Diagnostic and Therapeutic Challenges for Patients with Pulmonary Hypertension due to Lung Diseases

Pulmonary hypertension (PH) associated to chronic lung disease (CLD) is relatively frequent in end-stage disease but can be also present at early stage, sometimes associated with low carbon monoxide diffusion capacity (DLCO) and relatively preserved lung volume in a pulmonary vascular phenotype. PH worsens prognosis, decreases exercise capacity, and impairs quality of life. Echocardiography, chest high-resolution computed tomography (HRCT) and cardiopulmonary exercise test may help for PH screening in CLD. The diagnostic is based on right heart catheterisation. Treatment of CLD-PH includes treatment of the underlying condition, long-term oxygen therapy and lung transplantation. Pulmonary arterial hypertension (PAH)-specific medications may be useful but due to lack of evidences, heterogeneity of the studies and concerns regarding side effects, cannot be recommended. In this review, we summarize epidemiological data, clinical features and treatments of CLD-PH, especially in chronic obstructive pulmonary disease (COPD) and idiopathic pulmonary fibrosis (IPF)