Chylopericardium is the collection of lymph fluid inside the pericardial cavity. The incidence of chylopericardium is very low, as this diagnosis is rarely reported following cardiac procedures in children. While some reports were published worldwide on isolated chylopericardium after cardiac surgeries for diverse reasons, it has never been reported after repair for partial anomalous pulmonary venous return. In addition, management of this diagnosis ends up being surgical with minimal concentration on medical treatment which proved unsuccessful. We present a medical approach with corticosteroids as an effective method to treat isolated chylopericardium.

In this manuscript, we present an approach to treat isolated post-operative chylopericardium in a child following repair of partial anomalous pulmonary venous return. Chylous drainage responded to corticosteroids and completely ceased. There was no need for surgical intervention.

Until now, isolated chylopericardium has never been reported to occur with partial anomalous pulmonary venous return repair. A review of the literature showed that most patients follow a conservative approach consisting of diuretics and non-steroidal anti-inflammatory agents with some of them undergoing surgical re-intervention. With future research on the topic still needed, we hope that this will encourage physicians worldwide to consider administering a trial of corticosteroids as an option to treat chylopericardium.

The Fontan procedure is the final stage of surgical palliation for a single-ventricle circulation. Significant complications are common including rhythm disturbance necessitating implantation of a permanent pacemaker. This has been widely considered a negative prognostic indicator.

This single-centre, retrospective case control study involved all patients who underwent the Fontan procedure at the Leeds Congenital Heart Unit between 1990 and 2015 and have had regular follow-up in Yorkshire and Humber, United Kingdom. 167 Fontan patients were identified of which 2 were excluded for having a pre-procedure pacemaker. Of the remainder, 23 patients required a pacemaker. Outcomes were survival, early and late complications, need for further intervention and oxygen saturation in long-term follow-up.

There was no difference in survival (30-day survival pacemaker 92.6%, sinus rhythm 90.5%, p = 0.66, 1-year pacemaker 11.1%, sinus rhythm 10.1%, p = 1). The pacemaker group was more likely to have cerebral or renal complications in the first-year post-procedure (acute kidney injury: sinus rhythm 0.8%, pacemaker 19.1%, p = 0.002). No difference was observed in longer term complications including protein losing enteropathy (sinus rhythm 3.5%, pacemaker 0% p = 1). There was no difference in saturations between the two groups at follow-up. Paced patients were more likely to have required further intervention, with a higher incidence of cardiopulmonary bypass procedures (sinus rhythm 6.3%, pacemaker 35%, p < 0.001).

Despite an increase in early complications and the need for further interventions, pacemaker requirement does not appear to affect long-term survival following the Fontan procedure.

Pain control is an important element of care for patients after surgery, leading to better outcomes, quicker transitions to recovery, and improvement in quality of life. The purpose of this study was to evaluate the safety and efficacy of non-steroidal anti-inflammatory drugs in children after cardiac surgery

Patients between the ages of 1 month and 18 years of age, who received intravenous or oral non-steroidal anti-inflammataory drugs after cardiac surgery, from November 2015 until September 2017 were included in this study. The primary endpoints were non-steroidal anti-inflammataory drug-associated renal dysfunction and post-operative bleeding. Secondary endpoints examined the effect of non-steroidal anti-inflammataory drug use on total daily dose of narcotics, number of intravenous PRN narcotic doses received, and pain assessment score. Data were analysed using descriptive statistics for frequencies and ranges. Multivariate analysis was performed to measure the association of all predictors and outcomes. Wilcoxon singed-rank test was performed for secondary outcomes.

There was no association between the incidence of renal dysfunction and the use of or duration of non-steroidal anti-inflammataory drugs; in addition no association was found with increased chest tube output. There was a statistically significant reduction of patients’ median Face, Legs, Activity, Cry, Consolability (FLACC) scores (2–0; p = 0.003), seen within first 24 hours after initiation of ketorolac, and a significant reduction of morphine requirements seen from day 1 to day 2 (0.3 mg/kg versus 0.1 mg/kg; p < 0.001) and number of as-needed doses.

