Platypnea-Orthodeoxia Syndrome Two Decades after Definitive Surgical Repair of Pulmonary Atresia with Intact Ventricular Septum

A 20-year-old female had undergone definitive surgical repair for pulmonary atresia with intact ventricular septum soon after birth. She was referred to our institution with the chief complaint of clubbing fingers. A thorough examination revealed platypnea-orthodeoxia syndrome due to an interatrial right-to-left shunt through a secundum atrial septal defect. Percutaneous closure with an Amplatzer Septal Occluder resulted in resolution of the syndrome.

Platypnea-orthodeoxia syndrome in a patient with a pre-existing patent foramen ovale successfully treated with an atrial septal occluder / 老年心脏病学杂志（英文版）

Platypnea orthodeoxia syndrome is associated with dyspnea and arterial oxygen desaturation accentuated by an upright posture. It can be secondary to an intracardiac shunt. We report a case of platypnea-orthodeoxia syndrome (POS) in a 58-year old male patient who had a pre-existing patent foramen ovale (PFO) and substantial pulmonary pathologies. He was successfully treated by percutaneous transcatheter closure of the PFO. Our case highlights the importance of recognition of this rare syndrome in patients who present with unexplained hy-poxia for whom transcatheter closure of the interatrial shunt can be safely carried out.

A Case of Platypnea-Orthodeoxia Syndrome Caused by a Patent Foramen Ovale / 日本心臓血管外科学会雑誌

We encountered a patient with platypnea-orthodeoxia syndrome. This rare syndrome is characterized by right-to-left shunt, which appears in the upright position. A 76-year-old woman with symptomatic hypoxemia was referred for evaluation and treatment. She did not complain of dyspnea while in a supine position, but experienced dyspnea with severe hypoxemia in a sitting or standing position. She did not have any pulmonary diseases that can be cause of dyspnea. Echocardiography revealed a patent foramen ovale and mild left-to-right shunt when the patient was supine. However in an upright position, right-to-left shunt appeared and the arterial oxygen saturation dropped from 95% to 80% with dyspnea. Cardiac catheterization revealed normal pulmonary artery pressure and right-to-left shunt through the patent foramen ovale in the sitting position. We then diagnosed platypnea-orthodeoxia syndrome. The chest CT showed deformity of the right atrium caused by compression of the elongated ascending aorta. The patent foramen ovale was closed and the ascending aorta was shortened by open heart surgery. Her dyspnea and hypoxemia in the upright position was completely resolved after surgery.