Almudena Ortiz Garrido

Bakcgroung: Training in pediatric and congenital echocardiography has progressed substantially over the past two decades, supported by structured recommendations in North America and Europe. However, while North American training pathways are generally more standardized and closely linked to accreditation and certification, European training remains heterogeneous. Differences in healthcare systems, regulatory frameworks, institutional organization, supervision, case exposure, and access to advanced imaging may result in unequal acquisition of competencies, with potential implications for patient care. The aim of this expert consensus document is to update European standards for training in pediatric and congenital echocardiography and to promote harmonization through a competency-based framework that complements, rather than replaces, national regulatory systems and existing certification pathways. Methods: Developed by the Imaging Working Group of the Association for European Paediatric and Congenital Cardiology (AEPC), in collaboration with the Congenital Heart Disease Shared Interest Group of the European Association of Cardiovascular Imaging (EACVI), the document defines three progressive levels of expertise: Basic, Advanced, and Specialized/Tertiary. Each level is characterized by specific competencies, indicative procedural experience, and expected professional responsibilities. Building on previous European recommendations and the recent joint statement addressing laboratory organization and service governance, this document focuses specifically on the educational pathway, progressive acquisition of clinical competencies, and assessment of individual practitioners. Results: The proposed framework emphasizes comprehensive training across the lifespan of congenital heart disease, including competencies bridging pediatric and adult cardiology. It incorporates contemporary imaging modalities, including three-dimensional echocardiography, myocardial deformation imaging, transesophageal and fetal echocardiography, and emerging blood speckle tracking and artificial intelligence applications. Minimum requirements for training centres, structured assessment, quality assurance, continuous professional development, research activity, and multidisciplinary collaboration are also addressed. Procedural volumes are proposed as indicative benchmarks to support training and assessment, not as stand-alone evidence of competence or universally mandatory thresholds. Conclusions: This consensus document provides a shared European reference framework designed to reduce unwarranted variability in training, facilitate professional mobility, promote competency-based progression, and ensure that pediatric and congenital echocardiography remain aligned with current clinical practice and technological innovation.

This document has been developed to provide a practical guide for the application of speckle tracking echocardiography (STE) in paediatric and congenital heart diseases. In the present document, the following topics are reviewed and discussed: (i) the basic principles of STE, its potential applications, and limitations in the paediatric population; (ii) normal paediatric reference values for STE; and (iii) the use of STE in various congenital and acquired heart diseases. The potential role of routine STE in the management and follow-up of paediatric and congenital heart disease is explored, with practical examples illustrating how STE can provide additional insights beyond conventional echocardiographic parameters. Limitations regarding clinical interpretation, prognostic value, the lack of standardized timing for measurements, and the absence of universally accepted thresholds for STE-derived strain parameters are also highlighted. Overall, this document is intended to serve as a comprehensive guide to (i) orient clinicians regarding the strengths and limitations of STE in paediatric and congenital heart disease; (ii) identify specific clinical scenarios where STE may be particularly informative; and (iii) encourage future research and multicentre collaborative studies. By providing both practical guidance and a framework for further investigation, the document aims to support standardized use of STE, improve patient care, and foster the integration of this technique into routine paediatric and congenital cardiology practice.

Although echocardiography remains the primary imaging modality for assessing Fontan palliation, a standardized systematic approach for evaluating the univentricular heart throughout the different stages of palliation has yet to be established. This document aims to provide a narrative review and practical guide for the echocardiographic assessment of the univentricular heart at various phases of Fontan palliation. Additional objectives include highlighting the potential of advanced three-dimensional (3D) and four-dimensional (4D) imaging modalities, as well as outlining a systematic strategy for detecting thromboembolic complications. We propose a sequential framework for echocardiographic evaluation, encompassing key anatomical and functional components of the univentricular heart. This includes the atrial septum and pulmonary veins, ventricular function, atrioventricular valve, aorta and neo-aorta, sub-aortic region, aortic arch, and pulmonary arteries. Furthermore, we detail the assessment of pulmonary blood supply at different stages of palliation, including the modified Blalock–Taussig–Thomas (mBTT) shunt, Sano conduit, Glenn procedure, and total cavo-pulmonary connection (TCPC). A comprehensive analysis of potential thrombus formation sites is provided, along with diagnostic pitfalls. Additionally, we outline methods for screening for extracardiac clots following Glenn and TCPC procedures. This document serves as a practical guide for the systematic echocardiographic evaluation of the univentricular heart across all stages of Fontan palliation, offering guidance for clinical practice. It also explores the capabilities of emerging 3D and 4D echocardiographic techniques in univentricular heart assessment and provides an in-depth review of thromboembolic complications, emphasizing key diagnostic challenges.

