Glenn procedure is performed for patients with cyanotic CHD and univentricular physiology and has a survival rate above 90%.

To evaluate the risk factors associated with a poor outcome after Glenn procedure.

The data for this retrospective analysis were collected from a regional Brazilian registry of congenital heart surgeries (ASSIST initiative) from 2014 to 2019. Data from 97 patients who underwent the Glenn procedure were considered. The primary outcomes were prolonged mechanical ventilation (>24 hours post-operatively) or in-hospital death, alone and combined.

The overall in-hospital mortality was 13.4% (n = 13). Prolonged mechanical ventilation occurred in 52% (n = 51) of the patients. Our analysis found that risk factors for death or prolonged post-operative mechanical ventilation were cardiopulmonary bypass duration and post-operative lactate, whereas weight-for-age z-score and age at surgery were risk factors for prolonged mechanical ventilation. Cardiopulmonary bypass duration increased (adjusted odds ratio: 1.02; 95 % CI: 1.01, 1.03) and age at surgery decreased (adjusted odds ratio: 0.96; 95 % CI: 0.93, 0.99) the odds of the combined outcome.

Age at surgery, post-operative lactate, and low-weight-for-age z-score are associated with prolonged mechanical ventilation and death following the Glenn procedure.

Prognosis of fetuses with hydrops and tachyarrhythmia has been portrayed as poor in most published reports. This might lead to biased counselling, unnecessary caesarean section, preterm delivery, and even termination of pregnancy.

To evaluate contemporary fetal and postnatal outcomes of hydropic fetuses with fetal tachyarrhythmia when it is treated effectively and monitored systematically.

This is a retrospective review of a single centre experience at the University Hospital of Wales over a 20-year period. All fetuses received high doses of flecainide and digoxin combination treatment. Tachycardia response rate, time to arrhythmia and hydrops resolution, fetal and postnatal morbidity, and mortality rates were analysed.

Twenty fetuses were diagnosed with hydrops fetalis and received treatment. The mechanism of fetal tachyarrhythmia was supraventricular tachycardia in thirteen and atrial flutter in eight cases. Among the 20 fetuses treated, the overall tachycardia response rate was 90% (18/20) with the restoration of sinus rhythm in 85% (17/20) of the cases. The median time to restore sinus rhythm or to rate control of the arrhythmia was 1.5 days (range 12 hours to 13 days). Hydrops resolved in 17 of the 20 fetuses, with a median time of 12 days (range 3–21 days). Four fetuses went into spontaneous preterm birth and one fetus was delivered early due to worsening hydrops. No significant neurological morbidity was observed in surviving neonates and infants on clinical examination. There was one postnatal death due to respiratory complications of prematurity in the non-responsive supraventricular tachycardia case.

High-dose flecainide and digoxin combination offers effective treatment strategy in fetuses with hydrops and tachyarrhythmia with favourable outcomes. This study may guide more realistic counselling for pregnancies complicated by tachyarrhythmia and hydrops.

The pathophysiology of idiopathic chest pain remains unclear. Studies evaluating pain pressure thresholds in other idiopathic pain syndromes have revealed pain sensitivity in both affected and unaffected areas. The present study aimed to evaluate thoracic and extrathoracic pain pressure thresholds and their correlation with patients’ pain characteristics and quality of life.

This cross-sectional, single-blind, controlled study included children and adolescents with idiopathic chest pain. The patients’ pain characteristics, including their symptom duration, type of pain, mean pain intensity, mean duration of painful periods, pain frequency, and Pediatric Quality of Life Inventory Child Version scores, were assessed by a paediatric cardiologist via a face-to-face interview. Pain pressure thresholds were measured using an algometer by an algologist who was blinded to the study groups.

There was a statistically significant difference in pain pressure thresholds in the trapezius; supraspinatus; thoracic 2, 4, and 10 areas; deltoid; and tibia between the patient and healthy control groups. In the patient group, while there was a positive correlation between the mean all-region pain pressure thresholds and age (p = 0.047, r = 0.235), there was no correlation between pain pressure thresholds and symptom duration, pain intensity, and quality of life.

