TY -的T1 -肺通气/灌注扫描pulmonary veno-occlusive disease JF - European Respiratory Journal JO - Eur Respir J SP - 75 LP - 83 DO - 10.1183/09031936.00097911 VL - 40 IS - 1 AU - Seferian, Andrei AU - Helal, Badia AU - Jaïs, Xavier AU - Girerd, Barbara AU - Price, Laura C. AU - Günther, Sven AU - Savale, Laurent AU - Dorfmüller, Peter AU - Parent, Florence AU - Sitbon, Olivier AU - Humbert, Marc AU - Simonneau, Gérald AU - Montani, David Y1 - 2012/07/01 UR - //www.qdcxjkg.com/content/40/1/75.abstract N2 - 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. ER -