Chronic pulmonary regurgitation (PR) after the repair of tetralogy of Fallot (TOF) and other right ventricular outflow tract (RVOT) interventions leads to progressive right ventricular (RV) dilatation, altered ventricular–ventricular interaction, and an increased risk of arrhythmia and heart failure. Pulmonary valve replacement (PVR), whether surgical or transcatheter, effectively eliminates or reduces PR and is associated with short- and mid-term improvement in RV size, symptoms, and electrocardiographic markers. However, the optimal timing of intervention remains unresolved: operating late can result in irreversible myocardial damage and arrhythmogenic substrates, whereas operating early can lead to repeated reinterventions, the impact of which on hard outcomes is uncertain. This review summarizes contemporary evidence on ventricular remodelling after PVR, focusing on cardiovascular magnetic resonance (CMR) and echocardiographic markers, and critically appraises proposed criteria for timing PVR. Classic CMR-derived thresholds (RV end-diastolic volume index [RVEDVi] 150–170 mL/m², RV end-systolic volume index [RVESVi] 80–90 mL/m²) and QRS duration cut-offs are discussed alongside emerging markers of risk, including the RV mass-to-volume ratio, diffuse myocardial fibrosis (extracellular volume fraction), strain imaging, and diastolic dysfunction. Meta-analyses show consistent reverse remodelling and symptomatic benefit after PVR, but no conclusive survival benefit has been demonstrated, and data on arrhythmic outcomes remain conflicting. Key gaps include (i) the lack of prospective randomized or carefully matched comparative studies of “early” versus “deferred” PVR; (ii) limited understanding of how myocardial fibrosis, RV hypertrophy, and diastolic dysfunction interact with volume load and timing to influence long-term outcomes; (iii) under-representation of adult and older adult TOF cohorts; and (iv) insufficient integration of multiparametric risk scores and machine-learning approaches into clinical decision-making. Future research should prioritize multicentre longitudinal cohorts with standardized imaging, electrophysiological and clinical endpoints, incorporate advanced imaging techniques (e.g., strain, 3D late gadolinium enhancement, and T1 mapping), and explore precision-medicine strategies to individualize PVR timing.

Introduction and objective Double-chambered left ventricle (DCLV) is an extremely rare congenital heart disease in which a muscular band or fibromuscular structure divides the left ventricle into two compartments. This condition is less frequent than double-chambered right ventricle and is usually not associated with other congenital heart defects. The aim of this study is to review reported cases in the literature and clarify the diagnosis, clinical features, and management strategies for this anomaly. Methods We performed a literature review of previously reported cases, focusing on anatomical features, clinical presentation, diagnostic techniques, and therapeutic approaches for DCLV. Results DCLV may present in asymptomatic patients or manifest with clinical symptoms such as dyspnea, chest pain, or arrhythmias. Diagnosis relies primarily on imaging, with cardiac magnetic resonance (CMR) being the gold standard due to its ability to assess anatomical features, ventricular function, and myocardial viability. Management is typically conservative, although medical therapy may be required for symptomatic patients. Surgical intervention is indicated in cases of significant obstruction. Conclusion Despite its rarity, DCLV may have significant clinical implications, including arrhythmogenic risk, making timely diagnosis and appropriate follow-up essential for patient management. Graphical Abstract

Cross-sectional cardiac imaging including cardiac computed tomography (CCT) and cardiac magnetic resonance imaging (CMR) allows three-dimensional imaging of intracardiac and extracardiac structures. This study was undertaken to better understand current European practice of pediatric cardiology cross-sectional imaging, in addition to how training is undertaken in different centers. A structured detailed 64-question survey focusing on cross-sectional imaging was circulated to all Imaging Working Group members of the 95 AEPC affiliated centers. Consultants from 42 centers (44%) from 25 different European countries completed the survey, between 7th July 2024 and 28th October 2024. Ninety percent of centers have been performing pediatric CCT for > 10 years, with median 135 scans/year. Comparatively, 84% of centers have been performing CMR for > 10 years, with a median 200 scans/year. CCT was performed by a pediatric radiologist, whereas CMR was more often performed by a pediatric cardiologist, but frequently both modalities were undertaken by a combination of pediatric cardiologists and radiologists. Less than two-third of centers surveyed train fellows in CMR or CCT. Many fellows travel abroad to obtain specialist training. Just over half of the centers surveyed have institutional guidelines for pediatric CCT/CMR, with the majority agreeing that provision of European (AEPC-led) guidelines for pediatric CCT/CMR would be helpful. Across Europe there is significant variation in how CCT/CMR is performed and how pediatric cardiologists are trained in cross-sectional imaging. This survey highlights the current state of cross-sectional imaging in Europe, challenges faced, but also explores potential solutions to standardize European cross-sectional imaging.