To evaluate exercise performance and body mass index in healthy children over an 8-year period in order to determine whether cardiorespiratory fitness has decreased relative to changes in body mass index in our patient population.

This study is a retrospective review of anthropometric and cardiorespiratory fitness data from our hospital's Exercise Physiology Laboratory's database on 1201 healthy children (6–18 years) with structurally normal hearts who performed one cycle ergometry test between 1999 and 2006. Subjects were stratified by gender and age. Body mass index was compared between 1999–2002 (Early Group) and 2003–2006 (Late Group) and with national averages. Exercise outcome variables were maximal oxygen consumption, anaerobic threshold, physical working capacity indexed to body weight. Analysis of variance was used to detect differences between groups for each outcome variable.

Body mass index was not significantly different from national data (p-value equals 0.79) or between groups. A negative trend (p-value greater than 0.15 less than 0.20) was observed among most indices of exercise performance between Early and Late Groups. Significantly lower anaerobic threshold was found among males between Early and Late Group (25.7 plus or minus 0.9 versus 23.4 plus or minus 1.6 millilitres per kilogram per minute, p-value less than 0.01), and in physical working capacity among females between Early and Late Groups (2.9 plus or minus 0.1 versus 2.7 plus or minus 0.1, p-value less than 0.05).

Cardiorespiratory fitness in males and working capacity in females declined over time independent of body mass index. This suggests that other measures may need to be evaluated for promotion of overall cardiovascular health.

Cardiac anomalies are among the most frequent congenital malformations, but the basic underlying causes for most cardiac defects remains undetermined. Some 40 years ago, a higher incidence of blood group B was reported in a small number of African-American children with congenital cardiac defects. In this study, we sought to re-evaluate this association using a larger population.

We collected data from 1985 patients undergoing cardiac surgery from July, 2000, through December, 2004. We divided the patients into 6 subgroups according to their diagnosis. We then compared the prevalence of ABO phenotypes between the patients and the general population of the United States of America by chi-square analysis. There were no significant differences in the distribution of the ABO phenotypes amongst the subgroups of those with congenital cardiac disease, or any for subgroup compared to the general population.

While statistical significance is influenced by the size of the population within the United States of America and the small numbers within each of our subgroups of patients with congenital cardiac disease, we have been unable to show any relationship between the distribution of ABO phenotypes and the existence of congenital cardiac disease.

Stenoses in the pulmonary arterial system can have a significant negative impact on the early postoperative course in infants. Early recognition and aggressive management are mandatory.

We describe our experience with 8 infants, with ages ranging from 3 to 9 months, weighing from 4.5 to 7.7 kilograms, who presented in the up to 18 days following construction of a shunt from the superior caval vein to the pulmonary arteries with clinical symptoms of obstructed pulmonary flow. We include also 2 infants in whom pulmonary arterial stents were implanted in the operating room. Cardiac catheterization showed significant stenoses or occlusion of the left pulmonary arteries in 9 infants, the right pulmonary arteries in 2, or the superior caval vein in 1, the investigation being prompted by the findings of supraphysiological superior caval venous pressures and systemic hypoxaemia. We implanted a variety of stents mounted on balloons ranging in diameter from 6 to 13 millimetres, with 7 placed across a newly created surgical anastomotic site.

All stenoses were crossed successfully, and stents implanted satisfactorily in all patients, albeit that 1 infant suffered an acute tear of the left pulmonary artery, requiring immediate reoperation. This patient died 72 hours later due to a diffuse coagulopathy. All other patients demonstrated sustained clinical improvement following the procedure. At follow-up, 7 of the 9 survivors have progressed to completion of the Fontan circulation. Redilation of the stents was required in the interim, prior to completion of the Fontan circulation, in 4 of them. In 2 patients, the previously implanted stents were incised during the Fontan completion, permitting placement of the extracardiac Goretex conduit from the inferior caval vein to the pulmonary arteries.

Stents can successfully be implanted perioperatively in the pulmonary arterial system during infancy, and redilated, with improvement in clinical outcome in the majority of those with clinically relevant obstruction.

Tetralogy of Fallot is a common CHD. Studies have shown a close link between heart failure and myocardial fibrosis. Interleukin-6 has been suggested to be a post-independent factor of heart failure. This study aimed to explore the relationship between IL-6 and myocardial fibrosis during cardiopulmonary bypass.

We downloaded the expression profile dataset GSE132176 from Gene Expression Omnibus. After normalising the raw data, Gene Set Enrichment Analysis and differential gene expression analysis were performed using R. Further, a weighted gene correlation network analysis and a protein–protein interaction network analysis were used to identify HUB genes. Finally, we downloaded single-cell expression data for HUB genes using PanglaoDB.

