Platypnea-Orthodeoxia Syndrome: To Shunt or Not to Shunt, That is the Question.
Platypnea-orthodeoxia syndrome is a rare disease defined by dyspnea and deoxygenation, induced by an upright position, and relieved by recumbency. Causes include shunting through a patent foramen ovale and pulmonary arteriovenous malformations. A 79-year-old woman experienced 2 syncopal episodes at rest and presented at another hospital. In the emergency department, she was hypoxic, needing 6 L/min of oxygen. Her chest radiograph showed nothing unusual. Transthoracic echocardiograms with saline microcavitation evaluation were mildly positive early after agitated-saline administration, suggesting intracardiac shunting. She was then transferred to our center. Right-sided heart catheterization revealed no oximetric evidence of intracardiac shunting while the patient was supine and had a low right atrial pressure. However, her oxygen saturation dropped to 78% when she sat up. Repeat transthoracic echocardiography while sitting revealed a dramatically positive early saline microcavitation-uptake into the left side of the heart. Transesophageal echocardiograms showed a patent foramen ovale, with right-to-left shunting highly dependent upon body position. The patient underwent successful percutaneous patent foramen ovale closure, and her oxygen supplementation was suspended. In patients with unexplained or transient hypoxemia in which a cardiac cause is suspected, it is important to evaluate shunting in both the recumbent and upright positions. In this syndrome, elevated right atrial pressure is not necessary for significant right-to-left shunting. Percutaneous closure, if feasible, is first-line therapy in these patients.
Michael R
MR
Klein
Todd L
TL
Kiefer
Eric J
EJ
Velazquez
D000368:Aged; D006328:Cardiac Catheterization; D006348:Cardiac Surgical Procedures / Q000379:methods*; D003657:Decision Making; D003914:Dextrocardia / Q000150:complications* / Q000175:diagnosis / Q000601:surgery; D004417:Dyspnea / Q000175:diagnosis / Q000209:etiology* / Q000601:surgery; D005260:Female; D054092:Foramen Ovale, Patent / Q000150:complications* / Q000175:diagnosis / Q000601:surgery; D006344:Heart Septal Defects, Atrial / Q000150:complications*; D006801:Humans; D000860:Hypoxia / Q000175:diagnosis / Q000209:etiology* / Q000601:surgery; D013577:Syndrome
Congenital heart defects; dyspnea/diagnosis/radiography/therapy; echocardiography, transesophageal/color-flow; foramen ovale, patent; heart septal defects, atrial/ultrasonography; hypoxemia; magnetic resonance imaging, cardiac/cine; oxygen/blood; posture; respiration insufficiency/etiology