Complications from pulmonary hypertension are one of the leading contributors to morbidity and mortality post-cardiopulmonary bypass surgery in children with CHD. Pulmonary vasodilator therapies are commonly used post-operatively, but the optimal target patient population, therapy choice, timing of therapy initiation, and duration of therapy are not well defined.

We used PubMed and EMBASE to identify studies from 2000 to 2020 investigating the use of pulmonary vasodilator therapy post-cardiopulmonary bypass in children aged 0–18 years. To ensure eligibility criteria, studies were systematically reviewed by two independent reviewers.

We identified 26 studies of 42,971 children across four medication classes; 23 were single centre, 14 were prospective, and 11 involved randomisation (four of which employed a placebo-control arm). A disproportionate number of children were from a single retrospective study of 41,872 patients. Definitions varied, but change in pulmonary haemodynamics was the most common primary outcome, used in 14 studies. Six studies had clinical endpoints, with mortality the primary endpoint for two studies. Treatment with inhaled nitric oxide, iloprost, and sildenafil all resulted in improved haemodynamics in specific cohorts of children with post-operative pulmonary hypertension, although improved outcomes were not consistently demonstrated across all treated children. Iloprost may be a cheaper alternative to inhaled nitric oxide with similar haemodynamic response.

Studies were predominantly single-centre, a control arm was rarely used in randomised studies, and haemodynamic endpoints varied significantly. Further research is needed to reduce post-operative morbidity and mortality from pulmonary hypertension in children with CHD.

To evaluate the relationship between religious beliefs and mental state, care burden, and quality of life in parents of infantile patients with CHD.

A cross-sectional study was conducted at a provincial hospital in Fujian, China. In this study, 114 parents of infant patients with CHD were successfully enrolled. Data were collected using the Duke University Religion Index, Hospital Anxiety and Depression Scale, Zarit Caregiver Burden Interview, and 36-Item Short-Form Health Survey.

The organisational religious activity, non-organisational religious activity, and intrinsic religiosity of parents were significantly related to the care burden and quality of life, and the two dimensions of non-organisational religious activity and intrinsic religiosity of parents were significantly related to their anxiety symptoms. No association was found between parents’ religious beliefs and their depressive symptoms. Among Buddhist parents, non-organisational religious activity and intrinsic religiosity reduced the care burden and improved quality of life. Among Christian parents, organisational religious activity and non-organisational religious activity were found to reduce the care burden, while organisational religious activity and intrinsic religiosity were found to improve quality of life. There was no correlation between the sub-dimensions of religious beliefs and a negative impact on the care process in Muslim parents.

Religious beliefs have a protective effect on the parents of infant patients with CHD. They help relieve parents’ anxiety, reduce their care burden, and improve their quality of life. In addition, different religious beliefs have different dimensions of influence on caregivers.

The most serious manifestation of rheumatic fever is carditis, as it can lead to chronic rheumatic heart disease. Neutrophil-to-lymphocyte ratio, platelet-to lymphocyte ratio and mean platelet volume have been accepted as novel indicators of the continuing inflammation that is correlated with the severity of valvular involvement in patients with rheumatic carditis.

As a diagnostic study to investigate the correlation of neutrophil-to-lymphocyte ratio, platelet-to-lymphocyte ratio and mean platelet volume with the severity of carditis in children with rheumatic fever.

This was a cross-sectional study at Sanglah Hospital, Bali. Data were collected retrospectively from medical records. Severity of carditis was characterised by a clinical, audible murmur during physical examination and demonstration of valvular involvement by echocardiography. Neutrophil-to-lymphocyte ratio, platelet-to-lymphocyte ratio and mean platelet volume were calculated from complete blood count. The correlation between parameters was assessed using Spearman’s correlation tests.

From January 2018 to December 2020, we found 45 cases admitted to Sanglah Hospital. Carditis was present in 40 (88.9%) subjects. Neutrophil-to-lymphocyte ratio had a strong positive correlation with severity of carditis (r = 0.66, p = 0.001), while platelet-to-lymphocyte ratio had a weak positive correlation with the severity of carditis (r = 0.23, p = 0.13) and mean platelet volume had a very weak negative correlation with the severity of carditis (r = −0.01, p = 0.95). Using a cut-off of 3.4, neutrophil-to-lymphocyte ratio predicted severe carditis with a sensitivity of 81.8% and specificity of 91.3%.

There was a positive correlation between neutrophil-to-lymphocyte ratio and severity of carditis in children with rheumatic fever. Neutrophil-to-lymphocyte ratio might be used as a diagnostic tool to predict the diagnosis of carditis severity.

