To describe the impact of CHD surgery in early childhood on quality of life in children aged 10–16 years with surgically corrected Ventricular Septal Defect, Transposition of the Great Arteries, and Tetralogy of Fallot.

A cross-sectional survey study of quality of life survey on 161 children and adolescents aged 10–16 years with surgically corrected Ventricular Septal Defect, Transposition of the Great Arteries, and Tetralogy of Fallot. The international Paediatric Quality of Life 4.0 quality of life questionnaires were applied and collected for assessment from patients and parents. The endpoints were total, physical, emotional, social, and school quality of life scores.

The quality of life total and school scores was significantly lower in children with CHD than their healthy peers. There was no significant difference in quality of life between the three CHD groups. All three CHD groups had a significantly lower total (7.7–13.2%, p<0.001) and school scores (21.1–31.6%, p<0.001) than the control group. The tetralogy of Fallot group was the only group that had significantly lower scores in the physical subscale (p<0.001) than the controls.

Children and adolescents with surgically corrected CHD show losses in quality of life in total and school scores compared to healthy controls. The tetralogy of Fallot group was the only CHD group that had significantly lower physical score than the controls.

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.

To study physical activity and sleep in Fontan patients and healthy controls before and after an endurance training program, and after 1 year.

Fontan patients (n = 30) and healthy controls (n = 25) wore accelerometers for seven consecutive days and nights during a school week before and after a 12-week endurance training program and after 1 year.

Patients had similar sleep duration and sleep efficiency as healthy controls. Latency to sleep onset in minutes was longer for patients than controls (22.4 (4.3–55.3) minutes versus 14.8 (8.6–29.4) minutes, p < 0.01). More time in moderate-to-vigorous activity daytime was correlated with increased sleep time (p < 0.05; r2 = 0.20), improved sleep efficiency (p < 0.01; r2 = 0.24) and less time as wake after sleep onset (p < 0.05; r2 = 0.21) for patients but not controls. Sleep variables did not change after the exercise intervention for patients or controls. After 1 year, patients had decreased total sleep time, decreased sleep efficiency, increased accelerometer counts during sleep and more time as wake after sleep onset during sleep time, but not controls.

Fontan patients have prolonged latency to sleep onset compared with controls. More time in physical activities was correlated with better sleep quality for the patients. Also, subjects with low sleep efficiency and long latency to sleep onset may benefit most from physical exercise. These patients should be encouraged to engage in individually designed physical exercise as this could improve sleep quality.

To assess the effectiveness of radiofrequency catheter ablation for lone atrial fibrillation in young adults.

This single-centre, retrospective, observational study enrolled 75 consecutive patients (86.7% men) under 35 (median, 30) years old with lone atrial fibrillation (68% paroxysmal, 26.7% persistent, and 5.3% long-standing persistent) without other cardiopulmonary diseases who underwent catheter ablation between April 2009 and May 2017. Procedural endpoints were circumferential pulmonary vein ablation for atrial fibrillation with pulmonary vein trigger, and target ablation or bidirectional block of lines and disappearance of complex fractionated atrial electrograms for atrial fibrillation with clear and unclear non-pulmonary vein triggers, respectively.

Main study outcome was rate of survival free from atrial tachyarrhythmia recurrence, which at median 61 (range, 5–102) months follow-up was 62.7% (64.7 and 58.3% for paroxysmal and non-paroxysmal atrial fibrillation, respectively) after single ablation, and 69.3% (68.6 and 70.8% for paroxysmal and non-paroxysmal atrial fibrillation, respectively) after mean 1.2 ablations (two and three ablations in 11 and 2 patients, respectively). In multivariate analysis, non-pulmonary vein trigger was a significant independent predictor of recurrent atrial tachyarrhythmia (OR, 10.60 [95%CI, 2.25–49.96]; p = 0.003). There were no major periprocedural adverse events.

In patients under 35 years old with lone atrial fibrillation, radiofrequency catheter ablation appeared effective particularly for atrial fibrillation with pulmonary vein trigger and regardless of left atrial size or atrial fibrillation duration or type. Atrial tachyarrhythmia recurrence after multiple ablations warrants further study.

Although sleep apnoea is an important disorder associated with cardiac events, data regarding its prevalence and risk factors in adult patients with CHD are limited.

In this study, patients underwent a sleep study in the hospital. Indications for admission were classified as heart failure, diagnostic catheterisation, interventional catheterisation, or arrhythmia. The prevalence, characteristics, and risk factors of sleep apnoea using a type 3 portable overnight polygraph in adult patients with CHD were evaluated.

This study comprised 104 patients of median age 36 years with interquartile range of 28–48 years, admitted for heart failure 34% (n = 36), diagnostic catheterisation 26% (n = 27), interventional catheterisation 18% (n = 19), or arrhythmia 22% (n = 23). The prevalence of sleep apnoea, defined as a respiratory disturbance index ≥5, was 63% (n = 63), with a distribution of 37, 16, and 10% for mild (5≤ respiratory disturbance index <15), moderate (15⩽ respiratory disturbance index <30), and severe (respiratory disturbance index ≥30) sleep apnoea, respectively. A large majority of the sleep apnoea cases were categorised as obstructive sleep apnoea (92%, n = 58). The respiratory disturbance index ≥15 group had a significantly higher proportion of male patients and higher body mass index, noradrenaline level, and aortic blood pressure than the group without sleep apnoea (respiratory disturbance index <5). Multi-variable analysis showed that NYHA class ≥II, whose odds ratio 4.36, 95% confidence interval 1.09–20.87, and body mass index ≥25, whose odds ratio 4.29, 95% confidence interval 1.32–15.23, were independent risk factors for a respiratory disturbance index ≥15.

