This study aimed to assess the results of endomyocardial biopsy from the right ventricle to establish the possible cause for drug-refractory arrhythmias in children.

We enrolled 19 consecutive young patients with drug-refractory arrhythmia, from 2010 to 2013, who underwent endomyocardial biopsy. Inclusion criteria were as follows: age <18 years with a structurally normal heart or mild changes in a structure of the heart initially diagnosed as arrhythmia-induced cardiomyopathy. Overall, 86 biopsies were performed in 19 patients. Histopathological analysis, immunohistochemistry, and polymerase chain reaction were used for the interpretation of the endomyocardial biopsy.

The mean age of the patient population was 14.1±2.9 year (range from 7 to 17 years). All these patients had a history of drug-refractory arrhythmia for >5 months (mean 30 months). Patients underwent a complete history investigation, physical examination, laboratory studies, echocardiography, electrocardiography, treadmill test, and Holter monitoring before endomyocardial biopsy; two patients with arrhythmogenic right ventricular dysplasia had implantable cardioverter defibrillator implantation and further appropriate successful device shocks. Myocarditis was diagnosed based on histopathological and immunohistological analyses in nine (47.4%) patients. Polymerase chain reaction was positive for viral genome in four of them; five patients had active myocarditis. Radiofrequency ablation was performed in 17 patients; five out of six (83%) endomyocardial biopsy-proved myocarditis patients had successful radiofrequency ablation. No significant complication was reported during ablation and endomyocardial biopsy.

Approximately half of the children with drug-refractory arrhythmia had unsuspected myocarditis according to the results of the endomyocardial biopsy.

Canadian Cardiovascular Society consensus guidelines recommend that tetralogy of Fallot patients be seen by a congenital cardiologist every 2 years. In Atlantic Canada, tetralogy of Fallot patients are followed up at either tertiary or satellite clinics, which are held in the community and attended by paediatric cardiologists. The effectiveness of satellite clinics in congenital cardiac disease follow-up is unproven. Our objective was to compare patient-reported quality of life measures to determine whether these were impacted by the site of follow-up.

We included patients with tetralogy of Fallot undergoing surgical repair at the Izaak Walton Killam Health Centre from 1 November, 1972 to 31 May, 2002. Quality of life surveys, SF-10 or SF-36v2, were administered to consenting patients. We analysed the subjective health status by patient age and site of follow-up.

Of the 184 eligible patients, 72 were lost to follow-up. Of the locatable patients, 61% completed the questionnaires. In all, 90% (101 out of 112) were followed up at recommended intervals. Of the 112 (68%) patients, 76 were followed up at a tertiary clinic. These patients were older, with a mean age of 18.4 years versus 14.7 years, and scored higher on the SF-36 physical component summary (52.6 versus 45.7, p = 0.02) compared with satellite clinic patients. The SF-36 mental component summary scores were similar for patients regardless of the site of follow-up. SF-10 physical and psychosocial scores were similar regardless of the site of follow-up.

Tetralogy of Fallot patients followed at either satellite or tertiary clinics have similar subjective health status.

To assess the outcomes of congenital tracheal stenosis among children.

A retrospective review of all children who underwent surgical repair of congenital tracheal stenosis reviewing charts, operative notes, echocardiograms, CT and MRI data from January 2002 to February 2019.

Twenty-six children underwent surgical treatment for tracheal stenosis. The median age was 3 months (range 0.3–35 months) and the median weight was 4.7 kg (range 2.5–13 kg) at the time of surgical intervention. Stridor was the most common presenting symptom in 17 patients (65% of patients). Twenty-one patients (81%) had concurrent cardiac anomalies, with pulmonary arterial sling being the most common, present in nine patients (34%). Extracorporeal life support was utilised in seven patients (27%) pre-operatively. Laryngeal release was required in 16 patients. In 7 patients an end-to-end anastomosis was performed, in 18 patients slide tracheoplasty, and 1 patient had a double slide tracheoplasty. The median cardiopulmonary bypass time was 106 minutes (range 25–255 minutes). The median cross-clamp time was 30 minutes (range 5–67 minutes). The median post-operative duration of ventilation was 5 days (range 0.5–16 days). The median ICU length of stay was 12.5 days (range 2–60 days). There were three hospital mortalities with 88% survival. One patient only required reintervention with balloon dilation. Twenty-two patients (85%) remained symptom-free on median follow-up at 7.6 years (range 0.2–17 years). Two patients since 2017 had 3D printed tracheas produced from CT imaging to assist surgical planning.

Congenital tracheal stenosis can be managed effectively with excellent outcomes and 3D printed models assist in planning the optimal surgical intervention.

CHD is defined as structural defect(s) in the heart and proximal blood vessels present at birth. The National Cardiothoracic Center of Excellence, University of Nigeria Teaching Hospital (UNTH), Enugu, through the aid of visiting Cardiac Missions has managed a significant number of patients within the last 3.5 years.

The objective of this study was to review surgical options and outcome of complex CHD among patients attending UNTH, Enugu, Enugu.

During the period of 3.5 years (March, 2013 to June, 2016), a total of 20 cases of complex CHD were managed by cardiac missions that visited UNTH, Enugu. Their case notes and operating register were retrieved, reviewed, and analysed using SPSS version 19 (Chicago).

There were eight females and 12 males, with a ratio of 2:5. The age range was from 5 months to 34 years with a mean of 1.7. Among all, five patients died giving a mortality rate of 25%. The operative procedures ranged from palliative shunts to complete repair. The outcome was relatively good.

