To describe the association between successful weaning of inhaled nitric oxide and trends in dead space ratio during such weans in patients empirically initiated on nitric oxide therapy out of concern of pulmonary hypertensive crisis.

Children in a cardiac intensive care unit initiated on inhaled nitric oxide out of clinical concern for pulmonary hypertensive crisis retrospectively over 2 years.

Twenty-seven patients were included, and nitric oxide was successfully discontinued in 23/27. These patients exhibited decreases in dead space ratio (0.18 versus 0.11, p = 0.047) during nitric oxide weaning, and with no changes in dead space ratio between pre- and post-nitric oxide initiation (p = 0.88) and discontinuation (p = 0.63) phases. These successful patients had a median age of 10 months [4.0, 57.0] and had a pre-existent diagnosis of CHD in 6/23 and pulmonary hypertension in 2/23. Those who failed nitric oxide discontinuation trended with a higher dead space ratio at presentation (0.24 versus 0.10), were more likely to carry a prior diagnosis of pulmonary hypertension (50% versus 8.7%), and had longer mechanical ventilation days (5 versus 12).

Patients empirically placed on nitric oxide out of concern of pulmonary hypertensive crisis and successfully weaned off showed unchanged or decreased dead space ratio throughout the initiation to discontinuation phases of nitric oxide therapy. Trends in dead space ratio may aid in determining true need for nitric oxide and facilitate effective weaning. Further studies are needed to directly compare trends between success and failure groups.

Transcatheter ventricular septal defect closure remains a complex procedure with potential complications like complete heart block and aortic regurgitation. The ideal device design for such intervention is still evolving.

To assess the safety, efficacy, and short-term outcome of ventricular septal defect closure using LifeTechTM multifunctional (KONAR-MFTM) VSD Occluder.

In a multicenre study, 44 patients with haemodynamically significant, restrictive ventricular septal defects underwent closure with the KONAR-MFTM device from April, 2019 to March, 2020. Clinical, echocardiographic, and angiographic data were collected and reviewed. Patients were followed up at 1, 3, 6, and 12 months.

The median age and weight were 8 (1.7–36) years and 20 (11–79) kg. Of 44 patients, 8 (18%) had a high muscular and 36 (82%) had a perimembranous defect, of which 6 had mild prolapse of the right coronary cusp. The median ventricular septal defect size was 8.8 (3.9–13.4) mm. A retrograde approach was adopted in 39 (88.6%) patients. Nine patients (20.5%) had a small residual leak and there was a slight increase in aortic regurgitation in one patient. One device, which embolised to pulmonary artery was retrieved, and the defect was closed with a larger device. At a median follow-up of 13 (5–18) months, the residual leak persisted in 1 (2.3%) patient. Mild aortic regurgitation in one patient remained unchanged. There were no major complications.

Percutaneous closure of ventricular septal defect using KONAR-MFTM device is safe and effective in short and midterm follow-up including selected patients with perimembranous defect and mild prolapse of the right coronary cusp.

Cyanotic CHD is one of many disorders in paediatrics that influence the health of children in different clinical aspects. One of the fundamental aspects that may be affected is bone mineral density.

The aim of our study is to assess bone mineral density in children with congenital cyanotic heart disease of different anatomical diagnoses.

Cross-sectional, observational study included 39 patients (20 males) with congenital cyanotic heart disease of different anatomical diagnoses following with the cardiology clinic in Mansoura University children’s hospital. All patients were subjected to anthropometric measures, oxygen saturation assessment, and lumber bone mineral density using dual-energy X-ray absorptiometry.

Six patients (15.4%) out of the 39 included patients showed bone mineral density reduction, 13 patients (33.3%) showed bone mineral density with Z-score between −1 and −2, while 20 patients (51.3%) showed bone mineral density with Z-score more than −1.

Low bone mineral density can be found in children with cyanotic CHD, making it important to consider bone mineral density assessment and early treatment if needed to avoid further complications.

Slide tracheoplasty for congenital tracheal stenosis (CTS) has been shown to improve post-operative outcomes, but the incidence and risk factors of vocal cord paralysis (VCP) following slide tracheoplasty remain unclear. This study aimed to review our experience of slide tracheoplasty for CTS with a focus on post-operative VCP.

