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.22,23

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.24,25 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.

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Idiopathic Pulmonary Fibrosis (an Update) and Progressive Pulmonary Fibrosis in Adults: An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline

Reference

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22. Glaser S, Obst A, Koch B, et al. Pulmonary hypertension in patients with idiopathic pulmonary fibrosis - the predictive value of exercise capacity and gas exchange efficiency. PLoS One. 2013;8:e65643.

23. Lettieri CJ, Nathan SD, Barnett SD, Ahmad S, Shorr AF. Prevalence and outcomes of pulmonary arterial hypertension in advanced idiopathic pulmonary fibrosis. Chest. 2006;129:746-752.

24. Grosse C, Grosse A. CT findings in diseases associated with pulmonary hypertension: a current review. Radiographics. 2010;30:1753-1777.

25. Frazier AA, Galvin JR, Franks TJ, Rosado-De-Christenson ML. From the archives of the AFIP: pulmonary vasculature: hypertension and infarction. Radiographics. 2000;20:491-524.

26. Zisman DA, Karlamangla AS, Ross DJ, et al. High-resolution chest CT findings do not predict the presence of pulmonary hypertension in advanced idiopathic pulmonary fibrosis. Chest. 2007;132:773-779.