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Ventilation perfusion lung scan in pulmonary veno-occlusive disease

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Published:25th Mar 2020
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 ERS/ESC guidelines, suggest that unmatched perfusion defects on ventilation/perfusion (V/Q) lung scan, 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 was 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 values >0.05). Furthermore, no differences were observed between confirmed (n=31) or highly-probable PVOD (n=25). Unmatched perfusion defects were found in 7 (10%) idiopathic PAH patients and 4 (7.1%) PVOD patients (p>0.05). Unmatched 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 scan is not useful in discriminating PVOD from idiopathic PAH.

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