Patients with pulmonary regurgitation after tetralogy of Fallot repair have impaired aerobic capacity; one of the reasons is the decreasing global ventricular performance at exercise, reflected by decreasing peak oxygen pulse. The aims of our study were to evaluate the impact of pulmonary valve replacement on peak oxygen pulse in a population with pure pulmonary regurgitation and with different degrees of right ventricular dilatation and to determine the predictors of peak oxygen pulse after pulmonary valve replacement.

The mean and median age at pulmonary valve replacement was 27 years. Mean pre-procedural right ventricular end-diastolic volume was 182 ml/m2. Out of 24 patients, 15 had abnormal peak oxygen pulse before pulmonary valve replacement. We did not observe a significant increase in peak oxygen pulse after pulmonary valve replacement (p=0.76). Among cardiopulmonary test/MRI/historical pre-procedural parameters, peak oxygen pulse appeared to be the best predictor of peak oxygen pulse after pulmonary valve replacement (positive and negative predictive values, respectively, 0.94 and 1). After pulmonary valve replacement, peak oxygen pulse was well correlated with left ventricular stroke and end-diastolic volumes (r=0.67 and 0.68, respectively).

Our study confirms the absence of an effect of pulmonary valve replacement on peak oxygen pulse whatever the initial right ventricular volume, reflecting possible irreversible right and/or left ventricle lesions. Pre-procedural peak oxygen pulse seemed to well predict post-procedural peak oxygen pulse. These results encourage discussions on pulmonary valve replacement in patients showing any decrease in peak oxygen pulse during their follow-up.

A 34-week premature neonate presented with drug-refractory permanent junctional incessant tachycardia and haemodynamic compromise. The patient underwent successful radiofrequency catheter ablation using a single-catheter approach. The child remains in sinus rhythm, without pharmacological treatment, 2 years after the procedure.

Despite several reports describing echocardiography for the guidance of ductal closure, two-dimensional angiography remains the mainstay imaging tool; three-dimensional rotational angiography has the potential to overcome some of the drawbacks of standard angiography, and reconstructed image overlay provides reliable guidance for device placement. We describe arterial duct closure solely from venous approach guided by live three-dimensional image overlay.

Background: We evaluated whether quality of life correlates to age and activity in children following heart transplantation. In addition, quality of life in children following heart transplantation was compared with previously reported values in children with congenital heart disease. Quality of life remains an important aspect of therapy. Methods: The Pediatric Quality of Life Inventory Generic Core Scales and Cardiac Module were administered to 14 children who had previously undergone heart transplantation. Patients wore a pedometer for 7 days to assess daily activity. Results: The age at assessment was 13.1±1.9 years. The patients were 7.1±5.7 years post heart transplantation. There was a negative correlation between age at first heart transplantation and emotional (r=−0.64; p<0.05) and school function (r=−0.57; p<0.05). A negative correlation between patient’s age at assessment and perceived physical appearance existed (r=−0.53; p<0.05). Daily steps negatively correlated with cognitive (r=−0.58; p<0.05), physical (r=−0.63; p<0.05), emotional (r=−0.62; p<0.05), and school function (r=−0.66; p<0.01). Heart transplantation patients reported better scores for treatment and symptoms (p<0.05) but lower physical health scores (p<0.01) than those with moderate congenital heart disease. Conclusions: Paediatric heart transplantation patients reported overall similar quality of life as patients with moderate congenital heart disease. Children receiving heart transplants at an older age may require additional emotional and educational support. Heart transplantation patients with higher activity levels may be more aware of their physical, emotional, and cognitive limitations, and thus score lower on these quality of life indicators.

The classical option for surgical repair in patients with congenitally corrected transposition takes advantage of the physiologic correction provided by nature. At the end of the surgical procedures, however, the morphologically right ventricle remains as the systemic ventricle. Surgical intervention is essentially the correction of associated lesions, including closure of ventricular septal defects, pulmonary valvotomy, placement of a conduit from the morphologically left ventricle to the pulmonary arteries, replacement of the morphologically tricuspid valve, and placement of pacemakers for third degree atrioventricular block. For many years, the classical approach was the “standard” surgical approach.1–4 More recently, newer alternatives have become available, including forms of anatomic repair, the “one-and-a half” ventricular option, and conversion to the Fontan circulation. The goal of anatomic repair is to craft connections such that the morphologically left ventricle becomes the systemic ventricle. Surgical techniques that accomplish this are a Rastelli procedure combined with an atrial baffle,5 and the combination of an arterial switch with an atrial baffle, be it a Mustard or Senning procedure.6