The present study is the first step towards investigating probable pain sensitivity mechanisms in children and adolescents with idiopathic chest pain. We noted lower thoracic and extrathoracic pain pressure thresholds in children and adolescents with idiopathic chest pain than in healthy controls.

In the absence of evidence-based guidelines, paediatric cardiologists monitor patients in the ambulatory care setting largely according to personal, patient, institutional, and/or financial dictates, all of which likely contribute to practice variability. Minimising practice variability may optimise quality of care while incurring lower costs. We sought to describe self-reported practice patterns and physician attitudes about factors influencing their testing strategies using vignettes describing common scenarios in the care of asymptomatic patients with tetralogy of Fallot and d-transposition of the great arteries.

We conducted a cross-sectional survey of paediatric cardiologists attending a Continuing Medical Educational conference and at our centre. The survey elicited physician characteristics, self-reported testing strategies, and reactions to factors that might influence their decision to order an echocardiogram.

Of 267 eligible paediatric cardiologists, 110 completed the survey. The majority reported performing an annual physical examination (66–82%), electrocardiogram (74–79%), and echocardiogram (56–76%) regardless of patient age or severity of disease. Other tests (i.e. Holter monitors, exercise stress tests or cardiac MRIs) were ordered less frequently and less consistently. We observed within physician consistency in frequency of test ordering. In vignettes of younger children with mild disease, higher frequency testers were younger than lower frequency testers.

These results suggest potential practice pattern variability, which needs to be further explored in real-life settings. If clinical outcomes for patients followed by low frequency testers match that of high frequency testers, then room to modify practice patterns and lower costs without compromising quality of care may exist.

T1 mapping is a recently developed imaging analysis method that allows quantitative assessment of myocardial T1 values obtained using MRI. In children, MRI is performed under free-breathing. Thus, it is important to know the changes in T1 values between free-breathing and breath-holding. This study aimed to compare the myocardial T1 mapping during breath-holding and free-breathing.

Thirteen patients and eight healthy volunteers underwent cardiac MRI, and T1 values obtained during breath-holding and free-breathing were examined and compared. Statistical differences were determined using the paired t-test.

The mean T1 values during breath-holding were 1211.1 ± 39.0 ms, 1209.7 ± 37.4 ms, and 1228.9 ± 52.5 ms in the basal, mid, and apical regions, respectively, while the mean T1 values during free-breathing were 1165.1 ± 69.0 ms, 1103.7 ± 55.8 ms, and 1112.0 ± 81.5 ms in the basal, mid, and apical regions, respectively. The T1 values were lower during free-breathing than during breath-holding in almost all segments (basal: p = 0.008, mid: p < 0.001, apical: p < 0.001). The mean T1 values in each cross section were 3.1, 7.8, and 7.7% lower during free-breathing than during breath-holding in the basal, mid, and apical regions, respectively.

We found that myocardial T1 values during free-breathing were about 3–8% lower in all cross sections than those during breath-holding. In free-breathing, it may be difficult to assess myocardial T1 values, except in the basal region, because of underestimation; thus, the findings should be interpreted with caution, especially in children.

To describe outcomes of acute coronavirus disease 2019 in paediatric and young adult patients with underlying cardiac disease and evaluate the association between cardiac risk factors and hospitalisation.

We conducted a retrospective single-institution review of patients with known cardiac disease and positive severe acute respiratory syndrome coronavirus 2 RT-PCR from 1 March, 2020 to 30 November, 2020. Extracardiac comorbidities and cardiac risk factors were compared between those admitted for coronavirus disease 2019 illness and the rest of the cohort using univariate analysis.

Forty-two patients with a mean age of 7.7 ± 6.7 years were identified. Six were 18 years of age or more with the oldest being 22 years of age. Seventy-six percent were Hispanic. The most common cardiac diagnoses were repaired cyanotic (n = 7, 16.6%) and palliated single ventricle (n = 7, 16.6%) congenital heart disease. Fourteen patients (33.3%) had underlying syndromes or chromosomal anomalies, nine (21%) had chronic pulmonary disease and eight (19%) were immunosuppressed. Nineteen patients (47.6%) reported no symptoms. Sixteen (38.1%) reported only mild symptoms. Six patients (14.3%) were admitted to the hospital for acute coronavirus disease 2019 illness. Noncardiac comorbidities were associated with an increased risk of hospitalisation (p = 0.02), particularly chronic pulmonary disease (p = 0.01) and baseline supplemental oxygen requirement (p = 0.007). None of the single ventricle patients who tested positive required admission.

