Coeliac disease is an autoimmune intestinal disease that develops with permanent intolerance to gluten and similar cereal proteins. It can damage to many tissues, including myocardium, by autoimmune mechanisms. In our study, we aimed to investigate the effect of coeliac disease on cardiac electrical activity by comparing the Tp-e interval and Qt dispersion values of coeliac patients with healthy children.

Fifty-seven coeliac patients and 57 healthy children were included in the study. Sociodemographic findings, physical examinations, symptoms, laboratory values, dietary compliance, endoscopy, and pathological findings were recorded into a standardised form. Electrocardiogram parameters were calculated, and echocardiography findings were noted.

No statistically significant difference was found between the two groups in terms of age, gender, heart rate, electrocardiogram parameters such as p wave, PR interval, QRS complex, QT interval, and QTc values. Tp-e interval, Tp-e / QT ratio, and Tp-e / QTc ratio were statistically significantly higher in the patient group compared to the control group. Ejection fraction and fractional shortening values were significantly lower in the patient group compared to the control group. In the patient group, Tp-e interval, Tp-e / QT ratio, Tp-e / QTc ratio, and QTc dispersion were statistically significantly higher in patients with tissue transglutaminase IgA positive compared to patients with tissue transglutaminase IgA negative.

Our study gives important findings in terms of detecting early signs of future cardiovascular events in childhood age group coeliac patients.

In this single-centre prospective study, we aimed to evaluate the role of growth differentiation factor-15 in children with acute rheumatic fever.

The study group included 25 children with acute rheumatic fever, and the control group included 25 healthy children. In addition to routine laboratory tests used in the diagnosis and treatment of acute rheumatic fever, growth differentiation factor-15 levels of the study group (at the time of diagnosis and after the treatment) and the control group were assessed and compared.

The mean growth differentiation factor-15 level of the study group at the time of diagnosis (918.40 ± 605.65 pg/ml) was significantly higher than the mean post-treatment level (653.08 ± 330.92 pg/ml) (p = 0.015). Similarly, the mean growth differentiation factor-15 level of the study group at the time of diagnosis was significantly higher than the control group (p = 0.04). However, mean growth differentiation factor-15 levels were similar between the groups after the treatment. Growth differentiation factor-15 was positively correlated with both C-reactive protein (p < 0.001) and erythrocyte sedimentation rate (p = 0.001) at the time of diagnosis.

Growth differentiation factor-15 levels are significantly increased in children with acute rheumatic fever at the time of diagnosis and return to similar levels with healthy children after treatment. Growth differentiation factor-15 is positively and significantly correlated with erythrocyte sedimentation rate and C-reactive protein at the time of diagnosis.

As part of a quality improvement project beginning in October 2011, our centre introduced changes to reduce radiation exposure during paediatric cardiac catheterisations. This led to significant initial decreases in radiation to patients. Starting in April 2016, we sought to determine whether these initial reductions were sustained.

After a 30-day trial period, we implemented (1) weight-based reductions in preset frame rates for fluoroscopy and angiography, (2) increased use of collimators and safety shields, (3) utilisation of stored fluoroscopy and virtual magnification, and (4) hiring of a devoted radiation technician. We collected patient weight (kg), total fluoroscopy time (min), and procedure radiation dosage (cGy-cm2) for cardiac catheterisations between October, 2011 and September, 2019.

A total of 1889 procedures were evaluated (196 pre-intervention, 303 in the post-intervention time period, and 1400 in the long-term group). Fluoroscopy times (18.3 ± 13.6 pre; 19.8 ± 14.1 post; 17.11 ± 15.06 long-term, p = 0.782) were not significantly different between the three groups. Patient mean radiation dose per kilogram decreased significantly after the initial quality improvement intervention (39.7% reduction, p = 0.039) and was sustained over the long term (p = 0.043). Provider radiation exposure was also significantly decreased from the onset of this project through the long-term period (overall decrease of 73%, p < 0.01) despite several changes in the interventional cardiologists who made up the team over this time period.

Introduction of technical and clinical practice changes can result in a significant reduction in radiation exposure for patients and providers in a paediatric cardiac catheterisation laboratory. These reductions can be maintained over the long term.