Basic scientists, as well as cardiologists, are caught by the idea of curing ischaemic heart disease with cardiac progenitor or stem cells. This short review provides an overview of our current knowledge on the potential use of stem cells for cardiac disease. Since, in infants and children, aetiologies and pathomechanisms of critical cardiac disease are fundamentally different from those in adults, we will also address the question as to whether such young patients could be a therapeutic target at all, and in which respect it may be necessary to view treatment with stem cells from a different stance in the developing organism.

Transcatheter creation of an interatrial communication using the Occlutech Atrial Flow Regulator Device for pulmonary hypertension or heart failure is well described. We report a case of an 8-year-old boy with a failing Fontan circulation, in whom the Atrial Flow Regulator was used to successfully create a fenestration between the pulmonary artery and left atrium, improving his clinical condition.

Coronary sinus defect is a rare type of atrial septal defect. We report two patients who had a coronary sinus atrial septal defect without persistent left superior caval vein, where the orifice of the coronary sinus was closed using the Amplatzer Septal Occluder. The procedure was successful, without any complications including conduction disturbance.

Objective: There are limited data on the outcomes of children receiving delayed (≥7 days) extracorporeal membrane oxygenation after cardiac surgery. The primary aim of this project is to identify the aetiology and outcomes of extracorporeal membrane oxygenation in children receiving delayed (≥7 days) extracorporeal membrane oxygenation after cardiac surgery. Patients and methods: We conducted a retrospective review of all children ≤18 years supported with delayed extracorporeal membrane oxygenation after cardiac surgery between the period January, 2001 and March, 2012 at the Arkansas Children’s Hospital, United States of America, and Royal Children’s Hospital, Australia. The data collected in our study included patient demographic information, diagnoses, extracorporeal membrane oxygenation indication, extracorporeal membrane oxygenation support details, medical and surgical history, laboratory, microbiological, and radiographic data, information on organ dysfunction, complications, and patient outcomes. The outcome variables evaluated in this report included: survival to hospital discharge and current survival with emphasis on neurological, renal, pulmonary, and other end-organ function. Results: During the study period, 423 patients undergoing cardiac surgery were supported with extracorporeal membrane oxygenation at two institutions, with a survival of 232 patients (55%). Of these, 371 patients received extracorporeal membrane oxygenation <7 days after cardiac surgery, with a survival of 205 (55%) patients, and 52 patients received extracorporeal membrane oxygenation ≥7 days after cardiac surgery, with a survival of 27 (52%) patients. The median duration of extracorporeal membrane oxygenation run for the study cohort was 5 days (interquartile range: 3, 10). In all, 14 patients (25%) received extracorporeal membrane oxygenation during active cardiopulmonary resuscitation with chest compressions. There were 24 patients (44%) who received dialysis while being on extracorporeal membrane oxygenation. There were eight patients (15%) who had positive blood cultures and four patients (7%) who had positive urine cultures while being on extracorporeal membrane oxygenation. There were nine patients (16%) who had bleeding complications associated with extracorporeal membrane oxygenation runs. There were 10 patients (18%) who had cerebrovascular thromboembolic events associated with extracorporeal membrane oxygenation runs. Of these, 19 patients are still alive with significant comorbidities. Conclusions: This study demonstrates that mortality outcomes are comparable among children receiving extracorporeal membrane oxygenation ≥7 days and <7 days after cardiac surgery. The proportion of patients receiving extracorporeal membrane oxygenation ≥7 days is small and the aetiology diverse.

Numerous data sets collect information on patients with paediatric cardiovascular disease, including paediatric heart failure and transplant patients. This review discusses methodologies available for linking and integrating information across data sets, which may help facilitate answering important questions in the field of paediatric heart failure and transplant that cannot be answered with individual data sets or single-centre data alone.

Neurocardiogenic syncope is a common disorder, which is considered as a benign condition. However, sudden loss of conscience and muscle tone causes anxiety among the family members due to its similarity to sudden death. Autonomic nervous system dysregulation is thought to be responsible in the aetiology. Heart rate variability is used for assessment of autonomic nervous system.

