European Heart Journal Supplements最新文献

Since the first operation performed in 1968, the worldwide population of patients living with Fontan circulation is dramatically growing, with 40% reaching adulthood in the current era. Despite this remarkable improvement in survival, some patients struggle with Fontan-related complications including heart failure, arrhythmias, end-organ dysfunction, and premature death. This review aims at describing common complications of the Fontan circulation, proposing a clinical and mortality risk score to better stratify this complex population as well as exploring the current state of art in catheter interventions and transplant.

Recent advances in congenital heart disease management reflect a paradigm shift towards less invasive, physiology-preserving, and durable interventions. Transcatheter pulmonary valve implantation has become an established therapy for right ventricular outflow tract dysfunction, supported by multiple valve systems tailored to increasingly complex anatomies. These devices have demonstrated high procedural success and sustained hemodynamic performance, although infective endocarditis and arrhythmic complications remain clinical concerns. In parallel, transcatheter edge-to-edge repair for atrioventricular valve regurgitation (AVVR) has expanded from acquired disease to congenital populations, particularly in patients with single-ventricle physiology or systemic AVVR where surgical risk is prohibitive. Early experiences with devices such as MitraClip™, TriClip™, and Pascal have shown promising results in improving valve competence and functional status. From a surgical perspective, the Ross procedure continues to offer a unique, physiological alternative for aortic valve replacement in selected young adults, achieving excellent long-term outcomes when performed in high-volume centres with reinforcement strategies to prevent autograft dilation. Similarly, percutaneous correction of sinus venosus atrial septal defect using covered stents has emerged as a safe and effective alternative to conventional repair, providing anatomical reconstruction with low complication rates. The modern Fontan circulation exemplifies the evolution of single-ventricle palliation, emphasizing conduit optimization, pro-active re-intervention, and individualized surgical planning to sustain function over time. Collectively, these innovations underscore the growing integration of transcatheter and surgical therapies, re-defining long-term management paradigms and improving survival and quality of life in patients with congenital heart disease.

Patients with a systemic right ventricle (sRV)-including those with transposition of the great arteries (TGA) following atrial switch repair (Mustard or Senning procedures) and congenitally corrected TGA-represent one of the greatest challenges in the management of adults with congenital heart disease (ACHD). The right ventricle is not anatomically designed to sustain systemic pressure over a lifetime. As a result, sRV failure is almost inevitable and follows a progressive, multifactorial process that initiates with maladaptive functional and structural changes. In later stages, arrhythmias, conduction delays, and pacing-induced dyssynchrony emerge, accelerating deterioration. Finally, lifestyle and environmental factors such as inactivity, obesity, or pregnancy may trigger clinical decompensation. Recognizing where a patient lies along this continuum and identifying those at highest risk is essential to anticipate deterioration and implement timely interventions. Despite significant advances in the management of ACHD, important gaps in knowledge and research remain regarding the sRV, particularly in understanding its long-term adaptation, predictors of failure, optimal medical therapy, prevention of dyssynchrony, and timing for transplant. We herewith summarize latest evidences on prognostic determinants, multimodality imaging, heart failure therapy, electrical therapy for heart failure, mechanical circulatory systems, and heart transplant in sRV.

In recent years, remarkable advancements in paediatric cardiology and surgical techniques have dramatically increased the survival of patients with congenital heart disease. As a result, adults with congenital heart disease (ACHD) are now living longer, and many, despite having residual lesions and sequelae, may have near-normal life expectancy. Adults with congenital heart disease patients should no longer be viewed merely as survivors; instead, medical care should shift its focus from simply prolonging life to enhancing quality of life and overall well-being. Moreover, as ACHD population ages, they are increasingly exposed to a burden of acquired cardiovascular and extracardiac disease, making early intervention on modifiable risk factors a key priority. Herewith, we provide an overview of the multiple aspects that should be considered in this holistic approach to guide everyday issues of ACHD patients, including nutrition, physical activity, sexual health, cancer prevention, respiratory disease, and infective endocarditis.

