The timing for intervention in patients with significant chronic aortic regurgitation is based on adult guidelines and criteria which may not apply to children. There is limited data on the use of cardiac MRI parameters to guide surgical decision-making in paediatrics. We examined associations between MRI quantification of aortic regurgitation and left ventricular volumetric function and the need for surgical intervention.

Forty children and young adults with aortic regurgitation who had undergone cardiac MRI were divided into two groups based on aortic valve surgery (n = 20) or no surgery (n = 20). Ventricular volumetric functional parameters and aortic regurgitant volume and fraction were collected. Differences in MRI parameters between the groups were compared using unpaired t-tests. Receiver operating characteristic analysis identified MRI cut-off values with discriminatory ability towards primary end point of surgery (area under the curve > 0.7).

Patients who underwent surgery had significantly larger ventricular volumes and aortic regurgitant fraction than those without surgery. Aortic regurgitant fraction and volume had the highest discriminatory power (0.93 and 0.92, respectively) between the two groups, followed by indexed left ventricular volumes (end-diastolic volume 0.85 and end-systolic volume 0.89).

Current guidelines for surgical intervention in children with chronic aortic regurgitation are limited. Our findings suggest potential MRI-based threshold values that may aid in surgical decision-making and highlight the need future research for aortic valve surgery in children with chronic aortic regurgitation.

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.

The limited efficacy of monotherapy and the insufficient clinical experience with triple therapy (levosimendan, dapagliflozin, and sacubitril/valsartan) warrant further investigation. The aim of this study was to evaluate the effects of triple therapy on left ventricular function in children with advanced heart failure whose left ventricular function had not improved despite classical heart failure treatment and who remained dependent on inotropes.

The study included children who were admitted to the hospital with advanced heart failure and who were still inotrope-dependent at a mean of 42 days after the start of classical heart failure treatment and then started triple therapy at our hospital.

The study included 18 patients, 8 (44%) males, with a median age of 4 years (2–7 years). Before and after classical treatment and after triple treatment, statistically significant improvement in two-dimensional left ventricular ejection fraction (%) (median values 30; 38; 55, respectively), left ventricular end-diastolic diameter (median values 44; 45; 40 mm), left ventricular end-systolic diameter (median values 38; 36; 29 mm), left ventricular end-diastolic diameter (z score) (median values 4.2; 3.2; 2.7), left ventricular end-systolic diameter (z score) (median values 5.8; 4.8; 3.2), Simpson left ventricular ejection fraction (%) (median values 29; 36.5; 55), Simpson left ventricular end-diastolic volume (median values 60; 55; 43 ml), left ventricular end-systolic volume (median values 43; 40; 18. 5 ml), left ventricular global longitudinal strain four-chamber (median values -8.1;-10;-19), left ventricular global longitudinal strain three-chamber (median values -5.9;-8.9;-14), and left ventricular global longitudinal strain mean (median values -6.9;-9.7;-19) values was observed (all values p < 0.05).

In children admitted to the hospital with advanced heart failure whose left ventricular function has not improved with classical therapy, it seems likely that both left ventricular systolic and diastolic function will improve, inotrope dependency will resolve, and patients can be discharged with the new triple drug therapy.

Tetralogy of Fallot is the most common cyanotic congenital malformation of the heart. The right ventricular outflow tract is of great interest in this setting, but most of the focus on this feature has been on the size of the so-called pulmonary valvar “annulus”. We aimed to characterise other aspects of the morphology of the pulmonary root in heart specimens with tetralogy of Fallot.

We reviewed archived hearts with tetralogy of Fallot from four registries. The pulmonary root was examined with specific attention to the number of sinuses, the number of leaflets, presence of any fusion of leaflets, and the direction of the zone of apposition between the leaflets. Cluster analyses were then conducted to see if the features permitted segregation into groups.

We examined a total of 155 hearts. The pulmonary valve had two leaflets in 62%, three leaflets in 34%, and one leaflet in 3%. Irrespective of leaflet morphology, most hearts had two sinuses. Cluster analysis permitted segregation into three groups, with the direction of the zone of apposition being the most important feature for segregation.

In two-thirds of our hearts with tetralogy of Fallot, the pulmonary valve had two leaflets. Most frequently there were three sinuses. In the setting of a valve with two sinuses, the zone of apposition between the leaflets pointing towards the aorta. Cluster analysis permitted statistically sound segregation of the heart and highlights the importance of delineating these features, specifically the leaflet and sinus morphology, with clinical imaging.

