Children with congenital cardiac disease experience challenges in developing healthy patterns of physical activity due to decreased exercise capacity and parental fear and confusion about what is permissible. The purpose of this study was to describe physical activity habits in children 10–14 years of age with congenital cardiac disease and the relationship of those habits to obesity as defined by body mass index.

This cross-sectional study used self-report measures and clinical data to describe the association between physical activity participation and body mass index in 10- to 14-year-old children with congenital cardiac disease. Further, physical activity levels were compared between children who were overweight or obese and those who were not.

Children (n = 84; 51 males; 33 females) reported low rates of physical activity compared to reports on healthy children. Only 9.5% were overweight (body mass index between the 85th and 94th percentile), and alarmingly 26% were obese (body mass index at or above the 95th percentile). Physical activity and body mass index were not significantly correlated (r = −0.11, p = 0.45) and there was no significant difference in mean physical activity (t = 0.67) between children who were overweight or obese and those who were not.

Children in this study reported low rates of physical activity and a higher obesity rate than was reported in previous studies. However, the two were not significantly correlated. Further research is indicated to determine the specific factors contributing to obesity and to test interventions to combat obesity in children with congenital cardiac disease.

The use of imaging that employs ionising radiation is increasing in the setting of paediatric cardiology. Children's high radiosensitivity and the lack of contemporary radiation data warrant a review of the radiation doses from the latest “state-of-the-art” angiography and computed tomography systems.

In children aged less than 16 years with congenital cardiac disease, we aimed to report: recent trends in the use of diagnostic angiography and cardiac dual-source computed tomography; the characteristics, lesions, and imaging histories of patients undergoing these procedures; and the average radiation doses imparted by each modality.

Retrospective review of consecutive cases undergoing cardiac computed tomography or diagnostic angiography in a teaching hospital between January, 2008 and December, 2009. Radiation doses were converted to effective doses (millisievert) using published conversion factors.

Angiography was performed 3.7 times more often than computed tomography. Computed tomography examinations increased by 92.5%, whereas angiography decreased by 26.4% in 2009 compared with 2008. Patients undergoing computed tomography were younger and weighed less than those undergoing angiography, but lesions were similar between the 2 groups. Multiple lifetime angiography was more prevalent than multiple lifetime computed tomography (p < 0.001). The median procedural dose – range – from angiography and computed tomography was 5 (0.2–27.8) and 1.7 (0.5–9.5) millisieverts, respectively (p < 0.001).

Despite not being completely analogous investigations, computed tomography should be considered prior to angiography and not withheld on radiation dose concerns, given that it imparts lower and more consistent doses than conventional angiography.

Differentiation of the so-called sinus venosus defect from other defects permitting shunting between the atrial chambers remains problematic. The lesion is not a true septal defect, and current theories to explain the existence of the sinus venosus defect fall short. The presence of persistent systemic to pulmonary venous connections has been proposed to explain the existence of the sinus venosus defect.

Clinical histories and radiological findings of six patients are reviewed. Three patients have veno-venous bridges, two have partial anomalous pulmonary venous connections, and one patient has a sinus venosus defect. The clinical information is reviewed, along with current developmental and morphological considerations.

We provide radiographic, developmental, and morphological evidence to support the theory that a so-called sinus venosus defect is the consequence of persistence of foetal systemic to pulmonary veno-venous bridges, rather than of deficiencies in atrial septation.

This study evaluates the efficacy and safety of sildenafil in patients with Eisenmenger's syndrome with special emphasis on haemodynamic parameters and its comparative efficacy in atrial septal defect versus ventricular septal defect patients.

Oral sildenafil was given to 22 patients with Eisenmenger's syndrome – eight with atrial septal defect and 14 with ventricular septal defect – after detailed baseline evaluation including a six-minute walk test, echocardiography, and cardiac catheterisation. Patients were followed up for a period of 6 months for functional class assessment and six-minute walk distance. Cardiac catheterisation was repeated in all patients.

A significant improvement in the World Health Organization functional class, six-minute walk distance, mean pulmonary arterial pressure, and pulmonary vascular resistance was noticed. Systemic arterial and mixed venous oxygen saturations were also significantly improved along with improvement in pulmonary blood flow. None showed any significant side effects or worsening of systemic arterial saturation. At baseline, mean pulmonary arterial pressure, pulmonary vascular resistance, and pulmonary/systemic vascular resistance ratios were significantly higher in ventricular septal defect patients than in atrial septal defect patients. Atrial septal defect patients showed better response in clinical as well as haemodynamic parameters.

