Versatility of Atrial Flow Regulator Device in Congenital Heart Disease: A Case Series.

The Atrial Flow Regulator (AFR) is a self-expandable double-disk device with a central fenestration, intended to maintain a calibrated communication across the interatrial septum. Only case reports and small case series have been published about its use in the pediatric and congenital heart disease (CHD) population. We described AFR implantation in three congenital patients with different anatomies and indications. In the first case, the AFR was deployed to create a stable fenestration in a Fontan conduit, while in the second, it was used to reduce a Fontan fenestration. In the third case, we implanted an AFR to decompress the left atrium of an adolescent with complex CHD in natural history, with complete mixing, ductal-dependent systemic circulation and combined pulmonary hypertension. This case series demonstrates the great potential of the AFR device in the CHD field, showing versatility, efficacy, and safety in establishing a calibrated and stable shunt, with promising hemodynamic and symptomatic benefits.

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.

Atrial Flow Regulator for Postcapillary Pulmonary Hypertension: First-in-Human Transcatheter AFR Device Implantations in RCM.

Restrictive cardiomyopathy (RCM) has a poor prognosis and limited treatment options apart from heart transplantation (HTx). We report on the first-in-human interventional atrial flow regulator (AFR) implantations in 3 children with RCM, leading to marked clinical and hemodynamic improvement. We propose the AFR as bridge to HTx or destination therapy in RCM. (Level of Difficulty: Advanced.).