Non-steroidal anti-inflammataory drugs in paediatric cardiac surgery patients are safe and effective for post-operative pain management.

Both transposition of the great arteries (TGA) previously submitted to a Senning/Mustard procedure and congenitally corrected TGA (cc-TGA) have the systemic circulation supported by the morphological right ventricle, thereby rendering these patients to heart failure events risk. The aim of this study was to evaluate cardiopulmonary exercise test parameters for stratifying the risk of heart failure events in TGA patients.

Retrospective evaluation of adult TGA patients with systemic circulation supported by the morphological right ventricle submitted to cardiopulmonary exercise test in a tertiary centre. Patients were followed up for at least 1 year for the primary endpoint of cardiac death or heart failure hospitalisation. Several cardiopulmonary exercise test parameters were analysed as potential predictors of the combined endpoint and their predictive power were compared (area under the curve).

Cardiopulmonary exercise test was performed in 44 TGA patients (8 cc-TGA), with a mean age of 35.1 ± 8.4 years. The primary endpoint was reached by 10 (22.7%) patients, with a mean follow-up of 36.7 ± 26.8 months. Heart rate at anaerobic threshold had the highest area under the curve value (0.864), followed by peak oxygen consumption (pVO2) (0.838). Heart rate at anaerobic threshold ≤95 bpm and pVO2 ≤20 ml/kg/min had a sensitivity of 87.5 and 80.0% and a specificity of 82.4 and 76.5%, respectively, for the primary outcome.

Heart rate at anaerobic threshold ≤95 bpm had the highest predictive power of all cardiopulmonary exercise test parameters analysed for heart failure events in TGA patients with systemic circulation supported by the morphological right ventricle.

Neonates may require increased red cell mass to optimise oxygen content after stage 1 palliation; however, data informing transfusion practices are limited. We hypothesise there is a patient-, provider-, and institution-based heterogeneity in red cell transfusion decision-making after stage 1 palliation.

We conducted an online survey of Pediatric Cardiac Intensive Care Society practitioners in 2016. Respondents answered scenario-based questions that defined transfusion indications and identified haematocrit transfusion thresholds. Respondents were divided into restrictive and liberal groups based on a haematocrit score. Fisher’s exact test was used to determine the associations between transfusion likelihood and patient, provider, and institutional characteristics. Bonferroni correction was applied to adjust the p-value to 0.004 for multiple comparisons.

There was a 21% response rate (116 responses). Most were male (58.6%), attending physicians (85.3%) with >5 year of intensive care experience (88.7%) and subspeciality training in critical care medicine (47.4%). The majority of institutions were academic (96.6%), with a separate cardiac ICU (86.2%), and performed >10 stage 1 palliation cases annually (68.1%). After Bonferroni correction, there were no significant patient, respondent, or institutional differences between the restrictive and liberal groups. No respondent or institutional characteristics influenced transfusion decision-making after stage 1 palliation.

Decision-making around red cell transfusion after stage 1 palliation is heterogeneous. We found no clear relationships between patient, respondent, or institutional characteristics and transfusion decision-making among surveyed respondents. Given the lack of existing data informing red cell transfusion after stage 1 palliation, further studies are necessary to inform evidence-based guidelines.

Distinguishing between hypertrophic cardiomyopathy and other causes ofleft ventricular hypertrophy can be difficult in children. We hypothesised that cardiac MRI T1 mapping could improve diagnosis of paediatric hypertrophic cardiomyopathy and that measures of myocardial function would correlate with T1 times and extracellular volume fraction.

Thirty patients with hypertrophic cardiomyopathy completed MRI with tissue tagging, T1-mapping, and late gadolinium enhancement. Left ventricular circumferential strain was calculated from tagged images. T1, partition coefficient, and synthetic extracellular volume were measured at base, mid, apex, and thickest area of myocardial hypertrophy. MRI measures compared to cohort of 19 healthy children and young adults. Mann–Whitney U, Spearman’s rho, and multivariable logistic regression were used for statistical analysis.

