Review

Platypnea-Orthodeoxia: Bilateral Lower-Lobe Pulmonary Emboli and Review of Associated Pathophysiology and Management

Authors: Michael Brunner, MD, Victor Tapson, MD

Abstract

Platypnea-orthodeoxia is a rare syndrome characterized by dyspnea and arterial deoxygenation, which is exacerbated in an upright position and relieved with recumbency. We report a case of platypnea-orthodeoxia in a 56-year-old male who was found to have bilateral lower-lobe pulmonary emboli. He did not have any evidence of an intracardiac shunt. A comprehensive review of the pathophysiology and management of platypnea-orthodeoxia is provided.

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References

1. Burchell HB, Helmholz HF Jr, Wood EH. Reflex orthostatic dyspnea associated with pulmonary hypertension. Am J Physiol 1949;159:563-564.
 
2. Godart F, Rey C. Platypnea-orthodeoxia syndrome: a probably underestimated syndrome? Chest 2001;119:1624-1625.
 
3. Chen GP, Goldberg SL, Gill EA Jr. Patent foramen ovale and the platypnea-orthodeoxia syndrome. Cardiol Clin 2005;23:85-89.
 
4. Mashman WE, Silverman ME. Platypnea related to constrictive pericarditis. Chest 1994;105:636-637.
 
5. Kotoulas C, Patris K, Tsintiris K, et al. Platypnea-orthodeoxia syndrome after pneumonectomy relieved by mediastinal repositioning. Ann Thorac Surg 2007;83:1524-1526.
 
6. Cheng TO. Mechanisms of platypnea-orthodeoxia: what causes water to flow uphill? Circulation 2002;105:e47.
 
7. Bashour T, Kabbani S, Saalouke M, et al. Persistent Eustachian valve causing severe cyanosis in atrial septal defect with normal right heart pressures. Angiology 1983;34:79-83.
 
8. Cordero PJ, Morales P, Mora V, et al. Transient right-to-left shunting through a patent foramen ovale secondary to unilateral diaphragmatic paralysis. Thorax 1994;49:933-934.
 
9. Murray KD, Kalanges LK, Weiland JE, et al. Platypnea-orthodeoxia: an unusual indication for surgical closure of a patent foramen ovale. J Card Surg 1991;6:62-67.
 
10. Schnabel TG Jr, Ratto O, Kirby CK, et al. Postural cyanosis and angina pectoris following pneumonectomy: relief by closure of an interatrial septal defect. J Thorac Surg 1956;32:246-250.
 
11. LaBresh KA, Pietro DA, Coates EO, et al. Platypnea syndrome after left pneumonectomy. Chest1981;79:605-607.
 
12. Bhattacharya K, Birla R, Northridge D, et al. Platypnea-orthodeoxia syndrome: a rare complication after right pneumonectomy. Ann Thorac Surg 2009;88:2018-2019.
 
13. Adolph EA, Lacy WO, Hermoni YI, et al. Reversible orthodeoxia and platypnea due to right-to-left intracardiac shunting related to pericardial effusion. Ann Intern Med 1992;116:138-139.
 
14. Courtis J, Marani L, Amuchastegui LM, et al. Cardiac lipoma: a rare cause of right-to-left interatrial shunt with normal pulmonary artery pressure. J Am Soc Echocardiogr 2004;17:1311-1314.
 
15. Shakur R, Ryding A, Timperley J, et al. Late emergence of platypnea orthodeoxia: Chiari network and atrial septal hypertrophy demonstrated with transoesophageal echocardiography. Eur J Echocardiogr 2008;9:694-696.
 
16. Hashimoto M, Okawa Y, Baba H, et al. Platypnea-orthodeoxia syndrome combined with constrictive pericarditis after coronary artery bypass surgery. J Thorac Cardiovasc Surg 2006;132:1225-1226.
 
17. Wright RS, Simari RD, Orszulak TA, et al. Eosinophilic endomyocardial disease presenting as cyanosis, platypnea, and orthodeoxia. Ann Intern Med 1992;117:482-483.
 
18. Patakas D, Pitsiou G, Philippou D, et al. Reversible platypnoea and orthodeoxia after surgical removal of an hydatid cyst from the liver. Eur Respir J 1999;14:725-727.
 
19. Rietveld AP, Merrman L, Essed CE, et al. Right to left shunt, with severe hypoxemia, at the atrial level in a patient with hemodynamically important right ventricular infarction. J Am Coll Cardiol1983;2:776-779.
 
