The RISE Study: Retrospective Registry for the International Safety and Efficacy Results of Patent Foramen Ovale Closure with Figulla Flex Il PFO and UNI Occluders.

Background: Transcatheter closure of a patent foramen ovale (PFO) is performed in cryptogenic stroke and other conditions. Information is lacking for some devices. Methods: We aimed to evaluate the Figulla Flex II PFO Occluder (FFP) and Figulla Flex UNI Occluder (FFU) through a retrospective multi-center registry. Results: 527 patients were included. Mean age was 48.9 (±13.8) years. The procedure was under transthoracic, transesophageal or intracardiac echocardiography in 185 (35.1%), 193 (36.6%) and 149 (28.3%) cases, respectively, and under general anesthesia in 191 patients (36.2%). The FFP and FFU were used in 408 (77.4%) and 119 (22.6%) cases, respectively. The success rate was 99.1%. Median follow-up was 1.1 (0.5-2.5) years. A new atrial fibrillation/flutter within six months occurred in 14 (2.7%) cases, with no difference between devices. One device embolization in the pulmonary artery was identified two years post-procedure. Residual shunts occurred in 18 (6.9%) cases at 1 year, with TIA in three (16.6%) patients. Out of 437 patients with stroke/TIA, 260 (59%) were followed more than one year after closure. Median follow-up was 2.1 (1.17-3.1) years, with four recurrent strokes/TIA. Conclusions: The FFP and FFU devices are safe and effective for PFO closure, with very few atrial fibrillation/flutter and neurologic events, except in cases with a residual shunt.

Outcomes of transcatheter pulmonary SAPIEN 3 valve implantation: an international registry.

BACKGROUND AND AIMS: Transcatheter pulmonary valve implantation (TPVI) is indicated to treat right-ventricular outflow tract (RVOT) dysfunction related to congenital heart disease (CHD). Outcomes of TPVI with the SAPIEN 3 valve that are insufficiently documented were investigated in the EUROPULMS3 registry of SAPIEN 3-TPVI. METHODS: Patient-related, procedural, and follow-up outcome data were retrospectively assessed in this observational cohort from 35 centres in 15 countries. RESULTS: Data for 840 consecutive patients treated in 2014-2021 at a median age of 29.2 (19.0-41.6) years were obtained. The most common diagnosis was conotruncal defect (70.5%), with a native or patched RVOT in 50.7% of all patients. Valve sizes were 20, 23, 26, and 29 mm in 0.4%, 25.5%, 32.1%, and 42.0% of patients, respectively. Valve implantation was successful in 98.5% [95% confidence interval (CI), 97.4%-99.2%] of patients. Median follow-up was 20.3 (7.1-38.4) months. Eight patients experienced infective endocarditis; 11 required pulmonary valve replacement, with a lower incidence for larger valves (P = .009), and four experienced pulmonary valve thrombosis, including one who died and three who recovered with anticoagulation. Cumulative incidences (95%CI) 1, 3, and 6 years after TPVI were as follows: infective endocarditis, 0.5% (0.0%-1.0%), 0.9% (0.2%-1.6%), and 3.8% (0.0%-8.4%); pulmonary valve replacement, 0.4% (0.0%-0.8%), 1.3% (0.2%-2.4%), and 8.0% (1.2%-14.8%); and pulmonary valve thrombosis, 0.4% (0.0%-0.9%), 0.7% (0.0%-1.3%), and 0.7% (0.0%-1.3%), respectively. CONCLUSIONS: Outcomes of SAPIEN 3 TPVI were favourable in patients with CHD, half of whom had native or patched RVOTs.

Mind the gap-missing device on the shelf? Retrospective experience with 5/7 Occlutech duct Occluder.

AIM: To describe our initial experience with the indications and results of the 5/7 Occlutech® duct Occluder (ODO, Occlutech International AB, Helsingborg, Sweden). A small incremental increase in occluder sizes is of utmost importance for successful outcomes, especially in smaller patients in whom protrusion of the distal disk towards the aorta should be minimised. METHODS: Retrospective study of all patients undergoing PDA closure with the 5/7 ODO in three institutions since 2018. RESULTS: The 5/7 ODO was used in 18 patients with median age and weight at the time of the procedure of 17.5 months (interquartile range 25th to 75th percentile 8 months- 4.4 years) and 13.6 kg (interquartile range 25th to 75th percentile 6.4-22.5 kg) respectively. All cases were successful. There were no cases of device embolisation, haemolysis, or flow disturbance of the LPA or the aorta. CONCLUSIONS: This small retrospective study demonstrated an excellent outcome of transcatheter PDA closure with the 5/7 ODO. The device is a beneficial complement to the existing sizes of PDA devices, filling the gap between the 4/6 and 6/8 ODO and avoiding protrusion of a larger disk in the aortic isthmus.

