Orthodeoxia and postural orthostatic tachycardia in patients with pulmonary arteriovenous malformations: a prospective 8-year series.
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.
NHLI Cardiovascular Sciences, Imperial College London, London, UK NHLI Respiratory Sciences, Imperial College London, London, UK Imperial College School of Medicine, Imperial College London, London, UK.
V
V
Santhirapala
NHLI Cardiovascular Sciences, Imperial College London, London, UK Imperial College School of Medicine, Imperial College London, London, UK.
B
B
Chamali
Respiratory Medicine, Hammersmith Hospital, Imperial College Healthcare NHS Trust, London, UK.
H
H
McKernan
Respiratory Medicine, Hammersmith Hospital, Imperial College Healthcare NHS Trust, London, UK.
H C
HC
Tighe
Respiratory Medicine, Hammersmith Hospital, Imperial College Healthcare NHS Trust, London, UK.
L C
LC
Williams
Respiratory Medicine, Hammersmith Hospital, Imperial College Healthcare NHS Trust, London, UK.
J T
JT
Springett
Imperial College School of Medicine, Imperial College London, London, UK.
H R
HR
Bellenberg
Imperial College School of Medicine, Imperial College London, London, UK.
A J
AJ
Whitaker
NHLI Cardiovascular Sciences, Imperial College London, London, UK Respiratory Medicine, Hammersmith Hospital, Imperial College Healthcare NHS Trust, London, UK.
C L
CL
Shovlin
D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001164:Arteriovenous Fistula / Q000150:complications* / Q000175:diagnosis / Q000503:physiopathology; D005260:Female; D005500:Follow-Up Studies; D006339:Heart Rate / Q000502:physiology*; D006801:Humans; D008297:Male; D008875:Middle Aged; D010092:Oximetry; D011187:Posture; D011446:Prospective Studies; D011651:Pulmonary Artery / Q000002:abnormalities* / Q000503:physiopathology; D011667:Pulmonary Veins / Q000002:abnormalities* / Q000503:physiopathology; D013610:Tachycardia / Q000175:diagnosis / Q000209:etiology* / Q000503:physiopathology; D013997:Time Factors; D055815:Young Adult
Hypoxaemia; Oxygen; Rare lung disease