Platypnoea-Orthodeoxia Syndrome Secondary to Patent Foramen Ovale (PFO): A Challenging Subset for PFO Percutaneous Closure

BackgroundPlatypnoea-Orthodeoxia Syndrome (P-OS) is a rare disease characterised by arterial desaturation exacerbated by the upright position and relieved by recumbency. Patent foramen ovale (PFO) may lead to a P-OS causing a right-to-left shunt in the course of particular diseases that induce atrial deformation. Percutaneous closure of the defect usually allows prompt improvement of the clinical status.MethodsA series of patients with P-OS was treated with percutaneous PFO closure, according to standard clinical practice. Procedural monitoring was performed by transoesophageal two-dimensional-echocardiography (2D-echo).ResultsPFO percutaneous closure was initially effective in only half of the patients because of high rates of acute residual shunt. This unexpected result was related to the very complex anatomy evaluation with 2D-echo, mainly due to a peculiar atrial deformation occurring in P-OS. A second device delivery allowed it to achieve complete defect closure in the remaining patients. Technical issues arising during the procedures are widely discussed.ConclusionsPercutaneous closure of PFO in patients with P-OS is feasible but some technical issues should be considered when PFO anatomy is not clear with traditional imaging techniques.