Background In 2014 a professional collaboration was established between IRCCS Policlinico San Donato, Milan, Italy (PSD) and the Institute for Cardiovascular Disease Dedinje, Belgrade, Serbia (ICDD) to support and improve the care of adult patients with congenital heart disease (ACHD) in the country. Since 2018, this collaboration has become more structured, with a specific focus on surgery for adults with complex congenital heart disease (CHD). Aim of the study was to evaluate the outcomes of this collaboration. Methods We conducted a retrospective study on all consecutive adult patients (aged over 18 years) with CHD who underwent surgery at the Institute for Cardiovascular Disease Dedinje (ICDD) over a ten-year period, from January 2014 to December 2024. Collected data included number of cases, mean age at surgery, types of surgical procedures performed, number of previous operations, distribution of cases based on complexity, patient outcomes, and follow-up. Results Between 2014 and 2024, a total of 244 ACHD patients underwent surgery at ICDD. Eighty-eight of these patients (88/244 = 36%) required complex procedures and were referred to the visiting PSD cardiac surgeon. Mean age at surgery was 34.8 years (±14 years). The most common diagnosis was right ventricular outflow tract (RVOT) pathology, accounting for 89% of cases. Seventy patients (80%) underwent reoperations following previous procedures. In-hospital mortality was 4.5% (4 patients). No additional deaths or reoperations occurred during a mean follow-up of 58 months (2-122 months). Conclusions This ten-year international partnership demonstrates that high-quality ACHD surgical care can be delivered sustainably in countries with limited case volumes and expertise. By combining local access with external specialist support, the model has achieved outcomes comparable to high-volume Western European centres. Increased access to ACHD surgery in their home country and reduced financial burden suggests that this model of care may potentially serve in other countries facing similar healthcare challenges.

BACKGROUND:Percutaneous atrial septal defect (ASD) closure is an established alternative to surgical repair for patients with secundum ASD, offering minimally invasive treatment with high procedural success rates. Despite its widespread adoption, clinically significant complications such as device embolization and erosion may occur. Our Survey aims at providing a comprehensive, multicenter perspective on the incidence, features, and management strategies for these complications, in order to improve procedural outcomes and patient safety through collaborative data collection. METHODS:This is an international, multicenter survey collecting retrospective data from 30 centers performing transcatheter ASD closures between 2011 and 2021. Participating institutions contributed anonymous aggregate data on patient demographics, procedural details, and clinical outcomes, focusing on the incidence, anatomical and procedural risk factors, and management of device embolization or erosion. RESULTS:A total of 30 institutions participated, providing data on 13.155 procedures of transcatheter closure of ASD, with 40% of them in children. Amplatzer device was used in 58% of cases, with balloon sizing performed routinely in 62% of institutions, mainly with stop flow technique. A total of 17 erosions (0.13%) were reported, of which 10 in children (incidence 0.19%) and 7 in adults (incidence 0.09%). Surgical repair was performed in all these cases, one patient died. Embolizations were reported in 91 cases (incidence 0.7%), with 60% of them in children. Most (80%) occurred within 24 h. Surgical retrieval was carried out in 29% of cases. Irrespective of management, no fatal complications occurred. CONCLUSIONS:Complications such as embolization and erosion after transcatheter ASD closure in children and adults are exceedingly rare but still present in clinical practice and require careful monitoring for prompt recognition and timely intervention, in order to obtain favorable outcomes.

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.