20. Strunk BL, Cheitlin MD, Stulbarg MS, et al. Right-to-left interatrial shunting through a patent foramen ovale despite normal intracardiac pressures. Am J Cardiol 1987;60:413-415.
 
21. Yeo KK, Rogers JH. Dual mechanism platypnea-orthodeoxia syndrome from severe right coronary artery stenosis and a patent foramen ovale. Catheter Cardiovasc Interv 2007;70:440-444.
 
22. Medina A, de Lezo JS, Caballero E, et al. Platypnea-orthodeoxia due to aortic elongation. Circulation 2001;104:741.
 
23. Popp G, Melek H, Garnett AR Jr. Platypnea-orthodeoxia related to aortic elongation. Chest1997;112:1682-1684.
 
24. Sanikommu V, Lasorda D, Poornima I. Anatomical factors triggering platypnea-orthodeoxia in adults. Clin Cardiol 2009;32:E55-E57.
 
25. Eicher JC, Bonniaud P, Baudouin N, et al. Hypoxaemia associated with an enlarged aortic root: a new syndrome? Heart 2005;91:1030-1035.
 
26. Laybourn KA, Martin ET, Cooper RA, et al. Platypnea and orthodeoxia: shunting associated with an aortic aneurysm. J Thorac Cardiovasc Surg 1997;113:955-956.
 
27. Faller M, Kessler R, Chaouat A, et al. Platypnea-orthodeoxia syndrome related to an aortic aneurysm combined with an aneurysm of the atrial septum. Chest 2000;118:553-557.
 
28. Tempelhof MW, Gray AL, Wallace TW. An interesting case of positional hypoxia: the effect of multiple synergistic pathological conditions. South Med J 2009;102:211-213.
 
29. Varkul M, Robinson T, Ng E, et al. Orthodeoxia and platypnea secondary to a patent foramen ovale despite normal right-sided cardiac pressures. Can Respir J 2001;8:105-107.
 
30. Coates EO Jr, Drake EH. Myxoma of the right atrium, with variable right-to-left shunt; clinical and physiologic observations and report of a case with successful operative removal. N Engl J Med1958;259:165-169.
 
31. Gallerstein PE, Belluscio RL, Berger M, et al. Right to left intracardiac shunt: a unique presentation of metastatic cardiac disease. J Am Coll Cardiol 1984;3:865-867.
 
32. Hsu PF, Leu HB, Lu TM, et al. Platypnea-orthodeoxia syndrome occurring after a blunt chest trauma with acute tricuspid regurgitation. Am J Med 2004;117:890-891.
 
33. Somers C, Slabbynck H, Paelinck BP. Echocardiographic diagnosis of platypnoea-orthodeoxia syndrome after blunt chest trauma. Acta Cardiol 2000;55:199-201.
 
34. Altman M, Robin ED. Platypnea (diffuse zone I phenomenon?). N Engl J Med 1969;281:1347-1348.
 
35. Michel O, Sergysels R, Ham H. Platypnea induced by worsening of VA/Q inhomogeneity in the sitting position in chronic obstructive lung disease. Chest 1988;93:1108-1110.
 
36. Tenholder MF, Russell MD, Knight E, et al. Orthodeoxia: a new finding in interstitial fibrosis. Am Rev Respir Dis 1987;136:170-173.
 
37. Crystal RG, Fulmer JD, Roberts WC, et al. Idiopathic pulmonary fibrosis. Clinical, histologic, radiographic, physiologic, scintigraphic, cytologic, and biochemical aspects. Ann Intern Med 1976;85:769-788.
 
38. Bourke SJ, Munro NC, White JE, et al. Platypnoea-orthodeoxia in cryptogenic fibrosing alveolitis. Respir Med 1995;89:387-389.
 
39. Papiris SA, Maniati MA, Manoussakis MN, et al. Orthodeoxia in amiodarone-induced acute reversible pulmonary damage. Chest 1994;105:965-966.
 
40. Katsoulis K, Minasidis I, Vainas A, et al. Platypnea and orthodeoxia associated with Pneumocystis jiroveci and Cytomegalovirus pneumonia: a case report. J Med Case Reports 2009;3:9319.
 
41. Newton PN, Wakefield AE, Goldin R, et al. Pneumocystis carinii pneumonia with pleurisy, platypnoea and orthodeoxia. Thorax 2003;58:185-186.
 
