Heart transplantation has been an option for children in Sweden since 1989. As our unit faced an increased rate of post-transplant lymphoproliferative disorder, the objective of the study was to identify possible risk factors.

This is a retrospective study of all children aged 0–18 years who underwent heart transplantation in Gothenburg from 1989 to 2014.

A total of 71 children underwent heart transplantation. The overall incidence of post-transplant lymphoproliferative disorder was 14% (10/71); however, 17% (6/36) of those undergoing transplantation after 2007 developed lymphoma, compared with only 10% (4/35) of transplantation cases before 2007 (p=0.85). The mean age at transplantation was 9 years (0–17). The mean post-transplant follow-up time was 5.5 years (0.5–21.9) in the group that developed post-transplant lymphoproliferative disorder, compared with 10.2 years (0.02–25.2) in those who did not. In our study group, risk factors for post-transplant lymphoproliferative disorder were surgically palliated CHD (p=0.0005), sternotomy during infancy (p⩽0.0001), hypoplastic left ventricle (p=0.0001), number of surgical events (p=0.0022), mismatch concerning Epstein–Barr virus infection – that is, a positive donor–negative recipient (p⩽0.0001) – and immunosuppressive treatment with tacrolimus compared with ciclosporine (p=0.028).

This study has three major findings. First, post-transplant lymphoproliferative disorder only developed in subjects born with CHD. Second, the vast majority (9/10) of the subjects developing the disorder had undergone sternotomy as infants. Third, the number of surgical events correlated with a higher risk for developing post-transplant lymphoproliferative disorder.

Joint programmes, as opposed to regionalisation of paediatric cardiac care, may improve outcomes while preserving accessibility. We determined the prevalence and nature of joint programmes.

We sent an online survey to 125 paediatric cardiac surgeons in the United States in November, 2009 querying the past or present existence of a joint programme, its mission, structure, function, and perceived success.

A total of 65 surgeon responses from 65 institutions met the criteria for inclusion. Of the 65 institutions, 22 currently or previously conducted a joint programme. Compared with primary institutions, partner institutions were less often children's hospitals (p = 0.0004), had fewer paediatric beds (p = 0.005), and performed fewer cardiac cases (p = 0.03). Approximately 47% of partner hospitals performed fewer than 50 cases per year. The median distance range between hospitals was 41–60 miles, ranging from 5 to 1000 miles. Approximately 54% of partner hospitals had no surgeon working primarily on-site, and 31% of the programmes conducted joint conferences. Approximately 67% of the programmes limited the complexity of cases at the partner hospital, and 83% of the programmes had formal contracts between hospitals. Of the six programmes whose main mission was to increase referrals to the primary hospital, three were felt to have failed. Of the nine programmes whose mission was to increase regional quality, eight were felt to be successful.

Joint programmes in paediatric cardiac surgery are common but are heterogeneous in structure and function. Programmes whose mission is to improve the quality of regional care seem more likely to succeed. Joint programmes may be a practical alternative to regionalisation to achieve better outcomes.

The objective of this study was to verify possible associations between heart rate variability indices and physical activity, body composition, and metabolic and cardiovascular parameters in individuals with type 1 diabetes.

A total of 39 young patients with type 1 diabetes were included. Body composition, physical activity, cardiovascular parameters, and metabolic parameters were assessed. For the heart rate variability analysis, heart rate was recorded beat-by-beat using a Polar S810i heart rate monitor for 30 minutes, with the volunteers in the supine position; subsequently, the following indices were considered: standard deviation of all normal RR intervals; root-mean square of differences between adjacent normal RR intervals in a time interval; percentage of adjacent RR intervals with a difference of duration >50 ms; high frequency component in milliseconds squared; high frequency component in normalised units; standard deviation of the instantaneous variability beat-to-beat; and standard deviation of the long-term variability. The association between the heart rate variability indices and independent variables was verified through linear regression in unadjusted and adjusted models (considering gender and age). The statistical significance was set at 5% and the confidence interval at 95%.

High values of at-rest heart rate were associated with reduced parasympathetic activity and global heart rate variability, and higher values of waist-to-hip ratio were related to lower parasympathetic activity, independent of age or gender.

For young patients with type 1 diabetes, increases in at-rest heart rate values are associated with reduced parasympathetic activity and global heart rate variability, whereas higher waist-to-hip ratio values are related to lower parasympathetic activity, both independent of age and gender.

Veno-arterial extracorporeal membrane oxygenation is frequently used in patients with cardiac disease. We evaluated short-term outcomes and identified factors associated with hospital mortality in cardiac patients supported with veno-arterial extracorporeal membrane oxygenation.

A retrospective review of patients supported with veno-arterial extracorporeal membrane oxygenation at a university-affiliated children’s hospital was performed.

