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.

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).

Background: Clinical thromboembolism in Fontan patients is often a catastrophic event resulting in death and adverse long-term outcomes. The treatment of acute thromboembolic complications in these patients is very controversial. Case summary: We describe the use of rheolytic thrombectomy in a Fontan patient with life-threatening pulmonary embolism, employing a cerebral protection system to reduce the risk of stroke through the fenestration. Discussion: Rheolytic thrombectomy may be a successful alternative to systemic thrombolytic therapy and open surgical resection for the treatment of acute high-risk pulmonary embolism in the Fontan population. Embolic protection device to capture and remove thrombus/debris may be an innovative tool to reduce the risk of stroke through the fenestration while performing a percutaneous procedure in fenestrated Fontan patient.

Aims: Congenital heart diseases (CHD) are the most common birth defects in humans. Current clinical newborn screening for CHD has limitations. Digital elaboration of cardiac sounds with diagnostic purposes has been explored in the recent past in adults and older children. We propose to develop a dedicated software for automatic dichotomous clinical classification of heart sounds in newborns. Methods and results: The Digital Diagnosis of cardiac SOUNDs in pediatric patients (DI_SOUND) study is a multicenter study with two sequential phases: 1) derivation/training; 2) validation. Each newborn will undergo a digital recording of cardiac sounds (stored as wav file using the Littmann Core Stethoscope) and comprehensive echocardiogram. In the development phase, sound tracings will undergo processing to remove background noise. They will be segmented to generate sound samples of homogeneous suitable tuned duration, and filtered records will be normalized for preventing inhomogeneity in the extracted features. Data analysis will be then implemented based on handcrafted feature extraction. A DI_SOUND classifier algorithm will be developed (Aim 1). In the validation phase, consecutive newborns will undergo clinical (standard) screening and digital screening using the DI_SOUND algorithm. Comprehensive echocardiogram will serve as the reference to assess diagnostic performance of the DI_SOUND algorithm (Aim 2). A cost-effective analysis will be used to model marginal cost-effectiveness of digital screening using clinical screening as comparison (Aim 3). The study will prove if development and validation of a digital classifier of neonatal cardiac sounds is feasible and how this will impact clinical outcomes, patient care, hospital admissions and screening efficiency.

Objectives: Melody TPV is the first percutaneous valvular bio–prosthesis approved for trans–catheter pulmonary valve implantation (TPVI). We describe our ten–years experience about Melody TPV implantation in patients with congenital heart disease (CHD). Methods: This is an observational retrospective single–center study. All patients undergone Melody TPV implantation were included. The early outcomes analyzed were: procedural failure, death of patient, life–threating adverse events. The long–term outcomes analyzed during follow–up were: infective endocarditis, trans–catheter redo–procedure, and surgical redo–procedure. Results: From 2012 to 2022, 39 patients were evaluated in our catheterization laboratory for TPVI with Melody TPV. Mean age and weight were 21,31±11,58 (range 8–66) years and 57,5±16,24 (range 23–96) kilograms. The patients were affected by the following CHD: tetralogy of Fallot (22 pts, 56,4%), pulmonary atresia with ventricular septal defect (VSD) (4 pts, 10,3%), aortic stenosis s/p Ross surgery (4 pts, 10,3%), transposition of the great arteries with VSD and PS (3 pts, 7,7%), pulmonary stenosis (PS) (2 pts, 5,1%), truncus arteriosus (2 pts, 5,1%), double outlet right ventricle (1 pt, 2,5%), Ebstein’s anomaly (1 pt, 2,5%) In 4 cases (10,3%), the procedure failed because of coronary compression during balloon RVOT interrogation. One patient (2,5%) died due to post–procedural septic shock. In the other 34 patients (87,2%), the Melody TPV was implanted effectively. No life–threating adverse events were recorded. In 3 cases, the Melody TPV was implanted off–label: in tricuspid position (one case) and in “small conduits” (<16 mm) (two cases). The mean follow–up was 4,41±2,82 years. During follow–up, 5 patients (14,7%) developed an infective endocarditis, 7 patients (20,6%) required a trans–catheter procedure (6 Melody balloon dilation and 1 Melody valve–in–valve), and 2 patients (5,9%) underwent surgical Melody TPV replacement. Conclusion: Trans–catheter Melody TPV implantation is effective to deal RVOT dysfunction. At a mid–term follow–up, the most of the implanted Melody TPV worked properly and only a few patients required surgical valve replacement.