Background. Validation data of the use of implantable cardioverter-defibrillators (ICD) in the pediatric population is insufficient, with limited follow-up periods. Objectives. The aim of the study was to report on 17 years of experience with implantable cardioverter-defibrillator (ICD) therapy in children and young adults. Materials and methods. This retrospective review included patients below the age of 18 years at the time of ICD implantation between May 2000 and December 2017. For the statistical analysis, the sample was divided into groups by gender and the type of indications for ICD implantation (primary vs secondary prevention). Results. The study group included 20 children (8 female, 12 male) who underwent ICD implantation for primary or secondary prevention of sudden cardiac death (SCD). The average age at the time of the initial procedure was 15.6 years (range: 3.8-17.7 years). Primary electrical disease (PED) was present in 9 patients, cardiomyopathy (CMP) in 9 and 2 others had congenital heart defects (CHD5). The median follow-up time was 6.7 years (range: 0.4-12.5 years). The outcomes of ICD therapy were analyzed. No differences between the sexes were found in terms of treatment strategy effectiveness (p > 0.05). The girls were more often treated as primary prevention (p = 0.009). After implantation, all the patients were on optimal pharmacotherapy. Alltogether there were 126 ICD interventions in 11 patients, including 23 inadequate interventions (IA) in 2 children (18.2%).Three children (15%) died due to electrical storms. In the per-procedure analysis, the overall freedom rate from ICD lead replacement was 90%, 80%, and 57% at 1, 5 and 10 years of observation, respectively. Conclusions. Implantable cardioverter-defibrillator implantation indications in children are more heterogeneous in comparison to adult population. In the pediatric population undergoing ICD implantation, the treatment strategy is influenced by gender. The rate of inappropriate ICD discharges (IA) in our group of pediatric patients was low. Rigorous pharmacotherapy and individual ICD programming seemed of paramount importance. Lead malfunctions LF constituted the most prevalent complication observed.

Atrial septal defect (ASD) is characterized by an abnormal opening between the left (LA) and right atria (RA). Even though it’s one of the most prevalent congenital heart defects, there are still many knowledge gaps and clinical uncertainties. This review aims to create a complex description of ASD and discuss its link with pulmonary arterial hypertension (PAH). An extensive literature search was conducted on sites such as PubMed, Google Scholar, and ScienceDirect. This study reviews the key findings of peer-reviewed articles with the keywords ASD, PAH, and congenital heart defects. The research showed that whilst there are multiple reviews, there is still a need for a deeper understanding, especially in areas of embryology, decision thresholds for closure, and pediatric-specific long-term outcomes. Current guidelines often extrapolate data only from adults or avoid certain topics. Those ambiguities often lead to variable medical practices, missed opportunities, and uncertainty for families. This review is divided into clear sections, creating a step-by-step characterization of the most important information, which can be useful to specialists. It touches on important subjects and controversies. It shows a clear timeline, starting with embryology, genetics, and anatomy, through pathophysiology and patient description, ending with recommendations and indications for specific treatment methods. Moreover, it creates a clear connection between ASD and PAH, outlining its danger and the need for prevention.