Infants with moderate-to-severe CHD frequently undergo cardiopulmonary bypass surgery in childhood. Morbidity and mortality are highest in those who develop post-operative low cardiac output syndrome. Vasoactive and inotropic medications are mainstays of treatment for these children, despite limited evidence supporting their use.

To help inform clinical practice, as well as the conduct of future trials, we performed a systematic review of existing literature on inotropes and vasoactives in children after cardiac surgery using the PubMed and EMBASE databases. We included studies from 2000 to 2020, and the patient population was defined as birth – 18 years of age. Two reviewers independently reviewed studies to determine final eligibility.

The final analysis included 37 papers. Collectively, selected studies reported on 12 different vasoactive and inotropic medications in 2856 children. Overall evidence supporting the use of these drugs in children after cardiopulmonary bypass was limited. The majority of studies were small with 30/37 (81%) enrolling less than 100 patients, 29/37 (78%) were not randomised, and safety and efficacy endpoints differed widely, limiting the ability to combine data for meta-analyses.

Vasoactive and inotropic support remain critical parts of post-operative care for children after cardiopulmonary bypass surgery. There is a paucity of data for the selection and dosing of vasoactives and inotropes for these patients. Despite the knowledge gaps that remain, numerous recent innovations create opportunities to rethink the conduct of clinical trials in this high-risk population.

The management of fluid overload after congenital heart surgery has been limited to diuretics, fluid restriction, and dialysis. This study was conducted to determine the association between peritoneal dialysis and important clinical outcomes in children undergoing congenital heart surgery.

A retrospective review was conducted to identify patients under 18 years of age who underwent congenital heart surgery. The data were obtained over a 16-year period (1997–2012) from the Kids’ Inpatient Database. Data analysed consisted of demographics, diagnoses, type of congenital heart surgery, length of stay, cost of hospitalisation, and mortality. Logistic regression was performed to determine factors associated with peritoneal dialysis.

A total of 46,176 admissions after congenital heart surgery were included in the study. Of those, 181 (0.4%) utilised peritoneal dialysis. The mean age of the peritoneal dialysis group was 7.6 months compared to 39.6 months in those without peritoneal dialysis. The most common CHDs were atrial septal defect (37%), ventricular septal defect (32.6%), and hypoplastic left heart syndrome (18.8%). Univariate analyses demonstrated significantly greater length of stay, cost of admission, and mortality in those with peritoneal dialysis. Regression analyses demonstrated that peritoneal dialysis was independently associated with significant decrease in cost of admission (−$57,500) and significant increase in mortality (odds ratio 1.5).

Peritoneal dialysis appears to be used in specific patient subsets and is independently associated with decreased cost of stay, although it is associated with increased mortality. Further studies are needed to describe risks and benefit of peritoneal dialysis in this population.

Outcome data of doubly committed subarterial ventricular septal defect closure in adults are limited.

A review was made of the inpatients >18 years of age who underwent doubly committed subarterial ventricular septal defect closure between June 2010 and June 2017.

The patients were categorised into two groups: The valve intervention group consisted of 31 patients who underwent aortic valvuloplasty, aortic valve replacement, or repair of sinus Valsalva aneurysm in addition to doubly committed subarterial ventricular septal defect closure; non-valvular intervention group comprised 58 patients who underwent only doubly committed subarterial ventricular septal defect closure. The groups did not differ by sex and age. Patients in the valve intervention group, with a larger ventricular septal defect size, were shorter and tended to be lighter. The valve intervention group had more patients with pneumonia perioperatively. No infective endocarditis and reoperation were noted during the study period. At last follow-up, 91 and 96.6% of the studied patients were free from left ventricle dilation and pulmonary hypertension. In patients without pre-operative aortic regurgitation, 12 developed new mild aortic regurgitation during the follow-up.

