Sudden cardiac death is a significant concern among patients with congenital heart disease (CHD). We assessed the risk of remote sudden cardiac death after congenital heart surgery.

Patients undergoing congenital heart surgery before 21 years of age between 1982 and 2003 in the Pediatric Cardiac Care Consortium registry were linked to National Death Index data through 2019. Sudden cardiac death was defined as death associated with a cardiac arrest or ventricular fibrillation diagnosis code. Standardised mortality ratios relative to the general population were calculated using Centers for Disease Control and Prevention data.

Among 30,566 patients discharged after their initial surgery, 2,718 deaths occurred over a median period of 23 years (IQR 19–27). Of 463 (17%) sudden cardiac deaths, the median age was 1.7 years (IQR 0.5–16.5). The mean incidence was 7 per 10,000 person-years (95% CI: 0.64–0.77), ranging from 2.7 for left-to-right shunt lesions to 37 for single-ventricle physiology. Cardiac comorbidities including heart failure (13.6%) and arrhythmias (7.1%) were more frequent among sudden cardiac death patients. Standard mortality ratios for sudden cardiac death were elevated across all CHD types, ranging from 8.0 (95% CI: 6.3–9.6) for left-to-right shunts to 107.7 (95% CI: 88.9–126.5) for single-ventricle physiology.

Sudden cardiac death risk is higher post-congenital heart surgery compared to the general population. Even patients with mild CHD are at risk, highlighting the need for long-term follow-up for all patients. Heart failure and arrhythmia prevalence suggest potential therapeutic targets to reduce sudden cardiac death risk.

Multisystem inflammatory syndrome in children is a rare, post-infectious complication of SARS-CoV-2 infection in children. We aimed to assess the long-term sequelae, particularly cardiac, in a large, diverse population.

We performed a retrospective cohort study of all children (aged 0–20 years, n = 304) admitted to a tertiary care centre with a diagnosis of multisystem inflammatory syndrome in children from March 1, 2020 to August 31, 2021 and had at least one follow-up visit through December 31, 2021. Data were collected at hospitalisation, 2 weeks, 6 weeks, 3 months, and 1 year after diagnosis, where applicable. Cardiovascular outcomes included left ventricular ejection fraction, presence or absence of pericardial effusion, coronary artery abnormalities, and abnormal electrocardiogram findings.

Population was median age 9 years (IQR 5–12), 62.2% male, 61.8% African American (AA), and 15.8% Hispanic. Hospitalisation findings included abnormal echocardiogram 57.2%, mean worst recorded left ventricular ejection fraction 52.4% ± 12.4%, non-trivial pericardial effusion 13.4%, coronary artery abnormalities 10.6%, and abnormal ECG 19.6%. During follow-up, abnormal echocardiogram significantly decreased to 6.0% at 2 weeks and 4.7% at 6 weeks. Mean left ventricular ejection fraction significantly increased to 65.4% ± 5.6% at 2 weeks and stabilised. Pericardial effusion significantly decreased to 3.2% at 2 weeks and stabilised. Coronary artery abnormalities significantly decreased to 2.0% and abnormal electrocardiograms significantly decreased to 6.4% at 2 weeks and stabilised.

Children with multisystem inflammatory syndrome in children have significant echocardiographic abnormalities during the acute presentation, but these findings typically improve within weeks. However, a small subset of patients may have persistent coronary abnormalities.

While most children with multisystem inflammatory syndrome in children have rapid recovery of cardiac dysfunction, little is known about the long-term outcomes regarding exercise capacity. We aimed to compare the exercise capacity among patients with multisystem inflammatory syndrome in children versus viral/idiopathic myocarditis at 3–6 months after initial diagnosis.

We performed a retrospective cohort study among patients with multisystem inflammatory syndrome in children in June 2020 to May 2021 and patients with viral/idiopathic myocarditis in August 2014 to January 2020. Data from cardiopulmonary exercise test as well as echocardiographic and laboratory data were obtained. Inclusion criteria included diagnosis of multisystem inflammatory syndrome in children or viral/idiopathic myocarditis, exercise test performed within 3–6 months of hospital discharge, and maximal effort on cardiopulmonary exercise test as determined by respiratory exchange ratio >1.10.

