AEPC-YIA session（I-AEPCYIA）

[I-AEPCYIA-05] Atrial flow regulator device in children and in patients with Congenital Heart Disease : an International Registry

^○Enrico Piccinelli^1,2, Grazyna Brzezinska Rajszys³, Adam Kolinski³, Kostantin Averin⁴, Biagio Castaldi⁵, Alain Fraisse⁶, Carles Bautista-Rodriguez⁶, Alban-Elouen Baruteau⁷, Pedro Betrián Blasco⁸ , Lisa Bianco^1,8, Andreas Eicken⁹, Matthew Jones¹⁰, James A Kuo, Mara Pilati¹, Micol Rebonato¹, Roberto Formigari¹, Gianfranco Butera¹ (1.Pediatric Cardiology Department, Ospedale Pediatrico Bambino Gesù, Roma, Italy, 2.Department of Mechanical and Aerospace Engineering, Politecnico di Torino, Italy, 3.The Children’s Memorial Health Institute, Varsavia, Poland, 4.Stollery Children’s Hospital, Edmonton, Canada, 5.Pediatric Cardiology Department, Padova University, Padova, Italy, 6.Pediatric Cardiology Department, Royal Brompton Hospital, London, UK, 7.Nantes Université, CHU Nantes, Department of Pediatric Cardiology and Pediatric Cardiac Surgery, FHU PreciCare, F-44000 Nantes, France, 8.Pediatric Interventional Cardiology Unit Vall d'Hebron Hospital, Barcellona, Spain, 9.Deutsches Herzzentrum München, München, Germany, 10.Evelina Children’s hospital, London, UK, 11.Children's Healthcare of Atlanta, Atlanta, US)

Background: The Occlutech Atrial Flow Regulator (AFR®) is a self-expandable double-disc nitinol device with a central fenestration. Once deployed percutaneously, the central portion of the device stents the atrial septum leaving a preselected calibrated atrial communication. Its use has been approved in Europe in the adult population with heart failure and described for pulmonary hypertension. Only case reports and small series have been published about its use in the pediatric population and congenital heart disease. Materials and methods: This is a multicenter retrospective registry involving ten centres worldwide. Patients at any age with congenital heart disease or patients aged < 18 years with pulmonary hypertension or cardiomyopathy needing AFR implantation were included. Results: From 2017 to 2022, 40 patients underwent AFR implantation. The median age of the population was 58,5 (IQR 31.5 – 142.5) months and the median weight was 17 (IQR 10 – 46) Kg. Twenty-six patients had congenital heart disease, 9 children had cardiomyopathy and 5 had a structurally normal heart. Indications for AFR implantation were left heart failure in 16 patients (40%), right heart hypertension in 11 patients (27,5%), severe desaturation in fenestrated Fontan in 5 patients (12,5%), Fontan failure in 4 patients (10%) and ECMO in 4 patients (10%). Almost all the procedures (95%) were performed under general anaesthesia. Venous access was femoral in 90% of patients, transhepatic in 2 pts (5%), subclavian in 1 patient (2,5%) and hybrid transatrial in 1 patient (2,5%). AFR implantation required balloon pre-dilatation in 65% of patients. The implantation success rate was 100%. The most used device was the 4 mm one in 13 patients (32,5%), followed by the 8 mm in 12 patients (30%), the 6 mm in 9 patients (22,5%), and the 10 mm in 6 patients (15%). There was a 5% rate of intra-procedural complications, consisting in two immediate AFR occlusions. No intra-procedural deaths were registered. Two patients with dilated cardiomyopathy on ECMO died during the hospital stay, while a third one was complicated by a cardiac arrest few hours after an hybrid procedure of pulmonary artery banding and AFR implantation. All the other patients were discharged without complications. At a median follow-up of 330 days (IQR 125 – 593), 92,5% patients were alive. A child died of sepsis in the local hospital more than 3 months after the procedure. All but one device maintained their patency at last follow-up. At follow-up 20 patients improved their NYHA class, 12 patients did not change their NYHA class and only one patient with idiopathic pulmonary hypertension worsened his NYHA class. Conclusions: AFR implantation in patients with congenital heart diseases and children with severe pulmonary hypertension or cardiomyopathy is promising and seems to have beneficial effects at short-term follow up. The AFR has the potential to provide benefits in terms of symptoms and survival to a variety of patients with limited treatment options and indeterminate prognosis.