The Transcatheter Closure of Patent Ductus Arteriosus in Extremely Low-Birth-Weight Infants: Technique and Results.

Persistent patent ductus arteriosus is a very common condition in preterm infants. Although there is no management agreed by consensus, despite numerous randomized controlled trials, hemodynamically significant patent ductus arteriosus increases morbidity and mortality in these vulnerable patients. Medical treatment is usually offered as first-line therapy, although it carries a limited success rate and potential severe adverse events. In recent years, transcatheter patent ductus arteriosus closure has fast developed and become widely accepted as a safe and efficient alternative to surgical ductal ligation in extremely low birth weight infants >700 g, using most often the dedicated Amplatzer Piccolo Occluder device. This article aims to provide an appraisal of the patients' selection process, and a step-by-step description of the procedure as well as a comprehensive review of its outcomes.

Transcatheter Closure of Superior Sinus Venosus Defects.

Superior sinus venosus defect is a communication between the right and left atrium located above the upper margin of the oval fossa, immediately inferior to the junction of the superior vena cava and the right atrium. It is systematically associated with partial anomalous pulmonary venous drainage, especially of the right upper pulmonary vein. Surgical repair has been the gold standard approach to close that defect. Introduced in 2014, percutaneous closure has gradually become a safe and effective alternative to surgery in carefully selected patients, although worldwide experience remains limited. This article provides an appraisal of the patients' selection process and a step-by-step description of the procedure as well as a comprehensive review of its outcomes.

Case report: septic embolism through patent foramen ovale in mitral valve endocarditis.

Background: Infective endocarditis with septic emboli is a well-recognized sequala. However, emboli almost invariably are found 'downstream' to the vegetation. In the absence of congenital heart disease, the embolization from the left heart to the pulmonary circulation has never been described. Case summary: A 4-year-old boy presents with a background history of pharyngitis treated with antibiotics. Upon ambulatory review, he is noted to have a new murmur. Transthoracic echocardiogram demonstrated mitral valve vegetation with severe mitral regurgitation; in addition, there was a patent foramen ovale (PFO), and there were no congenital heart defects. The patient was treated for infective endocarditis on high clinical suspicion. He subsequently developed septic pulmonary emboli in the absence of right-sided vegetation. Subsequent mitral valve vegetectomy, resection of infected native anterior mitral valve leaflet, mitral valve repair, and valvuloplasty. The patient made an excellent recovery following the completion of antibiotic therapy. Discussion: Although not possible to confirm with certainty, this case demonstrates the most plausible explanation for this child's presentation being septic pulmonary emboli originating from left-sided heart vegetation migrating through a PFO.

Infective endocarditis after transcatheter pulmonary valve implantation in patients with congenital heart disease: Distinctive features.

The introduction of transcatheter pulmonary valve implantation (TPVI) has greatly benefited the management of right ventricular outflow tract dysfunction. Infective endocarditis (IE) is a feared complication of TPVI that affects valve durability and patient outcomes. Current recommendations provide only limited guidance on the management of IE after TPVI (TPVI-IE). This article, by a group of experts in congenital heart disease in children and adults, interventional cardiology, infectious diseases including IE, and microbiology, provides a comprehensive review of the current evidence on TPVI-IE, including its incidence, risk factors, causative organisms, diagnosis, and treatment. The incidence of TPVI-IE varies from 13-91/1000 person-years for Melody valves to 8-17/1000 person-years for SAPIEN valves. Risk factors include history of IE, DiGeorge syndrome, immunosuppression, male sex, high residual transpulmonary gradient and portal of bacteria entry. Staphylococci and streptococci are the most common culprits, whereas Staphylococcus aureus is associated with the most severe disease. In addition to the modified Duke criteria, a high residual gradient warrants a strong suspicion. Imaging studies are helpful for the diagnosis. Intravenous antibiotics guided by blood culture results are the mainstay of treatment. Invasive re-intervention may be required. TPVI-IE in patients with congenital heart disease exhibits several distinctive features. Whether specific valve types are associated with a higher risk of TPVI-IE requires further investigation. Patient and parent education regarding IE prevention may have a role to play and should be offered to all patients.

Transcatheter closure with a "single lobe" Amplatzer Vascular Plug of a tubular patent ductus arteriosus in a neonate with transposition of the great arteries and heart failure.

A patent ductus arteriosus in patients with transposition of the great arteries is usually beneficial to allow shunting between pulmonary and systemic circulations. However, if the duct is too large, it can cause haemodynamic instability, pulmonary oedema and compromised organ perfusion. We present a neonate in whom a 5 mm short and tubular ductus arteriosus was causing significant cardiac failure with necrotising enterocolitis and liver impairment, leaving him too unstable for the arterial switch operation. At day 14 of life (3.1 kg), the baby underwent successful transcatheter closure using an Amplatzer vascular plug (Abbott, Chicago, Il, USA) delivered through a 5Fr catheter from the aortic side. The procedure was uncomplicated and successful as the neonate was extubated 2 days later. He subsequently underwent successful arterial switch surgery.