We investigated the efficacy and complication profile of intranasal dexmedetomidine for transthoracic echocardiography sedation in patients with single ventricle physiology and shunt-dependent pulmonary blood flow during the high-risk interstage period.

A single-centre, retrospective review identified interstage infants who received dexmedetomidine for echocardiography sedation. Baseline and procedural vitals were reported. Significant adverse events related to sedation were defined as an escalation in care or need for any additional/increased inotropic support to maintain pre-procedural haemodynamics. Minor adverse events were defined as changes from baseline haemodynamics that resolved without intervention. To assess whether sedation was adequate, echocardiogram reports were reviewed for completeness.

From September to December 2020, five interstage patients (age 29–69 days) were sedated with 3 mcg/kg intranasal dexmedetomidine. The median sedation onset time and duration time was 24 minutes (range 12–43 minutes) and 60 minutes (range 33–60 minutes), respectively. Sedation was deemed adequate in all patients as complete echocardiograms were accomplished without a rescue dose. When compared to baseline, three (60%) patients had a >10% reduction in heart rate, one (20%) patient had a >10% reduction in oxygen saturations, and one (20%) patient had a >30% decrease in blood pressure. Amongst all patients, no significant complications occurred and haemodynamic changes from baseline did not result in need for intervention or interruption of study.

Intranasal dexmedetomidine may be a reasonable option for echocardiography sedation in infants with shunt-dependent single ventricle heart disease, and further investigation is warranted to ensure efficacy and safety in an outpatient setting.

Acute rheumatic fever is an autoimmune disease that develops due to streptococcal infection. The positive effect of breastfeeding on the development of the child’s immune system is well documented. In this study, we aimed to investigate the effect of breast milk intake period on the development of carditis.

Patients (n: 182) who were diagnosed with acute rheumatic fever between 2010 and 2019 were enrolled in the study. The patients were divided into groups according to carditis development. The demographic, socio-economic, and breastfeeding data were compared between groups.

The mean age of the patients was 10.5 ± 3.4, and 43.4 % (n: 79) of them were female. Independent predictors of the development of carditis in the first acute rheumatic fever episode were the number of children at home (OR: 1.773, CI 95%: 1.105, 2.845; p: 0.018) and breast milk intake less than 6 months (OR: 0.404, CI 95%: 0.174, 0.934; p: 0.034). Independent predictors of the development of carditis in any of the acute rheumatic fever episodes were the number of children at home (OR: 1.858, CI 95%: 1.100, 3.137; p: 0.021) and female gender (OR: 3.504, CI 95%: 1.227, 10.008; p: 0.019). The only independently predictor of the development of chorea during acute rheumatic fever was female gender (OR: 3.801, CI 95%: 1.463, 9.874; p: 0.006).

Although the occurrence of carditis is less common during the first acute rheumatic fever attack in patients with breast milk intake less than six months, this advantage is lost in recurrent attacks. This study showed that breast milk does not have a negative effect on acute rheumatic fever carditis.

This study aimed to analyse the results of paediatric aortic valve repairs in our institution.

The data of 57 patients under 18 years of age who underwent aortic valve repair between 2014 and 2019 were retrospectively analysed. Early postoperative reoperation and hospital mortality rates were evaluated based on the ages of the patients, their preoperative diagnoses, and the surgical techniques used. Survival curves for groups of patients were calculated by Kaplan–Meier analysis.

The rate of reoperation was 14% (n = 8), and there were no significant differences regarding valve pathologies and preoperative diagnoses (p > 0.05). Among the repair techniques, tricuspidisation was considered to be a risk factor for reoperation (p < 0.05). Augmentation, the material used (0.1 PTFE or pericardium), and the number of cusps were not found to have significant effects on reoperation or mortality. The mean follow-up period was 29.86 ± 21.30 months. The survival rates of the patients were 88%, 100%, and 88.2% for those with aortic stenosis, aortic insufficiency, and mixed disease, respectively, and no significant difference was found when these rates were evaluated with the log-rank test (p > 0.05). The mortality rate was 8.8% (n = 5), and undergoing surgery before the age of 1 year was found to be significant in terms of mortality (p = 0.032, p < 0.05). The bicuspid aortic valve group had the lowest mortality risk, while the Shone complex group had the highest.