This study aimed to evaluate the predictive accuracy of Paediatric Risk of Mortality-III, Paediatric Index of Mortality-II, and Paediatric Logistic Organ Dysfunction scoring systems for major adverse events following congenital heart surgery.

This prospective observational study included patients under 18 years of age who were admitted to the ICU for at least 24 hours postoperatively following congenital heart surgery. Major adverse events were defined as a composite of 30-day mortality, ICU readmission, reintubation, acute neurologic events, requirement for extracorporeal membrane oxygenation, cardiac arrest requiring cardiopulmonary resuscitation, need for a permanent pacemaker, acute kidney injury, or unplanned reoperation.

A total of 116 patients, with a median age of 17.5 months (interquartile range: 5.4–60.0) were included in the study. Major adverse events occurred in 34 patients (29.3%). Paediatric Risk of Mortality-III (11.5 [8.0–18.8] vs. 7.0 [2.3–11.0]; p = 0.001), Paediatric Index of Mortality-II (3.8 [2.8–6.6] vs. 2.2 [1.7–2.8]; p < 0.001), and Paediatric Logistic Organ Dysfunction (12.0 [10.0–21.0] vs. 1.0 [1.0-10.0]; p < 0.001) scores were significantly higher in patients with major adverse events than in those without. The Paediatric Logistic Organ Dysfunction score (area under the curve 0.83; 95% confidence interval: 0.74–0.92) demonstrated the highest discrimination capacity compared to Paediatric Risk of Mortality-III (area under the curve 0.70; 95% confidence interval: 0.60–0.81) and Paediatric Index of Mortality-II (area under the curve 0.77; 95% confidence interval: 0.66–0.88) with good calibration (Hosmer–Lemeshow p > 0.05 for all). Based on the logistic regression model evaluation metrics, Paediatric Logistic Organ Dysfunction demonstrated better performance in predicting major adverse events compared with Paediatric Risk of Mortality-III and Paediatric Index of Mortality-II.

The Paediatric Logistic Organ Dysfunction score outperformed the Paediatric Index of Mortality-II and Paediatric Risk of Mortality-III scores in predicting major adverse events in paediatric patients admitted to the ICU after congenital heart surgery.

Transcatheter pulmonary valve implantation has emerged as a minimally invasive and preferred therapeutic option for patients with dysfunction of previously repaired right ventricular outflow tracts. The Myval™ Octacor valve is a new device designed for this purpose, though limited reports exist regarding its use in the pulmonary position.

To report the immediate and short-term outcomes of percutaneous pulmonary valve implantation using the Myval™ Octacor valve in patients with severe right ventricular–pulmonary artery conduit or pulmonary valve bioprosthesis dysfunction.

This was a single-centre retrospective review of data obtained from case files.

The Myval™ Octacor valve was used in ten patients with a mean age of 34.5 ± 7.4 years. The median procedure duration and fluoroscopy time were 146 minutes and 30.5 minutes, respectively. The median Z-score for valves used was −0.5. The median right ventricular systolic pressure decreased from 68.5 mmHg pre-procedure to 33 mmHg post-procedure. The median peak instantaneous gradient across the right ventricular outflow tract or conduit decreased from 30 mmHg to 6.5 mmHg. There were no reported incidences of frame fracture, conduit rupture, device embolisation, or endocarditis.

This is the first UK experience of using the new-generation Myval™ Octacor valve in percutaneous pulmonary valve implantation. The results demonstrate the valve’s safety and clinical efficacy, with favourable outcomes in terms of procedural success, haemodynamic improvement, and echocardiographic findings.

Neurodevelopmental follow-up programmes for children with CHD help identify neurodevelopmental impairments and support the delivery of recommended interventions. The Cardiac Neurodevelopmental Outcome Collaborative, Diversity, Equity, and Inclusion Special Interest Group surveyed members to assess perceived patient barriers to neurodevelopmental follow-up, previous diversity and inclusion education, and confidence in caring for historically marginalised populations.

A link to a Redcap online survey was emailed to Cardiac Neurodevelopmental Outcome Collaborative members on 23 April 2022, with 4 weeks given to complete.

