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Pulmonary Artery Band And Fenestrated Septal Occluder For Unilateral Pulmonary Arteriovenous Malformations After Heart-liver Transplantation
Syed Faaz Ashraf, MD1, Laura Seese2, Richard Tang2, Lauren Carlozzi2, Luciana Da Fonseca Da Silva2, Mario Castro-Medina2, Shawn West2, Kirsten Rose-Felker2, Brian Feingold2, Sara Trucco2, Bryan Goldstein2, Victor Morell2.
1University of Pittsburgh Medical Center, Pittsburgh, PA, USA, 2UPMC Children’s Hospital of Pittsburgh, Pittsburgh, PA, USA.


Objective(s):Pulmonary arteriovenous malformations (PAVMs) in heterotaxy with single ventricle physiology typically regress following heart-liver transplantation, but the months-long remodeling period can produce exaggerated, preferential shunting through the low-resistance AVM-affected lung. We describe a novel technique using a manually fenestrated septal occluder as a titratable flow restrictor to manage this physiology.
Methods:A 13-year-old girl with heterotaxy, unbalanced AVC, and diffuse left-sided PAVMs underwent en bloc heart-liver transplantation for Fontan-associated liver disease. On postoperative day 11, refractory hypoxemia from preferential left-lung shunting necessitated venovenous ECMO. Diffuse anatomy precluded coil embolization. Left PA banding with right PA stenting allowed decannulation but she remained oxygen-dependent. Catheter-based vascular plugs failed: occlusion eliminated left lung perfusion; repositioning caused embolization requiring VV ECMO recannulation. A 20mm Gore Cardioform Septal Occluder was electrocautery-fenestrated and deployed proximal to the band, preserving antegrade flow for hepatic factor delivery.
Results:She was decannulated four days after device placement, with RV systolic pressure one-third systemic. At nine months, progressive RV dysfunction (systolic pressure 60% systemic) prompted catheterization. The fenestrations were dilated with a 12mm balloon, after which LPV saturation reached 100% on room air—confirming AVM regression—and RV pressure returned to one-third systemic. Perfusion imaging at ten months demonstrated 35% left lung perfusion.
Conclusions:Surgical PA banding with a proximally positioned, manually fenestrated septal occluder offers a reproducible strategy for titratable flow restriction in refractory unilateral PAVMs following cardiac transplantation.

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