title: Usefulness of Transcranial Doppler for Detecting Pulmonary Arteriovenous Malformations in Hereditary Hemorrhagic Telangiectasia.
abstract: The aim of this study was to assess transcranial Doppler (TCD) as a screening test for pulmonary arteriovenous malformation (PAVM) in patients with hereditary hemorrhagic telangiectasia (HHT). This retrospective study included suspected patients with HHT who were screened for PAVM with a TCD and a chest computed tomography (CT) study. The results of TCD and CT were compared to evaluate the usefulness of TCD for detecting PAVM. A TCD Spencer grade ≥3 was defined as positive for a significant right-to-left shunt (RLS). The diameter of the pulmonary arteries feeding the PAVM was measured by calipers from the CT study. In 86 subjects from 74 families with HHT, the sensitivity of TCD for identifying a PAVM at rest was 98% and post-Valsalva was 100%. Specificity was 58% and 35%, respectively, presumably due to pulmonary shunts too small to recognize on CT. Of the patients with HHT who were referred for embolization therapy for their PAVMs, all 20 had TCD grade ≥3. In patients who were diagnosed with a PAVM by chest CT, patients with TCD grade ≥5 had a significantly larger sum of artery diameters feeding the PAVMs compared to those with grade ≤4 (5.0 ± 3.2 mm vs 2.6 ± 1.9 mm, p = 0.01). In conclusion, a TCD examination for evaluating RLS is sensitive for identifying PAVM in patients with HHT and is useful in quantitating the degree of RLS flow. The sensitivity of the TCD examination makes it a useful screening test without radiation in HHT subjects to determine which patients need to undergo CT evaluation to identify PAVMs.
authors.affiliation: Department of Medicine, Program in Interventional Cardiology, David Geffen School of Medicine at UCLA, Los Angeles.
authors.forename: Yasufumi
authors.initials: Y
authors.lastname: Kijima
authors.affiliation: Department of Medicine, Program in Interventional Cardiology, David Geffen School of Medicine at UCLA, Los Angeles.
authors.forename: Rubine
authors.initials: R
authors.lastname: Gevorgyan
authors.affiliation: Department of Radiology, David Geffen School of Medicine at UCLA, Los Angeles.
authors.forename: Justin Pryce
authors.initials: JP
authors.lastname: McWilliams
authors.affiliation: Department of Medicine, Program in Interventional Cardiology, David Geffen School of Medicine at UCLA, Los Angeles.
authors.forename: Nicholas
authors.initials: N
authors.lastname: Miller
authors.affiliation: Department of Medicine, Program in Interventional Cardiology, David Geffen School of Medicine at UCLA, Los Angeles.
authors.forename: Nabil
authors.initials: N
authors.lastname: Noureddin
authors.affiliation: Department of Medicine, Program in Interventional Cardiology, David Geffen School of Medicine at UCLA, Los Angeles. Electronic address: jtobis@mednet.ucla.edu.
authors.forename: Jonathan Marvin
authors.initials: JM
authors.lastname: Tobis
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001164:Arteriovenous Fistula / Q000150:complications / Q000175:diagnosis* / Q000628:therapy; D001165:Arteriovenous Malformations / Q000150:complications / Q000175:diagnosis* / Q000628:therapy; D002648:Child; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D011651:Pulmonary Artery / Q000002:abnormalities*; D011667:Pulmonary Veins / Q000002:abnormalities*; D012189:Retrospective Studies; D012680:Sensitivity and Specificity; D013683:Telangiectasia, Hereditary Hemorrhagic / Q000150:complications / Q000000981:diagnostic imaging* / Q000628:therapy; D014057:Tomography, X-Ray Computed; D017585:Ultrasonography, Doppler, Transcranial; D055815:Young Adult