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(Circulation. 2002;105:e47.)
© 2002 American Heart Association, Inc.

Correspondence

Mechanisms of Platypnea-Orthodeoxia: What Causes Water to Flow Uphill?

Tsung O. Cheng, MD

Professor of Medicine, George Washington University, Washington, DC

To the Editor:

Platypnea-orthodeoxia is a relatively uncommon but striking clinical syndrome characterized by dyspnea and deoxygenation accompanying a change to a sitting or standing from a recumbent position. Since Burchell et al¹ described this rare syndrome over half a century ago, no more than 50 cases have been reported in the literature.²

Two conditions must coexist to cause platypnea-orthodeoxia: an anatomical component in the form of an interatrial communication and a functional component that produces a deformity in the atrial septum and results in a redirection of shunt flow with the assumption of an upright posture. The former may be an atrial septal defect, a patent foramen ovale, or a fenestrated atrial septal aneurysm. The latter may be cardiac, such as pericardial effusion or constrictive pericarditis; pulmonary, such as emphysema, arteriovenous malformation, pneumonectomy, or amiodarone toxicity; abdominal, such as cirrhosis of the liver or ileus; or vascular, such as aortic aneurysm or elongation.²

Under normal conditions an interatrial communication allows blood to shunt from left to right due to a higher pressure in left atrium than right atrium and a greater compliance of the right ventricle than the left ventricle. Right-to-left interatrial shunting is usually associated with spontaneous or induced pulmonary hypertension and, therefore, in the absence of a right-to-left pressure gradient, what is the mechanism for a right-to-left shunt? Or put in another way, what causes water to flow uphill?² A persistent Eustachian valve can cause interatrial right-to-left shunting with a normal right atrial pressure.³ Platypnea-orthodeoxia could be explained on the basis of positional modification of abnormal shunting. Standing upright could stretch the interatrial communication, be it a patent foramen ovale, an atrial septal defect, or a fenestrated atrial septal aneurysm, thus allowing more streaming of venous blood from inferior vena cava through the defect, whether or not a persistent Eustachian valve coexists.³ This redirection of flow caused by an anatomic distortion of the right atrium or the atrial septum also might occur from a loculated pericardial effusion, an aortic aneurysm, or aortic elongation.²

The elegant echocardiographic demonstration by Medina et al⁴ is an example of the last mentioned situation. The readers are referred to my recent editorial for a more detailed discussion of the etiology, differential diagnosis, and management of platypnea-orthodeoxia.² Suffice it to say that the definitive treatment for platypnea-orthodeoxia is closure of the interatrial communication, which can be carried out nowadays by transcatheter techniques,⁵ as was done in the case of Medina et al.⁴

References

1. Burchell HB, Helmholz HF Jr, Wood EH. Reflex orthostatic dyspnea associated with pulmonary hypertension. Am J Physiol. 1949; 159: 563–564.
   
2. Cheng TO. Platypnea-orthodeoxia syndrome: etiology, differential diagnosis, and management. Cathet Cardiovasc Interv. 1999; 47: 64–66.[CrossRef][Medline] [Order article via Infotrieve]
   
3. Cheng TO. Reversible orthodeoxia. Ann Intern Med. 1992; 116: 875.
   
4. Medina A, Suarez de Lezo J, Caballero E, et al. Platypnea-orthodeoxia due to aortic elongation. Circulation. 2001; 104: 741.[Free Full Text]
   
5. Cheng TO. Transcatheter closure of patent foramen ovale: a definitive treatment for platypnea-orthodeoxia. Cathet Cardiovasc Interv. 2000; 51: 120.[CrossRef][Medline] [Order article via Infotrieve]
   

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Response

Alfonso Medina, MD

Chief of Cardiology Department, Hospital Dr. Negrín;, University of Las Palmas, Las Palmas, Spain, E-mail amedina@idecnet.com

Jose Suarez de Lezo, MD

Hospital Reina Sofia, University of Córdoba, Córdoba, Spain

Eduardo Caballero, MD; Jose Ramon Ortega, MD

Hospital Dr. Negrín, Las Palmas, Spain

We appreciate very much Dr Cheng’s comments regarding the definition, pathophysiology, differential diagnosis, and management of platypnea-orthodeoxia syndrome, and we completely agree with them.¹

From our point of view, Dr Cheng’s articles^2–4 help explain the underlying mechanisms of this condition and offer current recommendations for its treatment.

References

1. Medina A, Suarez de Lezo J, Caballero E, et al. Platypnea-orthodeoxia due to aortic elongation. Circulation. 2001; 104: 741.
   
2. Cheng TO. Platypnea-orthodeoxia syndrome: etiology, differential diagnosis, and management. Cathet Cardiovasc Interv. 1999; 47: 64–66.
   
3. Cheng TO. Reversible orthodeoxia. Ann Intern Med. 1992; 116: 875.
   
4. Cheng TO. Transcatheter closure of patent foramen ovale: definitive treatment for platypnea-orthodeoxia. Cathet Cardiovasc Interv. 2000; 51: 120.
   

This Article Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Request Permissions Citing Articles Citing Articles via Google Scholar Google Scholar Articles by Cheng, T. O. Articles by Ortega, J. R. Search for Related Content PubMed PubMed Citation Articles by Cheng, T. O. Articles by Ortega, J. R. Related Collections Cardiac development Catheter-based coronary and valvular interventions: other