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.
- Guidelines
- pulmonary arterial hypertension
- pulmonary veno-occlusive disease
- ventilation/perfusion lung scan
Footnotes
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Statement of Interest
Statements of interest for O. Sitbon, M. Humbert, G. Simonneau and D. Montani can be found at www.erj.ersjournals.com/site/misc/statements.xhtml
- Received June 7, 2011.
- Accepted November 7, 2011.
- ©ERS 2012