Real-world treatment patterns and all-cause mortality of pulmonary arterial hypertension in Europe

<bold>Background:</bold> Pulmonary Arterial Hypertension (PAH) is a complex disease with suboptimal treatment responses and limited survival rates. Understanding real-world treatment patterns and outcomes is important for optimizing patient care. We investigated PAH treatment patterns and mortality rates in selected DARWIN-EU^® data partners. <bold>Methods:</bold> We conducted a retrospective cohort study utilizing primary care and hospital data from four European countries (Estonia, France, Germany, and the UK) between 2012 and 2022. All participating databases were mapped to the OMOP Common Data Model, enhancing standardized data analytics. Study participants included children (1–17 years old) and adults diagnosed with PAH. PAH cases were identified using the SNOMED CT identifier 4013643 and its descendant concepts. <bold>Results:</bold> Among 9,474 patients with newly diagnosed PAH (median age 62–77 years; 51–65% female), cardiovascular diseases were the most common comorbidity. Dyspnea and chest pain were the main presenting symptoms. The predominant PAH treatment in primary care was phosphodiesterase-5 inhibitor monotherapy (83%), mostly sildenafil (74%). Combination therapy was more commonly prescribed in hospitals (53%), and it consisted mainly of tadalafil combined with specific endothelin receptor antagonists, particularly bosentan (17%) and ambrisentan (13%). The all-cause mortality rates were 7-8% at 1 year, increased to 14–32% at 3 years, and reached 17–43% at 5 years post-index. <bold>Conclusions:</bold> This study observed setting-specific differences in PAH treatment and progressively high mortality rates. The findings underscore the urgent need for more effective treatment strategies to improve patient outcomes and survival.