With its acceptable reoperation and mortality rates, aortic valve repair should be the first choice of treatment in the paediatric age group. Early results were satisfactory in all groups.

Although serum lactate levels are widely accepted markers of haemodynamic instability, an alternative method to evaluate haemodynamic stability/instability continuously and non-invasively may assist in improving the standard of patient care. We hypothesise that blood lactate in paediatric ICU patients can be predicted using machine learning applied to arterial waveforms and perioperative characteristics.

Forty-eight post-operative children, median age 4 months (2.9–11.8 interquartile range), mean baseline heart rate of 131 beats per minute (range 33–197), mean lactate level at admission of 22.3 mg/dL (range 6.3–71.1), were included. Morphological arterial waveform characteristics were acquired and analysed. Predicting lactate levels was accomplished using regression-based supervised learning algorithms, evaluated with hold-out cross-validation, including, basing prediction on the currently acquired physiological measurements along with those acquired at admission, as well as adding the most recent lactate measurement and the time since that measurement as prediction parameters. Algorithms were assessed with mean absolute error, the average of the absolute differences between actual and predicted lactate concentrations. Low values represent superior model performance.

The best performing algorithm was the tuned random forest, which yielded a mean absolute error of 3.38 mg/dL when predicting blood lactate with updated ground truth from the most recent blood draw.

The random forest is capable of predicting serum lactate levels by analysing perioperative variables, including the arterial pressure waveform. Thus, machine learning can predict patient blood lactate levels, a proxy for haemodynamic instability, non-invasively, continuously and with accuracy that may demonstrate clinical utility.

Assessing the hepatic status of children with CHD is very important in the post-operative period. This study aimed to assess the usefulness of paediatric liver T1/T2 values and to evaluate the impact of respiration on liver T1/T2 values.

Liver T1/T2 values were evaluated in 69 individuals who underwent cardiac MRI. The mean age of the participants was 16.2 ± 9.8 years. Two types of imaging with different breathing methods were possible in 34 participants for liver T1 values and 10 participants for liver T2 values.

The normal range was set at 620–830 msec for liver T1 and 25–40 ms for liver T2 based on the data obtained from 17 healthy individuals. The liver T1/T2 values were not significantly different between breath-hold and free-breath imaging (T1: 769.4 ± 102.8 ms versus 763.2 ± 93.9 ms; p = 0.148, T2: 34.9 ± 4.0 ms versus 33.6 ± 2.4 ms; p = 0.169). Higher liver T1 values were observed in patients who had undergone Fontan operation, tetralogy of Fallot operation, or those with chronic viral hepatitis. There was a trend toward correlation between liver T1 values and liver stiffness (R = 0.65, p = 0.0004); and the liver T1 values showed a positive correlation with the shear wave velocity (R = 0.62, p = 0.0006).

Liver T1/T2 values were not affected by breathing patterns. Because liver T1 values tend to increase with right heart overload, evaluation of liver T1 values during routine cardiac MRI may enable early detection of future complications.

Cardiovascular disease is one of the most important problems in long-term follow-up for Noonan syndrome. We examined cardiovascular issues and clinical manifestations, with a focus on the cardiovascular disease and prognosis of patients with Noonan syndrome.

This single-centre study evaluated patients who were clinically and genetically diagnosed with Noonan syndrome.

Forty-three patients diagnosed with Noonan syndrome were analysed. The most prevalent responsible mutation was found in PTPN11 (25/43). The second and third most prevalent causative genes were SOS1 (6/43) and RIT1 (5/43), respectively, and 67.4% of genetically diagnosed patients with Noonan syndrome had structural cardiovascular abnormalities. Pulmonary valve stenosis was prevalent in patients with mutations in PTPN11 (8/25), SOS1 (4/6), and RIT1 (4/5). Hypertrophic cardiomyopathy was found in two of three patients with mutations in RAF1. There was no difference in the cardiovascular events or cardiovascular disease prevalence in patients with or without PTPN11 mutations. The proportion of RIT1 mutation-positive patients who underwent intervention due to cardiovascular disease was significantly higher than that of patients with PTPN11 mutations. Patients who underwent any intervention for pulmonary valve stenosis exhibited significantly higher pulmonary flow velocity than patients who did not undergo intervention, when they visited our hospital for the first time. All patients who underwent intervention for pulmonary valve stenosis had a pulmonary flow velocity of more than 3.0 m/s at first visit.

