosis, and pulmonary hypertension. The metabolic needs of these children differ from those of healthy children, and malnutrition is common. Protein-losing enteropathy has been reported in 5 to 13% of patients after the Fontan procedure. Serum albumin and total protein levels, which are indicators of the quality of post-operative care, can be useful tools for monitoring and examining the intensive care treatment strategies of these patients. In our retrospective study, the effects of albumin and total protein values, which are two of the markers that give us an idea about diet, nutritional status, and inflammation, on the prognosis of children who underwent the Fontan procedure were investigated.

In our study, 127 patients who underwent Fontan procedure in our clinic between 2012 and 2021 were analysed retrospectively. Of the patients, 52.7% (n = 67) were male and 47.3% (n = 60) were female. The mean age is 5.83 ± 4.63 years. Patients who underwent albumin replacement were not included in the study.

Although the relationship between pre-operative albumin and total protein values and post-operative mortality was not statistically significant, the inverse correlation of post-operative albumin 1st, 2nd, and 3rd-day values and post-operative total protein 1st, 2nd, and 3rd-day values with mortality was found to be statistically significant. In addition, we found that mortality was statistically high in patients whose total protein amount was below 6.65 mg/dl in the early post-operative period.

Albumin and total protein, whose blood levels can vary with diet, can be used as predictors in the early post-operative prognosis of Fontan patients. In addition, when we examined the exitus patients, it was observed that the total protein amount was below 6.65 mg/dl on the post-operative 1st day. Based on this, we think that a diet with high protein content before surgery will help reduce post-operative early mortality.

We assessed the effect of anomalous aortic origin of a coronary artery on the risk of early and late postoperative events after aortic or mitral valve replacement in adults.

Between 2005 and 2022, 29,579 adults underwent surgical aortic or mitral valve replacement at Cleveland Clinic. Among these, 29 had an unrepaired coronary artery rising anomalously from the aorta that was not intervened upon during valve surgery, 19 (65%) an anomalous circumflex, and 9 (31%) an anomalous right. Operative outcomes were compared between the 29 patients with anomalous coronary arteries and 87 balancing score (1:3) matched patients with normal coronary origin. Median follow-up was 6.5 years.

Among matched groups, major morbidity and mortality 24% (n = 7) in patients with anomalous coronaries and 20% (n = 17) among patients with normal coronary origin (P = .7). Ten-year freedom from coronary reintervention was 83% versus 100% (P[log-rank] = .005), and 10-year survival was 59% versus 53% (P[log-rank] = .8). One patient experienced a coronary injury from valve surgery, in which the incidentally found anomalous retroaortic circumflex was immediately repaired without further complication. There was no coronary reintervention after discharge in the normal coronary origin group and three in the anomalous coronary group; however, only one of these patients required intervention on the anomalous coronary.

Anomalous coronaries were uncommon in surgical valve replacement patients at a high-volume centre. The origin and course of each coronary should be assessed before valve replacement. With careful planning, valve replacement does not result in a significantly higher prevalence of postoperative ischaemia, mortality, or reintervention.

Derived from the National Pediatric Cardiology Quality Improvement Collaborative registry, the NEONATE risk score predicted freedom from interstage mortality or heart transplant for patients with single ventricle CHD and aortic arch hypoplasia discharged home following Stage 1 palliation.

We sought to validate the score in an external, modern cohort.

This was a retrospective cohort analysis of single ventricle CHD and aortic arch hypoplasia patients enrolled in the National Pediatric Cardiology Quality Improvement Collaborative Phase II registry from 2016 to 2020, who were discharged home after Stage 1 palliation. Points were allocated per the NEONATE score (Norwood type—Norwood/Blalock–Taussig shunt: 3, Hybrid: 12; extracorporeal membrane oxygenation post-op: 9, Opiates at discharge: 6, No Digoxin at discharge: 9, Arch Obstruction on discharge echo: 9, Tricuspid regurgitation ≥ moderate on discharge echo: 12; Extra oxygen plus ≥ moderate tricuspid regurgitation: 28). The composite primary endpoint was interstage mortality or heart transplant.

