The Use of Vasodilator Therapy in Fontan Patients: A Single-Centre Experience.

Background: The aim of this study was to describe our centre experience in the use of pulmonary vasodilator therapy in Fontan patients.

Methods: We retrospectively enrolled patients that underwent Fontan operation between 2000 and 2024, reporting demographic and operative data and noting complications and the use of pulmonary vasodilators.

Results: A total of 117 patients were followed for a median time of 150 months (90-207).

Multi-Modality Imaging in Coronary Anomalies: Focus on Anomalous Aortic Origin of Coronary Arteries.

Coronary artery anomalies represent a series of congenital heart diseases characterized by the abnormal circulation of coronary arteries. Of particular interest is the anomalous aortic origin of coronary arteries (AAOCA), an underdiagnosed but potentially fatal anomaly, specifically relevant in young adult athletes. During the last decades, knowledge of the pathophysiology of AAOCA-related ischemic mechanisms has increased, and major risk conditions have been discovered.

Left ventricular longitudinal strain in the follow-up of arterial switch operation: a fingerprint of the patient's history.

Background: Left ventricular function after arterial switch operation for d-transposition of the great arteries is notoriously compromised because of abnormal coronary artery anatomy or altered loading conditions. We sought to longitudinally investigate the performance of the left ventricle in a cohort of d-transposition of the great artery patients after arterial switch operation, by using advanced echocardiographic deformation imaging and grouping patients according to pre- and post-surgery variables, labelled as risk factors.

Methods: Longitudinal single-centre study involving 53 d-transposition of the great artery patients (81.

Developing consensus outcome measures in juvenile systemic sclerosis: a global survey of pediatric rheumatologists and literature review.

Background: Juvenile systemic sclerosis (JSSc) is a rare multisystemic disease with high morbidity and mortality rates. Treatment options remain limited, and there is a significant unmet need for effective therapies. This study aims to address this gap by investigating current JSSc management practices and identifying key outcome measures that can be used to inform the development of standardized assessment tools for future clinical trials.

Hypertension in aortic coarctation.

Aortic coarctation (AoC) is a common congenital heart defect, affecting 5%-8% of patients with structural congenital anomalies. Despite advances in surgical and percutaneous interventions, hypertension remains a significant complication in AoC patients, even after successful repair. Chronic hypertension develops in 20%-70% of patients and is a leading cause of long-term cardiovascular morbidity.

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.

Pulmonary artery banding to treat end-stage heart failure in infants and young children: A multicenter study.

Background: Conventional treatment options for end-stage heart failure (ESHF) in children include heart transplantation (HT) and ventricular assist devices (VADs), both with significant drawbacks in the pediatric population. Pulmonary artery banding (PAB) has been effectively used as bridge to transplant or recovery in pediatric ESHF. We herein describe the early and mid-term clinical outcomes from a multicenter international experience.

Mini-invasive surgical approach for hybrid pulmonary valve implantation: an option for very high-risk patients.

Transcatheter pulmonary valve replacement is the first choice to treat residual or recurrent right ventricular outflow tract dysfunction. Surgery is an effective option when anatomy is not permissive for transcatheter procedures. When surgical risk is too high, hybrid procedures might be considered.

Cardiovascular function shows early impairment in asymptomatic adolescents diagnosed with type 1 diabetes mellitus: an ultrasound-derived myocardial work study.

Background: Cardiac dysfunction and endothelial damage are known complications of type 1 diabetes mellitus (T1D) mainly affecting adults. However, some studies have shown that subclinical myocardial impairment already present during adolescence. Myocardial work (MW) has emerged as an afterload-independent tool that allows early identification of subclinical damage.

Pediatric Three-dimensional Transesophageal Echocardiography: A Game Changer in Congenital Heart Disease.

Background: Despite its potential, pediatric three-dimensional (3D) transesophageal echocardiography (TEE) faced technical limitations due to the size and rigidity of early probes designed for adults.

Aim: In this paper, we present our experience in using the new pediatric 3D TEE probe in complex congenital heart disease (CHD).

Methods: Recent advancements in the field of miniaturization have led to the development of a new pediatric 3D TEE probe, specifically designed for young children (weight ≥4.

Balloon atrioseptostomy for transposition of the great arteries in Europe: characteristics and outcomes.

Introduction And Objectives: Balloon atrial septostomy (BAS) improves oxygenation in neonates with transposition of the great arteries (TGA) and restrictive foramen ovale. Currently, there is a global shortage of dedicated BAS catheters, while new unmarked catheters have recently become available at some European centers. This study aimed to characterize BAS outcomes using the currently available BAS catheters in Europe.

Advantages of using a TorqVue LP catheter for patent arterial duct stenting in newborn with a duct-dependent pulmunary circulation: a case report.

Almost 20% of children with simple or complex types of CHD have a duct-dependent circulation The systemic-to-pulmonary shunt can be performed surgically, e.g., Blalock-Taussing shunt or with a patent duct arteriosus stenting.