No established risk prediction tool exists in United Kingdom and Irish Paediatric Cardiology practice for patients undergoing cardiac catheterisation. The Catheterisation RISk score for Paediatrics is used primarily in North American practice to assess risk prior to cardiac catheterisation. Validating the utility and transferability of such a tool in practice provides the opportunity to employ an already established risk assessment tool in everyday practice.

To ascertain whether the Catheterisation RISk score for Paediatrics assessment tool can accurately predict complications within United Kingdom and Irish congenital catheterisation practice.

Clinical and procedural data including National Institute for Cardiovascular Outcomes Research derived outcome data from 1500 patients across five large congenital cardiology centres in the United Kingdom and Ireland were retrospectively collected. Catheterisation RISk score for Paediatrics were then calculated for each case and compared with the observed procedural outcomes. Chi-square analysis was used to determine the relationship between observed and predicted events.

Ninety-eight (6.6%) patients in this study experienced a significant complication as qualified by National Institute for Cardiovascular Outcomes Research classification. 4% experienced a moderate complication, 2.3% experienced a major complication and 0.3% experienced a catastrophic complication resulting in death. Calculated Catheterisation RISk score for Paediatrics scores correlated well with all observed adverse events for paediatric patients across all CRISP categories. The association was also transferable to adult congenital heart disease patients in lower Catheterisation RISk score for Paediatrics categories (CRISP 1–3).

The Catheterisation RISk score for Paediatrics score accurately predicts significant complications in congenital catheterisation practice in the United Kingdom and Ireland. Our data validated the Catheterisation RISk score for Paediatrics assessment tool in five congenital centres using National Institute for Cardiovascular Outcomes Research-derived outcome data.

Vascular rings cause respiratory symptoms in children. Treatment consists of surgical division; however, data regarding mid-term results are scarce. The purpose of this study was to evaluate clinical results of vascular ring surgery.

Retrospective chart review of consecutive patients who underwent vascular ring surgery. Mid-term follow-up consisted of clinic visits and telephone questionnaire over a 1-year period sampling at five points in time.

Follow-up of 85 patients who underwent vascular rings surgery revealed significant symptomatic improvement within 6 months. In total, 50% were symptomatic to some degree at their last documented clinic visit complaining of stridor (36.8%), cough (34.2%), wheezing (10.5%), dyspnoea on exertion (10.5%), or recurrent respiratory infections (23.7%). By telephone questionnaires, 83% described a significant improvement in respiratory symptoms, 63.4% had some residual symptoms, 36.6% stridor, 38.8% chronic cough, 32.4% bronchodilator use,18.3% had at least one episode of pneumonia per year, 31% of children had dyspnoea or respiratory limitation, and 12.7% while doing physical activities. We found no association between the age at surgery or anatomic variant to the presence of symptoms at mid-term follow-up.

Surgical division of vascular rings results in significant clinical improvement within 1 year; nevertheless, some patients remain symptomatic to some degree. We found no association between the age at surgery or anatomic variant to the presence of symptoms at mid-term follow-up. Further evaluation is warranted to assess the nature of residual symptoms and explore whether anatomical causes can be identified leading to surgical modifications.

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.

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.

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.

The coronary flow velocity reserve of the left anterior descending artery was examined by transthoracic Doppler echocardiography in 189 patients (male 125, female 64) with a history of Kawasaki disease and 10 volunteers (control). Their ages ranged from 6 to 40 years (median 22 years). The patients were divided into nine groups based on the left anterior descending artery lesions. The coronary flow velocity reserve was measured by intravenous administration of adenosine triphosphate (0.15 mg/kg/minute) while fasting. The coronary flow velocity reserve was calculated as the ratio of hyperaemic to basal mean diastolic flow velocities. The respective groups were as follows: control, no coronary artery lesions (n = 39), no coronary artery lesions in the right coronary artery (n = 29), regression (n = 11), aneurysm at the bifurcation of the left coronary artery (n = 26), aneurysm of the left anterior descending artery (n = 15), localised stenosis <75% (n = 12), localised stenosis ≥75% (n = 17), segmental stenosis (n = 5) and coronary artery bypass grafting (n = 36). One-factor ANOVA followed by Tukey’s test was used to compare the coronary flow velocity reserve among the groups.

The coronary flow velocity reserve was significantly lower in the localised stenosis ≥75%, segmental stenosis and coronary artery grafting groups than in the other groups (p < 0.05).

The endothelial function in the epicoronary artery was preserved in patients with a history of Kawasaki disease and dilated coronary artery lesions.

