Pulmonary hypertension (PH) is a rare and life-threatening disorder marked by considerable morbidity and mortality. Off-label drug use is widespread, with approved drugs available only for the treatment of pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) patients. In PAH patients, phosphodiesterase type 5 (PDE-5) inhibitors and endothelin receptor antagonists (ERAs) are most commonly prescribed as first-line treatment, with the more-efficacious prostacyclin therapies used in later lines. Branded therapies dominate the PAH treatment algorithm, and combination therapy is common. Adempas is the only approved therapy for CTEPH patients. PH is a highly lucrative market despite the relatively small patient population.
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