Twenty-eight patients, who underwent tracheal reconstruction with or without cardiovascular repair at Kobe Children’s Hospital between June, 2016 and March, 2020 were enrolled in this retrospective observational study. They were divided into two groups based on the presence of a pulmonary artery sling (PA sling). Perioperative variables were compared between the two groups.

Twenty-one of the 28 patients underwent concomitant repair for associated cardiovascular anomalies, including 15 patients with PA sling. The overall incidence of VCP following slide tracheoplasty was 28.6%. The incidences of VCP were 46.7% in patients with CTS and PA sling, which were 14.3% in CTS patients without cardiovascular anomalies. The only risk factor associated with VCP following slide tracheoplasty was a concomitant repair for PA sling. Post-operatively, the duration of nasogastric tube feeding in patients with VCP was significantly longer than that in patients without VCP.

The incidence of VCP following slide tracheoplasty for CTS was high, especially in concomitant repair cases for PA sling. Routine screening and evaluation of VCP soon after post-operative extubation is required for its appropriate management.

In patients with right ventricular diastolic dysfunction after complete repair of tetralogy of Fallot, some employ the use of beta-blockade. The theoretical benefit of this therapy is felt to be one of the two: 1) reduction in heart rate with subsequent increase in diastolic filling time and stroke volume; 2) halting or reversal of right ventricular remodelling. This study aimed to characterise the use of beta-blockade in paediatric admissions with complete repair of tetralogy of Fallot and characterise the effects of beta-blockade on admission characteristics.

Admissions from 2004 to 2015 in the Pediatric Health Information System database with complete repair of tetralogy of Fallot were identified. Characteristics between admissions with and without beta-blockade were compared by univariate analysis. Next, regression analyses were conducted to determine the independent association of beta-blockade on length of admission, billed charges, cardiac arrest, and inpatient mortality while controlling for demographic variables and comorbidities.

A total of 3594 admissions were included in the final analyses. Of these, 371 employed beta-blockade. Admissions with beta-blockade were more likely to have heart failure and tachyarrhythmias. These admissions also tended to be longer by univariate analysis. Regression analyses demonstrated that beta-blockade was independently associated with a 2.8-day increase in length of stay and no statistically significant change in billed charges, cardiac arrest, or inpatient mortality.

Beta-blockade after complete repair of tetralogy of Fallot is associated with a longer length of stay but did not statistically significantly impact billed charges, cardiac arrest, or inpatient mortality.

The aim of this cross-sectional study was to examine the prevalence of the multidimensional phenomenon of fatigue in adults with congenital heart disease.

Adults with congenital heart disease are a growing population, and patient-reported outcomes can provide valuable information about the patient’s experience of living with CHD. Fatigue is a multidimensional phenomenon that can be described as an overwhelming feeling of exhaustion with a reduced capacity of mental and physical work. Fatigue can be observed clinically in adults with congenital heart disease, but the actual prevalence is unknown.

Fatigue was assessed by the Multidimensional Fatigue Inventory which enables the respondent to report the presence of fatigue according to five dimensions: “general fatigue,” “physical fatigue,” “mental fatigue,” “reduced motivation,” and “reduced activity.” The questionnaire was sent to 463 patients in Lund and Umeå. Four groups with complex CHD and two groups with moderately complex CHD were included. The reliability (internal consistency) of the Multidimensional Fatigue Inventory was tested for all dimensions and groups of diagnosis.

The response rate was 56.6% (n= 262). In patients with complex CHD, 40.0–59.4% reported severe to very severe general fatigue, and patients with a single ventricle reported the highest prevalence (59.4%). Among patients with complex CHD, 29.2–40.0% reported severe to very severe mental fatigue. The Multidimensional Fatigue Inventory had a high reliability measured with Cronbach’s alpha.

The study findings show a relatively high prevalence of fatigue in adults with congenital heart disease, and general fatigue was the most prevalent. Further studies are needed regarding fatigue and its causes and consequences in adults with congenital heart disease.

