title: Pulmonary venous occlusion and death in pulmonary arterial hypertension: survival analyses using radiographic surrogates.
abstract: BACKGROUND
  Recent studies find that a considerable number of patients with pulmonary arterial hypertension (PAH) develop fibrous obstruction of the pulmonary veins. Such obstruction more commonly accompanies connective tissue disorder (CTD)-associated PAH than idiopathic PAH. However, few researchers have gauged the risk of death involving obstruction of the pulmonary veins.
  
  
  METHODS
  Thirty-seven patients with PAH were enrolled (18 patients, idiopathic PAH; 19 patients, CTD-associated PAH). The patients were 49 ± 18 years and had a World Health Organization functional class of 3.2 ± 0.6. Thickening of the interlobular septa, centrilobular ground-glass attenuation, and mediastinal adenopathy were surrogates for obstruction of the pulmonary veins, and were detected by a 16-row multidetector computed tomography scanner.
  
  
  RESULTS
  The follow-up period was 714 ± 552 days. Fifteen deaths occurred. Thickening of the interlobular septa, centrilobular ground-glass attenuation, and mediastinal adenopathy were found in 37.8%, 24.3%, and 16.2% of patients, respectively. Cox proportional hazard analysis revealed an increased risk of death with each radiographic surrogate (mediastinal adenopathy: p < 0.0001, hazard ratio = 13.9; thickening of interlobular septa: p < 0.001, hazard ratio = 12.0; ground-glass attenuation: p = 0.02, hazard ratio = 3.7). The statistical significance of these relationships was independent of the cause of PAH and plasma concentration of brain natriuretic peptide.
  
  
  CONCLUSIONS
  The results of this study imply that obstruction of the pulmonary veins is associated with an increased risk of death in patients with PAH.
authors.affiliation: Department of Cardio-Renal Medicine and Hypertension, Nagoya City University Graduate School of Medical Sciences, Nagoya, Japan. takeday@med.nagoya-cu.ac.jp
authors.forename: Yasuko
authors.initials: Y
authors.lastname: Takeda
authors.forename: Yutaka
authors.initials: Y
authors.lastname: Takeda
authors.forename: Koji
authors.initials: K
authors.lastname: Yamamoto
authors.forename: Shigehiro
authors.initials: S
authors.lastname: Tomimoto
authors.forename: Tomomitsu
authors.initials: T
authors.lastname: Tani
authors.forename: Hitomi
authors.initials: H
authors.lastname: Narita
authors.forename: Nobuyuki
authors.initials: N
authors.lastname: Ohte
authors.forename: Genjiro
authors.initials: G
authors.lastname: Kimura
mesh_terms: D000328:Adult; D000368:Aged; D065627:Familial Primary Pulmonary Hypertension; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000150:complications* / Q000000981:diagnostic imaging / Q000401:mortality; D053208:Kaplan-Meier Estimate; D008168:Lung / Q000000981:diagnostic imaging; D008297:Male; D008875:Middle Aged; D016016:Proportional Hazards Models; D011668:Pulmonary Veno-Occlusive Disease / Q000000981:diagnostic imaging* / Q000209:etiology* / Q000401:mortality; D014057:Tomography, X-Ray Computed