Eighty-four participants from 37 institutions in North America completed the survey. Respondents acknowledged that education on the importance of neurodevelopmental follow-up and frequent accommodations for cancellations or rescheduling clinic visits is essential. Language interpretation and written materials were available in languages other than English, but a limited number provided fully translated evaluation reports. Driving distance and the caregiver’s lack of understanding of the rationale for neurodevelopmental follow-up were the top perceived barriers to programme attendance. At the institutional level, training for cultural competency was typically provided, and most respondents felt comfortable caring for patients from diverse backgrounds. However, many agreed their programmes could do more to make evaluations accessible to historically marginalised/underserved populations.

Multiple barriers exist to cardiac neurodevelopmental follow-up, particularly for patients from under-represented minorities and for those whose primary language is not English. Surveying families will be valuable to understand how we may overcome these barriers. Further education about the importance of neurodevelopmental follow-up programmes continues to be essential.

Guidelines recommend screening for psychiatric co-morbidities in patients with congenital heart defects alongside cardiac outpatient follow-ups. These recommendations are not implemented in Denmark. This study aimed to investigate the psychiatric co-morbidities in children and adolescents with Fontan circulation in Denmark and to evaluate the feasibility of an online screening measure for psychiatric disorders.

Children, adolescents, and their families answered the Development and Well-Being Assessment questionnaire and a questionnaire about received help online. Development and Well-Being Assessment ratings present psychiatric diagnoses in accordance with ICD-10 and DSM-5. Parent-reported received psychiatric help is also presented. Feasibility data are reported as participation rate (completed Development and Well-Being Assessments) and parental/adolescent acceptability from the feasibility questionnaire.

The participation rate was 27%. Of the participating children and adolescents, 53% (ICD-10)/59% (DSM-5) met full diagnostic criteria for at least one psychiatric diagnosis. Of these, 50% had not received any psychiatric or psychological help. Only 12% of participants had an a priori psychiatric diagnosis.

We found that a large proportion of children and adolescents with Fontan circulation are underdiagnosed and undertreated for psychiatric disorders. The results from our study emphasise the need for psychiatric screening in this patient group. Development and Well-Being Assessment may be too comprehensive for online electronic screening in children and adolescents with CHD.

Patients with cyanotic CHD and those with metabolic dysfunction–associated steatotic liver disease are at risk of liver fibrosis. We compared hepatic extracellular volumes and native T1 values to better understand the burden of liver disease in these populations.

The sample comprised 136 patients in 5 groups: control (n = 23), metabolic dysfunction–associated steatotic liver disease [mild (F0–F1) and significant (F2–F4) fibrosis; n = 45], repaired tetralogy of Fallot (n = 30), and Fontan circulation (n = 38). Differences were assessed using linear regression models, with adjustment for the body mass index and sex.

The hepatic extracellular volume was significantly larger in the Fontan group (43.96% ± 4.22%) than in the other groups, even with adjustment. Patients with Fallot had significantly larger extracellular volumes (36.77% ± 5.63%) than did controls and mild liver disease (p < 0.001 and p = 0.011, respectively), although smaller extracellular volumes than patients with significant liver disease (p = 0.042). These trends were corroborated by native T1 values, which were highest in patients with Fontan (1013.7 ± 86.1 ms), although not significantly different from patients with F2–F4 steatotic liver disease.

The potential burden of CHD-related hepatic injury and steatotic liver disease highlights the importance of early identification. Given the possible additional risk of liver fibrosis in patients with coexisting metabolic dysfunction and CHD, comprehensive clinical management should prioritise regular metabolic risk assessment and the promotion of a healthy lifestyle to reduce the likelihood of liver disease development in this vulnerable population.

Physiologic changes in the peripartum period put women with CHD at increased risk for morbidity. This study examines factors associated with peripartum complications and length of stay compared to patients without CHD.

This single-institution retrospective case-control study included women with CHD (2000–2017) and a control population without CHD. A review of clinical and echocardiographic data was used to assign baseline characteristics, disease severity, and adverse outcomes. Primary outcomes were composite variables of cardiac and obstetric adverse events, along with peripartum length of stay. The relationship between maternal CHD, baseline characteristics, and peripartum adverse events was evaluated by multivariable regression.

The cohort and control groups included 162 deliveries among 113 women and 321 deliveries among 321 women, respectively. Cardiac complications, including arrhythmia, heart failure, pulmonary oedema, and thromboembolic events, occurred in 8.6% of the cohort (RR 2.52, 95% CI 1.17–5.42), with the most common event being arrhythmia. Obstetric events, such as caesarean delivery, assisted vaginal delivery, preterm birth, and pre-eclampsia, occurred in 67.9% versus 56.1% in the control group (RR 1.21, 95% CI 1.05–1.40). In multivariable models, increasing age was associated with increased composite cardiac events. Length of stay was longer in the cohort group (p < 0.001) and significantly associated with modified World Health Organization classification (p = 0.016).