In total, 1026 patients met inclusion criteria; 61 (6%) met the primary outcome. Interstage mortality occurred in 44 (4.3%) patients at a median of 129 (IQR 62,195) days, and 17 (1.7%) were referred for heart transplant at a 167 (114,199) days of life. The median NEONATE score was 0(0,9) in those who survived to Stage 2 palliation compared to 9(0,15) in those who experienced interstage mortality or heart transplant (p < 0.001). Applying a NEONATE score cut-off of 17 points that separated patients into low- and high-risk groups in the learning cohort provided 91% specificity, negative predictive value of 95%, and overall accuracy of 87% (85.4–89.5%).

In a modern cohort of patients with single ventricle CHD and aortic arch hypoplasia, the NEONATE score remains useful at discharge post-Stage 1 palliation to predict freedom from interstage mortality or heart transplant.

Patients with univentricular hearts can only be palliated by a staged surgical procedure that carries a high morbidity and mortality risk. The aim of this study was to examine the emotional demands, psychosocial burden, and quality of life of parents with children with univentricular hearts compared to parents of children with a simple heart defect, those with no heart defect and children with chronic diseases.

An anonymous questionnaire was created to interview parents about their quality of life, stressors, needs, strategies for coping with illness, and partnership satisfaction.

73 families participated in the study. Parents of children with univentricular hearts experience a significantly higher psychosocial burden, limitations in daily life, and distress in family interactions, as well as greater emotional distress compared to the other study groups. When comparing the families of children with other chronic diseases (e.g. cystic fibrosis, chronic arthritis and diabetes), these differences remained significant.

The study confirms a higher psychosocial burden, restrictions in daily life and a lower quality of life of parents with children with univentricular hearts, compared to parents of children with simple heart defects and parents of heart-healthy children or those with other chronic diseases. Since this condition persists until adolescence and adulthood, the families are exposed to special challenges and stresses throughout their lives. This has yet to be adequately addressed in the management of these families.

Pain management is essential in the immediate post-surgical period. We sought to describe the ketorolac dose regimen in neonates and infants following cardiac surgery. Secondary outcomes included renal dysfunction, bleeding, and pain management.

We performed a single-centre retrospective cohort study of neonates and infants (aged < 12 months) who received ketorolac following cardiac surgery, from November 2020 through November 2021 (inclusive). Ketorolac was administered at 0.5 mg/kg every 6 hours. Safety was defined by absence of a clinically significant decline in renal function (i.e., increase in serum creatinine [SCr] by ≥ 0.3 mg/dL from baseline within 48 hours and/or urine output ≤ 0.5 mL/kg/hour for 6 hours) and absence of clinically significant bleeding defined as major by International Society on Thrombosis and Hemostasis paediatric criteria or Severe/Fatal Bleeding Events by Nellis et al. Efficacy measures included pain scores and opioid utilisation.

Fifty-five patients met eligibility criteria. The median (range) dose and duration of ketorolac administration was 0.5 mg/kg/dose for 48 (6–90) hours. Among all patients, there was not a statistically significant difference observed in median SCr within 48 hours of baseline (p > .9). There were no major or severe bleeding events. The median (range) opioid requirements (morphine intravenous equivalents per kg per day) at 48 hours post-ketorolac initiation was 0.1 (0–0.8) mg/kg/day.

If validated prospectively, these findings suggest that a ketorolac regimen 0.5 mg/kg/dose every 6 hours in neonates and infants post-cardiac surgery may be safe with regard to renal function and bleeding risk, and effective regarding opioid-sparing capacity.

Individuals with single ventricle physiology who are palliated with superior cavopulmonary anastomosis (Glenn surgery) may develop pulmonary arteriovenous malformations. The traditional tools for pulmonary arteriovenous malformation diagnosis are often of limited diagnostic utility in this patient population. We sought to measure the pulmonary capillary transit time to determine its value as a tool to identify pulmonary arteriovenous malformations in patients with single ventricle physiology.

