To assess the outcomes in children and adolescents with rheumatic fever of the implantation of mechanical as opposed to biological heart valves.

We assessed 73 patients with rheumatic heart disease under the age of 18 years, who underwent replacement of heart valves between January, 1996, and December, 2005, at the National Institute of Cardiology in Rio de Janeiro, Brazil. Of the group, 71 patients survived, and were divided into a group of 52 receiving mechanical prostheses, and 19 with biological prostheses. We compared endpoints between the groups in terms of mortality, reoperation, haemorrhage, and stroke. Survival curves were estimated using the Kaplan-Meier method and were compared by the Mantel (log-rank) test.

Overall mortality was 8.2%. In those receiving mechanical prostheses, 2 (3.8%) patients died, 5 (9.6%) underwent reoperation, 2 (3.8%) suffered severe haemorrhage, and 3 (5.8%) had strokes. In those receiving biological valves, 2 (10.5%) patients died, and 4 (21%) underwent reoperation. After 2, 4, and 8 years, overall survival was 96%, 93% and 86%, respectively, with a borderline difference between the groups (p = 0.06). The probabilities of remaining free from reoperation (p = 0.13), and from combined endpoints, showed no statistically significant difference between the groups (p = 0.28).

Patients with mechanical prostheses had lower mortality and required fewer reoperations, but when all combined endpoints were considered, the groups did not differ. The biological prosthesis proved to be a good option for cardiac surgery in children and adolescents with difficulties or risks of anticoagulation.

The purpose was to examine the relationship between the complexity of structural heart disease and psychological well-being in adults with congenital cardiac disease.

A total of 380 patients registered at the Adult Congenital Heart Clinic in Calgary, Alberta, Canada were surveyed. The patients were mailed a socio-demographic questionnaire and an instrument to measure psychological well-being. The instrument of psychological well-being measures six dimensions: positive relations with others, autonomy, environmental mastery, personal growth, purpose in life, and self-acceptance. Of the 380 patients surveyed, 85 of 205 patients with simple structural malformation responded, giving a response rate of 41%, and 78 of 175 patients with complex malformations, thus giving a response of 45%.

There was no statistically significant difference in the mean scores of each of the six dimensions of psychological well-being between those patients with simple and complex malformations. Two-way analysis of variance, and multivariate analysis of variance, found significant differences in mean scores for dimensions when other socio-demographic variables were included in the analysis. For example, significantly higher mean scores for the dimension of purpose in life was found in patients who obtained a higher level of education (p = 0.009), and in patients who were employed (p < 0.001). We present the socio-demographic variables that statistically impact the mean scores for the dimension of psychological well-being.

Psychological well-being is not affected by the complexity of the structural congenital cardiac disease. Certain socio-demographic variables that impact psychological well-being, nonetheless, must be considered when developing multidisciplinary programmes to care for young adults with congenitally malformed hearts.

To describe the motor proficiency of 5-year-old children who underwent early infant cardiac surgery and had atypical infant gross motor development. To identify risk factors for motor dysfunction at 5 years of age.

A total of 33 children (80.5% participation rate) were re-assessed by a physiotherapist blinded to the diagnosis and previous clinical course, using standardised motor assessment tools.

Motor proficiency was categorised as below average or well below average in 41% of the study patients. Approximately 30% of the cohort had balance deficits. Motor abilities at 4 months and 2 years of age were associated with motor proficiency at age 5; however, atypical motor development in infancy was not predictive of below-average or well below-average scores at age 5. Risk factors associated with motor ability at age 5 included respiratory support and intensive care length of stay in the 1st year of life, asymmetrical crawling in infancy, and cyanotic CHD at age 5.

Despite differences from other reported studies in terms of cohort diagnoses and age at surgery, the rate of motor dysfunction was similar, with rates much higher than expected in typical children. Further assessment is needed in later childhood to determine the significance of these findings.

Anthracycline cardiotoxicity is an important side-effect in long-term childhood cancer survivors. We evaluated the incidence of and factors associated with anthracycline cardiotoxicity in a population of patients diagnosed with bone or soft tissue sarcoma.