Thirty-one patients with multisystem inflammatory syndrome in children and 25 with viral/idiopathic myocarditis were included. The mean percent predicted peak VO2 was 90.84% for multisystem inflammatory syndrome in children patients and 91.08% for those with viral/idiopathic myocarditis (p-value 0.955). There were no statistically significant differences between the groups with regard to percent predicted maximal heart rate, metabolic equivalents, percent predicted peak VO2, percent predicted anerobic threshold, or percent predicted O2 pulse. There was a statistically significant correlation between lowest ejection fraction during hospitalisation and peak VO2 among viral/idiopathic myocarditis patients (r: 0.62, p-value 0.01) but not multisystem inflammatory syndrome in children patients (r: 0.1, p-value 0.6).

Patients with multisystem inflammatory syndrome in children and viral myocarditis appear to, on average, have normal exercise capacity around 3–6 months following hospital discharge. For patients with viral/idiopathic myocarditis, those with worse ejection fraction during hospitalisation had lower peak VO2 on cardiopulmonary exercise test.

Loss of follow-up is a barrier to providing adequate care to paediatric cardiac patients. The purpose of this study was to determine variables associated with loss of appropriate paediatric cardiology follow-up, including potentially modifiable factors. We hypothesised having earlier recommend follow-up intervals was associated with less likelihood of loss of follow-up.

We performed a retrospective cohort study of patients >5 years old seen in a large, outpatient paediatric practice from 2013 to 2016. Subjects were considered to be lost to follow-up if they did not have a subsequent outpatient encounter by 6 months after their recommend follow-up time interval.

Of the 8940 eligible patients, 45.9% were lost to follow-up. Recommended follow-up interval of 1 year was associated with less loss of follow-up (41.4%) as compared to 2-year intervals (51.6%) and 3 years (55.7%) (p < 0.001 for both). Other significant predictors of loss of follow-up included less severe heart disease, older age, and non-Hispanic Black race/ethnicity. Sex and payor type were not significant predictors. In the stratified analyses by severity of disease and age, longer recommended follow-up time was associated with greater loss of follow-up among all severity and age categories.

Almost half of the patients in our cohort did not return to clinic within the recommended timeline. Shorter follow-up time was associated with less loss of follow-up among all categories of disease severity and age groups. Recommending shorter follow-up intervals may be one initiative for paediatric cardiologists to improve rates of follow-up.

To determine whether racial/ethnic differences exist for the treatment of Marfan syndrome aortopathy. The 2014 Pediatric Heart Network randomised trial of losartan versus atenolol in Marfan syndrome paediatric and young adult patients showed no treatment differences in the rate of aortic root growth over 3 years; however, they did not examine racial/ethnic differences, and recent data suggest that angiotensin receptor blockers may have different pharmacologic effects in different racial/ethnic populations.

We performed a secondary analysis of public-use data from the Pediatric Heart Network randomised trial comparing the differences by race/ethnicity (non-Hispanic White, non-Hispanic Black, and Hispanic patients) amongst the treatment groups for the primary outcome of rate of aortic root enlargement by z score and secondary outcome of rate of change of absolute diameter of aortic root, z score and absolute diameter of ascending aorta, and blood pressure changes.

For aortic root enlargement by z score amongst non-Hispanic White patients, patients on losartan exhibited an annual z score change of –0.090 ± 0.016, compared to –0.146 ± 0.015 for those on atenolol (p = 0.01), favouring atenolol. For Hispanic and non-Hispanic Black patients, there was no difference in primary or secondary outcomes between treatment groups.

Non-Hispanic White patients had a small, but statistically significantly greater decrease in aortic root z score favouring atenolol over losartan. There were no significant differences amongst Hispanic or non-Hispanic Black patients, which may be due to relatively small size numbers. These findings may have important implications for medication selection by race/ethnicity in Marfan syndrome patients, which has not previously been evaluated in studies.

The prevalence of attention deficit/hyperactivity disorder in the general population is common and is now diagnosed in 4%–12% of children. Children with CHD have been shown to be at increased risk for attention deficit/hyperactivity disorder. Case reports have led to concern regarding the use of attention deficit/hyperactivity disorder medications in children with underlying CHD. We hypothesised that medical therapy for patients with CHD and attention deficit/hyperactivity disorder is safe.

A single-centre, retrospective chart review was performed evaluating for adverse events in patients aged 4–21 years with CHD who received attention deficit/hyperactivity disorder therapy over a 5-year span. Inclusion criteria were a diagnosis of CHD and concomitant medical therapy with amphetamines, methylphenidate, or atomoxetine. Patients with trivial or spontaneously resolved CHD were excluded from analysis.

