Platypnoea-orthodeoxia syndrome, an underdiagnosed cause of hypoxaemia: four cases and the possible underlying mechanisms

M Nassif; H Lu; T C Konings; B J Bouma; A Vonk Noordegraaf; B Straver; N A Blom; S A Clur; A P C M Backx; M Groenink; S M Boekholdt; D R Koolbergen; M G Hazekamp; B J M Mulder; R J de Winter

doi:10.1007/s12471-015-0714-5

Platypnoea-orthodeoxia syndrome, an underdiagnosed cause of hypoxaemia: four cases and the possible underlying mechanisms

Neth Heart J. 2015 Nov;23(11):539-45. doi: 10.1007/s12471-015-0714-5.

Authors

M Nassif¹, H Lu², T C Konings³, B J Bouma², A Vonk Noordegraaf⁴, B Straver^{2

5}, N A Blom^{2

5

6}, S A Clur⁵, A P C M Backx², M Groenink², S M Boekholdt², D R Koolbergen^{7

8}, M G Hazekamp^{7

8}, B J M Mulder^{2

9}, R J de Winter²

Affiliations

¹ Department of Cardiology, Cardiac Catheterization Laboratory, Academic Medical Center-University of Amsterdam, PO Box 22660, 1100 DD, Amsterdam, The Netherlands. r.j.dewinter@amc.uva.nl.
² Department of Cardiology, Cardiac Catheterization Laboratory, Academic Medical Center-University of Amsterdam, PO Box 22660, 1100 DD, Amsterdam, The Netherlands.
³ Department of Cardiology, VU University Medical Center, Amsterdam, The Netherlands.
⁴ Department of Pulmonary Medicine, VU University Medical Center, Amsterdam, The Netherlands.
⁵ Department of Pediatric Cardiology, Academic Medical Center, Amsterdam, The Netherlands.
⁶ Department of Pediatric Cardiology, Leiden University Medical Center, Leiden, The Netherlands.
⁷ Department of Cardiothoracic Surgery, Academic Medical Center, Amsterdam, The Netherlands.
⁸ Department of Pediatric Cardiac Surgery, Leiden University Medical Center, Leiden, The Netherlands.
⁹ Interuniversity Cardiology Institute of the Netherlands (ICIN), Utrecht, The Netherlands.

Abstract

Cardiac platypnoea-orthodeoxia syndrome (POS) is a position-dependent condition of dyspnoea and hypoxaemia due to right-to-left shunting. It often remains unrecognised in clinical practice, possibly because of its complex underlying pathophysiology. We present four consecutive patients with POS and patent foramen ovale (PFO) who underwent a successful percutaneous PFO closure, describe the mechanism of their POS and provide a review of the literature.

Keywords: Atrial septum; Dyspnoea; Foramen ovale, patent; Mechanism; Platypnoea-orthodeoxia.