Development of the fetal myocardium and changes in myocardial fibers orientation.

Background And Aims: The mature left ventricular myocardium is arranged in a complex three-dimensional network of fibers that form a counterclockwise helix in the endocardial layer and a clockwise helix in the epicardial layer. There are no data in the literature on the development of left ventricular myocardium during the fetal life. The aims of this paper were to study the physiological maturation steps of the LV myocardium in fetuses from 17 to 40 gestational weeks, by means of speckle tracking applied to the endocardial and epicardial aspect of the left ventricle, and, to confirm our finds, through the histologic study of the myocardium of demised fetuses.

Enhancing paediatric and congenital cardiology e-learning postgraduate education: insights from the Association for European Paediatric and Congenital Cardiology directed webinar series.

Objective: A series of webinars covering widespread knowledge on paediatric cardiology and cardiac surgery topics was initiated by Association for European Paediatric and Congenital Cardiology, serving towards preparation for the Association for European Paediatric and Congenital Cardiology certification in paediatric and congenital cardiology. This study investigated the impact of webinars as educational tools for junior paediatric cardiologists in the post-COVID-19 pandemic era.

Materials And Methods: A cross-sectional survey design study using an online survey as a tool for the assessment of trainees.

Adult congenital heart disease training in Europe: current status, disparities and potential solutions.

Objectives: This study aimed to determine the status of training of adult congenital heart disease (ACHD) cardiologists in Europe.

Methods: A questionnaire was sent to ACHD cardiologists from 34 European countries.

Results: Representatives from 31 of 34 countries (91%) responded.

Assessment for learning of paediatric cardiology trainees in 41 centres from 19 European countries.

Background: Limited data exist on how trainees in paediatric cardiology are assessed among countries affiliated with the Association of European Paediatric and Congenital Cardiology.

Methods: A structured and approved questionnaire was circulated to educationalists/trainers in 95 Association for European Paediatric and Congenital Cardiology training centres.

Results: Trainers from 46 centres responded with complete data in 41 centres.

Paediatric and adult congenital cardiology education and training in Europe.

Background: Limited data exist on training of European paediatric and adult congenital cardiologists.

Methods: A structured and approved questionnaire was circulated to national delegates of Association for European Paediatric and Congenital Cardiology in 33 European countries.

Results: Delegates from 30 countries (91%) responded.

Multi-Parametric Diagnostic Approach and Potential Markers of Early Onset Subclinical Cardiovascular Disease in a Cohort of Children, Adolescents and Young Adults Vertically Infected with HIV on cART.

Background: HIV infection and lifelong cART are responsible of an increase in cardiovascular risk. The aim of this study was to describe the subclinical cardiovascular disease and to identify early markers of cardiovascular damage in adolescents and young adults vertically infected with HIV on cART, through an innovative multi-parametric approach.

Methods: We enrolled 52 patients vertically infected with HIV.

COVID-19 FAQs in paediatric and congenital cardiology: AEPC position paper.

The COVID-19 pandemic has had a huge influence in almost all areas of life, affecting societies, economics, and health care systems worldwide. The paediatric cardiology community is no exception. As the challenging battle with COVID-19 continues, professionals from the Association for the European Paediatric and Congenital Cardiology receive many questions regarding COVID-19 in a Paediatric and Congenital Cardiology setting.

End-of-life care for children with complex congenital heart disease: Parents' and medical care givers' perceptions.

Aim: In complex congenital heart diseases (CHD), patients may remain affected by significant morbidity and mortality after surgery. We analysed the end-of-life (EoL) care in children with severe CHD who died in our institution and investigated perspectives of parents and health-care professionals (HCPs).

Methods: Medical records of all children (age < 18 years old) affected by a severe CHD who died in a tertiary cardiac care centre were reviewed.

Association for European Paediatric and Congenital Cardiology recommendations for basic training in paediatric and congenital cardiology 2020.

The recommendations of the Association for European Paediatric and Congenital Cardiology for basic training in paediatric and congenital cardiology required to be recognised as a paediatric cardiologist by the Association for European Paediatric and Congenital Cardiology are described below. Those wishing to achieve more advanced training in particular areas of paediatric cardiology should consult the training recommendations of the different Association for European Paediatric and Congenital Cardiology Working Groups available on the Association for European Paediatric and Congenital Cardiology website (www.aepc.

Typical values for pediatric interventional cardiology catheterizations: A standardized approach towards Diagnostic Reference Level establishment.

Purpose: To define weight-stratified Diagnostic Reference Levels (DRL) typical values for pediatric interventional cardiology (IC) procedures adopting standardized methodologies proposed by ICRP135 and RP185.

Methods: Procedures performed at the pediatric catheterization room of the University-Hospital of Padua were analysed. Patients were stratified into body weight (BW) classes and DRL quantities were analysed for the most performed procedures.

Early detection of ventricular dysfunction in juvenile systemic sclerosis by speckle tracking echocardiography.

Objective: Cardiac involvement is the most important cause of mortality in juvenile systemic sclerosis (JSSc). Recent reports in adult patients underline that traditional techniques of imaging are inadequate to assess the subclinical cardiac involvement, while speckle tracking echocardiography (STE) is able to identify ventricular dysfunctions in the early stages. The aim of our study was to assess the role of STE in JSSc.