Short-term outcome of late gadolinium changes detected on cardiovascular magnetic resonance imaging following coronavirus disease 2019 Pfizer/BioNTech vaccine-related myocarditis in adolescents.

BACKGROUND: Rare cases of cardiac inflammation following vaccination for severe acute respiratory coronavirus 2 (SARS-CoV-2) have been reported. OBJECTIVE: To study paediatric patients with clinical findings of acute inflammation post coronavirus disease 2019 (COVID-19) Pfizer/BioNTech vaccination using cardiovascular magnetic resonance imaging (MRI) in acute and subacute phases. MATERIALS AND METHODS: We enrolled adolescents younger than 18 years who presented at one of two institutions between July 2021 and August 2022 with clinical and laboratory findings of acute myocarditis shortly following COVID-19 Pfizer/BioNTech vaccination. They all underwent cardiovascular MRI using the institutional myocarditis protocol. RESULTS: Five adolescents (four boys) underwent eight scans between 3 days and 109 days (mean 49 days) after the onset of symptoms following COVID-19 vaccination. Myocardial oedema appeared on short tau inversion recovery (STIR) T2-weighted images in three adolescents at presentation (3-12 days after symptom onset). In these children, the myocardial oedema/acute inflammation had resolved at follow-up cardiovascular MRI (53-68 days after first MRI). However, in all three adolescents, a persistent area of late gadolinium enhancement was evident at follow-up, suggesting post-myocarditic fibrosis. One adolescent scanned only once, 66 days after being symptomatic, had no acute inflammation but persistent fibrotic changes. This last adolescent, who underwent the first scan 109 days after symptom onset, had findings compatible with an episode of previous myocarditis, with mild ongoing regional myocardial oedema/inflammation. CONCLUSION: This study on post-vaccine myocarditis demonstrates residual lesions with persistent areas of late gadolinium enhancement/myocardial fibrosis with ongoing myocardial oedema after resolution of the initial myocardial oedema a few weeks after Pfizer/BioNTech vaccination. There is an urgent need to recognise and fully investigate the outcome of post-vaccination myocarditis.

Feasibility, safety, and efficacy of Atrial Flow Regulator in children under 10 kg.

INTRODUCTION: The Atrial Flow Regulator (AFR) is a new self-expandable percutaneous-delivered fenestrated device providing an interatrial shunt. Its use in pediatric population has been reported in failing Fontan, pulmonary hypertension, or in patients with cardiomyopathy and venoarterial extracorporeal membrane oxygenator (ECMO) support. Its use in small children under 10 kg has not been reported. METHODS: We report the initial single center experience of the AFR implantation in children below 10 kg. RESULTS: Four children underwent AFR implantation. Patients' age and weight ranged between 9 and 22 months and 5-8.7 kgs. Indications were to unload the left atrium during ECMO support for end-stage cardiomyopathy and to enlarge a restrictive interatrial shunt in two other patients with complex congenital heart diseases. Devices implanted were AFR-6 and AFR-8. Delivery sheaths used via venous femoral access were undersized and ranged from 9 to 11 Fr. Devices were successfully implanted and provided unrestrictive interatrial shunt in all cases. One child developed a nonocclusive thrombus in the inferior vena cava which resolved within 1 month. Clinical improvement and femoral vein patency were observed in all cases. CONCLUSION: AFR implantation is feasible in selected children under 10 kg. The AFR can be safely deployed through sheaths that are 1-2 Fr smaller than the recommended manufacturer size. More studies are needed to confirm safety and efficacy of the device in selected pediatric patients.

Pathogenesis, immunology, and immune-targeted management of the multisystem inflammatory syndrome in children (MIS-C) or pediatric inflammatory multisystem syndrome (PIMS): EAACI Position Paper.

Multisystem inflammatory syndrome in children (MIS-C) is a rare, but severe complication of coronavirus disease 2019 (COVID-19). It develops approximately 4 weeks after severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection and involves hyperinflammation with multisystem injury, commonly progressing to shock. The exact pathomechanism of MIS-C is not known, but immunological dysregulation leading to cytokine storm plays a central role. In response to the emergence of MIS-C, the European Academy of Allergy and Clinical Immunology (EAACI) established a task force (TF) within the Immunology Section in May 2021. With the use of an online Delphi process, TF formulated clinical statements regarding immunological background of MIS-C, diagnosis, treatment, follow-up, and the role of COVID-19 vaccinations. MIS-C case definition is broad, and diagnosis is made based on clinical presentation. The immunological mechanism leading to MIS-C is unclear and depends on activating multiple pathways leading to hyperinflammation. Current management of MIS-C relies on supportive care in combination with immunosuppressive and/or immunomodulatory agents. The most frequently used agents are systemic steroids and intravenous immunoglobulin. Despite good overall short-term outcome, MIS-C patients should be followed-up at regular intervals after discharge, focusing on cardiac disease, organ damage, and inflammatory activity. COVID-19 vaccination is a safe and effective measure to prevent MIS-C. In anticipation of further research, we propose a convenient and clinically practical algorithm for managing MIS-C developed by the Immunology Section of the EAACI.