Hypertrophic cardiomyopathy patients had increased left ventricular ejection fraction and indexed mass. Hypertrophic cardiomyopathy patients had decreased global strain and increased native T1 (−14.3% interquartile range [−16.0, −12.1] versus −17.3% [−19.0, −15.7], p < 0.001 and 1015 ms [991, 1026] versus 990 ms [972, 1001], p = 0.019). Partition coefficient and synthetic extracellular volume were not increased in hypertrophic cardiomyopathy. Global native T1 correlated inversely with ejection fraction (ρ = −0.63, p = 0.002) and directly with global strain (ρ = 0.51, p = 0.019). A logistic regression model using ejection fraction and native T1 distinguished between hypertrophic cardiomyopathy and control with an area under the receiver operating characteristic curve of 0.91.

In this cohort of paediatric hypertrophic cardiomyopathy, strain was decreased and native T1 was increased compared with controls. Native T1 correlated with both ejection fraction and strain, and a model using native T1 and ejection fraction differentiated patients with and without hypertrophic cardiomyopathy.

This prospective observational study evaluates the utility of expert focused cardiac ultrasound (eFCU) with spectral Doppler to inform decision making in established patients with specific, selected congenital cardiac defects in outreach clinics. Secondary objectives include determining if the addition of eFCU expands capacity in paediatric cardiology outreach clinics and if it improves the patient experience.

Patients aged 2 months to 19 years old with a diagnosis of ventricular septal defect, atrial septal defect, atrioventricular septal defect, patent ductus arteriosus, aortic valve stenosis, or pulmonary valve stenosis with the need for follow-up echocardiography in an outreach clinic from August 2017 to June 2018 were studied. A novel assessment tool was used to determine the success of eFCU.

Forty-two patients from 11 clinics underwent eFCU with one unsuccessful exam making the failure rate 2.3% (95% CI 0.0006–0.1256). Addition of eFCU led to a significant increase in volume of patients able to be seen 19 versus 15.5 (p < 0.01). A majority of parents/patients reported a positive experience with eFCU.

Expert focused cardiac ultrasound with spectral Doppler can be used successfully for follow-up in patients with select CHD and the addition of eFCU permits increased patient capacity in outreach clinics and has the potential to improve the patient experience.

The air gap technique (AGT) is an approach to radiation dose optimisation during fluoroscopy where an “air gap” is used in place of an anti-scatter grid to reduce scatter irradiation. The AGT is effective in adults but remains largely untested in children. Effects are expected to vary depending on patient size and the amount of scatter irradiation produced.

Fluoroscopy and cineangiography were performed using a Phillips Allura Fluoroscope on tissue simulation anthropomorphic phantoms representing a neonate, 5-year-old, and teenager. Monte Carlo simulations were then used to estimate effective radiation dose first using a standard recommended imaging approach and then repeated using the AGT. Objective image quality assessments were performed using an image quality phantom.

Effective radiation doses for the neonate and 5-year-old phantom increased consistently (2–92%) when the AGT was used compared to the standard recommended imaging approaches in which the anti-scatter grid is removed at baseline. In the teenage phantom, the AGT reduced effective doses by 5–59%, with greater dose reductions for imaging across the greater thoracic dimension of lateral projection. The AGT increased geometric magnification but with no detectable change in image blur or contrast differentiation.

The AGT is an effective approach for dose reduction in larger patients, particularly for lateral imaging. Compared to the current dose optimisation guidelines, the technique may be harmful in smaller children where scatter irradiation is minimal.

Prenatal diagnosis and planned peri-partum care is an unexplored concept for care of neonates with critical CHDs in low-middle-income countries.

To report the impact of prenatal diagnosis on pre-operative status in neonates with critical CHD.

Prospective observational study (January 2017–June 2018) in tertiary paediatric cardiac facility in Kerala, India. Neonates (<28 days) with critical CHDs needing cardiac interventions were included. Pre-term infants (<35 weeks) and those without intention to treat were excluded. Patients were grouped into those with prenatal diagnosis and diagnosis after birth. Main outcome measure was pre-operative clinical status.