We evaluated 24 children between 6 and 18 years (mean plus or minus standard deviation is equal to 12.5 plus or minus 3.28, with neurocardiogenic syncope and 10 healthy controls, mean plus or minus standard deviation is equal to 12.48 plus or minus 3.27) by using 24 hour Holter monitorisation and head-up tilt test. Heart rate variability analysis was performed using the Holter recordings obtained both during head-up tilt test and throughout the day.

Our results revealed that, there is no significant difference between the study and the control groups in terms of the mean heart rate and all indices of the heart rate variability (p > 0.05). However, during the first 5 minutes of the head-up tilt test, standard deviation of all RR intervals and root mean square of successive differences were significantly lower in the syncope group compared with the control group, 42.17 plus or minus 12.56 versus 60.10 plus or minus 33.10 and 21.26 plus or minus 8.87 versus 36.80 plus or minus 31.03; p-values 0.02 and 0.03, respectively.

In conclusion; autonomic functions in children with neurocardiogenic syncope are similar to healthy children. However, sympathetic hyperactivation occurs during the early phase of orthostatic stress in children with neurocardiogenic syncope comparing to healthy controls. Parasympathetic innervation is not sufficient in compensation of this sympathetic hyperactivation. Management strategy in neurocardiogenic syncope should be based on these pathophysiologic mechanisms.

We report a case of anomalous origin of the right pulmonary artery from the ascending aorta that was diagnosed by fetal ultrasound at 21 weeks of gestation. The clue to the diagnosis was present in the three-vessel view, this being one of the views that we use for fetal cardiac screening. The anomaly was corrected surgically at 11 days of age. We discuss the importance of prenatal diagnosis in the management of this rare anomaly.

Obesity is increasingly prevalent, and abnormal body mass index is a risk factor for cardiovascular disease. There are limited data published regarding body mass index and CHD. We tested the hypothesis that body mass index and obesity prevalence are increasing in patients with tetralogy of Fallot over time by analysing time since surgery, age, height, weight, and body mass index among tetralogy of Fallot patients and demographic data from age-matched controls. NYHA class and left ventricular ejection fraction were analysed in adults. Body mass index was categorised into normal, overweight, and obese in this single-centre, retrospective chart review. Data were collected from 137 tetralogy of Fallot patients (71 men:66 women), of whom 40 had body mass index >25 kg/m2. Tetralogy of Fallot patients aged <6 years had lower body mass index (15.9 versus 17.1; p=0.042) until 16–20 years of age (27.4 versus 25.4; p=0.43). For adult tetralogy of Fallot patients, the mean body mass index was 26.5 but not statistically significantly different from the control cohort. Obese adult patients had significantly higher average NYHA class compared with those of normal weight (p=0.03), but no differences in left ventricular ejection fraction by echocardiography (p=0.55) or cardiac MRI (p=0.26) were noted. Lower body mass index was observed initially in tetralogy of Fallot patients, but by late adolescence no significant difference was observed. As adults, tetralogy of Fallot patients with higher body mass index had increased NYHA class but similar left ventricular ejection fraction.

Maternal hyperthermia induces malformations in human and laboratory animals, but there are only a few reports of cardiovascular anomalies induced by maternal hyperthermia. Pregnant Wistar Imamichi rats were exposed to hyperthermia on day nine of gestation by immersion in a hot-water bath (43.0–43.5 °C) twice a day. Their fetuses were examined on day 18 after fixing with 2.5% glutaraldehyde solution. According to the average body temperature of the dam, the fetuses were divided into five groups, each group increasing by 0.1 °C from 42.5 °C to 42.9 °C. The incidence of cardiovascular anomalies was increased from 24 to 100% with the rise in body temperature of the dams. In the groups with average temperatures of 42.8 and 42.9 °C, most of the fetuses (70–80% of cases with anomalies) showed visceroatrial heterotaxy associated with severe cardiovascular anomalies, including common atrioventricular orifices with double outlet right ventricle or discordant ventriculo-arterial connections. The visceral structures of those fetuses showed a tendency to left isomerism, such as bilateral left atrial appendages, single lobation of the lung on both sides, a symmetrical liver with interruption of the posterior caval vein, hypoplasia of the spleen, and malrotation of the intestinal loop. Day nine of gestation in the rat may be a critical period for determining left-right sidedness of the viscera. The results indicate that the determination of morphological left-sidedness of visceral structures is related in some way with maternal hyperthermia.