Positional oximetry to screen for pulmonary arteriovenous malformations in HHT.
نویسندگان
چکیده
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.
منابع مشابه
Sensitivity and specificity of radioisotope right-left shunt measurements and pulse oximetry for the early detection of pulmonary arteriovenous malformations.
STUDY OBJECTIVES To assess the effectiveness of pulse oximetry and radioisotope measurement of right-to-left (R-L) shunt for the early detection of pulmonary arteriovenous malformations (PAVMs) in patients with hereditary hemorrhagic telangiectasia (HHT). DESIGN Patients with HHT had serial measurements of the following: (1) arterial oxygen saturation (SaO2) by pulse oximetry in erect and sup...
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Hereditary haemorrhagic telangiectasia (HHT) is a rare autosomal dominant disorder, characterised by the presence of vascular malformations. The pulmonary vascular complications of HHT include pulmonary arteriovenous malformations, pulmonary hypertension associated with high-output heart failure and liver vascular malformations and, finally, pulmonary arterial hypertension secondary to HHT. In ...
متن کاملClinical heterogeneity in hereditary haemorrhagic telangiectasia: are pulmonary arteriovenous malformations more common in families linked to endoglin?
Pulmonary arteriovenous malformations (PAVMs) occur in up to 27% of patients with hereditary haemorrhagic telangiectasia (HHT) and are associated with a rate of paradoxical cerebral embolism at presentation of up to 36%. At least two different loci have been shown for HHT. Mutations in endoglin have been found in some families and the locus designated ORW1. In other families this locus has been...
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ورودعنوان ژورنال:
- Thorax
دوره 69 11 شماره
صفحات -
تاریخ انتشار 2014