Sildenafil is an effective and safe agent for patients with Eisenmenger's syndrome. It improves their functional capacity as well as haemodynamic parameters. The beneficial effects are greater in patients with Eisenmenger's syndrome secondary to atrial septal defect than ventricular septal defect.

Successful paediatric cardiac surgery and cardiology treatment has resulted in an increase in the use of surgery as a method of treatment of congenital cardiac disease in adult population. However, late detection and lower socio-economic condition in developing countries might change patients’ characteristics by the time they come for treatment. This study aimed to elaborate the long-term surgical results of adult congenital cardiac disease in Indonesia as a developing country.

We reviewed retrospectively all adult congenital cardiac disease patients with a mean age of 28 years plus or minus 9.5 years, who underwent surgery at National Cardiovascular Center. The types of procedures used were corrective in 338 patients (89.2%), palliative in 10 patients (2.6%), and re-operations in 31 patients (8.2%). The overall hospital mortality rate was 2.6% but as high as 20% with palliative surgery. Post-operative New York Heart Association class III–IV is the only independent predictor of death at 60 months (hazard ratio 61.48, 95% confidence interval 9.41–401.69, p<0.001). The survival rates were 96.3% and 95% for overall and non-atrial septal defect in patients at 60 months, which was highest in corrective procedures (97.6%). The percentage of patients free of re-operation at 5 years’ follow-up was 85.4% and 42.7% at 10 years.

In developing countries, surgical treatment of adult congenital cardiac disease is effective and safe, with an overall survival rate of 96.3% at 60 months. Due to high mortality rate, palliative surgery of a non-atrial septal defect patient is recommended to be discontinued. The independent predictor of mortality was post-operative New York Heart Association functional class III–IV.

Congenital cardiac disease is the most common malformation, and a substantial source of mortality and morbidity in children and young adults. A role for genetic factors is recognised for these malformations, but overall few predisposing loci have been identified. Here we report the rationale, design, and first results of a multi-institutional congenital cardiac disease cohort, assembled mainly from the French-Canadian population of the province of Quebec and centred on families with multiple affected members afflicted by cardiac malformations.

Families were recruited into the study, phenotyped and sampled for DNA in cardiology clinics over the first 3 years of enrolment. We performed segregation analysis and linkage simulations in the subgroup of families with left ventricular outflow tract obstruction (LVOTO).

A total of 1603 participants from 300 families were recruited, with 169 out of 300 (56.3%) families having more than one affected member. For the LVOTO group, we estimate heritability to be 0.46–0.52 in our cohort. Simulation analysis demonstrated sufficient power to carry out linkage analyses, with an expected mean log-of-odds (LOD) score of 3.8 in 67 pedigrees with LVOTO.

We show feasibility and usefulness of a population-based biobank for genetic investigations into the causes of congenital cardiac disease. Heritability of LVOTO is high and could be accounted for by multiple loci. This platform is ideally suited for multiple analysis approaches, including linkage analysis and novel gene sequencing approaches, and will allow to establish segregation of risk alleles at family and population levels.

Classical Atriopulmonary Fontan connections tend to fail in the long term due to progressive anastomotic site obstruction, right atrial enlargement, and refractory atrial arrhythmias. Conversion to total cavopulmonary connection with concomitant arrhythmia surgery is a promising treatment but optimal timing of the procedure remains controversial.

Between the years 2002 and 2009, 15 patients with a median age of 26.2 (12–43) years underwent Fontan conversion operation with concomitant arrhythmia surgery. All were symptomatic and 14 out of the 15 patients had refractory arrhythmias. The duration of pre-operative arrhythmia and the outcome of surgery were correlated to study the impact of delay in surgical intervention on post-operative survival and arrhythmia control.

There were two patients who died in the early post-operative period (13.3%). At the mid-term follow-up, 53 (20–86) months, late atrial arrhythmias had recurred in two of the 13 surviving patients (15.30%) and one patient developed late sinus node dysfunction. The need for anti-arrhythmic drugs decreased considerably from 93.5% to 15.3% on mid-term follow-up. There was no late death or need for cardiac transplantation. The duration of arrhythmia before surgery was prolonged for more than 10 years in patients who died as well as in those who had complications like late recurrence of arrhythmias, dependence on anti-arrhythmic medications, and worsening of ventricular dysfunction.

Fontan conversion is a well-established treatment option for salvaging the failing atriopulmonary connections. Concomitant arrhythmia surgery effectively resolves the refractory atrial arrhythmias and improves survival, but we need to optimise the timing of Fontan conversion to improve the long-term outcome.