There were 119 differentially expressed genes in right atrium tissues comparing the post-CPB group with the pre-CPB group. IL-6 was found to be significantly up-regulated in the post-CPB group. Six genes (JUN, FOS, ATF3, EGR1, IL-6, and PTGS2) were identified as HUB genes by a weighted gene correlation network analysis and a protein–protein interaction network analysis. Gene Set Enrichment Analysis showed that IL-6 affects the myocardium during CPB mainly through the JAK/STAT signalling pathway. Finally, we used PanglaoDB data to analyse the single-cell expression of the HUB genes.

Our findings suggest that high expression of IL-6 and the activation of the JAK/STAT signalling pathway during CPB maybe the potential mechanism of myocardial fibrosis. We speculate that the high expression of IL-6 might be an important factor leading to heart failure after ToF surgery. We expect that these findings will provide a basis for the development of targeted drugs.

Infective endocarditis is a major threat after prosthetic pulmonary valve replacement. Early diagnosis may improve outcomes.

A structured patient education programme for prevention and early diagnosis of infective endocarditis was developed at our institution since 2016. Time delay between onset of symptoms of prosthetic pulmonary valve endocarditis and its diagnosis (defined as initiation of appropriate high-dose intravenous antibiotic treatment) was compared for patients presenting before (cohort 1) and after (cohort 2) initiation of the patient education programme.

Between 2008–2019, 26 patients (median age 24.9, range: 16.8–62.0 years, 73% male) were diagnosed with prosthetic pulmonary valve endocarditis, 13 patients (cohort 1) before (1.7 cases/year) and 13 patients (cohort 2) after June 2016 (3.7 cases/year). There were no differences in baseline characteristics or clinical presentation between the study cohorts. Overall, the median delay between onset of symptoms and diagnosis of infective endocarditis was 6 days (range: 0–133 days) with a significantly longer delay among patients in cohort 1, compared to cohort 2 (25 days, range: 5–133 days versus 3 days, range: 0–13 days, p < 0.0001). A delay of >7 days was documented in 11/13 patients (85%) in cohort 1 as compared to 1/13 (8%) in cohort 2 (p < 0.001). Need for urgent valve replacement or permanent deterioration of prosthetic valve function was higher in cohort 1, compared to cohort 2 (11/13, 85% versus 5/13, 39%; p = 0.041).

Prosthetic pulmonary valve endocarditis is increasingly recognised. A structured patient education programme may improve early diagnosis and clinical outcomes.

In the era of multi-modality imaging, this study compared contemporary, pre-operative echocardiography and cardiac MRI in predicting the need for intervention on additional lesions before surgical bidirectional cavopulmonary connection.

A total of 72 patients undergoing bidirectional cavopulmonary connection for single-ventricle palliation between 2007 and 2012, who underwent pre-operative assessment using both echocardiography and MRI, were included. The pre-determined outcome measure was any additional surgical or catheter-based intervention within 6 months of bidirectional cavopulmonary connection. Indices assessed were as follows: indexed dimensions of right and left pulmonary arteries, coarctation of the aorta, adequacy of interatrial communication, and degree of atrioventricular valve regurgitation.

Median age at bidirectional cavopulmonary connection was 160 days (interquartile range 121–284). The following MRI parameters predicted intervention: Z score for right pulmonary artery (odds ratio 1.77 (95% confidence interval 1.12–2.79, p=0.014)) and left pulmonary artery dimensions (odds ratio 1.45 (1.04–2.00, p=0.027)) and left pulmonary artery report conclusion (odds ratio 1.57 (1.06–2.33)). The magnetic resonance report predicted aortic arch intervention (odds ratio 11.5 (3.5–37.7, p=0.00006)). The need for atrioventricular valve repair was associated only with magnetic resonance regurgitation fraction score (odds ratio 22.4 (1.7–295.1, p=0.018)). Echocardiography assessment was superior to MRI for predicting intervention on interatrial septum (odds ratio 27.7 (6.3–121.6, p=0.00001)).

For branch pulmonary arteries, aortic arch, and atrioventricular valve regurgitation, MRI parameters more reliably predict the need for intervention; however, echocardiography more accurately identified the adequacy of interatrial communication. Approaching bidirectional cavopulmonary connection, the diagnostic strengths of MRI and echocardiography should be acknowledged when considering intervention.