Segmental and global longitudinal strain differences between Kawasaki disease and multi-system inflammatory syndrome in children.

BACKGROUND: Multi-system inflammatory syndrome in children and Kawasaki disease have overlapping clinical features but comparative echocardiographic studies are lacking. METHODS: We reviewed echocardiography findings of all multi-system inflammatory syndrome cases between 1st April and 31st July, 2020 and typical Kawasaki disease patients with coronary arteries abnormalities consecutively followed between 1st October, 2016 and June 30th, 2019. RESULTS: We included 40 multi-system inflammatory syndrome children (25 males, 62.5%) and 45 Kawasaki disease patients (31 males, 68.9%) at a mean age of 6.4 years old and 8 years old, respectively. Four out of 40 multi-system inflammatory syndrome children had coronary arteries abnormalities. Left ventricle ejection fraction was normal in both groups. Global longitudinal strain was normal although Kawasaki disease group had significantly lower values (-20.0 versus -21.7%; p = 0.02). Basal segments were the most affected in Kawasaki disease patients with significant differences in the basal anterior, anterolateral, and anteroseptal strain: -18.2 versus -23.0% (p = 0.002), -16.7 versus -22.0% (p < 0.001), -16.7 versus -19.5% (p = 0.034), respectively. The basal anterolateral and anteroseptal segments in Kawasaki disease patients were the only ones with an absolute reduction of longitudinal strain (-16.7% both) consistent with the greater left main coronary involvement in this cohort. CONCLUSIONS: Our findings are consistent with the transient cardiac involvement in multi-system inflammatory syndrome, as opposed to the subtle and chronic myocardial involvement in Kawasaki disease children with coronary arteries abnormalities. We speculate that the mechanism of cardiac impairment in the few multi-system inflammatory syndrome children with reduced global longitudinal strain is not related to coronary arteries abnormalities.

Implantation of atrial flow regulator devices in patients with congenital heart disease and children with severe pulmonary hypertension or cardiomyopathy-an international multicenter case series.

Background: The Occlutech Atrial Flow Regulator (AFR) is a self-expandable double-disc nitinol device with a central fenestration. Its use has been approved in the adult population with heart failure and described for pulmonary hypertension (PH). Only case reports and small series have been published about its use in the paediatric population and for congenital heart disease (CHD). Objectives: The authors sought to investigate the feasibility, safety, and short-term follow-up of AFR implantation in patients with CHD or children with PH or cardiomyopathy. Methods: This is a multicenter retrospective study involving 10 centers worldwide. Patients of any age with CHD or patients aged < 18 years with PH or cardiomyopathy needing AFR implantation were included. Results: A total of 40 patients underwent AFR implantation. The median age of the population at the time of the procedure was 58.5 months (IQR: 31.5-142.5) and the median weight was 17 kg (IQR: 10-46). A total of 26 (65.0%) patients had CHD, nine (22.5%) children, a cardiomyopathy, and five (12.5%), a structurally normal heart. The implantation success rate was 100%. There were two early and one late device thrombosis. Two patients (5.0%) with dilated cardiomyopathy on extracorporeal membrane oxygenator (ECMO) died during the hospital stay. At a median follow-up of 330 days (IQR: 125-593), 37 (92.5%) patients were alive. At follow-up, 20 patients improved their New York Heart Association (NYHA) class, 12 patients did not change their NYHA class, and one patient with idiopathic PH worsened. Conclusions: AFR implantation in patients with CHD and children with severe PH or cardiomyopathy is promising and seems to have beneficial effects at short-term follow-up.

Transcatheter closure of large perimembranous ventricular septal defects with inlet to outlet extension with the Amplatzer Vascular Plug-II.

Transcatheter closure of perimembranous ventricular septal defect is an alternative to surgery in selected small to medium defects with a reasonable distance between the defect and the aortic or tricuspid valves. Surgical closure is generally preferred for large defects with an inlet to outlet extension, aortic malalignment, or deficient aortic rim. We report two successful cases of percutaneous closure of large perimembranous ventricular septal defects with inlet to outlet extension using an Amplatzer Vascular Plug-II via a retrograde approach. Transcatheter closure of large perimembranous ventricular septal defect with inlet to outlet extension is feasible and facilitated by the characteristics of the Amplatzer Vascular Plug-II device and the specific implantation technique.