Hospitalisations for coronavirus disease 2019 were rare among children and young adults with underlying cardiac disease. Extracardiac comorbidities like pulmonary disease were associated with increased risk of hospitalisation while cardiac risk factors were not.

CHD influences many aspects of life in affected individuals. Puberty, a major aspect of development, is a concern for patients and families.

We investigated pubertal status in children and adolescents with CHD.

Patients with CHD aged 6–18 were enrolled. Cardiac diagnoses were confirmed using history, examination, and paraclinical tools including echocardiography. An endocrinologist determined pubertal stages, and the second Tanner stages for pubarche (P2), thelarche (B2), and gonadarche (G2) were considered as the pubertal onset. A study with a large sample size on pubertal onset in a normal population was used for comparison.

Totally, 451 patients (228 girls and 223 boys) at a median (10th–90th percentile) age of 10.79 (8.02–14.28) years for the girls and 10.72 (8.05–14.03) years for the boys were enrolled. The median (10th–90th percentile) ages at B2 and P2 in the girls with CHD were 10.77 (9.55–12.68) and 10.53 (9.39–12.28) years, respectively, which were higher than the median ages of 9.74 (8.23–11.94) and 10.49 (8.86–12.17) years in the normal girls.

The median (10th–90th percentile) ages at G2 and P2 in the boys with CHD were 11.04 (8.85–13.23) and 11.88 (9.78–13.46) years, correspondingly, which were higher than the median ages of 9.01 (6.00–11.84) and 10.34 (6.84–13.10) years in the normal boys.

Pubertal onset could be delayed in children with CHD when compared with the normal population.

Recurrent laryngeal nerve injury leading to vocal cord paralysis is a known complication of cardiothoracic surgery. Its occurrence during interventional catheterisation procedures has been documented in case reports, but there have been no studies to determine an incidence.

To establish the incidence of left recurrent laryngeal nerve injury leading to vocal cord paralysis after left pulmonary artery stenting, patent ductus arteriosus device closure and the combination of the procedures either consecutively or simultaneously.

Members of the Congenital Cardiovascular Interventional Study Consortium were asked to perform a retrospective analysis to identify cases of recurrent laryngeal nerve injury after the aforementioned procedures. Twelve institutions participated in the analysis. They also contributed the total number of each procedure performed at their respective institutions for statistical purposes.

Of the 1337 patients who underwent left pulmonary artery stent placement, six patients (0.45%) had confirmed vocal cord paralysis. 4001 patients underwent patent ductus arteriosus device closure, and two patients (0.05%) developed left vocal cord paralysis. Patients who underwent both left pulmonary artery stent placement and patent ductus arteriosus device closure had the highest incidence of vocal cord paralysis which occurred in 4 of the 26 patients (15.4%). Overall, 92% of affected patients in our study population had resolution of symptoms.

Recurrent laryngeal nerve injury is a rare complication of left pulmonary artery stent placement or patent ductus arteriosus device closure. However, the incidence is highest in patients undergoing both procedures either consecutively or simultaneously. Additional research is necessary to determine contributing factors that might reduce the risk of recurrent laryngeal nerve injury.

Improvements in mortality after congenital heart surgery have necessitated a shift in focus to postoperative morbidity as an outcome measure. We examined late morbidity after congenital heart surgery based on prescription medication use.

Between 1953 and 2009, 10,635 patients underwent congenital heart surgery at <15 years of age in Finland. We obtained 4 age-, sex-, birth-time, and hospital district-matched controls per patient. The Social Insurance Institution of Finland provided data on all prescription medications obtained between 1999 and 2012 by patients and controls. Patients were assigned one diagnosis based on a hierarchical list of cardiac defects and dichotomised into simple and severe groups. Medications were divided into short- and long-term based on indication. Follow-up started at the first operation and ended at death, emigration, or 31 December, 2012.