These findings suggest that genetic information can provide a clinical prognosis for cardiovascular disease and may be part of genotype-based follow-up in Noonan syndrome.

Beta-thalassemia major requires regular blood transfusions throughout life, which in turn leads to iron accumulation in the body. While cardiac T2* MRI is the gold standard in determining cardiac iron accumulation, it is not always feasible, which has led to the search for new biomarkers. Herein, the value of growth differentiation factor-15, galectin-3, and N-terminal pro-B-type natriuretic peptide in predicting cardiac iron accumulation is investigated in asymptomatic children with beta-thalassemia major.

Forty-one patients aged 11–21 years and 41 age-, gender-, body mass index-matched healthy controls were included. Serum growth differentiation factor-15, galectin-3, and N-terminal pro-B-type natriuretic peptide levels were compared between the patients and controls. Additionally, the relations of these biomarkers with cardiac and liver T2 * MRI were investigated in the patients.

In the patients, growth differentiation factor-15, galectin-3, and N-terminal pro-B-type natriuretic peptide levels were higher than healthy controls (p < 0.001, p = 0.025, p < 0.001, respectively). There were no significant correlations of growth differentiation factor-15 and N-terminal pro-B-type natriuretic peptide levels with both cardiac and liver T2 * MRI measurements. While there was no significant correlation of serum galectin-3 with cardiac T2 * MRI measurements, a negative correlation was found with liver T2 * MRI measurements (p = 0.040, rho = –0.325).

All three biomarkers investigated in this study failed to predict myocardial iron accumulation in asymptomatic children with beta-thalassemia major. However, a weak relation between serum galectin-3 level and hepatic iron accumulation was demonstrated.

To investigate UK variability in prenatal and postnatal management strategy of right aortic arch and double aortic arch (RAA/DAA).

Online surveys were sent to senior physicians (consultants) of the National Fetal Cardiology Working Group regarding prenatal diagnosis, counselling, and perinatal management of antenatally diagnosed RAA/DAA and to the British Congenital Cardiovascular Association regarding postnatal management strategies.

There were 28 prenatal and 90 postnatal surveys completed. Prenatally, there was consensus for potential associated chromosomal/genetic anomalies, but there was variation in the risk quoted. Confidence in defining aortic arch morphology was reported by 43% (12/28) of fetal cardiologists. There was variation in what was felt to be possible symptoms/signs of a compressive vascular ring, postnatal investigation, postnatal management, follow-up duration of asymptomatic patients, and indications for surgical intervention.

This study has highlighted important areas for future research: improving accuracy of prenatal diagnosis, clarification of potential symptoms, optimal investigation strategies, and indications for surgery.

The incidence of prosthetic valve implantation is increasing in the paediatric population. Prosthetic valve thrombosis leading to obstruction could potentially be a life-threatening complication. There is a debate regarding optimal management of this complication, and there is limited use of thrombolytic therapy in childhood in the setting of valve thrombosis.

We aim to share our experience of successfully using fibrinolytic therapy in terms of alteplase for paediatric prosthetic mitral valve thrombosis and to propose a management algorithm.

This retrospective analysis of the database was conducted at our hospital including patients who underwent thrombolysis (alteplase) for prosthetic mitral valve thrombosis from June, 2011 to June, 2021. A total of 10 patients with 20 attempts of alteplase infusion were found in our record.

Alteplase was successful in 19 attempts to relieve valve thrombosis. The safe and effective dose of alteplase was between 0.1 and 0.3 mg/kg/hour. There were no associated major bleeding complications and alteplase was administered either by central or peripheral line.

Thrombolysis by alteplase infusion was found to be successful in relief of prosthetic mitral valve thrombosis in paediatric population without major bleeding complications.