Pulmonary arterial hypertension: thin-section CT predictors of epoprostenol therapy failure.

Abstract

To correlate pretherapeutic thin-section computed tomographic (CT) findings in patients with pulmonary hypertension with the risk of fatality with treatment with epoprostenol. Seventy-three consecutive patients with severe pulmonary hypertension treated with epoprostenol were retrospectively separated into two groups. The first group included 12 patients who had a fatal outcome with epoprostenol therapy. The second group (n = 61) was a reference group of patients with epoprostenol-induced clinical improvement. Pretherapeutic thin-section CT scans of each patient were reviewed. Poorly defined nodular opacities (P =.003), septal lines (P =.04), pleural effusion (P =.01), and adenopathy (P =.009) strongly correlated with a risk of clinical worsening with treatment. In six patients in group 1, postmortem examination of the lung revealed either pulmonary veno-occlusive disease or pulmonary capillary hemangiomatosis. On pretherapeutic thin-section CT scans, poorly defined nodular opacities, septal lines, pleural effusion, and adenopathy should raise suspicion for pulmonary veno-occlusive disease or pulmonary capillary hemangiomatosis and provoke possible further evaluation before epoprostenol therapy.