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Case Reports
. 2014 Mar 7:2014:bcr2013201284.
doi: 10.1136/bcr-2013-201284.

Platypnoea-orthodeoxia syndrome: novel cause for a known condition

Rajendra Takhar  1 , Rakesh Biswas, Ashish Arora, Vipin Jain
Affiliations
Case Reports

Platypnoea-orthodeoxia syndrome: novel cause for a known condition

Rajendra Takhar et al. BMJ Case Rep. .

Abstract

A 50-year-old man presented with dyspnoea while sitting, standing and walking but resolved completely in supine position. On cardiorespiratory examinations, fine crackles were noted over bibasal area. Chest X-ray showed bilateral reticulonodular shadows, restrictive pattern on spirometry, elevated alveolar arterial O2 gradient on arterial blood gas. High-resolution CT of the thorax revealed pattern as 'confident' or 'certain' radiographic diagnosis of idiopathic pulmonary fibrosis (IPF). Bubble-contrast echocardiography in recumbent, sitting and upright positions revealed no intracardiac (right to left shunt) or intrapulmonary shunts. This case highlights the necessity of awareness of this syndrome in cases of interstitial lung diseases (ILDs) also. Although 188 cases have been described thus far of platypnoea-orthodeoxia syndrome (P-OS) of various aetiologies, to the best of our knowledge, it is the first ever case of P-OS in ILD/IPF. Both lung bases were predominantly affected in this patient, platypnoea and orthodeoxia were attributed to areas of low/zero ventilation/perfusion (V/Q) ratio (zone 1 phenomena) as no other obvious explanation was found.

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Figures

Figure 1
Figure 1
Posteroanterior view of the chest X-ray showing bilateral diffuse interstitial infiltrates and few reticulonodular shadows in lower and mid zones.
Figure 2
Figure 2
X-ray of the hands showing partial loss of joint space between carpal bones of distal row.
Figure 3
Figure 3
High-resolution CT of the thorax showing peripheral patchy predominantly subpleural, and bibasilar reticular opacities with minimal ground-glass opacities. Bilateral interstitial septal thickening, subpleural fibrosis with traction bronchiectasis and multiple peripherally arranged (subpleural) honeycombing also seen.
Figure 4
Figure 4
Still image from bubble-contrast echocardiography showing no right-to-left shunting of bubbles.
See this image and copyright information in PMC

References

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