Myocardial hypertrophy (MH) represents a complex and heterogeneous condition in the pediatric and young adult population. While rare in children, MH encompasses a wide spectrum of physiological and pathological entities, ranging from transient hypertrophy in the infants of diabetic mothers to progressive genetic hypertrophic cardiomyopathies (HCM) with significant morbidity and mortality. Differential diagnosis is critical, as many phenocopies-including metabolic, mitochondrial, and syndromic diseases-can mimic HCM.
View Article and Find Full Text PDFInfective endocarditis (IE) remains a significant cause of morbidity and mortality in pediatric patients with congenital heart disease (CHD), however, data on its epidemiology, risk factors, symptoms, complications, and outcomes remain scarce. This retrospective multicenter study analyzed pediatric patients (< 18 years) with CHD diagnosed with IE between 2008 and 2023. Clinical data-including demographic information, cardiac history, echocardiographic findings, microbiological results, treatment strategies, and outcomes-were collected and analyzed.
View Article and Find Full Text PDFBackground: Double-chambered left ventricle (DCLV) is a rare congenital heart anomaly and can be easily misdiagnosed with transthoracic echocardiography. Cardiac magnetic resonance (CMR) can help in the diagnostic process and risk stratification.
Case Summary: A 15-year-old girl diagnosed with left ventricular noncompaction and unsustained ventricular tachycardia was referred to our institution for further characterization.
Children (Basel)
June 2025
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).
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.
View Article and Find Full Text PDFJ Cardiovasc Echogr
April 2025
Critically ill patients in the intensive care unit (ICU) require continuous hemodynamic monitoring to guide therapeutic decisions and prevent clinical deterioration. Echocardiography has emerged as a cornerstone for noninvasive hemodynamic assessment, offering real-time, bedside evaluation of key parameters such as venous congestion, pulmonary pressures, left atrial pressure (LAP), systemic vascular resistances, cardiac output, and ventricular-arterial coupling. Systemic venous congestion and right atrial pressure (RAP) can be assessed through inferior vena cava diameter measurement and respiratory variation, with additional accuracy provided by the VeXUS score, which incorporates hepatic, portal, and renal vein Doppler profiles.
View Article and Find Full Text PDFUnlabelled: Despite significant advances in knowledge and the development of guidelines, the management of hypoplastic left heart syndrome (HLHS) remains highly variable. A structured questionnaire was circulated across European Association of Paediatric & Congenital Cardiology (AEPC) affiliated centres. The aims were to evaluate standards in pre-operative assessment, types of surgery, follow-up and medical practices in children with HLHS.
View Article and Find Full Text PDFVeno-arterial extracorporeal life support (VA ECLS) is crucial for neonatal and paediatric patients with severe circulatory collapse but may cause left ventricular (LV) overload, affecting myocardial recovery and causing complications. Evidence on optimal LV decompression strategies in paediatric patients remains limited. We conducted a systematic review following PRISMA guidelines, including articles published between 1993 and 2024 focused on VA ECLS and LV decompression in patients under 18 years of age.
View Article and Find Full Text PDFBackground: 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.
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.
View Article and Find Full Text PDFBackground 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.
View Article and Find Full Text PDFEur Heart J Imaging Methods Pract
January 2025
Paediatric myocarditis is a challenging and heterogeneous condition, with varied clinical presentations ranging from mild symptoms to life-threatening complications such as heart failure, arrhythmias, and sudden cardiac death. Effective management hinges on early diagnosis, appropriate treatment, and ongoing monitoring, which can be significantly enhanced through multimodal imaging techniques. This review emphasizes the crucial role of advanced imaging in the diagnosis, prognostication, and management of paediatric myocarditis.
View Article and Find Full Text PDFEur Heart J Cardiovasc Imaging
June 2025
Congenital heart disease (CHD) is the most common inborn defect affecting up to 1% of newborns. The prevalence of CHD has shifted from childhood to adulthood, and the number of adult patients living with CHD continues to increase. This patient population presents unique challenges in diagnostic imaging and management due to complex underlying cardiac morphology, previous operations and interventions, and haemodynamic conditions.
View Article and Find Full Text PDFBackground: 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.
View Article and Find Full Text PDFAlthough advances in medical and surgical management have significantly improved clinical outcomes, infective endocarditis (IE) remains a significant threat to patients with congenital heart disease (CHD). The complexity of cardiac anatomy, the presence of prosthetic materials, and the emergence of novel pathogens pose unique diagnostic challenges in this specific population. However, the use of personalized imaging, integrating the strengths of each modality, has the potential to refine the diagnostic process, thereby optimizing diagnostic accuracy, guiding therapeutic decisions, and, ultimately, improving patient clinical outcomes.
View Article and Find Full Text PDFFontan-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.
View Article and Find Full Text PDFTranscatheter 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.
View Article and Find Full Text PDFBackground: Despite lung ultrasound (LUS) gaining consensus for the diagnosis of pulmonary complication in paediatric acute care setting and in adult cardiology, its use in paediatric cardiology remains limited.
Aim: The aim of the present investigation is to provide an expert opinion on the applications of LUS in neonates and children with congenital heart disease, with a special focus on the post-surgical period.
Methods And Results: A complete guide for identification of landmarks and major signs (A and B lines) and their characteristics is provided.
J Clin Med
February 2025
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.
View Article and Find Full Text PDFFront Cardiovasc Med
February 2025
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.
View Article and Find Full Text PDFJ Cardiovasc Echogr
December 2024
The latest ESC guidelines advocate for a multimodality imaging approach in diagnosing infective endocarditis, incorporating echocardiography, computed tomography (CT), magnetic resonance imaging, positron emission tomography/CT, and single-photon emission CT/CT to enhance diagnostic accuracy. This case study examines the limitations of this approach in a patient with suspected prosthetic valve endocarditis, suggesting that while multimodality imaging is valuable, a clinically oriented strategy may be more effective in certain situations.
View Article and Find Full Text PDFJ Cardiovasc Echogr
December 2024
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.
Eur Heart J Cardiovasc Imaging
March 2025
With this document, the European Association of Cardiovascular Imaging provides an Expert Consensus on the role of multi-modality imaging (MMI) in the management of patients with multiple valvular heart disease (MVD). Emphasis is given to the use of MMI to unravel the diagnostic challenges that characterize these patients and to improve risk stratification. Complementing the last European Society of Cardiology and European Association of Cardio-Thoracic Surgery guidelines on valvular heart disease, this Expert Consensus document also outlines how MMI assessment should form an integral part of the multi-disciplinary heart team discussion for patients with MVD to help with complex decision-making regarding the choice and timing of treatment.
View Article and Find Full Text PDFAlmost 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.
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