Pulmonary veno-occlusive disease: the role of CT

The authors sought to evaluate the diagnostic accuracy of high-resolution computed tomography (HRCT) in the detection of pulmonary veno-occlusive disease (PVOD) in patients with pre-capillary pulmonary arterial hypertension (PAH) of unknown aetiology, and to identify the role of CT in diagnosis and therapy.

The CT scans of 96 patients were retrospectively reviewed and assessed for specific HRCT findings: ground-glass opacities, septal lines and mediastinal lymph nodal enlargement (short diameter ≥1 cm). According to the HRCT findings, patients were divided into PVOD-suspicious and not PVOD-suspicious. Subsequently, a clinical-instrumental evaluation was performed, and the response to therapy and histopathological reports were evaluated.

Radiological evaluation based on HRCT findings revealed 29 patients as PVOD-suspicious and 67 as not PVOD-suspicious. The final diagnosis was PVOD in 22 patients and idiopathic PAH in 74 patients. The CT scan showed 95.5 % sensitivity, 89% specificity, 72.5% positive predictive value, and 98.5% negative predictive value, with a diagnostic accuracy of 90.5% in identifying patients with PVOD.

Chest CT can be considered a screening test in the assessment of patients with PAH of unknown aetiology, and the radiologist can help the clinician to identify patients with CT findings that make PVOD highly probable.