Total 119 neonates included; 39 (32.8%) had prenatal diagnosis. Eighty infants (67%) underwent surgery while 32 (27%) needed catheter-based interventions. Pre-operative status was significantly better in prenatal group; California modification of transport risk index of physiological stability (Ca-TRIPS) score: median 6 (0–42) versus 8 (0–64); p < 0.001; pre-operative assessment of cardiac and haemodynamic status (PRACHS) score: median 1 (0–4) versus 3 (0–10), p < 0.001. Age at cardiac procedure was earlier in prenatal group (median 5 (1–26) versus 7 (1–43) days; p = 0.02). Mortality occurred in 12 patients (10%), with 3 post-operative deaths (2.5%). Pre-operative mortality was higher in postnatal group (10% versus 2.6%; p = 0.2) of which seven (6%) died due to suboptimal pre-operative status precluding surgery.

Prenatal diagnosis and planned peri-partum care had a significant impact on the pre-operative status in neonates with critical CHD in a low-resource setting.

In any country, the development of open heart surgery programme parallels stable political climate, economic growth, good leadership, and prudent fiscal management. This is lacking in a country like Nigeria without a functional cardiac hospital.

To review and compare the various models being adopted towards establishing a sustainable open heart surgery programme in Nigeria.

For ethnic and cultural reasons, Nigeria is divided into six geopolitical regions. Each region has one or more Federal Teaching Hospitals including medical centres. The hospitals have trained cardiothoracic surgeons and cardiologists as well as other auxiliary staff. After attainment of democratic rule in 1999, individual hospitals have devised various models to establish sustainable open heart surgery programme. The number of hospitals in each region, the models devised, and the limitations including the outcome were studied and analysed.

Each geopolitical zone has about three to four centres, either public or private, trying to establish the programme. There are six different models. Each centre has been trying the different models since the year 2000. The oldest of the model is cardiac mission and the newest is employment of highly skilled retired expatriate consultant cardiac surgeon to help develop the local team. Inadequate funds, lack of governmental support, and brain drain syndrome have largely affected the programme.

Anthracycline-associated cardiotoxicity in childhood cancer survivors may relate to global or segmental left ventricular abnormalities from associated thromboembolic events and myocardial microinfarcts. We characterized left ventricular segmental changes by two-dimensional speckle-tracking echocardiography in anthracycline-treated asymptomatic childhood cancer survivors.

Childhood cancer survivors’ echocardiograms with normal left ventricular fractional shortening >1 year after anthracycline chemotherapy were studied. Cancer-free control children had normal echocardiograms. Apical two-, three-, and four-chamber peak systolic left ventricular longitudinal and global longitudinal strain, and peak systolic left ventricular radial and circumferential strain at papillary muscle levels were analyzed. The mean (standard deviation) age was 12.7 (3.8) years in 41 childhood cancer survivors. The median (interquartile range) follow-up after anthracycline chemotherapy was 4.73 (2.15–8) years. The median (range) cumulative anthracycline dose was 160.2 (60–396.9) mg/m2. In childhood cancer survivors, the mean (standard deviation) left ventricular longitudinal strain was lower in two- (−18.6 [3.2] versus −21.3 [2.5], p < 0.001), three- (−16.3 [6.0] versus −21.7 [3.0], p < 0.001), and four- (−17.6 [2.7] versus −20.8 [2.0], p < 0.001) chamber views compared to controls. The left ventricular global longitudinal strain (−17.6 [2.7] versus −21.3 [2.0]) and circumferential strain (−20.8 [4.3] versus −23.5 [2.6], p < 0.001) were lower in childhood cancer survivors. Among childhood cancer survivors, 12 out of 16 left ventricular segments had significantly lower longitudinal strain than controls.

Asymptomatic anthracycline-treated childhood cancer survivors with normal left ventricular fractional shortening had lower global longitudinal and circumferential strain. The left ventricular longitudinal strain was lower in majority of the segments, suggesting that anthracycline cardiotoxicity is more global than regional.

Extracorporeal membrane oxygenation has been widely used after paediatric cardiac surgery due to increasing complex surgical repairs in neonates and infants having complex CHDs.

We reviewed retrospectively the medical records of all patients with CHD requiring corrective or palliative cardiac surgery at King Abdulaziz University Hospital that needed extracorporeal membrane oxygenation support between November 2015 and November 2018.