Complex CHD are present in our environment. Their surgical management in our centre is being made possible by periodic visits of foreign cardiac missions.

To investigate UK variability in prenatal and postnatal management strategy of right aortic arch and double aortic arch (RAA/DAA).

Online surveys were sent to senior physicians (consultants) of the National Fetal Cardiology Working Group regarding prenatal diagnosis, counselling, and perinatal management of antenatally diagnosed RAA/DAA and to the British Congenital Cardiovascular Association regarding postnatal management strategies.

There were 28 prenatal and 90 postnatal surveys completed. Prenatally, there was consensus for potential associated chromosomal/genetic anomalies, but there was variation in the risk quoted. Confidence in defining aortic arch morphology was reported by 43% (12/28) of fetal cardiologists. There was variation in what was felt to be possible symptoms/signs of a compressive vascular ring, postnatal investigation, postnatal management, follow-up duration of asymptomatic patients, and indications for surgical intervention.

This study has highlighted important areas for future research: improving accuracy of prenatal diagnosis, clarification of potential symptoms, optimal investigation strategies, and indications for 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.

Parents may experience anxiety and stress when their children undergo cardiac catheterisation. The goal of this study was to assess the level of anxiety in parents of children undergoing cardiac catheterisation and to identify factors that were associated with level of anxiety.

This was a cross-sectional survey of parents of children who underwent cardiac catheterisation. Anxiety levels were measured using a validated self-report questionnaire – State-Trait Anxiety Inventory, which generates state anxiety scores on the current state of anxiety and trait anxiety scores on the stable aspects of anxiety proneness. One sample t-test was used to compare the data with normative data. Multiple linear regression was used to assess the factors associated with the state score.

A total of 113 parents completed the survey. The mean age of parents was 34.0±7.7 years and the mean age of children undergoing catheterisation was 6.7±5.7 years. Compared with normative data, mean state score was significantly higher in our cohort (p<0.05) despite no difference in the trait score. Final multivariate model showed that the state score was significantly associated with child age group (<1 year [coefficient β 7.2] and 10–18 years [6.3], compared to 1 to <10 years of age [reference]) and history of previous catheterisation (−5.2) (p<0.05).

Concurrent state anxiety level was high among parents of children undergoing cardiac catheterisation, whereas trait anxiety level was not. Higher anxiety was experienced by parents of infants and adolescents without a history of previous catheterisation.

Systemic inflammation has been suggested to underlie in part the elevated risk of arrhythmias and myocardial dysfunction during the first weeks after cardiac surgery. Recent transthoracic Doppler studies from our centre indicated increased basal coronary arterial flow in children 5 days after cardiopulmonary bypass surgery. In these children, we investigated whether the inflammatory mediator, C-reactive protein, could influence this association.

The peak flow velocity, velocity time-integral in diastole and systole, and basal blood flow in the proximal part of the left anterior descending artery, were assessed by transthoracic Doppler echocardiography 1 day before, and 5 days after, cardiac surgery in 17 children with ventricular and atrioventricular septal defects whose mean age at surgery was 6 months. Levels of C-reactive protein in the plasma were measured at both time-points.

Prior to surgery, all children had levels of C-reactive protein under the limit for detection, that is less than 0.8 milligrams per litre. The levels of the protein had increased significantly by the second day, when the median value was 25, and the range from 20 to 142 milligrams per litre. They remained elevated on the fifth day after surgery, when the median was 11, and the range from 3 to 20 milligrams per litre. On the fifth day, the percentage increase in velocity time integral corrected for left ventricular mass was significantly lower in those patients with C-reactive protein greater than or equal to 10 milligrams per litre than in the remaining patients. Also, both the velocity time integral and the velocity of diastolic peak flow correlated inversely with log C-reactive protein, r being equal to −0.54 and p less than 0.02 and r equal to −0.74 and p less than 0.01, respectively, particularly among those patients in whom clamping of the aorta lasted for more than 1 hour, r for this statistic being equal to −0.8.

The postsurgical increase in the velocity of coronary arterial flow in children is inversely associated with rising levels of C-reactive protein. The duration of the aortic cross-clamping during surgery strengthens the association between levels of C-reactive protein and the microcirculatory changes.

To describe endovascular stent placement using partially covered stents to preserve flow in head and neck vessels.

Endovascular stent placement has become established as a first-line therapy for native coarctation of the aorta or re-coarctation in older children and adults. Increasingly covered stents are becoming the preferred option over bare-metal stents because of the perceived lower risk of aneurysm formation. Open-cell bare-metal stents are chosen when there is a high likelihood of jailing a head and neck vessel. Here we describe partial uncovering of a covered stent before implantation to allow flow through the uncovered portion of the stent to the branch vessel but preserve the covering over the majority of the remaining stent.

We describe two cases with aortic arch hypoplasia and re-coarctation, both of which required two partially uncovered stents for a satisfactory result.

Endovascular stent placement is becoming the preferred option in the management of coarctation of the aorta in older children and adults. Strategies to deal with transverse arch hypoplasia and multiple levels of aortic arch obstruction frequently involving branch vessels or aneurysms need to be considered before these procedures are embarked upon. Partially uncovering stents may afford more protection than using bare-metal stents in the transverse and distal arch while preserving flow in head and neck branches, and is a technically straightforward procedure.