Evidence-based pulmonary arterial hypertension treatment algorithm for patients with idiopathic, heritable, drug-associated, and connective tissue disease-associated pulmonary arterial hypertension.

DLCO, Lung diffusion capacity for carbon monoxide; ERA, endothelin receptor antagonist; I/H/D-PAH, idiopathic, heritable, or drug-associated pulmonary arterial hypertension; i.v., intravenous; PAH-CTD, PAH associated with connective tissue disease; PCA, prostacyclin analogue; PDE5i, phosphodiesterase 5 inhibitor; PH, pulmonary hypertension; PRA, prostacyclin receptor agonist; ReCo, recommendation; s.c., subcutaneous; sGCs, soluble guanylate cyclase stimulator. ^aCardiopulmonary comorbidities are conditions associated with an increased risk of left ventricular diastolic dysfunction, and include obesity, hypertension, diabetes mellitus, and coronary heart disease; pulmonary comorbidities may include signs of mild parenchymal lung disease and are often associated with a low DLCO (<45% of the predicted value). ^bIntravenous epoprostenol or i.v./s.c. treprostinil.