Pulmonary Arterial Hypertension and Cardiopulmonary Comorbidities

In the new guidelines on pulmonary arterial hypertension (PAH, group 1) the differentiation between PAH patients with and without comorbidities is recommended before treatment initiation for the first time. The recommendation is based on the fact that patients with comorbidities have been underrepresented or were excluded from the approval studies, but represent the biggest group of PAH patients in clinical practice. While PAH patients with comorbidities are mainly treated with phosphodiesterase-5 inhibitors as monotherapy, PAH targeted treatment is not indicated in patients with PH due to left heart (group 2) or lung disease (group 3) as it may aggravate the disease or lead to an increased mortality. This article displays how PAH-patients with comorbidities may be differentiated from patients with group 2 and group 3. Differentiation and therapeutic decisions are often complicated and should be made in a pulmonary hypertension expert centre.