Women with CHD experience increased cardiac and obstetric morbidity compared to controls during peripartum admission. Those with CHD have longer hospital stays around delivery, which is associated with disease severity.

Despite patent ductus arteriosus closure in premature infants is a relatively fast procedure, it involves specific steps for equipment exchange and the use of a 4-Fr catheter, which may be large for tiny infants, potentially causing haemodynamic instability or tricuspid valve regurgitation.

This study aims to describe the early experience with a new technique designed to simplify transcatheter patent ductus arteriosus closure in premature infants. It enables the closure using the KA micro plug device via a single microcatheter without equipment exchanges and contrast exposure. This technique involves a simple modification of the microcatheter by creating a curved tip.

This is a retrospective, single-institution study involving premature infants who underwent attempted transcatheter patent ductus arteriosus closure using a manually modified microcatheter of the KA micro plug device at Stead Family Children’s Hospital.

Five patients underwent transcatheter patent ductus arteriosus closure using a KA micro plug with a modified microcatheter. The procedure was successful in all patients, with no haemodynamic instability. The median (range) for procedural weight, time, and radiation dose were 1055 g (730–1330), 12 minutes (11–19), and 0.49 µGy.m2 (0.14–1), respectively. At a median follow-up of 1.1 months (0.17–2.6), all patients maintained good device position without residual shunt or other concerning echocardiogram findings.

Transcatheter patent ductus arteriosus closure in infants weighing less than 1500 g can be successfully performed using a KA micro plug device through a microcatheter with zero-contrast use, low radiation exposure, and a short procedure time.

Scimitar syndrome is an uncommon congenital malformation of pulmonary venous drainage to the junction of the right atrium and inferior caval vein. Treatment is usually surgical, depending on the morphological variant. When there is dual drainage of the anomalous veins to the inferior caval vein and the left atrium, a transcatheter procedure may represent an alternative to surgery.

We report a series of four patients with the scimitar variant with dual pulmonary venous drainage treated with a transcatheter approach.

All four patients (three children, one adult) had dual drainage of right pulmonary veins into the inferior caval vein and to the left atrium via a connecting vein. All patients underwent a successful catheter occlusion of the anomalous connection to the systemic vein without complication. Vascular plug devices were used in two patients: a left atrial appendage closure device in one patient and a ventricular septal defect closure device in one patient. All the procedures resulted in complete occlusion of the anomalous venous drainage to the inferior caval vein and unobstructed drainage to the left atrium.

The treatment for proximal aortic arch hypoplasia in paediatric patients is still controversial. While some authors favours direct tissue anastomosis, others state that patch augmentation may also be a good alternative. The aim of this study is to compare the results of arch reconstructions using bovine pericardium with the direct anastomosis technique.

Paediatric patients who underwent arch reconstruction via median sternotomy between 2019 and 2023 were evaluated. Patients were divided into two groups according to the repair method of arch reconstructions: direct native tissue anastomosis and bovine pericardial patch augmentation. Using perioperative data, the relationship between the surgical method and postoperative morbidity, in-hospital mortality, and the risks for early reintervention was investigated.

Between August 2019 and August 2023, 38 paediatric patients underwent arch reconstruction. The average age and weight of the patients were 40 days (15–157.5 days, interquartile) and 3.78 kg (3.2–6.0 kg, interquartile range), respectively. While completely native tissue anastomosis was applied in 18 of the patients (47.4%), bovine pericardial patch was used in arch reconstruction in 20 patients (52.6%). Cross-clamp time was found to be significantly longer in patients using bovine patches (p = .016). No difference was detected between the two surgical methods in terms of postoperative mortality and morbidity factors (p > .05). There was no significant difference between the two surgical procedures in terms of reintervention in the early period after discharge (p = .177).

Although early results of both reconstruction techniques may be promising, their reliability needs to be evaluated in detail with large-scale prospective studies.

Amiodarone is a frequently used medication in paediatric patients to manage atrial and ventricular arrhythmias, but its acute haemodynamic effects, particularly in children, remain underexplored. This retrospective, single-centre study aimed to characterise the clinical impact of amiodarone intravenous infusions on heart rate, blood pressure, oxygen delivery, and transaminase levels within the first 48 hours of amiodarone initiation in paediatric cardiac ICU patients.