Our results showed a high prevalence of sleep apnoea in adult patients with CHD. Its unique haemodynamics may be associated with a high prevalence of sleep apnoea. Congestive heart failure and being overweight are important risk factors for sleep apnoea. Management of heart failure and general lifestyle improvements are important for controlling sleep apnoea symptoms in these patients.

Kawasaki disease is an acute vasculitis of childhood and is the leading cause of acquired heart disease in the developed countries.

Data from hospital discharge records were obtained from the National Kids Inpatient Database for years 2009 and 2012. Hospitalisations by months, hospital regions, timing of admission, insurance types, and ethnicity were analysed. Length of stay and total charges were also analysed.

There were 10,486 cases of Kawasaki disease from 12,678,005 children hospitalisation. Kawasaki disease was more common between 0 and 5 years old, in male, and in Asian. The January–March quarter had the highest rate compared to the lowest in the July–September quarter (OR=1.62, p < 0.001). Admissions on the weekend had longer length of stay [4.1 days (95 % CI: 3.97–4.31)] as compared to admissions on a weekday [3.72 days (95 % CI: 3.64–3.80), p < 0.001]. Blacks had the longest length of stay and whites had the shortest [4.33 days (95 % CI: 4.12–4.54 days) versus 3.60 days (95 % CI: 3.48–3.72 days), p < 0.001]. Coronary artery aneurysm was identified in 2.7 % of all patients with Kawasaki disease. Children with coronary artery aneurysm were hospitalised longer and had higher hospital charge. Age, admission during weekend, and the presence of coronary artery aneurysm had significant effect on the length of stay.

This report provides the most updated epidemiological information on Kawasaki disease hospitalisation. Age, admissions during weekend, and the presence of coronary artery aneurysm are significant contributors to the length of stay.

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.

Left atrial congestion results from backward failure in dilated cardiomyopathy. We aimed to evaluate feasibility and efficacy of percutaneous atrioseptostomy to create a restrictive atrial septum defect in management of dilated cardiomyopathy.

From June 2009 to December 2016, 27 interventions comprised left atria decompressions in 22 dilated cardiomyopathy patients; 9 females; age: 24 days to 36.9 years; weight: 3–50 kg; NYHA-/Ross class IV (n=16). Mean left ventricular ejection fraction was 21.5±9.7% and brain natriuretic peptide was 2291±1992 pg/ml. Dilated cardiomyopathy was classified as chronic (n=9); acute (n=1) myocarditis; idiopathic (n=5); left ventricular non-compaction (n=4); mitochondriopathy, pacemaker induced, and arrhythmogenic (n=3). Atrioseptostomy was concomitantly performed with myocardial biopsies 6.5 days (±11.7) after admission (n=11). Trans-septal puncture was used in 18 patients; foramen ovale dilatation was done in four patients. Mean balloon size was 11 mm (range 7–14 mm); total procedure time was 133±38 minutes. No procedural complications were observed. Mean left atrial pressure decreased from 15.8±6.8 to 12.2±4.8 mmHg (p=0.005), left/right atrial pressure gradient from 9.6±5.6 to 5±3.5 mmHg; brain natriuretic peptide (n=18) decreased from 1968±1606 to 830±1083 pg/ml (p=0.01). One patient unsuitable for heart transplantation died at home despite additionally performed pulmonary artery banding and three further left atrial decompressions; five patients were bridged to transplantation, two died afterwards. Functional recovery occurred in the remaining 14 patients and in six after additional pulmonary artery banding. No patient required assist device.

Percutaneous left atrial decompression is an age-independent, effective palliation treating patients with dilated cardiomyopathy.

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.

This study was performed to explore the clinical features of Kawasaki disease shock syndrome and analyse the association between the left ventricular ejection fraction and Kawasaki disease shock syndrome.

We retrospectively reviewed the medical records of all consecutive inpatients with Kawasaki disease at Wenzhou Medical University Second Affiliated Hospital and Yuying Children’s Hospital in Wenzhou, China from January 2009 to December 2016. We compared the clinical characteristics, laboratory data, and left ventricular ejection fraction between patients with and without Kawasaki disease shock syndrome and analysed the effect of the left ventricular ejection fraction on Kawasaki disease shock syndrome under different clinical conditions of Kawasaki disease.

In total, 1147 patients were diagnosed with Kawasaki disease. Of these 1147 patients, 17 were diagnosed with Kawasaki disease shock syndrome; 68 patients admitted to the hospital at the same time, ±2 weeks, with Kawasaki disease but without Kawasaki disease shock syndrome served as the control group. Compared with the control group, the Kawasaki disease shock syndrome group had a significantly higher incidence of coronary artery lesions, cardiac troponin I concentration, N-terminal prohormone of brain natriuretic peptide concentration, neutrophil count and ratio, alanine aminotransferase concentration, aspartate aminotransferase concentration, and C-reactive protein concentration and a significantly lower platelet count, serum albumin concentration, and left ventricular ejection fraction. A low left ventricular ejection fraction was associated with Kawasaki disease shock syndrome under different conditions of Kawasaki disease.

Among patients with Kawasaki disease, cardiac injury is more likely in those with Kawasaki disease shock syndrome than without, and a low left ventricular ejection fraction may be associated with the development of Kawasaki disease shock syndrome.