Fontan-Associated Liver Disease (FALD) in the EUROFontan Experience. An Insight into European Awareness.

Fontan-Associated Liver Disease (FALD) is a dramatically emerging problem even if not precisely defined in term of debated diagnosis and surveillance protocols. We analyze FALD prevalence, clinical impact and implications in a European cohort of patients. It's a retrospective observational multicenter study including Fontan patients operated between 1990 and 2022.

Heart-Liver Interplay in Patients with Fontan Circulation.

The Fontan procedure has provided pediatric patients suffering from severe congenital heart disease the opportunity to reach adulthood. Increasingly, we encounter the liver repercussions of Fontan circulation, alongside a decline in heart function and exercise performance. This study aims to identify the univentricular heart malformations that are most susceptible to liver dysfunction; assess which markers of liver injury are essential for multidisciplinary clinical follow-up of Fontan patients; determine the optimal approach for evaluating liver function in Fontan patients; and explore how a congenital cardiology team can interpret the data and respond effectively to signs of organ failure.

Postoperative Outcomes of Fontan Operation in a Multicenter Italian Study. How Far Have We Gone? Early Outcomes After Fontan Operation.

Despite the clinical results of the Fontan operation have certainly improved, it still presents with an inherent surgical risk of death and early morbidities. This is a retrospective clinical study of children undergoing Fontan operation in 9 congenital cardiac centers in Italy between 1990 and 2023. Clinical and surgical data were collected via a dedicated RedCap database.

Utility of Fetal Cardiac Resonance Imaging in Prenatal Clinical Practice: Current State of the Art.

The field of prenatal cardiac imaging has revolutionized the way we understand and manage congenital heart diseases (CHD) in the developing fetus. In the prenatal period, cardiac imaging plays a pivotal role in the diagnostic pathway, from screening to classification and follow-up of CHD. The ability to visualize the fetal heart in utero allows healthcare professionals to detect abnormalities early, thus enabling timely interventions and informed decision-making processes for both the mother and the medical team.

Childhood Obesity and Congenital Heart Disease: A Lifelong Struggle.

Congenital heart disease (CHD) affects approximately one in every one hundred infants worldwide, making it one of the most prevalent birth abnormalities globally. Despite advances in medical technology and treatment choices, CHD remains a significant health issue and necessitates specialized care throughout an individual's life. Childhood obesity has emerged as a novel global epidemic, becoming a major public health issue, particularly in individuals with lifelong conditions such as CHD.

Mid- and Long-Term Atrio-Ventricular Functional Changes in Children after Recovery from COVID-19.

Background: Although most children may experience mild to moderate symptoms and do not require hospitalization, there are little data on cardiac involvement in COVID-19. However, cardiac involvement is accurately demonstrated in children with MISC. The objective of this study was to evaluate cardiac mechanics in previously healthy children who recovered from severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) infection in a long-term follow-up by means of two-dimensional speckle-tracking echocardiography (STE).

Evolution of echocardiographic and cardiac magnetic resonance imaging abnormalities during follow-up in patients with multisystem inflammatory syndrome in children.

Aims: Multisystem inflammatory syndrome in children (MIS-C) with cardiovascular manifestations are frequent. However, there is lacking evidence regarding cardiological follow-up of this cohort of patients. The aim of our study was to describe the early and mid-term cardiac abnormalities assessed by standard and speckle-tracking echocardiography (STE), and cardiac MRI (CMR).

Role of Transient Elastography to Stage Fontan-Associated Liver Disease (FALD) in Adults with Single Ventricle Congenital Heart Disease Correction.

Fontan-associated liver disease (FALD) is an arising clinical entity that can occur long after a successful Fontan operation for correction of single ventricle (SV) congenital heart disease (CHD). Occurrence of FALD is characterized by liver cirrhosis and other hepatic complications, and determinates an increased morbidity and mortality. Currently, there is no consensus on how to stage FALD.

Effectiveness of Repair of Aortic Coarctation in Neonates: A Long-Term experience.

To evaluate early and long-term results of surgical treatment of aortic coarctation (CoAo) in neonates. This is a retrospective clinical review of neonates with CoAo, who underwent surgery between 1995 and 2019. Data were retrieved from our institutional database, to identify preoperative and postoperative characteristics.

Pulmonary Artery Banding for Ventricular Rehabilitation in Infants With Dilated Cardiomyopathy: Early Results in a Single-Center Experience.

Pulmonary artery banding (PAB) is reported as an innovative strategy for children with end-stage heart failure (ESHF) to bridge to transplantation or recovery. We report our early experience with PAB to evaluate outcomes, indications, and limitations. This is a single-center prospective clinical study, including infants and children admitted for ESHF owing to dilated cardiomyopathy (DCM) with preserved right ventricular function after failure of maximal conventional therapy.

JAG1 Loss-Of-Function Variations as a Novel Predisposing Event in the Pathogenesis of Congenital Thyroid Defects.