To assess the perception of the quality of life of adolescents and young adults with congenital heart disease and to examine the variables that have a negative impact on it and that add a resilience effect.

A total of 22 male and 18 female patients, aged 12–26 years, of whom 27 were admitted to surgery and 13 were not, participated in this study. All patients had complete medical records and were interviewed once; demographic and clinical data were collected, and patients filled a questionnaire on quality of life, the WHOQOL-BREF, and underwent an interview on social support, educational style, self-image, functional limitations, and emotional adjustment.

Our patients showed a better perception of quality of life than did the general population, on the basis of psychological, social relationship and environment scales. Older patients hold a better perception of quality of life on the psychological scale. Cyanosis did not show any significant impact over perception of quality of life decay; however, the number of surgical procedures and the persistence of moderate-to-severe residual injuries had considerable detrimental effect. Social support had an impact on increasing resilience, promoting adjustment to illness. Several factors may play a role in adjustment to congenital heart disease, either improving the perception of quality of life or worsening it. We may conclude that some buffer variables on congenital heart disease may play roles in increasing the perception of quality of life of patients during their lifetime, social support probably explaining why the perception of quality of life is better than in the normal population. The number of surgeries and the moderate-to-severe residual injuries, however, reverted that effect.

The left ventricle in patients with hypoplastic left heart syndrome may influence right ventricular function and outcome. We aimed to investigate differences in right ventricular deformation and intraventricular dyssynchrony between hypoplastic left heart syndrome patients with different anatomical subtypes and left ventricle sizes after Fontan surgery using two-dimensional speckle tracking.

We examined 29 hypoplastic left heart syndrome patients aged 5.4 plus or minus 2.8 years after Fontan surgery and compared 15 patients with mitral and aortic atresia with the remaining 14 patients with other anatomic subtypes. We used two-dimensional speckle tracking to measure the global and regional systolic longitudinal strain and strain rate as well as intraventricular dyssynchrony.

Global strain (−19.5, 2.8% versus −17.4, 3.9%) and global strain rate (−1.0, 0.2 per second versus −0.9, 0.3 per second) were not different between groups. The mitral and aortic atresia group had higher strain in the basal septal (−13.0, 5.0% versus −3.9, 9.3%, p = 0.003) and mid-septal (−19.4, 4.7% versus −13.2, 6.5%, p = 0.009) segments, and higher strain rates in the mid-septal segment (−1.14, 0.3 per second versus −0.95, 0.4 per second, p = 0.047), smaller left ventricle area (0.18, 0.41 square centimetre versus 2.83, 2.07 square centimetre, p = 0.0001), and shorter wall-to-wall delay (38, 29 milliseconds versus 81, 57 milliseconds, p = 0.02).

Significant differences in regional deformation and intraventricular dyssynchrony exist between the mitral and aortic atresia subtype with small left ventricles and the other anatomic subtypes with larger left ventricles after Fontan surgery.

Successful management of bleeding disorders after congenital heart surgery requires detection of specific coagulation disturbances. Whole-blood rotation thromboelastometry (RoTEM®) provides continuous qualitative haemostatic profiles, and the technique has shown promising results in adult cardiac surgery.

To compare the performance of RoTEM® with that of conventional coagulation tests in children, we conducted a descriptive study in children undergoing congenital cardiac surgery. For that purpose, 60 children were enrolled and had blood samples taken before, immediately after, and 1 day after surgery. Conventional coagulation tests included: activated partial thromboplastin time, prothrombin time, fibrinogen, fibrin D-dimer, thrombin clotting time, factor XIII, and platelet count.

Post-surgical haemostatic impairment was present to some degree in all children, as seen by pronounced changes in activated partial thromboplastin time, prothrombin time, thrombin clotting time, and platelet count, as well as RoTEM® analysis. RoTEM® showed marked changes in clotting time – prolonged by 7–18% – clot formation time – prolonged by 46–71% – maximum clot firmness – reduced by 10–19%, and maximum velocity – reduced by 29–39%. Comparison of the two techniques showed that conventional coagulation tests and RoTEM® performed equally well with regard to negative predictive values for excessive post-operative drain production – more than 20 millilitres per kilogram per 24 hours after surgery – with an area under the curve of approximately 0.65.

RoTEM® can detect haemostatic impairments in children undergoing cardiac surgery and the method should be considered as a supplement in the perioperative care of the children where targeted transfusion therapy is necessary to avoid volume overload.