Pulmonary hypertension and chronic lung disease: where are we headed?

Abstract

Pulmonary hypertension related to chronic lung disease, mainly represented by COPD and idiopathic pulmonary fibrosis, is associated with a worse outcome when compared with patients only affected by parenchymal lung disease. At present, no therapies are available to reverse or slow down the pathological process of this condition and most of the clinical trials conducted to date have had no clinically significant impact. Nevertheless, the importance of chronic lung diseases is always more widely recognised and, along with its increasing incidence, associated pulmonary hypertension is also expected to be growing in frequency and as a health burden worldwide. Therefore, it is desirable to develop useful and reliable tools to obtain an early diagnosis and to monitor and follow-up this condition, while new insights in the therapeutic approach are explored.

FIGURE 1

Measures obtained from the computed tomography scan that are useful for the radiological suspicion of pulmonary hypertension (PH). Main pulmonary artery (MPA) dilatation values >29 mm in men and >27 mm in women are strongly related to PH (sensitivity 87%, specificity 89%), when its value is >35 mm the specificity reaches 100%. Right and left pulmonary artery (RPA and LPA, respectively) diameter>18 mm is abnormal. MPA dilatation and an ascending aorta (AAO) diameter ratio >1, in individuals <50 years old and with diffuse pulmonary fibrosis, is highly specific for hypertension (specificity >90%, sensitivity 70%). In patients with severe COPD this ratio is more accurate than echocardiography.