Recommendations from the Association for European Paediatric and Congenital Cardiology for training in diagnostic and interventional electrophysiology

The field of electrophysiology (EP) in paediatric cardiology patients and adults with congenital heart disease is complex and rapidly growing. The current recommendations for diagnostic and invasive electrophysiology of the working group for Cardiac Dysrhythmias and Electrophysiology of the Association for European Paediatric and Congenital Cardiology acknowledges the diveristy of European countries and centers. These training recommendations can be fulfilled in a manageable period of time, without compromising the quality of training required to become an expert in the field of paediatric and congenital EP and are for trainees undergoing or having completed accredited paediatric cardiologist fellowship. Three levels of expertise, the training for General paediatric cardiology EP, for non-invasive EP and invasive EP have been defined. This Association for European EP curriculum describes the theoretical and practicsal knowledge in clinical EP; catheter ablation, cardiac implantable electronic devices, inherited arrhythmias and arrhythmias in adults with congenital heart defects for the 3 levels of expertise.Developmen

Cardiac resynchronisation therapy in paediatric and congenital heart disease: differential effects in various anatomical and functional substrates

BACKGROUND: Cardiac resynchronisation therapy (CRT) is increasingly used in children in a variety of anatomical and pathophysiological conditions, but published data are scarce. OBJECTIVE: To record current practice and results of CRT in paediatric and congenital heart disease. DESIGN: Retrospective multicentre European survey. SETTING: Paediatric cardiology and cardiac surgery centres. PATIENTS: One hundred and nine patients aged 0.24-73.8 (median 16.9) years with structural congenital heart disease (n = 87), congenital atrioventricular block (n = 12) and dilated cardiomyopathy (n = 10) with systemic left (n = 69), right (n = 36) or single (n = 4) ventricular dysfunction and ventricular dyssynchrony during sinus rhythm (n = 25) or associated with pacing (n = 84). INTERVENTIONS: CRT for a median period of 7.5 months (concurrent cardiac surgery in 16/109). MAIN OUTCOME MEASURES: Functional improvement and echocardiographic change in systemic ventricular function. RESULTS: The z score of the systemic ventricular end-diastolic dimension decreased by median 1.1 (p<0.001). Ejection fraction (EF) or fractional area of change increased by a mean (SD) of 11.5 (14.3)% (p<0.001) and New York Heart Association (NYHA) class improved by median 1.0 grade (p<0.001). Non-response to CRT (18.5%) was multivariably predicted by the presence of primary dilated cardiomyopathy (p = 0.002) and poor NYHA class (p = 0.003). Presence of a systemic left ventricle was the strongest multivariable predictor of improvement in EF/fractional area of change (p<0.001). Results were independent of the number of patients treated in each contributing centre. CONCLUSION: Heart failure associated with ventricular pacing is the largest indication for CRT in paediatric and congenital heart disease. CRT efficacy varies widely with the underlying anatomical and pathophysiological substrate

Cytomegalovirus seroprevalence among solid organ donors in hungary: correlations with age, gender, and blood group

BACKGROUND: Cytomegalovirus (CMV) is endemic throughout the world, affecting most of the population, but the seroprevalence of CMV is known to vary among countries. CMV causes a mild infection in persons with intact immunity; however, CMV infection in organ transplantation is associated with significant morbidity and mortality. The present retrospective study was designed to evaluate the age-, gender-, and blood group-adjusted CMV seroprevalence among solid organ donors, representing fairly the overall Hungarian population (according to Hungarian Central Statistic Institute). This information is important for calculating risk-factors for CMV-seronegative recipients. No nationwide estimates of CMV seroprevalence in Hungary (as a representative of Eastern Middle Europe) have been published yet. METHODS: We investigated 2070 organ donors for CMV seroprevalence by measuring the CMV-specific immunoglobulin G. The donors were divided into 3 age groups (2-20, 21-50, and 51-70 years old). A study was also conducted on a fourth group consisting of 200 residents from an old age home. CMV seroprevalence differences were searched according to age-, gender- and blood-group distribution. RESULTS: The CMV seroprevalence of organ donors is 85% and of all investigated persons is 86%. The age-specific prevalence increases, starting from 72% in the first group to 99% in the fourth group. Seroprevalence of females was found to be significantly higher than of males (P = .0001). CONCLUSION: We have shown that the overall CMV seroprevalence in the Hungarian population is moderately high at 86%. The opportunity for CMV-seronegative recipients to get a graft from a seronegative donor is statistically only 2%. The seroprevalence of the youngest age group is 72% and so it can be concluded that the Hungarian population acquires the infection mainly in childhood or in the early adulthood. Female gender is a risk factor for CMV infection. This fact must be taken into consideration during the planning of patients' follow-up, prophylaxis, and therapy

The SIDECAR project : S-IcD registry in European paediatriC and young Adult patients with congenital heaRt defects

AIMS: Subcutaneous-implantable cardiac defibrillators (S-ICDs) are used increasingly to prevent sudden cardiac death in young patients. This study was set up to gain insight in the indications for S-ICD, possible complications, and their predictors and follow-up results. METHODS AND RESULTS: A multicentre, observational, retrospective, non-randomized, standard-of-care registry on S-ICD outcome in young patients with congenital heart diseases (CHDs), inherited arrhythmias (IAs), idiopathic ventricular fibrillation (IVF), and cardiomyopathies (CMPs). Anthropometry was registered as well as implantation technique, mid-term device-related complications, and incidence of appropriate/inappropriate shocks (IASs). Data are reported as median (interquartile range) or mean ± standard deviation. Eighty-one patients (47% CMPs, 20% CHD, 21% IVF, and 12% IA), aged 15 (14-17) years, with body mass index (BMI) 21.8 ± 3.8 kg/m2, underwent S-ICD implantation (primary prevention in 59%). This was performed with two-incision technique in 81% and with a subcutaneous pocket in 59%. Shock and conditional zones were programmed at 250 (200-250) and 210 (180-240) b.p.m., respectively. No intraoperative complications occurred. Follow up was 19 (6-35) months: no defibrillation failure occurred, 17% of patients received appropriate shocks, 13% of patients received IAS (supraventricular tachycardias 40%, T-wave oversensing 40%, and non-cardiac oversensing 20%). Reprogramming, proper drug therapy, and surgical revision avoided further IAS. Complications requiring surgical revision occurred in 9% of patients, with higher risks in patients with three-incision procedures [hazard ratio (HR) 4.3, 95% confidence interval (95% CI) 0.5-34, P = 0.038] and BMI 20 showed better outcome

Arrhythmias in congenital heart disease: a position paper of the European Heart Rhythm Association (EHRA), Association for European Paediatric and Congenital Cardiology (AEPC), and the European Society of Cardiology (ESC) Working Group on Grown-up Congenital Heart Disease, endorsed by HRS, PACES, APHRS, and SOLAECE

Cardiolog

Lysophosphatidic Acid Receptor-dependent Secondary Effects via Astrocytes Promote Neuronal Differentiation*S⃞

Lysophosphatidic acid (LPA) is a simple phospholipid derived from cell membranes that has extracellular signaling properties mediated by at least five G protein-coupled receptors referred to as LPA1–LPA5. In the nervous system, receptor-mediated LPA signaling has been demonstrated to influence a range of cellular processes; however, an unaddressed aspect of LPA signaling is its potential to produce specific secondary effects, whereby LPA receptor-expressing cells exposed to, or “primed,” by LPA may then act on other cells via distinct, yet LPA-initiated, mechanisms