Recanalization of Thrombosed Atrial Flow Regulator in Failing Fontan: A Bridge to Transplant

We report the case of a 10-year-old boy (41 kg, 133 cm) with hypoplastic left heart syndrome (mitral and aortic atresia), who underwent staged palliation: Norwood-Sano at 6 days, Glenn at 7 months, and Fontan completion at 3 years using a 16 mm non-fenestrated extracardiac Gore-Tex conduit. By age 8, he developed Fontan failure with elevated pressures and protein-losing enteropathy, refractory to medical management, supportive therapy, and hepatic lymphatic embolization. At age 9, the Fontan conduit was needle-perforated, dilated with a 7 mm balloon, and a 6 × 5 mm Occlutech atrial flow regulator (AFR) was implanted. Complete AFR thrombosis occurred 4 months later. Recanalization was achieved using an Astato® 30 0.018" wire under carotid protection with 6 mm SpiderFX filters, followed by high-pressure ballooning and a 9 × 18 mm BeSmooth stent implantation. Despite a patent AFR and optimized therapy, the patient showed no clinical improvement and ultimately underwent successful heart transplantation 1 month later under more favorable conditions.