About 34.8% of adult patients with doubly committed subarterial ventricular septal defect required concurrent intervention on aortic valve or sinus Valsalva aneurysm. The midterm results of doubly committed subarterial ventricular septal defect closure in adult patients were favourable. However, the incidence of new mild aortic regurgitation after ventricular septal defect closure was high (27.3%). Long-term follow-up of aortic regurgitation progression is needed.

N-terminal pro-brain natriuretic peptide has an established role in the diagnosis and prognosis of heart failure. In Fontan patients, this peptide is often increased, but its diagnostic value in this particular non-physiologic, univentricular circulation is unclear. We investigated whether N-terminal pro-brain natriuretic peptide represents ventricular function or other key variables in Fontan patients.

Ninety-five consecutive Fontan patients ≥10 years old who attended the outpatient clinic of the Center for Congenital Heart Diseases in 2012–2013 were included. Time since Fontan completion was 16 ± 9 years. Median N-terminal pro-brain natriuretic peptide was 114 (61–264) ng/l and was higher than gender-and age-dependent normal values in 54% of the patients. Peptide Z-scores were higher in patients in NYHA class III/IV compared to those in class I/II, but did not correlate with ventricular function assessed by MRI and echocardiography, nor with peak exercise capacity. Instead, peptide Z-scores significantly correlated with follow-up duration after Fontan completion (p < 0.001), right ventricular morphology (p = 0.004), indexed ventricular mass (p = 0.001), and inferior caval vein diameter (p < 0.001) (adjusted R2 = 0.615).

N-terminal pro-brain natriuretic peptide levels in Fontan patients correlate with functional class, but do not necessarily indicate ventricular dysfunction. Increased peptide levels were associated with a longer existence of the Fontan circulation, morphologic ventricular characteristics, and signs of increased systemic venous congestion. Since the latter are known to be key determinants of the performance of the Fontan circulation, these findings suggest increase in N-terminal pro-brain natriuretic peptide levels to indicate attrition of the Fontan circulation, independent of ventricular function.

Secundum atrial septal defect is one of the most common congenital heart defects. Previous paediatric studies have mainly addressed echocardiographic and few clinical factors among children associated with adverse events. The aim of this study was to identify neonatal and other clinical risk factors associated with adverse events up to one year after closure of atrial septal defect.

This retrospective case–control study includes children born in Sweden between 2000 and 2014 that were treated surgically or percutaneously for an atrial septal defect. Conditional logistic regression was used to evaluate the association between major and minor adverse events and potential risk factors, adjusting for confounding factors including prematurity, neonatal sepsis, neonatal general ventilatory support, symptomatic atrial septal defects, and pulmonary hypertension.

Overall, 396 children with 400 atrial septal defect closures were included. The median body weight at closure was 14.5 (3.5–110) kg, and the median age was 3.0 (0.1–17.8) years. Overall, 110 minor adverse events and 68 major events were recorded in 87 and 49 children, respectively. Only symptomatic atrial septal defects were associated with both minor (odds ratio (OR) = 2.18, confidence interval (CI) 95% 1.05–8.06) and major (OR = 2.80 CI 95% 1.23–6.37) adverse events.

There was no association between the investigated neonatal comorbidities and major or minor events after atrial septal defect closure. Patients with symptomatic atrial septal defects had a two to four times increased risk of having a major event, suggesting careful management and follow-up of these children prior to and after closure.

In children, the first episode of supraventricular tachycardia occurs at various ages. The aim of this study is to describe age-specific tachycardia mechanisms, clinical findings, and outcome in a contemporary cohort of paediatric patients with supraventricular tachycardia.

Retrospective analysis of 531 consecutive patients with structurally normal hearts under the age of 18 years who underwent invasive electrophysiological study for supraventricular tachycardia. The study population was divided into two groups, early-onset group (n = 57) and late-onset group (n = 474), according to the age of the occurrence of the first tachycardia before or after the age of 12 months.

