OBJECTIVES: Pulmonary arterial hypertension (PAH) is a rare, life-threatening, and progressive disease characterized by remodeling and progressive narrowing of the pulmonary vessels. The current treatment strategies for PAH, depending on disease severity and treatment response, include therapy with high-dose calcium channel blockers (in vasoreactive patients) or PAH-specific agents (in non-vasoreactive patients) where treatment decision is individually at physician’s discretion. We aimed to describe treatment patterns with PAH-specific agents from the German payer’s perspective.

METHODS: A retrospective analysis using anonymized claims data from the InGef research database was conducted. The database includes approximately 4 million insured individuals (~ 5% of the German population) and is representative for the German population in terms of age, gender, and region of residence.

The study population comprised patients with a documented diagnosis of PAH treated with eligible PAH-specific agents in 2021. Identified patients needed to have ≥ 1 inpatient or ≥ 2 outpatient diagnosis codes for PAH (ICD-10-GM I27.0) in 2021. The patients were categorized into three subgroups: patients on monotherapy, dual therapy, or triple therapy for PAH.

RESULTS: In 2021, 403 PAH patients treated with PAH-specific agents were identified. About 62.3%, 29.0% and 5.0% received monotherapy, dual or triple therapy, respectively. The remaining 3.7% had a prescription of ≥ 2 of the eligible PAH-specific agents in 2021 but none of the pre-defined combinations.

As monotherapy, the following agents were most frequently prescribed: tadalafil (37.8%), sildenafil (30.3%) and riociguat (17.5%). The combinations of tadalafil+macitentan (31.6%), sildenafil+macitentan (23.1%) and tadalafil+ambrisentan (14.5%) were most frequently prescribed dual therapies. Sildenafil+macitentan+selexipag was the most prevalent triple therapy (35.0%).

CONCLUSIONS: Almost two-thirds of PAH patients treated with PAH-specific agents in 2021 in Germany received monotherapy. The treatment decision is multifactorial and must be made on a patient-individual basis, especially taking severity of disease and previous PAH-specific therapies into account.