• Beate Stubbe, Hans-Jürgen Seyfarth, Janina Kleymann, Michael Halank, Hussam Al Ghorani, Anne Obst, Susanna Desole, Ralf Ewert, and Christian F Opitz.
• Internal Medicine B, Pneumology, University Hospital Greifswald, Greifswald, Germany. beate.stubbe@med.uni-greifswald.de.
• BMC Pulm Med. 2021 Apr 21; 21 (1): 130.

BackgroundAlthough combination therapy is the gold standard for patients with pulmonary arterial hypertension (PAH), some of these patients are still being treated with monotherapy.MethodsWe conducted a retrospective analysis at four German PH centres to describe the prevalence and characteristics of patients receiving monotherapy.ResultsWe identified 131 incident PAH patients, with a mean age of 64 ± 13.8 years and a varying prevalence of comorbidities, cardiovascular risk factors and targeted therapy. As in other studies, the extent of prescribed PAH therapy varied with age and coexisting diseases, and younger, so-called "typical" PAH patients were more commonly treated early with combination therapy (48% at 4-8 months). In contrast, patients with multiple comorbidities or cardiovascular risk factors were more often treated with monotherapy (69% at 4-8 months). Survival at 12 months was not significantly associated with the number of PAH drugs used (single, dual, triple therapy) and was not different between "atypical" and "typical" PAH patients (89% vs. 85%).ConclusionAlthough "atypical" PAH patients with comorbidities or a more advanced age are less aggressively treated with respect to combination therapy, the outcome of monotherapy in these patients appears to be comparable to that of dual or triple therapy in "typical" PAH patients.

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