The impact of dominant ventricle morphology and additional ventricular chamber size on clinical outcomes in patients with Fontan circulation.

Objectives: The functional roles of ventricular dominance and additional ventricular chamber after Fontan operation are still uncertain. We aim to assess and correlate such anatomical features to late clinical outcomes.

Methods: Fontan patients undergoing cardiac MRI and cardiopulmonary exercise test between January 2020 and December 2022 were retrospectively reviewed.

Transforming Heart Failure Management: The Power of Strain Imaging, 3D Imaging, and Vortex Analysis in Echocardiography.

Heart failure (HF) remains a critical global health challenge, necessitating advancements in diagnostic and therapeutic strategies. This review explores the evolution of imaging technologies and their impact on HF management, focusing on three-dimensional echocardiography (3DE), myocardial strain imaging, and vortex dynamics imaging. Three-dimensional echocardiography enhances traditional echocardiography by providing more accurate assessments of cardiac structures, while myocardial strain imaging offers the early detection of subclinical myocardial dysfunction, crucial in conditions such as chemotherapy-induced cardiotoxicity and ischemic heart disease.

Stress Echocardiography in the Follow-Up of Young Patients with Repaired Aortic Coarctation.

Aortic coarctation (CoA) is a congenital heart disease affecting 5-8% of patients, with long-term complications persisting despite successful correction. Stress echocardiography (SE) is increasingly used for evaluating cardiac function under stress, yet its role in repaired CoA remains under-explored. This study aimed to assess the predictive value of SE and myocardial strain in repaired CoA patients with a history of hypertension without significant gradients or with borderline gradients at rest.

Feasibility, Safety, and Efficacy of the Atrial Flow Regulator in Pediatric Patients: A Single-Center Experience.

Background: The Atrial Flow Regulator (AFR; Occlutech) can be used to create interatrial communication with a predetermined diameter in various pathophysiological settings. In the pediatric population, the experience is limited to a few case reports. We aim to report the initial single-center experience of AFR implantation in children with congenital and acquired heart disease.

Standardized diagnostic reference levels for paediatric interventional cardiology: Data from an Italian referral centre.

Purpose: To provide data on radiation exposure in paediatric interventional cardiology procedures, addressing the scarcity of valuable Local Diagnostic Reference Levels (LDRLs),established according to the standardized approach proposed by the Radiation Protection 185 report (RP185).

Methods: Paediatric catheterization procedures conducted at the University-Hospital of Padua from September 2019 to December 2022 were stratified by body weight (BW) classes and procedure type. LDRLs were calculated for groups with at least 20 patients as the 75th percentile of Kerma-Area Product (P) and Air Kerma at reference point (K) values.

[Italian Society of Pediatric Cardiology (SICP) position paper on the prevention, diagnosis, treatment and follow-up of cardiotoxicity in pediatric patients with cancer].

The survival of pediatric cancer patients has significantly increased thanks to the improvement of oncological treatments. Therefore, it is of utmost importance to manage short- and long-term cardiovascular complications. In pediatric cardio-oncology, there are no recognized guidelines as in adults.

How Will Artificial Intelligence Shape the Future of Decision-Making in Congenital Heart Disease?

Improvements in medical technology have significantly changed the management of congenital heart disease (CHD), offering novel tools to predict outcomes and personalize follow-up care. By using sophisticated imaging modalities, computational models and machine learning algorithms, clinicians can experiment with unprecedented insights into the complex anatomy and physiology of CHD. These tools enable early identification of high-risk patients, thus allowing timely, tailored interventions and improved outcomes.

Cardiac Mechanics Evaluation in Preschool-Aged Children with Preterm Birth History: A Speckle Tracking and 4D Echocardiography Study.

The premature-born adult population is set to grow significantly, and prematurity has emerged as an important cardiovascular risk factor. We aimed to comprehensively assess cardiac mechanics and function in a cohort of ex-preterm preschoolers. Ex-preterm children (<30 weeks of gestation), aged 2 to 5 years, underwent transthoracic 2D, speckle-tracking, and 4D echocardiography.

Intracorporeal LVAD implantation in pediatric patients: A single-center 10 years' experience.

Background: Mechanical cardiac support is currently an effective strategy to reduce morbidity and mortality in pediatric patients. However, solid evidence regarding the feasibility of intracorporeal devices in children still needs to be provided. We report our 10-year experience with intracorporeal left ventricular assist devices (LVAD) in children.

Implantation of atrial flow regulator devices in patients with congenital heart disease and children with severe pulmonary hypertension or cardiomyopathy-an international multicenter case series.

Background: The Occlutech Atrial Flow Regulator (AFR) is a self-expandable double-disc nitinol device with a central fenestration. Its use has been approved in the adult population with heart failure and described for pulmonary hypertension (PH). Only case reports and small series have been published about its use in the paediatric population and for congenital heart disease (CHD).