Arteriovenous Malformations / surgery

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Case Report

Platypnea-Orthodeoxia Syndrome Due to Venovenous Malformation

Introduction

The platypnea-orthodeoxia syndrome (POS) is a rare clinical condition characterized by dyspnea occurring in the upright position and relieved in the supine position (platypnea) associated with oxygen desaturation noted in the upright position (orthodeoxia). A diagnosis of POS requires two of the following criteria: 1) orthodeoxia, 2) platypnea, 3) presence of an interatrial communication, 4) right-to-left shunt, and 5) absence of pulmonary arterial hypertension or right atrial hypertension.^, POS can originate in the heart, lungs, abdomen, or elsewhere due to either an intracardiac (cardiac POS) or intrapulmonary shunt, or a ventilation-perfusion mismatch. However, a venovenous malformation (VVM) as a cause of cardiac POS has never been reported in the literature.

We report here the case of a 24-year-old female patient who presented with cardiac POS due to a VVM. The syndrome emerged insidiously 16 years after an extracardiac total cavopulmonary connection (TCPC) for a complex of congenital cardiovascular malformations and was successfully treated with percutaneous transvenous coil embolization.

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Platypnea-Orthodeoxia Syndrome Due to Venovenous Malformation

Introduction

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