Accessory pathway-mediated tachycardia was more common (82.5 versus 50.1%, p < 0.001) and the proportion of left-sided accessory pathways was more pronounced (74.5 versus 53.7%, p = 0.01) in the early-onset group than in the late-onset group. The antegrade and retrograde refractory periods of the accessory pathways were similar in both groups, but pre-excitation was more common in the early-onset group (50.9 versus 31.9%, p = 0.007). Typical atrioventricular nodal re-entrant tachycardia was more common (36.7 versus 7.0%, p < 0.001) in the late-onset group. There was no difference among the two groups regarding overall outcome.

Accessory pathway-mediated re-entrant tachycardia is the most common mechanism of recurrent supraventricular tachycardia in infants with structurally normal hearts who are later referred to an electrophysiological study. These pathways often cause pre-excitation and tend to be located on the left side whereas their refractory period is not different from that of patients with late-onset tachycardia.

Liver dysfunction, associated with morbidity and mortality, is common in patients with CHD. We investigate risk factors for and outcomes of hyperbilirubinaemia in neonates and infants after cardiac surgery.

In a retrospective analysis of neonates and infants undergoing cardiac surgery at our institution between January 2013 and December 2017, we identified those with post-operative conjugated hyperbilirubinaemia. We tested various demographic and surgical risk factors, and use of post-operative interventions, for an association with conjugated hyperbilirubinaemia. We also tested hyperbilirubinaemia for association with post-operative mortality and prolonged length of stay.

We identified 242 post-operative admissions, of which 45 (19%) had conjugated hyperbilirubinaemia. The average conjugated bilirubin level in this group was 2.0 mg/dl versus 0.3 mg/dl for peers without hyperbilirubinaemia. The post-operative use of both extracorporeal membrane oxygenation (OR 4.97, 95% CI 1.89–13.5, p = 0.001) and total parenteral nutrition (OR 2.98, 95% CI 1.34–7.17, p = 0.010) was associated with conjugated hyperbilirubinaemia. No demographic variable analysed was found to be a risk factor. Hyperbilirubinaemia was associated with higher odds of mortality (OR 3.74, 95% CI 2.69–13.8, p = 0.005) and prolonged length of stay (OR 2.87, 95% CI 2.02–7.97, p = 0.005), which were independent of other risk factors.

We identified the post-operative use of total parenteral nutrition and extracorporeal membrane oxygenation as risk factors for hyperbilirubinaemia. These patients were more likely to experience morbidity and mortality than control peers. As such, bilirubin may be marker for elevated risk of poor post-operative outcomes and should be more frequently measured after cardiac surgery.

To characterise the mortality and neurological outcomes of paediatric cardiac patients requiring cardiopulmonary resuscitation for more than 30 minutes prior to extracorporeal membrane oxygenation cannulation and to identify risk factors associated with adverse outcomes in this population.

Observational retrospective cohort study in paediatric cardiac patients undergoing cardiopulmonary resuscitation for greater than 30 minutes prior to cannulation in a tertiary children’s hospital, from July 2000 to July 2013.

Seventy-three paediatric cardiac patients requiring cardiopulmonary resuscitation for more than 30 minutes prior to cannulation were included in the study. Survival to hospital discharge was 43.8%, with 75% of survivors having either normal neurologic function or only mild disability. Multivariable logistic regression analysis demonstrated that increased use of calcium during resuscitation (odds ratio 14.5, p 0.01), cardiopulmonary resuscitation duration >50 minutes (odds ratio 4.12, p 0.03), >6 interruptions of chest compressions during cannulation (odds ratio 6.40, p 0.03), the need for continuous renal replacement therapy (odds ratio 11.1, p 0.001), and abnormal pupillary response during extracorporeal membrane oxygenation (odds ratio 33.9, p 0.006) were independent predictors for hospital mortality.

Survival after cardiopulmonary resuscitation for more than 30 minutes prior to extracorporeal membrane oxygenation cannulation in our paediatric cardiac cohort was 43.8%. Factors associated with mortality included calcium use during resuscitation, longer cardiopulmonary resuscitation, increased chest compression pauses during cannulation, the use of continuous renal replacement therapy, and abnormal pupils during extracorporeal membrane oxygenation support. A prospective assessment of these factors in paediatric cardiac patients may be beneficial in improving outcomes.

