Platypnea-orthodeoxia is a poorly understood clinical syndrome resulting in dyspnea and hypoxemia in the upright position, which diminishes with recumbency. Recognition of the disease is limited by its low prevalence and decreased awareness among clinicians. However, understanding the disease, its pathophysiology, its clinical presentation, and the possible therapeutic options is vital in the management of these patients. Here, we present 2 cases of platypnea- orthodeoxia where oxygen saturations worsened with hemodialysis. After highlighting the common features in the clinical pattern of each patient, we present a pressure-mediated pathophysiologic mechanism (in contrast to a previously reported morphologically based hypothesis) to explain the characteristic effects of hemodialysis on hypoxia in platypnea-orthodeoxia. We present a novel diagnostic approach using balloon occlusion testing when the diagnosis is unclear and illustrate how treatments can be tailored to the comorbidities of a specific patient.

Keywords:
Diagnosis, Hemodialysis, Pathophysiology, Balloon occlusion testing, Platypnea orthodeoxia
1.
Hussain SF, Mekan SF: Platypnea-orthodeoxia: report of two cases and review of the literature. South Med J 2004;97:657-662.
2.
Kerut EK, Norfleet WT, Plotnick GD, et al: Patent foramen ovale: a review of associated conditions and the impact of physiological size. J Am Coll Cardiol 2001;38:613-623.
3.
Godart F, Rey C: Platypnea-orthodeoxia syndrome: a probably underestimated syndrome? Chest 2001;119:1624-1625.
4.
Cheng TO: Platypnea-orthodeoxia syndrome: etiology, differential diagnosis, and management. Catheter Cardiovasc Interv 1999;47:64-66.
5.
Adolph EA, Lacy WO, Hermoni YI, et al: Reversible orthodeoxia and platypnea due to right-to-left intracardiac shunting related to pericardial effusion. Ann Int Med 1992;116:138-139.
6.
Laybourn KA, Martin ET, Cooper RA, et al: Platypnea and orthodeoxia: shunt associated with an aortic aneurysm. J Thorac Cardiovasc Surg 1997;113:955-956.
7.
Bakris NC, Siddiqi AJ, Fraser CD Jr, et al: Right-to-left interatrial shunt after pneumonectomy. Ann Thorac Surg 1997;63:198-201.
8.
Mercho N, Stoller JK, White RD, et al: Right-to-left interatrial shunt causing platypnea after pneumonectomy: a recent experience and diagnostic value of dynamic magnetic resonance imaging. Chest 1994;105:931-933.
9.
Cheng TO: Mechanisms of platypnea-orthodeoxia: what causes water to flow uphill? Circulation 2002;105:e47.
10.
Lambrecht GL, Malbrain ML, Coremans P, et al: Orthodeoxia and platypnea in liver cirrhosis: effects of propranolol. Acta Clin Belg 1994;49:26-30.
11.
Desouza KA, Saraswat S, DeSouza SA, et al: Platypnea-orthodeoxia syndrome: a diagnostic challenge. South Med J 2009;102:1046-1048.
12.
Godart F, Rey C, Prat A, et al: Atrial right-to-left shunting causing severe hypoxaemia despite normal right-sided pressures: report of 11 consecutive cases corrected by percutaneous closure. Eur Heart J 2000;21:483-489.
13.
Mojadidi MK, Gevorgyan R, Noureddin N, Tobis JM: The effect of patent foramen oval closure in patients with platypnea-orthodeoxia syndrome. Catheter Cardiovasc Interv 2015;86:701-707.
© 2015 S. Karger AG, Basel
2015
Copyright / Drug Dosage / Disclaimer
Copyright: All rights reserved. No part of this publication may be translated into other languages, reproduced or utilized in any form or by any means, electronic or mechanical, including photocopying, recording, microcopying, or by any information storage and retrieval system, without permission in writing from the publisher.
Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug.
Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.
You do not currently have access to this content.