Background Ebstein’s anomaly (EA) exhibits significant anatomical and clinical heterogeneity, warranting a systematic approach to risk stratification. While the Carpentier classification (CC) is commonly employed for qualitative disease assessment, there is increasing interest in quantitative imaging parameters to personalise monitoring strategies and therapeutic interventions. We sought to evaluate the association between cardiovascular magnetic resonance (CMR) parameters, CC and symptoms of heart failure (HF). Also, we investigated whether CMR-derived markers may predict the need for bidirectional cavopulmonary anastomosis (BCPA) and the occurrence of haemodynamic complications or significant right ventricular (RV) dysfunction in the postoperative stay.Methods This retrospective study evaluated consecutive patients diagnosed with EA undergoing 1.5T CMR acquisition at a tertiary care centre. CC and quantitative indices were extracted from steady-state free precession sequences.Results In a total population of 60 patients (53% male, median age 22 years), CMR parameters most strongly associated (p<0.001) with CC included indexed displacement of the septal (SLDi) and inferior (ILDi) leaflets, Ebstein valve rotation angle, functional RV ejection fraction, indexed atrialised RV end-diastolic volume and the ratio of either atrialised or functional RV to anatomical RV. Decreasing left ventricular ejection fraction (OR 0.85, 95% CI 0.75 to 0.97, p=0.01) and increasing ILDi (OR 1.05, 95% CI 1.00 to 1.10, p=0.04) emerged as the most prominent variables associated with HF symptoms. Additionally, ILDi was significantly linked to the need for BCPA (OR 1.15, 95% CI 1.03 to 1.28), the occurrence of haemodynamic complications (OR 1.09, 95% CI 1.01 to 1.18) and significant RV dysfunction in the postoperative stay (OR 1.08, 95% CI 1.01 to 1.17).Conclusions Quantitative CMR indices proved to be effective in distinguishing between Carpentier classes and they may be valuable in an integrated CMR-based approach to assess EA severity. Among these, ILDi reflects both the extent of tricuspid valve abnormality and RV atrialisation and may serve as a useful metric in guiding personalised therapeutic strategies.

Anomalous coronary artery origin from pulmonary artery and coronary-pulmonary fistulas are the major causes of ischemic cardiopathy in children. Both anomalies are characterized by a connection between a higher and a lower pressure chamber causing coronary steal. However, several mechanisms and associated lesions may be responsible of the different presentations of the "coronary steal phenomenon". The aim of this review is to highlight the different embryology, anatomical features, clinical presentation, and the diagnostic and therapeutic strategy of these coronary anomalies, despite their similar pathophysiology.

The extracardiac Fontan procedure is the most widely adopted surgical approach for definitive palliation of single-ventricle anomalies, offering improved hemodynamics and reduced arrhythmogenic risk. However, limited access to the pulmonary venous atrium during follow-up poses significant challenges for electrophysiologic and interventional procedures. We describe a simple technical modification of the extracardiac Fontan designed to preserve these advantages while facilitating future percutaneous access.

AIMS:The growing population of adults with congenital heart disease (ACHD) has prompted global initiatives to define standards of care, training pathways, and institutional requirements for catheter-based interventions. High-quality care and effective training rely on specialized, multidisciplinary centres integrating paediatric and adult cardiologists, surgeons, and anaesthesiologists with congenital expertise, supported by advanced catheter labs and hybrid theatres. This work outlines essential elements for optimal ACHD catheterization training, emphasizing a structured, competency-based curriculum covering theory, procedural skills, and post-procedural care. METHODS AND RESULTS:Collaboration between paediatric and adult cardiologists is highlighted as vital for comprehensive care. To meet national and regional needs, a two-tier model of 'level 1' and 'level 2' centres-each with specific capacities-is proposed. Scientific societies play a key role in establishing guidelines, certifications, and facilitating trainee mobility and international collaboration. Emerging tools such as augmented reality and virtual case libraries can improve accessibility and quality of training. The framework also addresses global equity, considering regional and socio-economic differences. Proctorships and partnerships with industry are integral for introducing innovations and maintaining skills in evolving techniques. CONCLUSION:By fostering collaboration, harmonized standards, and multidisciplinary expertise, this model aims to improve ACHD training and meet growing patient needs.

Neonates undergoing surgery for congenital heart disease (CHD) are at high risk for brain function impairment. Reliable early predictors of postoperative neurological complications are lacking. We examined a retrospective cohort of 55 surgically treated CHD neonates systematically monitored by concomitant conventional electroencephalography (cEEG) and amplitude-integrated EEG (aEEG). Neonates underwent cEEG/aEEG at three time points: T0 (preoperative, duration: 90–120 min); T1 (24–48 h after cardiac surgery, duration: ≥11 h); and T2 (7–10 days post-surgery, duration: 90–120 min). For each patient, aEEG background activity was evaluated and scored, and clinical and surgical data were retrieved to establish short-term post-surgical outcomes. Patients with normal T0 monitoring had significantly higher aEEG bandwidths in T1. A lower Aristotle basic score was associated with an improvement in aEEG at T1. Inversely, a narrower aEEG bandwidth in T1 was associated with post-surgical neurological deterioration. The aEEG bandwidth accurately predicted short-term neurological outcome; in particular, a minimal aEEG amplitude above 17.5 µV excluded poor neurological outcome with a negative predictive value of 81.48%. Our results demonstrated that aEEG bandwidth and trend dynamics may be associated with surgical complexity and neurological outcomes. aEEG background trend monitoring may provide relevant prognostic information on neurological outcomes in surgically treated CHD neonates.

