Pulmonary Hypertension in Lung Diseases

Pulmonary hypertension (PH) is a common comorbidity of lung diseases such as chronic obstructive pulmonary disease or idiopathic pulmonary fibrosis and is associated with increased mortality. Pulmonary arterial hypertension (PAH), on the other hand, is a rare disease that also has a high mortality rate. Due to the often older and comorbid patient population of PAH, there are subgroups that differ from typical PAH patients in terms of their treatment response and mortality. In recent years it has been shown that, in addition to the cardiac or left heart phenotype, a pulmonary or cardiopulmonary phenotype can also be distinguished. Differentiation from severe PH in underlying lung disease can be difficult and is reserved for expert centers due to the different treatment strategies.