Atypical cardiac defects in patients with RASopathies: Updated data on CARNET study.

Background: RASopathies are a set of relatively common autosomal dominant clinically and genetically heterogeneous disorders. Cardiac outcomes in terms of mortality and morbidity for common heart defects (such as pulmonary valve stenosis and hypertrophic cardiomyopathy) have been reported. Nevertheless, also Atypical Cardiac Defects (ACDs) are described.

A case of AndraStent fracture in a patient with aortic coarctation: a review of the literature.

Percutaneous treatment of aortic coarctation is based on angioplasty and/or stenting of the isthmus. We report a case of a 28-year-old girl suffering from aortic coarctation syndrome (coarctation + ventricular septal defect + bicuspid aorta). She underwent coarctectomy with subclavian flap and pulmonary bandage followed by ventricular septal defect closure and bandage removal in her first year of life.

DATA in BRIEF of: Interventional Cardiac Catheterization in Neonatal Age: Results in a Multi-centre Italian Experience.

A comprehensive description of morbidity and mortality as well as risk factors of interventional cardiac catheterization performed in neonatal age was reported in our paper recently published on the (). Eight Italian high-volume centres of Paediatric Cardiology were involved in this observational, retrospective data collection and analysis. In this dataset, clinical and procedural characteristics of 1423 newborns submitted to 1551 interventional cardiac catheterization procedures were analyzed.

Interventional cardiac catheterization in neonatal age: results in a multicentre Italian experience.

Background: Despite recent technical advances, interventional cardiac catheterization is still challenging in neonatal age and no specific data concerning early outcome are so far published in literature.

Methods: Neonatal trans-catheter cardiac interventions performed in high-volume Italian referral centers were retrospectively analyzed. Primary outcomes were procedural major adverse events, in-hospital mortality and procedural failure.

A restrictive ventilatory pattern is common in patients with univentricular heart after Fontan palliation and associated with a reduced exercise capacity and quality of life.

Aim: The Fontan circulation is highly dependent on ventilation, improving pulmonary blood flow and cardiac output. A reduced ventilatory function is reported in these patients. The extent of this impairment and its relation to exercise capacity and quality of life is unknown and objective of this study.

Can myocardial remodeling be a useful surrogate predictor of myocardial iron load? A 3D echocardiographic multicentric study.

The relationship between myocardial iron load and eccentric myocardial remodeling remains an under-investigated area; it was thought that remodeling is rather linked to fibrosis. This study aims to determine whether or not measures of remodeling can be used as predictors of myocardial iron. For this purpose, 60 patients with thalassemia were studied with 3D echocardiography and myocardial relaxometry (T2*) by Cardiac MRI.

Data on cardiac defects, morbidity and mortality in patients affected by RASopathies. CARNET study results.

A comprehensive description of morbidity and mortality in patients affected by mutations in genes encoding for signal transducers of the RAS-MAPK cascade (RASopathies) was performed in our study recently published in the International Journal of Cardiology. Seven European cardiac centres participating to the CArdiac Rasopathy NETwork (CARNET), collaborated in this multicentric, observational, retrospective data analysis and collection. In this study, clinical records of 371 patients with confirmed molecular diagnosis of RASopathy were reviewed.

Early modifications of cardiac function in preterm neonates using speckle tracking echocardiography.

Background And Aims: Preterm newborns undergo hemodynamic challenges in the postnatal period. The aim of this study was to investigate myocardial mechanics changes in the postnatal period in preterm infants using speckle tracking echocardiography (STE).

Material And Methods: Thirty-nine preterm infants ≤34 weeks' gestation underwent cardiac ultrasound evaluation during the first 96 hours of life.

Hemodynamic impact of pulmonary vasodilators on single ventricle physiology.

Introduction: The Fontan procedure is the palliative procedure for single ventricle physiology. Pulmonary resistance plays a key role in the success of this operation. There are conflicting data concerning the impact of pulmonary vasodilators on survival and functional capacity among Fontan patients.

The natural history and surgical outcome of patients with scimitar syndrome: a multi-centre European study.

Aims: Treatment decisions in patients with scimitar syndrome (SS) are often challenging, especially in patients with isolated SS who are often asymptomatic and who might be diagnosed accidentally. We queried a large multi-institutional registry of SS patients to evaluate the natural history of this condition and to determine the efficacy of surgical treatment in terms of survival and clinical status.

Methods And Results: We collected data on 485 SS patients from 51 institutions; 279 (57%) patients were treated surgically (STPs) and 206 (43%) were clinically monitored (CMPs).

Nuclear Medicine in Pediatric Cardiology.

Accurate cardiovascular imaging is essential for the successful management of patients with congenital heart disease (CHD). Echocardiography and angiography have been for long time the most important imaging modalities in pediatric cardiology, but nuclear medicine has contributed in many situations to the comprehension of physiological consequences of CHD, quantifying pulmonary blood flow symmetry or right-to-left shunting. In recent times, remarkable improvements in imaging equipments, particularly in multidetector computed tomography and magnetic resonance imaging, have led to the progressive integration of high resolution modalities in the clinical workup of children affected by CHD, reducing the role of diagnostic angiography.