Context: The pathogenesis of congenital hypothyroidism (CH) is still largely unexplained. We previously reported that perturbations of the Notch pathway and knockdown of the ligand jagged1 cause a hypothyroid phenotype in the zebrafish. Heterozygous JAG1 variants are known to account for Alagille syndrome type 1 (ALGS1), a rare multisystemic developmental disorder characterized by variable expressivity and penetrance.

Pacemaker remote monitoring in the pediatric population: is it a real solution?

Background: Clinical utility of remote monitoring of implantable cardiac devices has been previously demonstrated in several trials in the adult population. The aim of this study was to assess the clinical utility of remote monitoring in a pediatric population undergoing pacemakers implantation.

Methods: The study population included 73 consecutive pediatric patients who received an implantable pacemaker.

Pulmonary artery branch stenosis in patients with congenital heart disease.

Objective: We sought to evaluate our recent experience with surgical treatment of branch pulmonary artery (PA) stenosis both for native and acquired lesions.

Materials And Methods: The postoperative course of patients who underwent surgical PA plasty augmentation between January 2004 and January 2012 were reviewed. Primary outcomes included the need for further surgical procedures or interventional maneuvers on the branch PAs for residual stenosis.

Medical and surgical management of primary cardiac tumours in infants and children.

Primary cardiac tumours in infants and children are extremely rare, with an estimated incidence of 0.2% according to echocardiographic studies. Owing to their rarity, there is very little literature available, and most knowledge is based on collections of case reports.

Iatrogenic aortopulmonary fistula occurring after pulmonary artery balloon angioplasty: a word of caution.

We describe the natural history of an adolescent patient who developed an aortopulmonary fistula (APF) after transcatheter stent placement for left pulmonary artery stenosis after neonatal repair of d-transposition of the great arteries. Due to its rarity, the APF was not initially diagnosed and treated until 4 months later. The APF was occluded with a covered stent.

The balloon dilation of the pulmonary valve during early repair of tetralogy of Fallot.

Background And Objective: Progressive pulmonary valve (PV) regurgitation leads to right ventricular failure after repair of tetralogy of Fallot (TOF). We sought to evaluate our results with the preservation of the PV in selected patients with TOF.

Methods: All patients with TOF who were scheduled for PV's preservation between June 2007 and May 2010 were enrolled.

Usefulness of fetal three-dimensional ultrasonography for detecting of congenital heart defects and associated syndromes.

Congenital heart defects (CHDs) occur in 1% of live-born infants and frequently are associated with extracardiac malformations. This study aimed to assess the feasibility and accuracy of three-dimensional ultrasonography (3DUS) in fetuses with CHD and to investigate whether 3DUS can add information about the heart and general fetal morphology that shows other congenital malformations or suggests syndromes. For 30 fetuses affected by CHD, 3DUS was performed using a Sonos 7500 ultrasound machine with a cardiac 3D transducer.

Cardiac operations after patent ductus arteriosus stenting in duct-dependent pulmonary circulation.

Background: Stenting of the patent ductus arteriosus (PDA) has been recently introduced to palliate patients with duct-dependent pulmonary circulations. We evaluated the surgical outcome of patients who had a previous PDA stent, focusing on their pulmonary arteries status.

Methods: This study included 15 patients (11 boys, 4 girls) who underwent cardiac operations after PDA stenting between August 2004 and April 2009.

Surgical repair of congenital mitral valve malformations in infancy and childhood: a single-center 36-year experience.

Objective: We sought to evaluate the results of surgical repair and determine predictors for the late outcome of congenital mitral valve dysplasia.

Methods: Preoperative, operative and postoperative data were obtained from an institutional database; follow-up data came from regular clinical evaluation at our institution or elsewhere. Patients were divided into isolated and complex cases according to the complexity of associated lesions.

Viral detection and tumor necrosis factor alpha profile in tracheal aspirates from children with suspicion of myocarditis.

Pediatric myocarditis is a serious disease resulting in significant morbidity and mortality. Tracheal aspirate (TA) has been demonstrated to be a sensitive diagnostic tool to detect viral agents responsible for respiratory disorders and myocardial dysfunction. Tumor necrosis factor alpha (TNFalpha) is thought to play an important role in the pathogenesis of these disorders.

Carotid artery approach as an alternative to femoral access for balloon dilation of aortic valve stenosis in neonates and infants.

Purpose: to evaluate the efficacy of a right common carotid artery cutdown as alternative access in neonates and small infants requiring a balloon dilation of aortic valve stenosis. In infants, the femoral approach is limited by difficulties in advancing the catheter across the valve and by the risk of femoral artery injuries.

Methods: from January 1997 to July 2000, 16 infants at our department underwent balloon dilation through a carotid artery cutdown.

Heart transplantation in pediatric age.

Heart transplantation, formerly the final option for terminally ill children, has now become the treatment of choice for a number of serious acquired or congenital cardiac conditions, which cannot be treated conservatively. Nevertheless, several problems remain unsolved. First of all the shortage of donors, mainly in the first months and years of life, which has become more and more significant with time, regardless of the country, religious belief or culture of the people.