PURPOSE:To address limitations in tricuspid regurgitation (TR) quantification by magnetic resonance imaging (MRI) or transthoracic echocardiography (TTE), a new method is proposed to directly quantify TR volume (TRV) using 4D Flow MRI with retrospective tricuspid valve (TV) annular tracking. METHODS:Thirty patients with TR were prospectively enrolled. TRV was quantified using four MRI-based methods: (i) indirect, (ii) direct (2D phase-contrast), (iii) 4D Flow with a static TV plane, and (iv) 4D Flow with dynamic annular tracking. Reproducibility and agreement were assessed in terms of TR grading against standard MRI- and TTE-based methods. RESULTS:The dynamic 4D Flow method correlated strongly with all other MRI methods (r = 0.77-0.81, p < 0.0001) but consistently yielded higher TRV values. Compared with the indirect method, it showed a Bland-Altman bias of 16 mL (limits of agreement: -19 to 51 mL), along with the highest agreement (intraclass correlation coefficient of 0.88). Agreement in TR severity grading was poor across methods (kappa ≤ 0.07), with dynamic 4D Flow classifying more cases as moderate or severe. Agreement with TTE was slight (kappa = 0.17, p = 0.27) with concordant TR grading in 50% of patients. Inter-observer reproducibility was excellent (r = 0.99, bias = 0.7 mL, kappa = 0.80). CONCLUSION:4D Flow with dynamic TV tracking offers a reproducible and potentially more sensitive method for TRV quantification than conventional MRI techniques. Despite limited alignment with TTE, this technique may help overcome key imaging limitations and improve TR clinical evaluation, especially in complex cases.

New cancer treatments led to a clear survival gain in childhood cancer patients in recent decades, but cardiovascular disease among long-term survivors represents the leading non-cancer cause of morbidity and mortality. The population of childhood, adolescent, and young adult (CAYA) cancer survivors is growing fast and is characterized by an increased cardiovascular risk, therefore requiring long-term cardiovascular surveillance for early diagnosis and timely treatment of cardiotoxicity. To increase follow-up adherence and implement preventive strategies, it is essential to ensure the transition process from pediatric to adult care. This document is the result of the collaboration between ANMCO and SICPED to ensure long-term care for CAYA cancer survivors and to guide clinicians in the transition phase.

Adolescents and young adults with congenital heart disease (CHD) face significant challenges when transitioning from pediatric to adult care. Despite growing recognition of its importance, transitional care remains inconsistently implemented across healthcare systems, and a summary of published literature in this regard is still missing. This scoping review aims to systematically map the literature on CHD transitional care and identify key topics and trends. Following Joanna Briggs Institute guidelines, we conducted a comprehensive search across seven electronic databases using the Population, Concept, and Context framework. A total of 73 studies were included. Data were extracted and analyzed using Latent Dirichlet Allocation to identify core topics, and Multiple Correspondence Analysis was applied to explore thematic relationships and validate topic structure. Lexicometric analysis assessed the linguistic complexity and specificity of the literature. Three major themes emerged: (1) Education, Self-Management, and Structured Support, (2) Timing, Knowledge Transfer, and Developmental Needs, and (3) Transition Program Implementation and Coordination. These themes reflect an increasing focus on structured educational strategies, developmentally tailored care, and system-level program delivery. Education-focused interventions were more frequently found in recent, high-quality experimental studies. In contrast, studies addressing timing and developmental needs and those focused on implementation were more common in earlier-phase or heterogeneous research contexts. Transitional care for individuals with CHD requires more standardized, evidence-based approaches. Improved documentation when reporting transitional care is essential to enhance fidelity, scalability, and long-term impact. This review provides a foundation for developing outcome-focused research and supports designing individualized, high-quality transition programs.