Single-centre, retrospective study of patients receiving amiodarone infusions, with measurements taken at baseline and at various intervals up to 48 hours after initiation. The primary outcome was the effect on heart rate, while secondary outcomes included blood pressure, arterial saturation, near-infrared spectroscopy values, central venous pressure, and transaminase levels. Several types of analysis models were employed to assess the results.

Data were collected from 87 paediatric patients. No significant changes in liver enzymes, blood pressure, or renal oxygen extraction were observed. These findings provide novel insights into the acute haemodynamic effects of amiodarone infusions in paediatric patients, suggesting that while amiodarone significantly lowers heart rate, it does not substantially affect oxygen delivery or necessitate increased vasoactive support.

Amiodarone infusions are associated with a significant decrease in heart rate without greatly impacting oxygen delivery or requiring increased vasoactive support. Heart rate decreases most until a cumulative dose of 30,000 mcg/kg, and heart rate decrease is most pronounced in those with ventricular tachycardia.

Coronary artery abnormalities in children that require bypass grafting are infrequent but represent a well-recognised entity with a broad spectrum of indications beyond Kawasaki disease. Although myocardial revascularisation in children is uncommon, studies have shown that it can yield favourable short- and long-term outcomes, allowing affected children to regain health and grow up to live normal lives.

Myocardial revascularisation in children is an extremely rare intervention in Western countries, accounting for less than 1% of all paediatric cardiac surgeries in this region. It is a highly technically demanding procedure that opens a new arena in cardiac surgery, for which cardiovascular surgeons need to be trained to achieve outcomes as good as those shown in the literature.

We present the experience of paediatric coronary artery bypass grafting in a middle-income country, with a wide range of indications.

A retrospective descriptive study was conducted on paediatric patients (under 18 years of age) who underwent coronary artery bypass grafting between 2004 and 2023 at a cardiovascular centre in Bogotá, Colombia. Data were collected from electronic medical records, including demographics, preoperative diagnoses, surgical details, and outcomes. Follow-up included clinical assessment and imaging with echocardiography. Ethical approval was obtained, and confidentiality was ensured.

Nine paediatric patients (ages 6–17) underwent coronary artery bypass grafting between 2004 and 2023. Kawasaki disease was the most common indication, but there are other aetiologies, including post-arterial switch coronary occlusion, anomalous origin of the LCA from the pulmonary artery, anomalous origin of coronary arteries from the aorta, Takayasu disease, and iatrogenic injury. The internal mammary artery was used in most cases, with successful completion of the planned revascularisation in all patients. There were no perioperative deaths or reinterventions. At a mean follow-up of 5.5 years, all patients showed clinical and biventricular improvement, and all grafts evaluated showed graft patency.

Paediatric coronary artery bypass grafting is a safe and effective treatment for selected congenital and acquired coronary pathologies, even in complex cases. Outcomes are optimised with the use of internal mammary arteries and a multidisciplinary heart team approach. In middle-income settings, favourable short- and mid-term results can be achieved despite follow-up challenges. Paediatric coronary artery bypass grafting should be considered a key component of congenital cardiac surgery training.

Pulmonary atresia with ventricular septal defect (PA/VSD) is a complex cyanotic CHD that requires an early diagnosis for optimal management and outcomes. The purpose of this study is to evaluate the effectiveness of an inter-hospital management protocol utilising the prenatal CHD diagnosis for achieving favourable postnatal outcomes in PA/VSD patients in Vietnam.

We described the protocol implemented between two tertiary medical settings in Vietnam for the prenatal diagnosis and postnatal management of PA/VSD infants. All PA/VSD patients with prenatal diagnosis between January 2016 and December 2022 were retrospectively reviewed. The primary outcome was postnatal survival, and the secondary outcome was the presence of major morbidities such as bleeding or the need for Extra Corporeal Membrane Oxygenation (ECMO) after total repair.

During the study period, 35 PA/VSD patients were identified including 29 infants who underwent surgical correction utilising a valved conduit and 6 infants who are still waiting for the next evaluation after the palliative surgery. No death prior to the surgery occurred. For 29 patients, one hospital death happened, two patients required ECMO initially in their postoperative course but both survived, one late mortality due to pneumonia, and three reoperations were due to conduit failure. In the mean follow-up time of 2.92 (0.51–7.92) years, all survivors had completed follow-up.

Our protocol including a multidisciplinary management and a close follow-up has shown promising short-term results in achieving favourable postnatal outcomes for PA/VSD patients.