A total of 253 patients with cardiac disease managed with extracorporeal membrane oxygenation were identified; survival to discharge was 48%, which significantly improved from 39% in an earlier era (1995–2001) (p=0.01). Patients were categorised into surgical versus non-surgical groups on the basis of whether they had undergone cardiac surgery before or not, respectively. The most common indication for extracorporeal membrane oxygenation was extracorporeal cardiopulmonary resuscitation: 96 (51%) in the surgical group and 45 (68%) in the non-surgical group. In a multiple covariate analysis, single-ventricle physiology (p=0.01), duration of extracorporeal membrane oxygenation (p<0.01), and length of hospital stay (p=0.03) were associated with hospital mortality. Weekend or night shift cannulation was associated with mortality in non-surgical patients (p=0.05).

We report improvement in survival compared with an earlier era in cardiac patients supported with extracorporeal membrane oxygenation. Single-ventricle physiology continues to negatively impact survival, along with evidence of organ dysfunction during extracorporeal membrane oxygenation, duration of extracorporeal membrane oxygenation, and length of stay.

To examine the utility of decline in arterial partial pressure of oxygen after exercise as a marker of pulmonary vascular obstructive disease in patients with atrial septal defect and pulmonary hypertension.

Treadmill exercise was performed in 18 patients with atrial septal defect and pulmonary hypertension. Arterial blood gas samples were obtained before and after peak exercise. A decline in the arterial pressure of oxygen of more than 10 millimetres of mercury after exercise was considered significant based on preliminary tests conducted on the controls. Cardiac catheterisation was performed in all patients and haemodynamic data sets were obtained on room air, oxygen, and a mixture of oxygen and nitric oxide (30–40 parts per million).

There were 10 patients who had more than a 10 millimetres of mercury drop in arterial partial pressure of oxygen after exercise and who had a basal pulmonary vascular resistance index of more than 7 Wood units per square metre. Out of eight patients who had less than a 10 millimetres of mercury drop in arterial partial pressure of oxygen after exercise, seven had a basal pulmonary vascular resistance index of less than 7 Wood units per square metre, p equals 0.0001. A decline in arterial partial pressure of oxygen of more than 10 millimetres of mercury predicted a basal pulmonary vascular resistance index of more than 7 Wood units per square metre with a specificity of 100% and a sensitivity of 90%.

A decline in arterial partial pressure of oxygen following exercise appears to predict a high pulmonary vascular resistance index in patients with atrial septal defect and pulmonary hypertension. This test is a useful non-invasive marker of pulmonary vascular obstructive disease in this subset.

Many studies have suggested that more women then men present with physical symptoms. There is no data available, however, on the differences in reporting of physical symptoms between teenage male and female athletes. Our objective was to evaluate the differences according to gender in physical symptoms in healthy teenage athletes.

A total of 1,465 high school athletes, between the ages of 13 and 19 years participated in a mass echocardiographic screening programme for detection of cardiac abnormalities. Screening was conducted using a hand-carried cardiac ultrasound device (OptiGo, Philips). All participants were actively involved in a high school sport programme. Each athlete was required to fill out a questionnaire before the screening. The athletes were asked to report the occurrence of physical symptoms with activity or exercise. A physical examination was not performed during screening.

There were 1,031 (70.4%) male and 434 (29.6%) female participants. Significantly more female teenage athletes reported physical symptoms (190/434, 43.8% versus 267/1,031, 25.9%, odds ratio: 2.28, confidence interval: 1.76–2.81, p less than 0.001). Symptoms did not correlate with any echocardiographically identified cardiac abnormalities in either gender. The differences in the reporting of symptoms were significant for all physical symptoms addressed by the questionnaire.

There is a high prevalence of reporting physical symptoms in young healthy athletes without any relation to cardiac abnormalities. Young female athletes report physical symptoms nearly twice as often as their male counterparts.

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.

The Coronavirus Disease 2019 (COVID-19) pandemic negatively impacted global healthcare. Consequences in Pediatric and Congenital Heart Surgery programmes and mortality of congenital heart patients infected with severe acute respiratory syndrome coronavirus-2 (SARS-Cov-2) is still to be determined.

To study the COVID-19 pandemic implications in Brazilian Pediatric and Congenital Heart Surgery programmes.

We conducted a national online survey covering all states that perform Pediatric and Congenital Heart Surgery from 10 November to 22 November, 2020, using a Google forms questionnaire. We formulated questions related to impact on surgical volume, case-mix, and mortality. Then we asked about short-term post-operative COVID-19 infection and outcomes.

We received responses from 46 centres representing all states where there were a Pediatric and Congenital Heart Surgery programme and all high-volume centres across the country. All but one centre experienced a significant decrease in surgical volume, and 23.9% of the responders revealed less than one-quarter of volume decrement. On the other hand, in over 70% of the centres, there was a significant surgical volume reduction. In addition to this, there was a shift in case-mix in 41 centres (89.1%) towards more complex cases. More than one-third of the responders revealed increased mortality in 2020 compared to previous years, and 43.5% of the programmes (20 centres) had at least one patient contaminated by SARS-Cov-2, accounting for 48 patients. Mortality in post-operative infected patients was 45.8% (22 patients).

In general, Brazilian Pediatric and Congenital Heart Surgery programmes were severely affected by decreased surgical volume, unbalanced case-mix towards more complex cases, and increased mortality. Almost half of the programmes related post-operative COVID-19 contamination with high mortality.