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Orthodeoxia and postural orthostatic tachycardia in patients with pulmonary arteriovenous malformations: a prospective 8-year series
  1. V Santhirapala1,2,3,
  2. B Chamali1,3,
  3. H McKernan4,
  4. H C Tighe4,
  5. L C Williams4,
  6. J T Springett4,
  7. H R Bellenberg3,
  8. A J Whitaker3,
  9. C L Shovlin1,4
  1. 1NHLI Cardiovascular Sciences, Imperial College London, London, UK
  2. 2NHLI Respiratory Sciences, Imperial College London, London, UK
  3. 3Imperial College School of Medicine, Imperial College London, London, UK
  4. 4Respiratory Medicine, Hammersmith Hospital, Imperial College Healthcare NHS Trust, London, UK
  1. Correspondence to Dr Claire L Shovlin, NHLI Cardiovascular Sciences, Imperial Centre for Translational and Experimental Medicine, Imperial College London, Hammersmith Campus, Du Cane Road, London, W12 0NN, UK; c.shovlin{at}


Postural changes in 258 patients with pulmonary arteriovenous malformations (PAVMs) reviewed between 2005 and 2013 were evaluated prospectively using validated pulse oximetry methods. Of the 257 completing the test, 75 (29%) demonstrated orthodeoxia with an oxygen saturation fall of at least 2% on standing. None described platypnoea (dyspnoea on standing). The heart rate was consistently higher in the erect posture: 74 (29%) had a postural orthostatic tachycardia of ≥20 min−1, and in 25 (10%) this exceeded 30 min−1. Orthostatic tachycardia was more pronounced in PAVM patients than controls without orthodeoxia (age-adjusted coefficient 5.5 (95% CIs 2.6, 8.4) min−1, p<0.001). For PAVM patients, the age-adjusted pulse rise was 0.79 min−1 greater for every 1% greater drop in oxygen saturation on standing (p<0.001). In contrast to the postural orthostatic tachycardia syndrome, in this population, there was a trend for more pronounced orthostatic tachycardia to be associated with better exercise tolerance.

  • Oxygen
  • Hypoxaemia
  • Rare lung disease
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