Management strategies for pulmonary atresia with intact ventricular septum are variable and are based on right ventricular morphology and associated abnormalities. Catheter perforation of the pulmonary valve provides an alternative strategy to surgery in the neonatal period. We sought to assess the long-term outcome in terms of survival, re-intervention, and functional ventricular outcome in the setting of a 26-year single-centre experience of low threshold inclusion criteria for percutaneous valvotomy.

Retrospective analysis of patients diagnosed with pulmonary atresia with intact ventricular septum from 1990 to 2016 at a tertiary referral centre, was performed. Of 71 patients, 48 were brought to the catheterisation laboratory for intervention. Catheter valvotomy was successful in 45 patients (94%). Twenty-three patients (51%) also underwent ductus arteriosus stenting. The length of intensive care and hospital stay was significantly shorter, and early re-interventions were significantly reduced in the catheterisation group. There were eight deaths (17%); all within 35 days of the procedure. Of the survivors, only one has required a Fontan circulation. Twenty-eight patients (74%) have undergone biventricular repair and nine patients (24%) have one-and-a-half ventricle circulation. Following successful valvotomy, 80% of patients required further catheter-based or surgical interventions.

A low threshold for initial interventional management yielded a high rate of successful biventricular circulations. Although mortality was low in patients who survived the peri-procedural period, the rate of re-intervention remained high in all groups.

Treatment of hypoplastic left heart syndrome varies across institutions. This study examined the impact of introducing a standardised programme.

This retrospective cohort study evaluated the effects of a comprehensive strategy on 1-year transplant-free survival with preserved ventricular and atrioventricular valve (AVV) function following a Norwood operation. This strategy included standardised operative and perioperative management and dedicated interstage monitoring. The post-implementation cohort (C2) was compared to historic controls (C1). Outcomes were assessed using logistic regression and Kaplan–Meier analysis.

The study included 105 patients, 76 in C1 and 29 in C2. Groups had similar baseline characteristics, including percentage with preserved ventricular (96% C1 versus 100% C2, p = 0.28) and AVV function (97% C1 versus 93% C2, p = 0.31). Perioperatively, C2 had higher indexed oxygen delivery (348 ± 67 ml/minute/m2 C1 versus 402 ± 102ml/minute/m2 C2, p = 0.015) and lower renal injury (47% C1 versus 3% C2, p = 0.004). The primary outcome was similar in both groups (49% C1 and 52% C2, p = 0.78), with comparable rates of death and transplantation (36% C1 versus 38% C2, p = 0.89) and ventricular (2% C1 versus 0% C2, p = 0.53) and AVV dysfunction (11% C1 versus 11% C2, p = 0.96) at 1-year. When accounting for cohort and 100-day freedom from hospitalisation, female gender (OR 3.7, p = 0.01) increased and ventricular dysfunction (OR 0.21, p = 0.02) and CPR (OR 0.11, p = 0.002) or ECMO use (OR 0.15, p = 001) decreased the likelihood of 1-year transplant-free survival.

Standardised perioperative management was not associated with improved 1-year transplant-free survival. Post-operative ventricular or AVV dysfunction was the strongest predictor of 1-year mortality.

The objective of this study was to evaluate the feasibility and effects of education on complex congenital heart diseases using patient-specific three-dimensional printed heart models.

Three-dimensional printed heart models were created using computed tomography data obtained from 11 patients with complex congenital heart disease. Fourteen kinds of heart models, encompassing nine kinds of complex congenital heart disease were printed. Using these models, a series of educational hands-on seminars, led by an experienced paediatric cardiac surgeon and a paediatric cardiologist, were conducted for medical personnel who were involved in the care of congenital heart disease patients. Contents of the seminars included anatomy, three-dimensional structure, pathophysiology, and surgery for each diagnosis. Likert-type (10-point scale) questionnaires were used before and after each seminar to evaluate the effects of education.

Between November 2019 and June 2020, a total of 16 sessions of hands-on seminar were conducted. The total number of questionnaire responses was 75. Overall, participants reported subjective improvement in understanding anatomy (4.8 ± 2.1 versus 8.4 ± 1.1, p < 0.001), three-dimensional structure (4.6 ± 2.2 versus 8.9 ± 1.0, p < 0.001), pathophysiology (4.8 ± 2.2 versus 8.5 ± 1.0, p < 0.001), and surgery (4.9 ± 2.3 versus 8.8 ± 0.9, p < 0.001) of the congenital heart disease investigated.

The utilisation of three-dimensional printed heart models for education on complex congenital heart disease was feasible and improved medical personnel’s understanding of complex congenital heart disease. This education tool may be an effective alternative to conventional education tools for complex congenital heart disease.