The extracorporeal membrane oxygenation population was 30 patients, which represented 4% of 746 children who had cardiac surgery during this period. The patients’ age range was from 1 day to 20.33 years, with a median age of 6.5 months. Median weight was 5 kg (range from 2 to 53 kg). Twenty patients were successfully decannulated (66.67%), and 12 patients (40%) were survived to hospital discharge. Patients with biventricular repair tended to have better survival rate compared with those with single ventricle palliation (55.55 versus 16.66%, p-value 0.058). During the first 24 hours of extracorporeal membrane oxygenation support, the flow rate was significantly reduced after 4 hours of extracorporeal membrane oxygenation connection in successfully decannulated patients.

Survival to hospital discharge in patients requiring extracorporeal membrane oxygenation support after paediatric cardiac surgery was better in those who underwent biventricular repair than in those who had univentricular palliation. Capillary leak on extracorporeal membrane oxygenation could be a risk of mortality in patients after paediatric cardiac surgery.

The Single Ventricle Reconstruction Trial randomised neonates with hypoplastic left heart syndrome to a shunt strategy but otherwise retained standard of care. We aimed to describe centre-level practice variation at Fontan completion.

Centre-level data are reported as median or median frequency across all centres and range of medians or frequencies across centres. Classification and regression tree analysis assessed the association of centre-level factors with length of stay and percentage of patients with prolonged pleural effusion (>7 days).

The median Fontan age (14 centres, 320 patients) was 3.1 years (range from 1.7 to 3.9), and the weight-for-age z-score was −0.56 (−1.35 + 0.44). Extra-cardiac Fontans were performed in 79% (4–100%) of patients at the 13 centres performing this procedure; lateral tunnels were performed in 32% (3–100%) at the 11 centres performing it. Deep hypothermic circulatory arrest (nine centres) ranged from 6 to 100%. Major complications occurred in 17% (7–33%). The length of stay was 9.5 days (9–12); 15% (6–33%) had prolonged pleural effusion. Centres with fewer patients (<6%) with prolonged pleural effusion and fewer (<41%) complications had a shorter length of stay (<10 days; sensitivity 1.0; specificity 0.71; area under the curve 0.96). Avoiding deep hypothermic circulatory arrest and higher weight-for-age z-score were associated with a lower percentage of patients with prolonged effusions (<9.5%; sensitivity 1.0; specificity = 0.86; area under the curve 0.98).

Fontan perioperative practices varied widely among study centres. Strategies to decrease the duration of pleural effusion and minimise complications may decrease the length of stay. Further research regarding deep hypothermic circulatory arrest is needed to understand its association with prolonged pleural effusion.

To perform the preliminary tests of coarctation of aorta repair trainer, evaluate the surgical properties of the simulation and to assess and enhance residents’ skills.

Single patient’s angio-CT anatomy data were converted into magnified 3D-printed model of aortic coarctation with hypoplastic aortic arch, serving for creation of a mould used during wax copies casting. Wax cores were painted with six layers of elastic silicone and melted, yielding phantoms that were consecutively fixed in a mounting with and without a thoracic wall. Simulation included: proximal and distal aortic arch clamping, incision of its lesser curvature, extended end-to-end anastomosis with 7-0 suture. A head-mounted camera video recording enabled anastomosis time and mean one suture bite time evaluation. Leakage assessment was done by a water test.

Two residents performed nine simulations each. Last four runs were performed with thoracic wall attached. All phantoms performed well, enabling tissue-like handling and cutting, excellent suture retention, and satisfactory elasticity. Median anastomosis times were 22′33″ and 24′47″ for phantoms without and with thoracic wall (p = not significant (NS)). Median times needed to pass suture through one side of anastomosis and regrasp needle were, respectively, 9″ and 13″ (p < 0.001). Median total number of leakages per phantom equalled 2 for both difficulty levels. There were no significant inter-resident differences in all assessed parameters.

This medium-fidelity aortic coarctation repair trainer showed its feasibility in replication of major critical steps of the real operation. Objective surgical efficiency parameters could be obtained from each simulation and compared between trainees and at different adjustable difficulty levels.