We retrospectively enrolled patients diagnosed with bone or soft tissue sarcoma, from 1995 to 2011, treated with anthracycline chemotherapy at our Centre and with a follow-up echocardiography carried out ⩾3 years from cardiotoxic therapy completion. Cardiac toxicity was graded using Common Terminology Criteria for Adverse Events version 4.0.

A total of 82 patients were eligible. The median age at treatment was 11.9 years (1.44–18). We evaluated the median cumulative anthracycline dose, age at treatment, sex, thoracic radiotherapy, hematopoietic stem cell transplantation, and high-dose cyclophosphamide treatment as possible risk factors for cardiotoxicity. The median cumulative anthracycline dose was 390.75 mg/m2 (80–580). Of the 82 patients, 12 (14.6%) developed cardiotoxicity with grade ⩾2 ejection fraction decline: four patients were asymptomatic and did not receive any treatment; six patients were treated with pharmacological heart failure therapy; one patient with severe cardiomyopathy underwent heart transplantation and did not need any further treatment; and one patient died while waiting for heart transplantation. The median time at cardiac toxicity, from the end of anthracycline frontline chemotherapy, was 4.2 years (0.05–9.6). Cumulative anthracycline dose ⩾300 mg/m2 (p 0.04) was the only risk factor for cardiotoxicity on statistical analyses.

In our population, the cumulative incidence of cardiotoxicity is comparable to rates in the literature. This underlines the need for primary prevention and lifelong cardiac toxicity surveillance programmes in long-term childhood cancer survivors.

To evaluate the presence of carotid thickening and its relationship with the Pathobiological Determinants of Atherosclerosis in Youth score.

We carried out a cross-sectional study involving 512 brazilian adolescents. Variables such as sex, body mass index, concentrations of non-high-density lipoprotein and high-density lipoprotein cholesterol, blood pressure, blood glucose and glycated haemoglobin A1c levels that make up the score, and carotid thickening through the intima-media complex measured by ultrasound were evaluated. We adopted two cut-off points to evaluate carotid thickening, being considered altered for those higher or equal to the z-score 2+ and ⩾75th percentile. The association was assessed using the χ2 test and univariate and multivariate logistic regression analyses.

High cardiovascular risk was present in 10.2% of the adolescents; carotid thickness was present in 4.3% determined by the z-score 2+ and in 25.0% determined by the 75th percentile. When measured by the z-score, carotid thickening was associated with high systolic blood pressure (p=0.024), high-non-high density lipoprotein cholesterol (p=0.039), and high cardiovascular risk assessed by the score and by the 75th percentile, with body mass index >30 (p=0.005). In the multivariate analysis, high cardiovascular risk was found to be independently associated with the presence of carotid thickness evaluated by the z-score, with risk four times greater (p=0.010) of presenting with this condition compared with individuals with low risk, and this fact was not observed when factors were analysed alone.

The presence of high cardiovascular risk in adolescents assessed by the Pathobiological Determinants of Atherosclerosis in Youth score was associated with marked thickening of the carotid artery in healthy adolescents.

Dilated cardiomyopathy is a rare but serious disorder in children. No effective diagnostic or treatment tools are readily available. This study aimed to evaluate the efficacy of intravenous immunoglobulins in children with new onset dilated cardiomyopathy.

In this retrospective cohort study, 94 children with new onset dilated cardiomyopathy were followed during a median period of 33 months. All patients with secondary dilated cardiomyopathy – for example, genetic, auto-immune or structural defects – had been excluded. Viral tests were performed in all patients and 18 (19%) children met the criteria for the diagnosis “probable or definite viral myocarditis”. Intravenous immunoglobulins were administered to 21 (22%) patients. Overall transplant-free survival was 75% in 5 years and did not differ between treatment groups. The treatment was associated with a higher recovery rate within 5 years, compared with non-treated children (70 versus 43%, log rank=0.045). After correction for possible confounders the hazard ratio for recovery with intravenous immunoglobulins was not significant (hazard ratio: 2.1; 95% CI: 1.0–4.6; p=0.056). Administration of intravenous immunoglobulins resulted in a greater improvement in the shortening fraction of the left ventricle.

