Congenital absence of the pericardium (CAP) is an unusual condition discovered, in most cases, incidentally but can potentially lead to fatal complications, including severe arrhythmias and sudden death. Recently, the use of modern imaging technologies has increased the diagnosis of CAP, providing important findings for risk stratification. Nevertheless, there is not yet consensus regarding therapeutic decisions, and the management of patients with CAP remains challenging. In this paper, we discuss the pathophysiological implication of CAP, review the current literature and explain the role of multimodality imaging tools for its diagnosis, management and treatment.
Pulmonary disfunction is frequent in repaired congenital heart diseases. Both pulmonary regurgitation and pulmonary stenosis are possible complications over time. In the past, the surgical approach was the only feasible management but exposed the patient to a redo-surgery with its consequent risks. Nowadays, the development of novel devices and techniques has made possible a transcatheter pulmonary valve implantation. The Melody Transcatheter Pulmonary Valve (TPV) (Medtronic Inc., Minneapolis, MN, USA) and the Edwards Sapien XT and S3 Transcatheter Heart Valve (Edwards Lifesciences LLC, Irvine, CA, USA) are balloon-expandable valvular bioprostheses approved for pulmonary position. Venus P-Valve (Venus Medtech, Shanghai, China) and Harmony TPV (Medtronic Inc., Minneapolis, MN, USA) are self-expandable pulmonary valves. Alterra Adaptive Prestent (Edwards Lifesciences LLC, Irvine, CA, USA) is an hourglass self-expandable stent that reduces the size of large right ventricular outflow tracts, creating a suitable landing zone to implant an Edwards Sapien S3 THV 29 mm. Novel stents and percutaneous valves are being planned and experimented with to widen the field of transcatheter approach. The aim of this review is to describe both the current approaches, strategies, and techniques as well as the future perspective to deal with the patients with significant pulmonary stenosis and/or regurgitation.
Advances in interventional cardiology have significantly broadened the range of congenital heart diseases (CHDs) amenable to trans-catheter interventions. Trans-oesophageal echocardiography (TOE) plays a pivotal role as a procedural guide in several percutaneous treatments. Enhanced imaging modalities and technological innovations in echocardiography have refined the precision and applicability of these approaches. This review explores the role, impact, and advancements of TOE in trans-catheter treatments for adult CHDs, including both common procedures (e.g., atrial septal defect closure, ventricular septal defect closure) and less frequent interventions (e.g., Mustard/Senning baffle leak closure, Fontan conduit fenestration, ruptured sinus of Valsalva embolization).
30 January 2026 in European Heart Journal Supplements
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.
Introduction: The Melody transcatheter pulmonary valve (TPV) was the first percutaneous bioprosthetic valve approved for transcatheter pulmonary valve implantation (TPVI). We report our single-centre experience with Melody TPV implantation in patients with congenital heart disease (CHD). Methods: This retrospective observational single-centre study included all patients evaluated in the catheterization laboratory for Melody TPV implantation. Early outcomes included procedural failure, life-threatening adverse events, and mortality. Long-term outcomes assessed during follow-up included infective endocarditis, transcatheter reintervention, and surgical reintervention. Results: Between 2015 and 2025, 50 consecutive patients were evaluated for TPVI with the Melody TPV at our institution. In four patients (8%), the procedure was aborted because of coronary artery compression detected during balloon interrogation of the right ventricular outflow tract (RVOT). One patient (2%) died of septic shock following acute pulmonary oedema in the immediate post-procedural period. The remaining 45 patients (90%) underwent successful Melody TPV implantation and were discharged from hospital. In six patients, the Melody TPV was implanted off-label: in the tricuspid position (n = 2) and in small conduits (<16 mm) (n = 4). Mean follow-up duration was 5.8 ± 3.6 years. One patient was lost to follow-up. Among the remaining 44 patients, seven (15.9%; 2.7% per patient-year) developed infective endocarditis, seven (15.9%; 2.7% per patient-year) underwent transcatheter reintervention (six balloon dilatations of the Melody valve and one valve-in-valve implantation), and four (9.1%; 1.5% per patient-year) required surgical replacement of the Melody TPV. Conclusions: Transcatheter implantation of the Melody TPV is an effective treatment for RVOT dysfunction. At mid-term follow-up, the majority of implanted Melody valves demonstrated satisfactory function, and only a minority of patients required surgical valve replacement.