Left ventricular non-compaction is an architectural abnormality of the myocardium, associated with heart failure, systemic thromboembolism, and arrhythmia. We sought to assess the prevalence of left ventricular non-compaction in patients with single ventricle heart disease and its effects on ventricular function.

Cardiac MRI of 93 patients with single ventricle heart disease (mean age 24 ± 8 years; 55% male) from three tertiary congenital centres was retrospectively reviewed; 65 of these had left ventricular morphology and are the subject of this report. The presence of left ventricular non-compaction was defined as having a non-compacted:compacted (NC:C) myocardial thickness ratio >2.3:1. The distribution of left ventricular non-compaction, ventricular volumes, and function was correlated with clinical data.

The prevalence of left ventricular non-compaction was 37% (24 of 65 patients) with a mean of 4 ± 2 affected segments. The distribution was apical in 100%, mid-ventricular in 29%, and basal in 17% of patients. Patients with left ventricular non-compaction had significantly higher end-diastolic (128 ± 44 versus 104 ± 46 mL/m2, p = 0.047) and end-systolic left ventricular volumes (74 ± 35 versus 56 ± 35 mL/m2, p = 0.039) with lower left ventricular ejection fraction (44 ± 11 versus 50 ± 9%, p = 0.039) compared to those with normal compaction. The number of segments involved did not correlate with ventricular function (p = 0.71).

Left ventricular non-compaction is frequently observed in patients with left ventricle-type univentricular hearts, with predominantly apical and mid-ventricular involvement. The presence of non-compaction is associated with increased indexed end-diastolic volumes and impaired systolic function.

The Pediatric Heart Network Normal Echocardiogram Database Study had unanticipated challenges. We sought to describe these challenges and lessons learned to improve the design of future studies.

Challenges were divided into three categories: enrolment, echocardiographic imaging, and protocol violations. Memoranda, Core Lab reports, and adjudication logs were reviewed. A centre-level questionnaire provided information regarding local processes for data collection. Descriptive statistics were used, and chi-square tests determined differences in imaging quality.

For the 19 participating centres, challenges with enrolment included variations in Institutional Review Board definitions of “retrospective” eligibility, overestimation of non-White participants, centre categorisation of Hispanic participants that differed from National Institutes of Health definitions, and exclusion of potential participants due to missing demographic data. Institutional Review Board amendments resolved many of these challenges. There was an unanticipated burden imposed on centres due to high numbers of echocardiograms that were reviewed but failed to meet submission criteria. Additionally, image transfer software malfunctions delayed Core Lab image review and feedback. Between the early and late study periods, the proportion of unacceptable echocardiograms submitted to the Core Lab decreased (14 versus 7%, p < 0.01). Most protocol violations were from eligibility violations and inadvertent protected health information disclosure (overall 2.5%). Adjudication committee reviews led to protocol changes.

Numerous challenges encountered during the Normal Echocardiogram Database Study prolonged study enrolment. The retrospective design and flaws in image transfer software were key impediments to study completion and should be considered when designing future studies collecting echocardiographic images as a primary outcome.

Sickle cell anaemia is characterised by frequent, sometimes serious events referred to as “crisis”. Cardiopulmonary consequences such as pulmonary hypertension and myocardial ischaemia may accompany a serious crisis.

To determine the cardiovascular changes that occur during a severe sickle cell crisis.

A cross-sectional comparative study of sickle cell anaemia in children (5–17 years) admitted during a severe crisis (cases) and those in steady state (controls) was conducted over a 2-year period. Effects of the crisis on the cardiopulmonary system were assessed. The diagnosis of myocardial ischaemia was made using electrocardiography and serological cardiac biomarkers, while cardiac dysfunction and the presence of pulmonary hypertension were determined using echocardiography. The presence of systemic hypertension and tachycardia was also evaluated.