The Multidimensional Fatigue Inventory proved to be an instrument with high reliability and low internal loss, which suggests that the instrument may be suitable to use as a patient-reported outcome in the care of adults with congenital heart disease, preferably at repeated occasions.

Approximate Entropy is an extensively enforced metric to evaluate chaotic responses and irregularities of RR intervals sourced from an eletrocardiogram. However, to estimate their responses, it has one major problem – the accurate determination of tolerances and embedding dimensions. So, we aimed to overt this potential hazard by calculating numerous alternatives to detect their optimality in malnourished children.

We evaluated 70 subjects split equally: malnourished children and controls. To estimate autonomic modulation, the heart rate was measured lacking any physical, sensory or pharmacologic stimuli. In the time series attained, Approximate Entropy was computed for tolerance (0.1→0.5 in intervals of 0.1) and embedding dimension (1→5 in intervals of 1) and the statistical significances between the groups by their Cohen’s ds and Hedges’s gs were totalled.

The uppermost value of statistical significance accomplished for the effect sizes for any of the combinations was −0.2897 (Cohen’s ds) and −0.2865 (Hedges’s gs). This was achieved with embedding dimension = 5 and tolerance = 0.3.

Approximate Entropy was able to identify a reduction in chaotic response via malnourished children. The best values of embedding dimension and tolerance of the Approximate Entropy to identify malnourished children were, respectively, embedding dimension = 5 and embedding tolerance = 0.3. Nevertheless, Approximate Entropy is still an unreliable mathematical marker to regulate this.

With increased survival, children with CHD are reaching adulthood, however, obesity amongst this cohort is an emerging problem. Making every contact count encourages clinicians to utilise contact to elicit behaviour change. The aim of this work was to identify whether the body habitus of children classified as obese was addressed during a clinical review.

A retrospective observational cohort study was completed using a cardiology outpatient dataset from 2010 to 2019. Inclusion criteria are all children with a body mass index z score classified as obese (≥ 2 z scores). Individual electronic patient records were reviewed to identify long-term anthropometric measures including (i) recognition of body habitus, (ii) prescription of physical activity or dietary intervention, and (iii) referral to a weight management programme or dietitian.

From the cohort of 95 patients, 285 “obese clinical encounters” were identified, at the time of a cardiology clinic attendance. Of those, obesity was acknowledged in 25 clinic letters (8.65%), but only 8 used the correct terms “obese” or “obesity” (2.77%). Action to tackle obesity was recorded in 9.3% of cases with a direct referral to a dietitian being made on 3 occasions (1.04%).

Body habitus is not being routinely addressed by cardiologists caring for paediatric and young adult cardiac patients. This study has recognised an alarmingly high incidence of missed opportunities to make every contact count, to manage those with obesity and associated risk factors.

Recent studies confirm the role of B vitamins deficiency and hyperhomocysteinaemia in the development of dysautonomia that has been considered to be the main factor in vasovagal syncope development. The aim of the study was to investigate serum pyridoxine, folate, cobalamin, and homocysteine levels in children presenting with vasovagal syncope and to analyse the correlation between them and main clinical parameters of syncope.

We studied 40 children, ages 8–17 years with a history of vasovagal syncope and 24 healthy volunteers. The serum pyridoxine, folate, cobalamin, and homocysteine levels were measured by a quantitative sandwich enzyme immunoassay technique using a commercial kit (Monobind, USA). Twenty-four-hour Holter monitoring and 24-hour ambulatory blood pressure monitoring were conducted for all participated patients.

Serum pyridoxine (9.42 ± 4.87, 16.11 ± 5.53 µg/L) and cobalamin (307.48 ± 95.50, 447.28 ± 108.85 ng/L) levels were reasonably low (p < 0.05) in patients with vasovagal syncope. Although there was no significant change in folate levels between syncope and healthy children (4.00 ± 1.34, 4.71 ± 1.73 µg/L; p = 0.20), we detected low folate-level association with longer duration of syncope (r = −0.42) and post syncope (r = −0.43) symptoms (p < 0.05). Finally, there was increased serum homocysteine level (13.55 ± 5.03, 7.81 ± 1.71 µmol/L; p < 0.05) in patients with vasovagal syncope. It was positively correlated with the average PQ interval (r = 0.35, p < 0.05) and average QTc interval (r = 0.49, p < 0.05).