In our population of children with new onset dilated cardiomyopathy, of either viral or idiopathic origin, intravenous immunoglobulins were administered to a minority of the patients and did not influence transplant-free survival, but were associated with better improvement of systolic left ventricular function and with better recovery. Our results support the concept that children with new onset dilated cardiomyopathy might benefit from intravenous immunoglobulins.

Growth failure is prevalent among infants with CHD. A Standardized Clinical Assessment and Management Plan was introduced at Boston Children’s Hospital’s cardiac medical ward to identify patients with growth failure, evaluate relevant contributing conditions, and recommend a management plan including collaboration with nutrition physicians.

The objective of this study was to determine whether enrolled patients had improved growth compared with historical controls.

A total of 29 patients were enrolled in the period July, 2013–June, 2014. In all, 42 historical controls who met eligibility criteria for enrolment were selected for comparison from patients admitted to the same ward in the period June, 2010–June, 2011. Patients with CHD aged <1 year , with growth failure defined as weight-for-age z-score <−2, or failure to sustain adequate weight gain were eligible for participation. Primary outcome was change in weight-for-age z-score from enrolment to most recent weight measurement among patients with at least 6 months of follow-up.

Control patients were older at baseline admission weight (118 versus 95 days, p=0.33), and had a higher weight-for-age z-score, −2.9 (−3.1, −2.6) versus −3.7 (−4.3, −3.0) (p=0.02), compared with enrolled patients. Enrolled patients had greater gain in weight-for-age z-score, 2.7 (2.0, 3.4) versus 1.8 (1.5, 2.2) (p=0.03), from baseline to most recent follow-up.

Patients enrolled in a nutrition-focused protocol had greater weight improvement than historical controls. Identification of growth failure and collaboration with a nutrition support team was associated with improved weight gain among CHD patients experiencing growth failure. CHD programmes should consider a structural approach, including nutrition expertise to address growth failure.

The optimal management of symptomatic tetralogy of Fallot in neonates and younger infants with unfavourable anatomy is unclear and is further constrained by resource limitations in low and middle income countries.

Retrospective medical record review of infants with tetralogy of Fallot undergoing corrective or palliative procedures between January 2016 and June 2019.

The study included 120 infants; of whom 83 underwent primary complete repair, four underwent surgical palliation, and 33 underwent catheter-based palliation, including balloon pulmonary valvuloplasty (n = 18), right ventricular outflow tract stenting (n = 14), and stenting of the patent arterial duct (n = 1). Infants undergoing catheter-based procedures were younger in age (median 32 days; inter-quartile range (IQR) 7–144 versus 210 days; IQR 158–250), with lower baseline saturation (65 ± 12% versus 87 ± 7%) and had smaller pulmonary artery z-scores compared to the complete repair cohort. Follow-up was available for 31/33 (94%) infants (median 7 months [IQR 4–11]) who underwent trans-catheter palliation; 12 underwent complete repair, 10 are well, awaiting repair, eight required further palliation (catheter: 6; surgical: 2), and one died post-discharge from non-cardiac causes.

Catheter-based palliation is a safe and effective alternative in infants with tetralogy of Fallot who are at high risk for primary surgical repair.

Multicentre research databases can provide insights into healthcare processes to improve outcomes and make practice recommendations for novel approaches. Effective audits can establish a framework for reporting research efforts, ensuring accurate reporting, and spearheading quality improvement. Although a variety of data auditing models and standards exist, barriers to effective auditing including costs, regulatory requirements, travel, and design complexity must be considered.

The Congenital Cardiac Research Collaborative conducted a virtual data training initiative and remote source data verification audit on a retrospective multicentre dataset. CCRC investigators across nine institutions were trained to extract and enter data into a robust dataset on patients with tetralogy of Fallot who required neonatal intervention. Centres provided de-identified source files for a randomised 10% patient sample audit. Key auditing variables, discrepancy types, and severity levels were analysed across two study groups, primary repair and staged repair.