A total of 176 patients were recruited, 92 in steady state (male:female ratio, 1.2:1) and 84 in severe crisis (male:female ratio, 1.3:1). The mean age was 10.4 ± 3.2 years for steady state and 10.5 ± 3.4 years for those in crisis. The mean heart rate in crisis was higher than in steady state (p < 0.0001). The blood pressures (systolic, p < 0.0001, diastolic, p < 0.0001, mean, p < 0.0001) as well as myocardial ischaemia scores (p < 0.0001) were higher in patients with crisis than in those in steady state. Similarly, conduction abnormalities, pulmonary hypertension, and ventricular dysfunction were more prevalent in the crisis than in the steady state.

The present data suggest that sickle cell crisis results in a derangement of clinical, electrocardiographical, and echocardiographical parameters in children with sickle cell anaemia. Further research on these cardiovascular events may improve the overall care of these patients.

Studies have suggested 5–20% of paediatric ICU patients may receive care felt to be futile. No data exists on the prevalence and impact of futile care in the Paediatric Cardiac ICU. The aim is to determine the prevalence and economic impact of futile care.

Retrospective cohort of patients with congenital cardiac disease 0–21 years old, with length of stay >30 days and died (2015–2018). Documentation of futility by the medical team was retrospectively and independently reviewed.

Of the 127 deaths during the study period, 51 (40%) had hospitalisation >30 days, 13 (25%) had received futile care and 26 (51%) withdrew life-sustaining treatment. Futile care comprised 0.69% of total patient days with no difference in charges from patients not receiving futile care. There was no difference in insurance, single motherhood, education, income, poverty, or unemployment in families continuing futile care or electing withdrawal of life-sustaining treatment. Black families were less likely than White families to elect for withdrawal (p = 0.01), and Hispanic families were more likely to continue futile care than non-Hispanics (p = 0.044).

This is the first study to examine the impact of futile care and characteristics in the paediatric cardiac ICU. Black families were less likely to elect for withdrawal, while Hispanic families more likely to continue futile care. Futile care comprised 0.69% of bed days and little burden on resources. Cultural factors should be investigated to better support families through end-of-life decisions.

Childhood onset inflammatory bowel disease is more aggressive and has rapidly progressive clinical course than adult inflammatory bowel disease. Early-onset inflammatory bowel disease has more severe clinical progression as a subspecialised group of monogenic inflammatory bowel disease. We studied cardiac functions and aortic elasticity in children with early- and late-onset inflammatory bowel disease in remission period.

Thirty-three paediatric patients were divided into subgroups according to age of disease onset (<10 and >10 years of age). Twenty-five healthy children were admitted as control group. M-Mode echocardiography and pulsed wave Doppler echocardiography were performed. Strain, distensibility, stiffness index of ascending, and abdominal aorta were evaluated.

Interventricular septum (mm) and left ventricular end-systolic diameter were higher (6.9 ± 1.2, 26.2 ± 4.6) in early-onset inflammatory bowel disease patients than control patients (6.1 ± 1.27, 22.7 ± 4.12) (p = 0.050, p = 0.050). Mitral E/E′ ratio and myocardial performance index were increased in inflammatory bowel disease and early-onset inflammatory bowel disease groups than control group (p = 0.046, p = 0.04; p = 0.023, p = 0.033). Diastolic functions were found to be impaired in inflammatory bowel disease and early-onset inflammatory bowel disease groups according to control group, while there was no difference between late-onset inflammatory bowel disease and control groups in terms of diastolic functions. Mitral E/A ratio was lower in inflammatory bowel disease patients and early-onset inflammatory bowel disease patients (1.46 ± 0.32, 1.4 ± 0.21) than control patients (1.70 ± 0.27) (p = 0.013, p = 0.004). Aortic elasticity did not differ between groups.

Chronic low-grade inflammation has effects on left ventricular diameters and diastolic function in remission period. Aortic elasticity is not affected in our study groups.