Totally, 8623 patients met inclusion criteria. Follow-up was 99.9%. In total, 8126 (94%) patients required prescription medications. Systemic anti-bacterials were the most common short-term prescriptions among patients (93%) and controls (88%). Patients required betablockers (simple hazard ratio 1.9, 95% confidence interval 1.7–2.1; severe hazard ratio 6.5, 95% confidence interval 5.3–8.1) and diuretics (simple hazard ratio 3.2, 95% CI 2.8–3.7; severe hazard ratio 38.8, 95% CI 27.5–54.7) more often than the general population. Both simple and severe defects required medication for cardiovascular, gastrointestinal, psychiatric, neurologic, metabolic, autoimmune, and infectious diseases more often than the general population.

The significant risk for postoperative cardiovascular and non-cardiovascular disease warrants close long-term follow-up after congenital heart surgery for all defects.

In this study, we assessed the acute changes in biventricular longitudinal strain after atrial septal defect transcatheter closure and its relation to the device size.

Hundred atrial septal defect patients and 40 age-matched controls were included. Echocardiography and strain study were performed at baseline and 24 hours and 1 month after the intervention. The study group was divided into two subgroups; group 1: smaller devices were used (mean device size = 1.61 ± 0.05 cm, n = 74) and group 2: larger devices were used (mean device size = 2.95 ± 0.07 cm, n = 26).

At baseline, there was a significant difference between the study group and controls as regards right ventricular global longitudinal strain with significant hyperkinetic apex (p = 0.033, p = 0.020, respectively). There was a significant immediate reduction in right ventricular global longitudinal strain (from −24.43 ± 0.49% to −21.62 ± 0.47%, p < 0.001), which showed insignificant improvement after 1-month follow-up. While only left ventricular global longitudinal strain increased after 1 month. Within 24 hours of device closure, all the basal- and mid-lateral segments strains and apical right ventricular strains showed a significant reduction. There was a significant negative correlation between the indexed large device size and an immediate change in the right ventricular global longitudinal strain (r = −0.425, p = 0.034).

Significant right ventricular global longitudinal strain reduction starts as early as 24 hours after transcatheter closure, irrespective of the device size used. The rapid impact of closure was mainly on the biventricular basal and lateral segments and right ventricular apical ones, especially with the large sized atrial septal defect.

Cardiopulmonary exercise testing represents the diagnostic tool for determining cardiopulmonary function. Especially in small children, exercise testing is extremely challenging. To address this problem, field testing has been implemented using small mobile devices. This study aims at using this protocol for developing normal values for cardiopulmonary exercise testing in very young children.

Healthy children aged 4–8 years were recruited. All children were tested according to an outdoor protocol, in which they were instructed to walk, then run slowly, then a little harder and at last run at full speed. Each step lasted for 2 minutes, except the last step, in which the children were instructed to maintain as long as possible.

A total of 104 children (64 female/35 male, mean age 6.6 years) performed outdoor cardiopulmonary exercise testing using a mobile device. Almost all tests were completed successfully (95%). Despite a predominance of female study subjects, anthropometric values did not differ between boys and girls. V̇O2peak/kg, respiratory exchange ratio, VT1, heart rate at VT1, and time of exercise were also comparable between sexes. Generally, a tendency of higher maximal oxygen uptake could be observed in older children.

Open field mobile cardiopulmonary exercise testing represents a novel approach in very young children. In this study, we were able to determine normal values of maximal oxygen uptake and OUES/kg for 4–8-year-old children. The method is easy to achieve and safe.

Data regarding preoperative mortality in neonates with critical CHD are sparse and would aid patient care and family counselling. The objective of this study was to utilise a multicentre administrative dataset to report the rate of and identify risk factors for preoperative in-hospital mortality in neonates with critical CHD across US centres.

The Pediatric Health Information System database was utilised to search for newborns ≤30 days old, born 1 January 2009 to 30 June 2018, with an ICD-9/10 code for d-transposition of the great arteries, truncus arteriosus, interrupted aortic arch, or hypoplastic left heart syndrome. Preoperative in-hospital mortality was defined as patients who died prior to discharge without an ICD code for cardiac surgery or interventional catheterisation.

