title: Ventilation/perfusion lung scan in pulmonary veno-occlusive disease.
abstract: Pulmonary veno-occlusive disease (PVOD), a rare form of pulmonary arterial hypertension (PAH), requires histological proof for definitive diagnosis; however, lung biopsy is not recommended in PAH. Recent conjoint European Respiratory Society/European Society of Cardiology guidelines suggest that nonmatched perfusion defects on ventilation/perfusion (V'/Q') lung scanning in PAH patients may suggest PVOD. The aim of our study was to evaluate V'/Q' lung scans in a large cohort of PVOD and idiopathic or heritable PAH patients. V'/Q' lung scans from 70 patients with idiopathic or heritable PAH and 56 patients with confirmed or highly probable PVOD were reviewed in a double-blind manner. The vast majority of V'/Q' lung scans were normal or without significant abnormalities in both groups. No differences in ventilation or perfusion lung scans were observed between PAH and PVOD patients (all p>0.05). Furthermore, no differences were observed between confirmed (n=31) or highly probable PVOD (n=25). Nonmatched perfusion defects were found in seven (10%) idiopathic PAH patients and four (7.1%) PVOD patients (p>0.05). Nonmatched perfusion defects were rarely seen in a large cohort of idiopathic or heritable PAH and PVOD patients. Future recommendations should be amended according to these results suggesting that V'/Q' lung scanning is not useful in discriminating PVOD from idiopathic PAH.
authors.affiliation: Université Paris-Sud, Faculté de Médecine, Le Kremlin-Bicêtre, France.
authors.forename: Andrei
authors.initials: A
authors.lastname: Seferian
authors.forename: Badia
authors.initials: B
authors.lastname: Helal
authors.forename: Xavier
authors.initials: X
authors.lastname: Jaïs
authors.forename: Barbara
authors.initials: B
authors.lastname: Girerd
authors.forename: Laura C
authors.initials: LC
authors.lastname: Price
authors.forename: Sven
authors.initials: S
authors.lastname: Günther
authors.forename: Laurent
authors.initials: L
authors.lastname: Savale
authors.forename: Peter
authors.initials: P
authors.lastname: Dorfmüller
authors.forename: Florence
authors.initials: F
authors.lastname: Parent
authors.forename: Olivier
authors.initials: O
authors.lastname: Sitbon
authors.forename: Marc
authors.initials: M
authors.lastname: Humbert
authors.forename: Gérald
authors.initials: G
authors.lastname: Simonneau
authors.forename: David
authors.initials: D
authors.lastname: Montani
mesh_terms: D004311:Double-Blind Method; D065627:Familial Primary Pulmonary Hypertension; D005260:Female; D006439:Hemodynamics; D006801:Humans; D006976:Hypertension, Pulmonary / Q000000981:diagnostic imaging* / Q000503:physiopathology; D008168:Lung / Q000000981:diagnostic imaging* / Q000473:pathology / Q000503:physiopathology; D008297:Male; D011379:Prognosis; D011668:Pulmonary Veno-Occlusive Disease / Q000000981:diagnostic imaging* / Q000473:pathology / Q000503:physiopathology; D011877:Radionuclide Imaging; D012189:Retrospective Studies; D014692:Ventilation-Perfusion Ratio