During tetralogy of Fallot repair, leaving or even create an interatrial communication may facilitate post-operative course particularly with right ventricle restrictive physiology. The aim of our study is to assess the influence of atrial communication on post-operative course of tetralogy of Fallot repair.

Retrospectively, we studied all children who had tetralogy of Fallot repair (2003–2018). We divided them into two groups: tetralogy of Fallot repair with interatrial communication (TOFASD) group and tetralogy of Fallot repair with intact atrial septum (TOFIAS) group. We performed propensity match score for specific pre- or intra-operative variables and compared groups for post-operative outcome variables. Secondarily, we looked for right ventricle restrictive physiology incidence and influence of early repair performed before 3 months of age on post-operative course.

One hundred and sixty children underwent tetralogy of Fallot repair including (93) cases of TOFIAS (58%) and (67) cases of TOFASD (42%). With propensity matching score, 52 patients from each group were compared. Post-operative course was indifferent in term of positive pressure ventilation time, vasoactive inotropic score, creatinine and lactic acid levels, duration and amount of chest drainage and length of intensive care unit and hospital stay. Right ventricle restrictive physiology occurred in 38% of patients with no effects on outcome. 12/104 patients (12%) with early repair needed longer pressure ventilation time (p = 0.003) and intensive care unit stay (p = 0.02).

Leaving interatrial communication in tetralogy of Fallot repair did not affect post-operative course. As well, right ventricle restrictive physiology did not affect post-operative course. Infants undergoing early tetralogy of Fallot repair may require longer duration of positive pressure ventilation time and intensive care unit stay.

Salih myopathy, characterised by both congenital myopathy and fatal dilated cardiomyopathy, is an inherited muscle disorder that affects skeletal and cardiac muscles. TTN has been identified as the main cause of this myopathy, the enormous size of this gene poses a formidable challenge to molecular genetic diagnostics.

In the present study, whole-exome sequencing, cardiac MRI, and metabolic parameter assessment were performed to investigate the genetic causes of Salih myopathy in a consanguineous Iranian family who presented with titinopathy involving both skeletal and heart muscles in an autosomal recessive inheritance pattern.

Two missense variants of TTN gene (NM_001267550.2), namely c.61280A>C (p. Gln20427Pro) and c.54970G>A (p. Gly18324Ser), were detected and segregations were confirmed by polymerase chain reaction-based Sanger sequencing.

The compound heterozygous variants, c.61280A>C, (p. Gln20427Pro) and c.54970G>A, (p. Gly18324Ser) in the TTN gene appear to be the cause of Salih myopathy and dilated cardiomyopathy in the family presented. Whole-exome sequencing is an effective molecular diagnostic tool to identify the causative genetic variants of large genes such as TTN.

Children with exposure to coronavirus disease 2019 in recent times (asymptomatic or symptomatic infection) approaching congenital heart surgery programme are in increasing numbers. Understanding outcomes of such children will help risk-stratify and guide optimisation prior to congenital heart surgery.

The objective of the present study was to determine whether convalescent coronavirus disease 2019 children undergoing congenital heart surgery have any worse mortality or post-operative outcomes.

Consecutive children undergoing congenital heart surgery from Oct 2020 to May 2021 were enrolled after testing for reverse transcription-polymerase chain reaction or rapid antigen test and immunoglobulin G antibody prior to surgery. Convalescent coronavirus disease 2019 was defined in any asymptomatic patient positive for immunoglobulin G antibodies and negative for reverse transcription-polymerase chain reaction or rapid antigen test anytime 6 weeks prior to surgery. Control patients were negative for any of the three tests. Mortality and post-operative outcomes were compared among the groups.

One thousand one hundred and twenty-nine consecutive congenital heart surgeries were stratified as convalescence and control. Coronavirus disease 2019 Convalescent (n = 349) and coronavirus disease 2019 control (n = 780) groups were comparable for all demographic and clinical factors except younger and smaller kids in control. Convalescent children had no higher mortality, ventilation duration, ICU and hospital stay, no higher support with extracorporeal membrane oxygenation, high flow nasal cannula, no higher need for re-intubations, re-admissions, and no higher infections as central line-associated bloodstream infection, sternal site infection, and ventilator-associated pneumonia on comparison with coronavirus disease 2019 control children.

Convalescent coronavirus disease 2019 does not have any unfavourable outcomes as compared to coronavirus disease 2019 control children. Positive immunoglobulin G antibody screening prior to surgery is suggestive of convalescence and supports comparable outcomes on par with control peers.