In 831 patients with CHD who received stimulants with a mean age of 12.9 years, there was only one adverse cardiovascular event identified. Using sensitivity analysis, our median follow-up time was 686 days and a prevalence rate of 0.21% of adverse events. This episode consisted of increased frequency of supraventricular tachycardia in a patient who had this condition prior to initiation of medical therapy; the condition improved with discontinuation of attention deficit/hyperactivity disorder therapy.

The incidence of significant adverse cardiovascular events in our population was similar to the prevalence of supraventricular tachycardia in the general population. Our single-centre experience demonstrated no increased risk in adverse events related to medical therapy for children with attention deficit/hyperactivity disorder and underlying CHD. Further population-based studies are indicated to validate these findings.

Using existing data from clinical registries to support clinical trials and other prospective studies has the potential to improve research efficiency. However, little has been reported about staff experiences and lessons learned from implementation of this method in pediatric cardiology.

We describe the process of using existing registry data in the Pediatric Heart Network Residual Lesion Score Study, report stakeholders’ perspectives, and provide recommendations to guide future studies using this methodology.

The Residual Lesion Score Study, a 17-site prospective, observational study, piloted the use of existing local surgical registry data (collected for submission to the Society of Thoracic Surgeons-Congenital Heart Surgery Database) to supplement manual data collection. A survey regarding processes and perceptions was administered to study site and data coordinating center staff.

Survey response rate was 98% (54/55). Overall, 57% perceived that using registry data saved research staff time in the current study, and 74% perceived that it would save time in future studies; 55% noted significant upfront time in developing a methodology for extracting registry data. Survey recommendations included simplifying data extraction processes and tailoring to the needs of the study, understanding registry characteristics to maximise data quality and security, and involving all stakeholders in design and implementation processes.

Use of existing registry data was perceived to save time and promote efficiency. Consideration must be given to the upfront investment of time and resources needed. Ongoing efforts focussed on automating and centralising data management may aid in further optimising this methodology for future studies.

Ebstein anomaly is a rare congenital heart defect (CHD) that, when severe, requires corrective surgery or other catheter-based intervention in the first year of life. Due to its rarity, risk factors for Ebstein anomaly remain largely unknown. Using national data, we examined 18 potential risk factors for Ebstein anomaly.

Using 1997–2011 data from the National Birth Defects Prevention Study, a population-based case–control study, we calculated crude and adjusted odds ratios and 95% confidence intervals for paternal age, maternal socio-demographics, reproductive history, and modifiable risk factors, and infant characteristics reported by mothers of 135 Ebstein anomaly cases and 11,829 controls.

Mothers of Ebstein anomaly cases had 4.1 (95% confidence interval: 1.8, 9.5) times the odds of reporting a family history of CHD compared with mothers of controls. Ebstein anomaly was associated with maternal second-hand cigarette smoke exposure at home (odds ratio = 2.2 [95% confidence interval: 1.1, 4.4]), but not maternal cigarette smoking (odds ratio = 1.3 [95% confidence interval: 0.8, 2.1]). Odds were elevated, but the 95% confidence interval included 1.0, for maternal marijuana use (odds ratio = 1.8 [95% confidence interval: 0.9, 3.8]) and paternal age ≥40 years at delivery (odds ratio = 1.9 [95% confidence interval: 1.0, 3.5]).

Maternal exposure to second-hand cigarette smoke at home and a family history of CHD were associated with elevated odds of Ebstein anomaly. Genetic analyses could clarify the potential heritability of Ebstein anomaly.

It is increasingly essential for medical researchers to be literate in statistics, but the requisite degree of literacy is not the same for every statistical competency in translational research. Statistical competency can range from ‘fundamental’ (necessary for all) to ‘specialized’ (necessary for only some). In this study, we determine the degree to which each competency is fundamental or specialized.

We surveyed members of 4 professional organizations, targeting doctorally trained biostatisticians and epidemiologists who taught statistics to medical research learners in the past 5 years. Respondents rated 24 educational competencies on a 5-point Likert scale anchored by ‘fundamental’ and ‘specialized.’

There were 112 responses. Nineteen of 24 competencies were fundamental. The competencies considered most fundamental were assessing sources of bias and variation (95%), recognizing one’s own limits with regard to statistics (93%), identifying the strengths, and limitations of study designs (93%). The least endorsed items were meta-analysis (34%) and stopping rules (18%).

We have identified the statistical competencies needed by all medical researchers. These competencies should be considered when designing statistical curricula for medical researchers and should inform which topics are taught in graduate programs and evidence-based medicine courses where learners need to read and understand the medical research literature.