The results suggested that pyridoxine, folate, cobalamin, and homocysteine may be involved in the pathogenesis of vasovagal syncope. This might provide a new approach for effective treatment of paediatric vasovagal syncope, requiring further study.

This study explored the effects of condition notification based on virtual reality technology on the anxiety levels of parents of children with simple CHD.

The subjects of the study were the parents of 60 children with simple CHD who were treated in our centre. The state anxiety scale was used to assess the anxiety status of the parents of the children before and after the condition notification in different ways (based on virtual reality explanations and written explanations).

In this study, a total of 55 parents of children with CHD successfully completed the survey. At the time of admission, there was no significant difference in the state anxiety scale scores of the parents between the two groups. The anxiety status of the virtual reality group was relieved after the condition notification assisted by virtual reality technology, and there was a significant difference compared with the control group.

Condition notification based on virtual reality technology can effectively alleviate the anxiety of parents of children with simple CHD, which is worthy of clinical application.

The usefulness of ultra-fast track cardiac anaesthesia may give great benefits to patients; however, its usefulness has not been completely evaluated in infants and toddlers, who are generally considered the most difficult group for ultra-fast track cardiac anaesthesia.

A total of 130 children were allocated randomly into to a ultra-fast track cardiac anaesthesia group (Group D) or a conventional anaesthesia group (Group C) (each n = 65). In Group D, dexmedetomidine was administrated at a dosage of 1 µg/kg/hour after induction. The patient- controlled intravenous analgesia was dexmedetomidine and sufentanil. In Group C, patients were infused with of the same volume of normal saline, and sufentanil alone for patient-controlled intravenous analgesia. The dosages of sufentanil, extubation time, haemodynamic parameters, postoperative hospitalisation conditions, pain and sedation scores, blood gas analysis, and inotropic scores were all recorded.

The dosage of sufentanil (1.49 ± 0.05 vs. 3.81 ± 0.04 µg, p < 0.001) and extubation time (2.63 ± 0.52 vs. 436.60 ± 22.19 minutes, p < 0.001) in Group D were all significantly lower than those in Group C. Moreover, cardiac intensive care unit stay time, total hospital stay, hospitalisation costs, postoperative lactate levels, and inotropic scores were also significantly lower in Group D.

Using of ultra-fast track cardiac anaesthesia in infants and toddlers is effective, it not only reduce the perioperative requirement for opioids and shorten the extubation time but also decreases the inotrope requirement and provide a better postoperative condition for young children.

Anorexia nervosa has a prevalence of 0.5–3% in adolescents, placing this population at increased risk of cardiac anomalies including arrhythmias, pericardial effusion, and myocardial dysfunction. Our objective is to describe cardiovascular anomalies observed by tissue Doppler imaging in patients with anorexia nervosa.

We retrospectively reviewed electrocardiogram, Holter, and echocardiography findings in 28 patients diagnosed with anorexia nervosa.

Electrocardiogram was abnormal in 71% of patients with sinus bradycardia observed in 57%. Holter confirmed sinus bradycardia without significant pauses. Prolonged QTc, low voltage, and ectopic beats were each seen in 14% of patients. Wenckebach atrioventricular block was observed in one patient. Supraventricular or ventricular tachycardia was not observed. Echocardiography showed structurally normal heart in all patients. Pericardial effusion was seen in 7.1% of patients and left ventricular mass was decreased in 10.7%. Mean ejection fraction was 0.73 and mean fractional shortening was 38.4%. Tissue Doppler imaging revealed systolic or diastolic dysfunction in four patients with e’, a’, and s’ velocities in the lateral and septal basal segments more than two standard deviations below the mean. Two patients had decreased left ventricular mass but no significant difference in disease duration from the group. Basal segment velocities below one standard deviation were also observed in an additional seven patients.

A trend for decreased tissue Doppler imaging velocities was seen in 25.0% of patients, while significant systolic and diastolic dysfunction was seen in 14.3% of patients, associated with a significant reduction in left ventricular mass and independent of disease duration.