The recent document from the American Heart Association updates the guidelines on the diagnosis and management of Kawasaki disease (KD), a severe acute systemic inflammatory and febrile illness with mucocutaneous manifestations and lymph node involvement primarily affecting children under 5 years of age. Coronary artery involvement, with dilation and aneurysms in approximately 25% of patients, and cardiovascular system involvement make KD the most common systemic vasculitides and the leading cause of acquired heart disease in pre-school children living in developed countries. Classic KD is diagnosed based on established clinical and laboratory criteria, which exclude other similar conditions. The etiology and pathogenesis of KD remain unknown, with the disease affecting genetically susceptible children through an immune-mediated mechanism. The leading theory is that an unidentified trigger initiates a multi-organ inflammatory pathological cascade, sometimes resulting in incomplete or atypical forms in younger patients. For this reason, the American guidelines review KD diagnostic criteria, cardiac imaging techniques (echocardiography, coronary computed tomography, magnetic resonance imaging), specific therapies (intravenous immunoglobulin, aspirin, and additional treatments for resistant cases), management of myocardial infarctions, and the transition of care from pediatric to adult age. This review article also highlights future research areas, the role of inflammation, the development of differential diagnostic algorithms for multisystem inflammatory syndrome in children associated with SARS-CoV-2 infection, and the use of new oral anticoagulants. Lastly, the most recent data on the long-term course of the disease and the regression of coronary aneurysms are revisited.

Nelle ultime decadi la sopravvivenza dei pazienti trattati per tumore pediatrico è notevolmente migliorata, ma tra i lungosopravviventi la malattia cardiovascolare rappresenta la prima causa non oncologica di morbilità e mortalità. La popolazione dei bambini, adolescenti e giovani adulti (childhood, adolescent, and young adult, CAYA) sopravvissuti al cancro è in aumento e presenta un maggior rischio cardiovascolare, per cui necessita di una sorveglianza cardiovascolare a lungo termine per identificare precocemente una cardiotossicità tardiva e iniziare un trattamento tempestivo. Per evitare la dispersione al follow-up e attuare strategie preventive personalizzate, è fondamentale assicurare il processo di transizione e il trasferimento delle cure dall’ospedale pediatrico a quello dell’adulto. Questo documento nasce dalla collaborazione tra ANMCO e SICPED per garantire la continuità di cure dei CAYA e promuovere percorsi di transizione, ancora poco strutturati nel nostro Paese, nonostante le linee guida internazionali.

Background. Cor triatriatum dexter (CTD) is a rare congenital heart defect where a membrane divides the right atrium into two chambers, resulting from the incomplete regression of the right valve of the sinus venosus. Due to its rarity, only individual case reports and a limited number of case series have been published to date. This study constitutes the most extensive comprehensive review conducted in this area. Eight factors were evaluated: age at diagnosis, sex, clinical presentation, electrocardiographic findings, imaging (ultrasound, CT, or MRI), associated cardiac anomalies, and patient outcomes. Methods. The electronic databases PubMed and Scopus were searched from their inception until 30 October 2025. Only case reports and case series were considered for inclusion. Studies involving foetuses, autopsies, and animals were excluded. The collected data were primarily presented as percentages. Results. One hundred fourteen studies were found encompassing 124 patients. The mean age at diagnosis was 33.3 ± 9.4 years The most common clinical presentations were dyspnoea (44.3%) and cyanosis (29.5%). The most commonly encountered ECG changes were supraventricular tachycardia/atrial flutter/atrial fibrillation (33.3%) and right bundle branch block (22.6%). On chest X-ray, cardiomegaly was noted in 46.5%. CTD was suspected or diagnosed by echocardiography in 95.2% of cases. The diagnosis was confirmed by CT and/or MRI in 34.1% of cases. A concomitant congenital heart defect was found in 67.7%, especially in the form of all kinds of atrial septal defect (38.1%) and of right valvular and right ventricular involvement (20.1%). An outcome was reported in 97/124. Surgical correction was the treatment of choice in 51.6%. Since 1991, a percutaneous approach has been employed in selected cases (5.1%). Conservative management was the treatment of choice in 43.3%. The mortality rate was 8.2%. Discussion. The principal limitation of this systematic review lies in its reliance solely on case reports and small case series, reflecting the absence of large-scale studies on CTD. Nonetheless, it constitutes the most comprehensive analysis available to date.