It is unclear whether cryoablation or radiofrequency ablation offers better value for treating atrioventricular nodal re-entrant tachycardia in children. We aimed to compare the value of these procedures for treating atrioventricular nodal re-entrant tachycardia in children, with value being outcomes relative to costs.

We performed a retrospective cohort study of all atrioventricular nodal re-entrant tachycardia ablations for children (age⩽18 years) from July, 2009 to June, 2011 at our institution. Costs included fixed costs, miscellaneous hospital costs, and labour costs, and key outcomes were acute and long-term success (6 months) of the ablations. We conducted T-tests and regression analyses to investigate the associations between the ablation procedure type and the cost and success of the ablations.

Of 96 unique cases performed by three paediatric electrophysiologists, 48 were cryoablation only, 42 radiofrequency ablation only, and six were a combination. Acute success was 100% for the cryoablation only and radiofrequency ablation only cases and 83% for the combination cases. There were no notable adverse events. The average total cost was $9636 for cryoablation cases, $9708 for radiofrequency ablation cases, and $10,967 for combination cases (p=0.51 for cryoablation only versus radiofrequency ablation only). The long-term success rate was 79.1% for cryoablation only, 92.8% for radiofrequency ablation only, and 66.7% for the combination (p=0.01 for cryoablation only versus radiofrequency ablation only), but long-term success varied notably by provider.

Cryoablation and radiofrequency ablation offer similar value in the short term for the treatment of atrioventricular nodal re-entrant tachycardia in children. Differences in long-term success may vary substantially by physician, and thus may lead to differences in long-term value.

Although much is known about morbidity and mortality, there are limited data focussing on the financial aspect of the Norwood operation. Our objective is to characterise the hospitalisation and detail the hospital costs.

We retrospectively studied 86 newborns with hypoplastic left heart syndrome who underwent Norwood palliation between 2008 and 2012. Clinical and financial data were collected. Financial data have been reported for 2011–2012.

At surgery, median age and weight of the patients were 4 days (range 1–13) and 3 kg (range 2–4.8), respectively. The median time from admission to surgery was 4 days (range 1–10), with the postoperative ICU stay and total length of stay at the hospital being 10 days (range 4–135) and 16 days (range 5–136), respectively. Discharge mortality was 14/86 (16%) patients.

For patients operated on between 2011 and 2012 (n=40), median hospital costs, charges, and collections per patient were $117,021, $433,054, and $198,453, respectively, and mean hospital costs, charges, and collections per patient were $322,765, $1,109,500, and $511,271, respectively.

A breakdown of total hospital costs (direct and indirect) by department showed that the top four areas of resource utilisation (excluding physician fees) were as follows: the cardiac ICU (35%), laboratory (12%), pharmacy (12%), and operating room (7%). Interestingly, point-of-care laboratory evaluations accounted for almost half of the laboratory total (5%). Extracorporeal membrane oxygenation, although only utilised in eight patients between 2011 and 2012, accounted for 7% of utilisation.General radiology only accounted for 2%, despite numerous radiographs.

Limited data are available that detail the hospitalisation and costs associated with the Norwood operation. We hope that this analysis will identify areas for quality and value improvement from both system and patient perspectives.

Necrotising enterocolitis is a rare, though catastrophic complication that may occur in term newborns with congenital heart disease. There is considerable controversy regarding the factors that lead to necrotising enterocolitis in this population. We sought to determine the incidence of necrotising enterocolitis among term and near-term newborns with congenital heart disease, focusing on the relationship of enteral feeding to this complication.

In this retrospective study, we identified the incidence of necrotising enterocolitis among 1551 newborns admitted to our cardiac intensive care unit between July 1, 2002 and July 1, 2010. In order to understand the impact of enteral feeding upon the development of necrotising enterocolitis, we undertook a nested 2:1 matched case–control analysis to compare feeding patterns in an age- and lesion-matched control population.

Necrotising enterocolitis developed in 45 term or near-term infants (3%). The majority of these cases, 27 (60%), occurred in the post-operative period after the introduction of enteral feeds. This subgroup was used for matched analysis. There were no differences in enteral feeding patterns among the patients who developed necrotising enterocolitis and their matched controls. The overall mortality rate for patients who developed necrotising enterocolitis was 24.4% (11 out of 45).

Despite numerous advances in the care of infants with congenital heart disease, necrotising enterocolitis remains a significant source of morbidity and mortality. In these infants, there is no clear relationship between enteral feeding patterns and the development of necrotising enterocolitis in the post-operative period. The benefits of graduated feeding advancements to avoid the development of necrotising enterocolitis remain unproven.