Low socio-economic status is associated with poorer quality of life among children with congenital heart disease (CHD), but this finding is based on disparities among children remaining under cardiology follow-up. We used a population-based health survey data set to analyse the impact of socio-economic status on health and functional status among children with CHD.

We used 2007–2018 National Health Interview Survey data, selecting children 2–17 years of age who had been diagnosed with CHD. Outcomes included caregiver-rated general health, presence of functional limitations, number of missed school days, need for special education, and need for special equipment related to the child’s health conditions. Socio-economic status measures included maternal educational attainment, food stamp programme participation, poverty status, and insurance coverage.

Based on a sample of 233 children with CHD, 10% had fair or poor health, 38% reported having any health-related limitation on their usual activities, 11% needed special equipment, and 27% received special education services. On multivariable analysis, lower maternal educational attainment was correlated with worse caregiver-rated health, and children without insurance were especially likely to experience functional limitations. Black children with CHD had significantly worse caregiver-rated health compared to White children (ordered logit odds ratio: 0.19; 95% confidence interval: 0.08, 0.45; p < 0.001).

In a population-based survey of children with CHD, race and several measures of socio-economic status disadvantage were associated with worse health outcomes. Further evaluation of social determinants of health during cardiology follow-up may help improve outcomes for children with CHD in socio-economically disadvantaged families.

Limited data exist on training of European paediatric and adult congenital cardiologists.

A structured and approved questionnaire was circulated to national delegates of Association for European Paediatric and Congenital Cardiology in 33 European countries.

Delegates from 30 countries (91%) responded. Paediatric cardiology was not recognised as a distinct speciality by the respective ministry of Health in seven countries (23%). Twenty countries (67%) have formally accredited paediatric cardiology training programmes, seven (23%) have substantial informal (not accredited or certified) training, and three (10%) have very limited or no programme. Twenty-two countries have a curriculum. Twelve countries have a national training director. There was one paediatric cardiology centre per 2.66 million population (range 0.87–9.64 million), one cardiac surgical centre per 4.73 million population (range 1.63–10.72 million), and one training centre per 4.29 million population (range 1.63–10.72 million population). The median number of paediatric cardiology fellows per training programme was 4 (range 1–17), and duration of training was 3 years (range 2–5 years). An exit examination in paediatric cardiology was conducted in 16 countries (53%) and certification provided by 20 countries (67%). Paediatric cardiologist number is affected by gross domestic product (R2 = 0.41).

Training varies markedly across European countries. Although formal fellowship programmes exist in many countries, several countries have informal training or no training. Only a minority of countries provide both exit examination and certification. Harmonisation of training and standardisation of exit examination and certification could reduce variation in training thereby promoting high-quality care by European congenital cardiologists.

Reflex-mediated syncope occurs in 15% of children and young adults. In rare instances, pacemakers are required to treat syncopal episodes associated with transient sinus pauses or atrioventricular block. This study describes a single centre experience in the use of permanent pacemakers to treat syncope in children and young adults.

Patients with significant pre-syncope or syncope and pacemaker implantation from 1978 to 2018 were reviewed. Data collected included the age of presentation, method of diagnosis, underlying rhythm disturbance, age at implant, type of pacemaker implanted, procedural complications and subsequent symptoms.

Fifty patients were identified. Median age at time of the first syncopal episode was 10.2 (range 0.3–20.4) years, with a median implant age of 14.9 (0.9–34.3) years. Significant sinus bradycardia/pauses were the predominant reason for pacemaker implant (54%), followed by high-grade atrioventricular block (30%). Four (8%) patients had both sinus pauses and atrioventricular block documented. The majority of patients had dual-chamber pacemakers implanted (58%), followed by ventricular pacemakers (38%). Median follow-up was 6.7 (0.4–33.0) years. Post-implant, 4 (8%) patients continued to have syncope, 7 (14%) had complete resolution of their symptoms, and the remaining reported a decrease in their pre-syncopal episodes and no further syncope. Twelve (24%) patients had complications, including two infections and eight lead malfunctions.

Paediatric patients with reflex-mediated syncope can be treated with pacing. Complication rates are high (24%); as such, permanent pacemakers should be reserved only for those in whom asystole from sinus pauses or atrioventricular block has been well documented.