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.

Pulmonary vein stenosis (PVS) is a difficult condition to treat due to recurrence and progression. In 2017, we developed a comprehensive PVS Program at our center to address the multidisciplinary needs of these patients. We discuss the components of our program and our approach to these patients, using a combination of primary (medical) therapy in addition to anatomic therapy to preserve vessel patency. A multidisciplinary approach to treating these challenging patients is critical.

Cardiovascular MRI with magnetic resonance angiography supports clinical indication for transcatheter pulmonary valve replacement. We aimed to: (1) assess the feasibility of generating virtual right ventricular outflow tract models directly from magnetic resonance angiography datasets and (2) compare transcatheter pulmonary valve replacement candidacy based on visualisation of anatomic and valve models in virtual reality to outcomes and dispositions suggested by industry fit analysis derived from cardiac CT. Patients with native or surgically palliated right ventricular outflow tracts considered for transcatheter pulmonary valve replacement with temporally related magnetic resonance angiography and cardiac CT were included. Magnetic resonance angiography models were generated using commercial software; virtual valves were created using industry dimensions. A blinded interventional cardiologist determined pulmonary valve replacement candidacy using virtual reality review. A total of 16 patients ( N = 7 males, 44%) with a median age 15.5 years (interquartile range [IQR] 13.9, 17.8) were identified. Median time for model generation was 20.6 minutes (IQR 18.5, 22.0). 11/16 (69%) patients passed industry screening fit analysis; 14/16 (88%) ultimately underwent transcatheter pulmonary valve replacement. Four patients who passed virtual reality screening failed industry screening but underwent successful transcatheter pulmonary valve replacement. One patient passed virtual reality and industry screening but did not undergo transcatheter pulmonary valve replacement. One patient passed virtual reality screening but failed industry screening and was not offered transcatheter pulmonary valve replacement. In conclusion, generating virtual models from clinical magnetic resonance angiography datasets is feasible. Modelling may help evaluate transcatheter pulmonary valve replacement candidacy, but must be used in conjunction with other data.

Background: Pediatric pulmonary vein stenosis (PVS) is a progressive and recurrent condition associated with high morbidity and mortality. Long-term outcomes of individual pulmonary veins remain poorly described. Methods: All pediatric (<18 years) PVS cases diagnosed between September 2005 and January 2020 with a minimum follow-up of 5 years were included. Vein-specific outcomes were assessed using competing-risk survival analysis adjusted for patient death and vein atresia. Veins with recurrent disease maintaining patency for ≥3 years were analyzed longitudinally to characterize reintervention burden as a surrogate for disease trajectory. Results: A total of 244 affected veins in 107 patients were identified. The cumulative incidence of vein loss progressively increased during the first 3 years, followed by plateauing thereafter. Overall, 147 of 244 veins (60%) were lost within 3 years of diagnosis, including 75 veins lost secondary to patient death. Vein attrition was greatest during the first year following diagnosis. Ninety-seven veins remained patent for ≥3 years, including 73 with recurrent disease. Among these veins, the median reintervention rate decreased significantly from 2 (IQR: 1–4) during the first 3 years to 1 per year (IQR: 1–3) thereafter (p < 0.01) while maintaining continued patency. Conclusions: In pediatric PVS, the first 3 years after diagnosis represent a critical period of active disease with high rates of vein attrition. Veins maintaining patency beyond this period demonstrate a reduced reintervention rate, suggesting stabilization of disease progression. Aggressive surveillance and intervention during this therapeutic window may improve long-term vein and patient outcomes.