Updated International Guidelines for IPF Diagnosis: A Radiology Perspective


Pulmonary Arterial Hypertension

Pulmonary arterial hypertension is seen in patients with IPF11 and is particularly common in patients with concomitant emphysema.21 Pulmonary arterial hypertension negatively impacts survival in patients with IPF.2223

Pulmonary arterial hypertension manifests on CT by the finding of an enlarged main pulmonary artery with a diameter greater than the aorta at the same level, right ventricular enlargement, and on occasion, displacement of the interventricular septum.2425 Attenuation of the peripheral pulmonary vessels can also be seen in certain cases. The extent of lung involvement does not, however, correlate with the magnitude pulmonary arterial hypertension.26 The soft-tissue images here were taken through the pulmonary artery to identify mediastinal structures. The clear finding is the dilation of the right main pulmonary artery, indicated in blue, evidenced by the fact that its diameter is wider than the aorta at the same level, marked in red. This is a strong CT sign of pulmonary arterial hypertension.