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 (V9/Q9) lung scanning in PAH patients may suggest PVOD. The aim of our study was to evaluate V9/Q9 lung scans in a large cohort of PVOD and idiopathic or heritable PAH patients.V9/Q9 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 V9/Q9 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 (n531) or highly probable PVOD (n525). 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 V9/Q9 lung scanning is not useful in discriminating PVOD from idiopathic PAH.