Multimodal Assessment in Univentricular Heart Disease and Fontan Circulation

Clinical management and decision-making in univentricular heart disease and Fontan circulation is one of the most complex challenges in congenital cardiology. The wide range of anatomic and hemodynamic presentations in single ventricle malformations together with the challenging management of Fontan patients from childhood onwards, results in substantial clinical and hemodynamic risks including the development of multi-organ complications and the failing Fontan circulation. Traditional assessment strategies often fall short in capturing the nuanced physiological and structural alterations that arise as pediatric patients progress from pre-Fontan stages through long-term post-Fontan follow-up. Recent advances in the understanding of univentricular and Fontan circulation include developments in lymphatic imaging and interventions, integration of multimodal cardiac and extracardiac imaging, and translational approaches incorporating novel biomarkers. These innovations demonstrate that combining diverse diagnostic modalities, enables early identification of clinical deterioration, guides patient-specific interventions, and has the potential to improve long-term outcomes and quality of life. However, robust evidence and structured integration of these promising strategies into everyday pediatric clinical practice remain limited, especially regarding the management of long-term complications in children and defining best practices for failing Fontan patients.Key unmet needs include evidence-based medical therapy for heart failure, pulmonary hypertension and anticoagulation in single-ventricle patients. Despite rapid progress in adult heart failure pharmacotherapy, data on novel agents such as SGLT2 inhibitors, sacubitril/valsartan, vericiguat, and emerging pulmonary hypertension therapies in pediatric and adolescent Fontan physiology remain scarce.Advanced interventional and surgical treatment strategies, including mechanical circulatory support and heart transplantation, represent the final therapeutic options for end-stage Fontan failure but continue to pose substantial challenges. Critical unresolved questions include optimal timing for transplant listing, management of complex anatomies such as isomerism, and perioperative risk stratification. In addition, early clinical experience with subpulmonary mechanical assistance using the Berlin Heart Fontan cannula is limited, and its role in stabilizing failing Fontan circulation remains to be defined.This Research Topic aims to highlight innovations in the comprehensive and dynamic assessment of pediatric patients with univentricular heart disease and Fontan physiology. The goal is to further define best practices and encourage research that unifies multimodal imaging techniques, such as echocardiography, cardiac MRI (including native and T2-weighted lymphatic MRI), or computed tomography, with functional testing and laboratory-based biomarkers. Of particular interest are studies further investigating lymphatic abnormalities and their contribution to complications such as protein-losing enteropathy and plastic bronchitis in pediatric patients.To gather further insights within the spectrum of multimodal pediatric assessment in univentricular and Fontan circulation, the scope of this Research Topic is centered on the unique challenges and advances for children and adolescents, from initial staging through long-term follow-up. We welcome articles addressing, but not limited to, the following themes:• Multidisciplinary pediatric follow-up protocols and longitudinal care models for single-ventricle and Fontan patients• Quantitative cardiac, vascular and lymphatic imaging metrics including longitudinal studies in children• Innovative interventional strategies guided with advanced imaging modalities for pediatric patients • Integration of imaging findings with hemodynamic data, patient phenotyping, and clinical outcomes• Cardiopulmonary exercise testing and wearable activity monitoring for prognostic stratification • Machine learning or algorithmic frameworks for multimodal pediatric data integration and individualized risk prediction• Translational research including, including biomarker and metabolomic approaches • Medical therapy in pediatric single-ventricle physiology, including heart failure, pulmonary hypertension and anticoagulation strategies• Mechanical circulatory support and heart transplantation in pediatric Fontan circulation• Novel interventional and surgical therapies for the Failing Fontan circulation.We welcome original research articles, reviews, brief reports, case studies and protocols, addressing these pediatric-focused themes. Topic editors: Julia Moosmann, Anastasia Schleiger, Enrico Piccinelli, Rouven Kubicki Source: https://www.frontiersin.org/research-topics/78170/multimodal-assessment-in-univentricular-heart-disease-and-fontan-circulation