Abstract

Orthodeoxia has been described in patients with hereditary haemorrhagic telangiectasia (HHT) and pulmonary arteriovenous malformations (PAVM), but its usefulness in screening children for PAVMs is unknown. We show here that while oximetry is overall lower in children with significant PAVMs, supine and upright oximetry is of no additional diagnostic value indicating that positional oximetry is not a useful screening test for PAVMs.