42. Limthongkul S, Charoenlap P, Nuchprayoon C, et al. Platypnea and orthodeoxia: a report of three cases and hypothesis in pathogenesis. J Med Assoc Thai 1983;66:417-424.
 
43. Fox JL, Brown E, Harrison JK, et al. Platypnea-orthodeoxia and progressive autonomic failure. Am Rev Respir Dis 1989;140:1802-1804.
 
44. Bouros D, Agouridakis P, Tsatsakis A, et al. Orthodeoxia and platypnoea after acute organophosphorus poisoning reversed by CPAP: a newly described cause and review of the literature. Respir Med 1995;89:625-628.
 
45. Ferry TG, Naum CC. Orthodeoxia-platypnea due to diabetic autonomic neuropathy. Diabetes Care1999;22:857-859.
 
46. Hussain SF, Mekan SF. Platypnea-orthodeoxia: report of two cases and review of the literature. South Med J 2004;97:657-662.
 
47. Robin ED, Laman D, Horn BR, et al. Platypnea related to orthodeoxia caused by true vascular lung shunts. N Engl J Med 1976;294:941-943.
 
48. Awan AN, Ashraf R, Meyerson MB, et al. Radiation-induced bronchial stenosis: a new cause of platypnea-orthodeoxia. South Med J 1999;92:720-724.
 
49. Schraufnagel DE, Kay JM. Structural and pathologic changes in the lung vasculature in chronic liver disease. Clin Chest Med 1996;17:1-15.
 
50. Lambrecht GL, Malbrain ML, Coremans P, et al. Orthodeoxia and platypnea in liver cirrhosis: effects of propranolol. Acta Clin Belg 1994;49:26-30.
 
51. Santiago SM Jr, Dalton JW Jr. Platypnea and hypoxemia in Laennec's cirrhosis of the liver. South Med J 1977;70:510-512.
 
52. Krowka MJ, Cortese DA. Hepatopulmonary syndrome. Current concepts in diagnostic and therapeutic considerations. Chest 1994;105:1528-1537.
 
53. DesJardin JA, Martin RJ. Platypnea in the intensive care unit. A newly described cause. Chest 1993;104:1308-1309.
 
54. Seward JB, Hayes DL, Smith HC, et al. Platypnea-orthodeoxia: clinical profile, diagnostic workup, management, and report of seven cases. Mayo Clin Proc 1984;59:221-231.
 
55. Marples IL, Heap MJ, Suvarna SK, et al. Acute right-to-left inter-atrial shunt; an important cause of profound hypoxia. Br J Anaesth 2000;85:921-925.
 
56. Natalie AA, Nichols L, Bump GM. Platypnea-Orthodeoxia, an uncommon presentation of patent foramen ovale. Am J Med Sci 2010;339:78-80.
 
57. Soliman OI, Geleijnse ML, Meijboom FJ, et al. The use of contrast echocardiography for the detection of cardiac shunts. Eur J Echocardiogr 2007;8:S2-S12.
 
58. Kim YJ, Hur J, Shim CY, et al. Patent foramen ovale: diagnosis with multidetector CT-comparison with transesophageal echocardiography. Radiology 2009;250:61-67.
 
59. Seward JB, Tajik AJ, Hagler DJ, et al. Peripheral venous contrast echocardiography. Am J Cardiol 1977;39:202-212.
 
60. Durand E, Bussy E, Gaillard JF. Lung scintigraphy in postpneumonectomy dyspnea due to a right-to-left shunt. J Nucl Med 1997;38:1812-1815.
 
61. Roxas-Timonera M, Larracas C, Gersony D, et al. Patent foramen ovale presenting as platypnea-orthodeoxia: diagnosis by transesophageal echocardiography. J Am Soc Echocardiogr 2001;14:1039-1041.
 
62. Pavoni D, Ragazzo S, Driussi M, et al. Transient platypnea-orthodeoxia-like syndrome induced by propafenone overdose in a young woman with Ebstein's anomaly. Ital Heart J 2003;4:891-894.
 
63. Khan F, Parekh A. Reversible platypnea and orthodeoxia following recovery from adult respiratory distress syndrome. Chest 1979;75:526-528.
 
64. Gacad G, Akhtar N, Cohn JN. Orthostatic hypoxemia in a patient with bronchogenic carcinoma. Arch Intern Med 1974;134:1113-1115.
 
65. Schwenk NR, Schapira RM, Byrd JC. Laryngeal carcinoma presenting as platypnea. Chest 1994;106:1609-1611.