Advances in paediatric cardiology and surgery have transformed congenital heart disease from a fatal condition into a chronic, lifelong one. As a result, the adult congenital heart disease (ACHD) population is rapidly expanding and aging, with rising medical complexity and growing workforce shortages. The ACHD patient of tomorrow will present with diverse needs-ranging from management of complex congenital repairs and heart failure (HF) to acquired comorbidities typical of aging populations. This shift necessitates a reimagined model of care emphasizing accessibility, precision, and patient partnership. Telemedicine, remote monitoring, and digital health platforms will decentralize ACHD care, improving follow-up and reducing disparities. Precision medicine, integrating genomics, proteomics, metabolomics, and microbiomics, will enable individualized diagnosis and therapy. Artificial intelligence (AI) will enhance early detection, risk prediction, and surgical planning while supporting patient education and engagement. Future ACHD patients will be empowered participants in shared decision-making, supported by AI-driven tools and online communities. Personalized strategies for HF management, especially in Fontan circulation and systemic right ventricles, along with advances in transcatheter interventions, will minimize surgical trauma and improve quality of life. Equally, mental health and social integration will be central to holistic care. Ultimately, tomorrow's ACHD care will merge technology and humanity-leveraging innovation to deliver compassionate, individualized treatment across a patient's lifespan.

The population of adult patients with congenital heart disease (ACHD) is rapidly growing thanks to advances in diagnostics and therapeutics. However, these patients now encounter new clinical and healthcare challenges. The 'New perspectives in ACHD management' session at the ACHD 2025 Congress highlighted the key areas of innovation aimed at improving prognosis and quality of life. The aging ACHD population places substantial demands on geriatric medicine, with significant implications for the healthcare system. Similarly, the increasing arrhythmic burden, in particular atrial fibrillation, requires specific preventive and therapeutic strategies, including ablation procedures tailored to the anatomical peculiarities of ACHD patients. Pulmonary hypertension is a major contributor to morbidity and mortality in ACHD, requiring precision medicine approaches, dedicated risk stratification, and multidisciplinary approach. A central role is played by adequate prevention of acquired cardiovascular diseases, whose incidence is higher and occur earlier than in the general population. Specific risk stratification tools and an integrated approach of lifestyle modification and innovative pharmacological therapy are necessary. Telemedicine offers new opportunities for remote monitoring, access to specialized expertise, and comprehensive support, particularly for frail patients or those living in remote areas. Lastly, emerging technologies such as 3D printing, virtual reality, computational modelling, and artificial intelligence are transforming treatment planning and enabling personalized care. A multidisciplinary and technologically advanced approach is essential for shifting the focus from merely prolonging survival to truly improving the quality of life of ACHD patients.

Arrhythmic sudden cardiac death (SCD) remains a major cause of late mortality in adults with congenital heart disease (ACHD), second only to heart failure. Risk prediction is challenging due to the heterogeneity of underlying defects and surgical histories. This review summarizes contemporary evidence on arrhythmic SCD prevention in ACHD, focusing on lesion-specific risk stratification, device-based therapies, and emerging technologies. Traditional markers-ventricular dysfunction, QRS prolongation, and prior arrhythmias-show limited predictive value. Recent models such as PREVENTION-ACHD and the Spanish ACHD Network score offer structured but evolving approaches to individualized risk assessment. Cardiac MRI-derived fibrosis quantification, electro-anatomical mapping, and machine-learning-based tools hold promise for refined prediction. Implantable cardioverter-defibrillators (ICDs) remain the cornerstone of secondary prevention, while subcutaneous ICDs and emerging extravascular systems mitigate lead-related complications. The wearable cardioverter-defibrillator offers a temporary protective option in high-risk patients awaiting definitive therapy. Catheter ablation-particularly in repaired Tetralogy of Fallot-can modify arrhythmic substrates, whereas bradyarrhythmias require vigilant monitoring and timely pacing interventions. In conclusion, effective prevention of arrhythmic SCD in ACHD demands a multidisciplinary, lesion-specific approach integrating advanced imaging, device innovation, and personalized risk modelling. Ongoing refinement of predictive tools and interventional strategies will be crucial to reduce mortality in this growing population.

[This retracts the article DOI: 10.1093/eurheartjsupp/suaf043.].

[This corrects the article DOI: 10.1093/eurheartjsupp/suaf003.].