Overall preoperative mortality rate was at least 5.4% (690/12,739) and varied across diagnoses (d-TGA 2.9%, TA 8.3%, IAA 5.5%, and HLHS 7.3%) and centres (0–20.5%). In multivariable analysis, risk factors associated with preoperative mortality included preterm delivery (<37 weeks) (OR 2.3, 95% CI: 1.8–2.9; p < 0.01), low birth weight (<2.5 kg) (OR 3.8, 95% CI: 3.0–4.7; p < 0.01), and genetic abnormality (OR 1.6, 95% CI: 1.2–2.2; p < 0.01). Centre average surgical volume was not a significant risk factor.

Approximately 1 in 20 neonates with critical CHD suffered preoperative in-hospital mortality, and rates varied across diagnoses and centres. Better understanding of the factors that drive the variation (e.g. patient factors, preoperative care models, surgical timing) could help identify patient care improvement opportunities and inform conversations with families.

This study was conducted to evaluate the persisting Covid-19-related symptoms of the cases included in our study and to assess their cardiac findings to determine the impact of Covid-19 on children’s cardiovascular health.

In this study, 121 children between the ages of 0– and 18 with Covid-19 were evaluated based on their history, blood pressure values, and electrocardiography and echocardiography results. These findings were compared with the findings of the control group which consisted of 95 healthy cases who were in the same age range as the study group and did not have Covid-19. The results were evaluated using the statistics program, SPSS 21.

There was no significant difference between the study group and the control group in terms of age, weight, and body mass index. The clinical symptoms (chest and back pain, dizziness, headache, palpitation, fatigue, shortness of breath, loss of balance, coughing) of 37.2% of the cases persisted for at least 1 month after Covid-19 recovery. Statistically significant differences were found in systolic blood pressure, left ventricular ejection fraction, relative wall thickness, and tricuspid annular plane systolic excursion.

The continuation of some cases’ clinical symptoms post-recovery indicates that long Covid infection can be observed in children. The fact that statistically significant differences were observed between the echocardiographic parameters of the study and control groups suggests that Covid-19 may have effects on the cardiovascular system. To shed light on the long Covid cases among children and the infection’s cardiac impacts, it would be beneficial to conduct more comprehensive studies on this matter.

Women carrying a fetus diagnosed with congenital heart disease often experience significant distress because of their medical diagnosis. Given the well-documented impact associated with elevated prenatal stress and critical importance of developing targeted interventions, this study aims to examine stressors, coping and resilience resources, and mental health treatment preferences in pregnant women receiving a congenital heart disease diagnosis to inform the development of a psychological intervention to reduce maternal distress prenatally.

Three groups of participants were included consisting of two pregnant women carrying a fetus with congenital heart disease, five women of children (4−16 months) with congenital heart disease, and five paediatric cardiology medical providers. Responses were gathered via semi-structured interviews and analysed using qualitative thematic analysis.

Information regarding four broad areas were analysed of emotional distress during pregnancy; experience of initial diagnosis; coping and resilience; and perspectives on a mental health intervention in pregnancy. Anxiety regarding baby’s future, guilt following diagnosis, and various coping strategies emerged as primary themes among the participant sample. Medical staff corroborated mothers’ heightened anxiety and viewed a psychotherapeutic intervention during the prenatal period as essential and complimentary to standard of care.

We identified salient themes and preferred components for a future psychological intervention delivered prenatally.

Patients’ and providers’ perspectives regarding the nature of maternal distress, resilience and treatment preferences can inform the development of interventions to support the emotional well-being of pregnant women carrying a fetus with congenital heart disease to optimise care and potentially improve outcomes for fetal brain development.

Young adults with heart disease constitute a growing group with the risk of cognitive and physical impairment. The knowledge of their academic performance and mental and physical health is, however, scant. This study aimed to compare young adults with CHDs or arrhythmia with their peers.

Information on physical health (Somatic Symptom Scale-8), mental health problems (Hopkins Symptoms Checklist-25), quality of life (Satisfaction With Life Scale), physical activity, and academic performance was collected online in a national cross-sectional survey in Norway among students in higher education (the SHoT2018 study).