Neonates with complex congenital heart disease (CHD) frequently require early and staged surgical interventions, which carry a high risk of postoperative complications. The coexistence of an interrupted aortic arch, ventricular septal defect (VSD), and mitral valve stenosis represents a rare and particularly high-risk constellation. This case is noteworthy for the integration of multistage cardiac surgery, prolonged extracorporeal membrane oxygenation (ECMO), and an innovative wound management strategy combining negative pressure wound therapy (NPWT) with topical oxygen-enriched gel. We report the case of a one-month-old Romanian female infant diagnosed with type A interrupted aortic arch, VSD, and mitral valve stenosis. The initial surgical procedure included aortic arch reconstruction, VSD closure with a bovine pericardial patch, and mitral valve repair. Severe residual mitral regurgitation necessitated immediate initiation of veno-arterial ECMO, followed by mitral valve replacement with a Melody bioprosthesis. On postoperative day 19, thrombotic obstruction of the prosthetic valve required a third surgical intervention for clot removal, after which ECMO was discontinued. Delayed sternal closure was performed on day 26, but wound dehiscence developed on day 33. Management included NPWT combined with topical oxygen-enriched gel for 33 days, followed by gel monotherapy for 38 additional days. Complete secondary intention healing was achieved after 73 days. Despite developing Pseudomonas aeruginosa sepsis and late-onset Staphylococcus haemolyticus bacteremia, the patient recovered fully without neurological deficits or wound-related sequelae. This case highlights the feasibility of combining advanced surgical management with innovative wound care strategies in critically ill neonates with complex CHD. The successful integration of NPWT and topical oxygen therapy promoted wound healing after delayed sternal closure, even in the context of severe infection and prolonged ECMO support. These findings suggest that tailored, multidisciplinary approaches may optimize outcomes in high-complexity neonatal cardiac cases.

New cancer treatments led to a clear survival gain in childhood cancer patients in recent decades, but cardiovascular disease among long-term survivors represents the leading non-cancer cause of morbidity and mortality. The population of childhood, adolescent, and young adult (CAYA) cancer survivors is growing fast and is characterized by an increased cardiovascular risk, therefore requiring long-term cardiovascular surveillance for early diagnosis and timely treatment of cardiotoxicity. To increase follow-up adherence and implement preventive strategies, it is essential to ensure the transition process from pediatric to adult care. This document is the result of the collaboration between ANMCO and SICPED to ensure long-term care for CAYA cancer survivors and to guide clinicians in the transition phase.

The recent document from the American Heart Association updates the guidelines on the diagnosis and management of Kawasaki disease (KD), a severe acute systemic inflammatory and febrile illness with mucocutaneous manifestations and lymph node involvement primarily affecting children under 5 years of age. Coronary artery involvement, with dilation and aneurysms in approximately 25% of patients, and cardiovascular system involvement make KD the most common systemic vasculitides and the leading cause of acquired heart disease in pre-school children living in developed countries. Classic KD is diagnosed based on established clinical and laboratory criteria, which exclude other similar conditions. The etiology and pathogenesis of KD remain unknown, with the disease affecting genetically susceptible children through an immune-mediated mechanism. The leading theory is that an unidentified trigger initiates a multi-organ inflammatory pathological cascade, sometimes resulting in incomplete or atypical forms in younger patients. For this reason, the American guidelines review KD diagnostic criteria, cardiac imaging techniques (echocardiography, coronary computed tomography, magnetic resonance imaging), specific therapies (intravenous immunoglobulin, aspirin, and additional treatments for resistant cases), management of myocardial infarctions, and the transition of care from pediatric to adult age. This review article also highlights future research areas, the role of inflammation, the development of differential diagnostic algorithms for multisystem inflammatory syndrome in children associated with SARS-CoV-2 infection, and the use of new oral anticoagulants. Lastly, the most recent data on the long-term course of the disease and the regression of coronary aneurysms are revisited.