Of the total 572 study patients, data from 58 patients (31 staged repairs and 27 primary repairs) were source data verified. Amongst the 1790 variables audited, 45 discrepancies were discovered, resulting in an overall accuracy rate of 97.5%. High accuracy rates were consistent across all CCRC institutions ranging from 94.6% to 99.4% and were reported for both minor (1.5%) and major discrepancies type classifications (1.1%).

Findings indicate that implementing a virtual multicentre training initiative and remote source data verification audit can identify data quality concerns and produce a reliable, high-quality dataset. Remote auditing capacity is especially important during the current COVID-19 pandemic.

Although sleep apnea is an important disorder associated with cardiac events, data regarding its prevalence and risk factors in adult patients with congenital heart disease are limited.

In this study, patients underwent a sleep study in the hospital. Indications for admission were classified as heart failure, diagnostic catheterisation, interventional catheterisation, or arrhythmia. The prevalence, characteristics, and risk factors of sleep apnea using a type-3 portable overnight polygraph in adult patients with congenital heart disease were evaluated.

There were 104 patients [median age: 36 (interquartile range: 28–48) years] who were admitted for heart failure 34% (n = 36), diagnostic catheterisation 26% (n = 27), interventional catheterisation 18% (n = 19), or arrhythmia 22% (n = 23). The prevalence of sleep apnea, defined as a respiratory disturbance index ≥5, was 63% (n = 63), with a distribution of 37%, 16%, and 10% for mild (5≤ respiratory disturbance index <15), moderate (15≤ respiratory disturbance index <30), and severe (respiratory disturbance index ≥30) sleep apnea, respectively. A large majority of the sleep apnea cases were categorised as obstructive sleep apnea (92%, n = 58). The respiratory disturbance index ≥15 group had a significantly higher proportion of male patients and higher body mass index, noradrenaline level, and aortic blood pressure than the group without sleep apnea (respiratory disturbance index <5). Multivariable analysis showed that New York Heart Association class ≥II (OR, 4.36; 95% CI, 1.09–20.87) and body mass index ≥25 (OR, 4.29; 95% CI, 1.32–15.23) were independent risk factors for a respiratory disturbance index ≥15.

Our results showed a high prevalence of sleep apnea in adult patients with congenital heart disease. Its unique haemodynamics may be associated with a high prevalence of sleep apnea. Congestive heart failure and being overweight are important risk factors for sleep apnea. Management of heart failure and general lifestyle improvements will be important for controlling sleep apnea symptoms in these patients.

To verify an association, if it exists, between obesity and blood pressure raised beyond the 90th percentile in children and adolescents, and to determine the measure of adiposity that best correlates with blood pressure in these subjects.

Cross-sectional study.

A school-based study in Belo Horizonte, Brazil.

We selected randomly 1,403 students, aged from 6 to 18 years, from 545,046 students attending 521 public and private schools. Those selected completed the study.

We recorded the weight, height, skin fold in the triceps, subscapular, and suprailiac areas, waist and hip circumference, body-mass index, and resting systolic and diastolic blood pressures using a mercury sphygmomanometer.

In univariate analyses, body mass index greater or lesser than 85th percentile, measurements of skin thickness in the subscapular and suprailiac areas, and the sum of all measurements of skinfold thickness, were associated with both systolic and diastolic measurements of blood pressure. After multivariate analyses that adjusted for all measurements of adiposity except itself, and age, race, and socioeconomic state, we found that the increased body mass index was associated with a 3.6-fold increased frequency of elevated systolic measurements of blood pressure, with 95% confidence intervals from 2.2 to 5.8, and a 2.7-fold increased frequency of elevated measurements of diastolic blood pressure, with 95% confidence intervals from 1.9 to 4.0.

Body-mass index serves as a better predictor of elevated blood pressure among children than do local measurements of adiposity.