Multiple Atrial Communications presenting as Platypnea-Orthodeoxia Syndrome

Abstract

We report a case of platypnea-orthodeoxia syndrome resulting from multiple inter-atrial communications with right-toleft shunting in a 73 year old woman. Our patient presented with progressive shortness of breath over the last eighteen months. She was on 10 liters of high flow oxygen at time of presentation to our hospital and was bed bound owing to severe dyspnea. Her history of chronic pulmonary emboli despite therapeutic coumadin levels and ulcerative colitis initially led to an exhaustive hepatopulmonary work-up. On clinical exam, she was noted to have normal oxygen saturation with lying down, but with sitting up or walking she developed significant hypoxia and respiratory distress. With no evidence of lung parenchymal or vascular diseases, right heart catheterization was performed and revealed normal right sided pressures. Intracardiac echocardiography confirmed a typical appearing patent foramen ovale (PFO) with atrial septal aneurysm and bidirectional shunting, and a small inferior secundum atrial septal defect (ASD). Although the ASD was congenital, aortic dilation with ageing could have altered the location of the atrial septum which facilitated laminar blood flow from the inferior vena cava to the left atrium. We proceeded to close both defects with an Amplatzer Cribiform Occluder. Her symptoms dramatically improved 48 hours following closure and she was ambulating on room air at time of discharge. One month follow-up was notable for no recurrence of symptoms. PFOs and small ASDs are generally innocuous but could cause right to left shunting even in the absence of elevated right sided pressures. Excluding other causes of platypnea-orthodeoxia, and closing the atrial communications can lead to resolution of symptoms. Key words: Multiple Atrial Communications, Platypnea-Orthodeoxia Syndrome.