Among 50,054 students, 172 (0.34%) reported CHD and 132 (0.26%) arrhythmias. Students reporting arrhythmias scored significantly higher than the control group on somatic symptoms (OR = 2.3 (95% CI: 1.62–3.27)), anxiety (OR = 1.60 (1.08–2.37)), depression (OR = 1.49 (1.05–2.11)), self-harm, and suicide attempt (OR = 2.72 (1.56–4.75)), and lower quality of life (OR 1.64 (1.16–2.32)) and more loneliness (OR = 1.99 (1.28–3.10)) compared to participants without heart disease. Participants with CHD reported an increased somatic symptom burden (OR = 1.58 (1.16–2.16)). Despite a tendency to a higher score, this group did not differ significantly from the control group on anxiety or depression, quality of life, or loneliness. However, the risk of self-harm thoughts and suicidality was significantly increased (OR for suicide attempt 2.22 (1.3–3.77)). There was no difference between the groups on academic performance.

Although Norwegian students with heart disease reported more somatic symptoms, their academic progress was not reduced compared to students without heart disease. Students with CHD or arrhythmias showed an increased risk of self-harm thoughts and suicidality.

When encountering adolescents with chest pain and a high troponin level but with no underlying coronary artery illness, it is advisable to consider myopericarditis. Though myopericarditis is a self-limiting, benign condition, it nevertheless causes anxiety in the patient and the family.

Thirty-nine patients diagnosed with myopericarditis were included. We retrospectively analysed the demographic and clinical features, laboratory tests, echocardiography, electrocardiograms, MRI findings, coronary CT angiography, and conventional angiography findings in these patients.

Of the 39 patients (female/male = 4/35) aged 7–17 years, 66.6% had viral infection in the 2 weeks preceding presentation. Eleven patients were tested for high-sensitivity cardiac troponin I, 28 for high-sensitivity cardiac troponin T, and 10 patients were tested for both biomarkers. The median hs-TnI and hs-TnT values were 6.3 (0.05–29.9) ng/mL and 586 (51–9398) ng/L, respectively. Twenty-three patients showed ST changes on electrocardiography, of whom 11 had ST-elevation in the leads supporting left ventricular involvement. Coronary CT angiography and catheter angiography evaluations performed for differential diagnosis of coronary anomaly and acute coronary syndrome were normal. Cardiac MRI was conducted on 28 patients, and the results in 10 (35.7%) were suggestive of myopericarditis.

Myopericarditis is common in the adolescent age group and is generally benign but should be carefully monitored for differential diagnosis and possible complications. Cardiac MRI, which has been used more frequently in recent years, has an important role in differential diagnosis and the follow-up of patients.

Cardiovascular magnetic resonance serves as a useful tool in diagnosing myocarditis. Current adult protocols are yet to be validated for children; thus, it remains unclear if the methods used can be applied with sufficient image quality in children. This study assesses the use of cardiovascular magnetic resonance in children with suspected myocarditis.

Image data from clinical cardiovascular magnetic resonance studies performed in children enrolled in Mykke between June 2014 and April 2019 were collected and analysed. The quality of the data sets was evaluated using a four-point quality scale (4: excellent, 3: good, 2: moderate, 1: non-diagnostic).

A total of 102 patients from 9 centres were included with a median age (interquartile range) of 15.4(10.7-16.6) years, 137 cardiovascular magnetic resonance studies were analysed. Diagnostic image quality was found in 95%. Examination protocols were consistent with the original Lake Louise criteria in 58% and with the revised criteria in 35%. Older patients presented with better image quality, with the best picture quality in the oldest age group (13-18 years). Sedation showed a negative impact on image quality in late gadolinium enhancement and oedema sequences. No such correlation was seen in cardiac function assessment sequences. In contrast to initial scans, in follow-up examinations, the use of parametric mapping increased while late gadolinium enhancement and oedema sequences decreased.

Cardiovascular magnetic resonance protocols for the assessment of adult myocarditis can be applied to children without significant constraints in image quality. Given the lack of specific recommendations for children, cardiovascular magnetic resonance protocols should follow recent recommendations for adult cardiovascular magnetic resonance.