We defined the angiographic pulmonary capillary transit time as the number of cardiac cycles required for transit of contrast from the distal pulmonary arteries to the pulmonary veins. Patients were retrospectively recruited from a single quaternary North American paediatric centre, and angiographic and clinical data were reviewed. Pulmonary capillary transit time was calculated in 20 control patients and compared to 20 single ventricle patients at the pre-Glenn, Glenn, and Fontan surgical stages (which were compared with a linear-mixed model). Correlation (Pearson) between pulmonary capillary transit time and haemodynamic and injection parameters was assessed using angiograms from 84 Glenn patients. Five independent observers calculated pulmonary capillary transit time to measure reproducibility (intraclass correlation coefficient).

Mean pulmonary capillary transit time was 3.3 cardiac cycles in the control population, and 3.5, 2.4, and 3.5 in the pre-Glenn, Glenn, and Fontan stages, respectively. Pulmonary capillary transit time in the Glenn population did not correlate with injection conditions. Intraclass correlation coefficient was 0.87.

Pulmonary angiography can be used to calculate the pulmonary capillary transit time, which is reproducible between observers. Pulmonary capillary transit time accelerates in the Glenn stage, correlating with absence of direct hepatopulmonary venous flow.

Timely prenatal diagnosis of CHD allows families to participate in complex decisions and plan for the care of their child. This study sought to investigate whether timing of initial fetal echocardiogram and the characteristics of fetal counselling were impacted by parental socio-economic factors.

Retrospective chart review of fetal cardiac patients from 1 January, 2017 to 31 December, 2018. We reviewed gestational age at first fetal echo, maternal age and ethnicity, zip code, rurality index, and hospital distance. Counselling was evaluated based on documentation regarding use of interpreter, time billed for counselling, and treatment option chosen.

Total of 139 maternal–fetal dyads were included, and 29 dyads had single-ventricle heart disease. There was no difference in income, hospital distance or rurality index, and first fetal echo timing. There was no significant difference between maternal ethnicity and maternal age, gestational age at initial visit, or follow-up. Patients in rural areas had increased counselling time (p < .05). There was no difference between socio-economic factors and ultimate parental choices (termination, palliative delivery, or cardiac interventions).

Oregon comprises a heterogeneous population from a large geographical catchment. While prenatal counselling and family decision-making are multifaceted, we demonstrated that dyads were referred from across the state and received care in a uniformly timely manner, and once at our centre received consistent counselling despite differences in parental socio-economic factors.

We aimed to assess cardiac autonomic balance with heart rate variability by using 24-hour Holter electrocardiography and also to assess susceptibility to ventricular arrhythmias by using microvolt T wave alternance in children with attention deficit hyperactivity disorder.

This study was conducted with age- and gender-matched groups of 40 patients taking long-acting methylphenidate for more than a year and 55 healthy controls. Heart rate variability analysis for cardiac autonomic functions and microvolt T wave alternance measurements for susceptibility to ventricular arrhythmias were evaluated by 24-hour Holter electrocardiography.

The mean age 10.9 ± 2.7 years, mean duration of therapy 22.76 months, and mean methylphenidate doses were 37.64 mg/day. The study group had considerably higher rMSSD, higher HF, and a lower LF/HF ratio (respectively, p : 0.02, p : 0.001 and p : 0.01). While parasympathetic activity parameters were elevated, sympathetic activity parameters were low during the sleep period. Increase in the microvolt T wave alternance values of the study group was not found to be statistically significant (p > 0.05).

In children taking long-acting methylphenidate, the autonomic balance was shown to be in favour of the parasympathetic system. Determination of the vulnerability to life-threatening ventricular arrhythmias has been evaluated for the first time in children with attention deficit hyperactivity disorder. Accordingly, microvolt T-wave alternance values give the notion that drug use is safe.