Pulmonary arterial hypertension (PAH) is a progressive, debilitating disorder associated with poor quality of life and shortened life span. For many years, medical therapy consisted of calcium channel blockers, warfarin, supplemental oxygen, and digitalis glycosides. A better understanding of the pathophysiology of PAH has led to the recent development of effective treatments for this disorder. Therapeutic agents target the pathophysiologic mechanisms of PAH: pulmonary vasoconstriction, pulmonary vascular remodeling, and in situ thrombosis. With better understanding of the pathogenesis of PAH, recent advances in pharmacotherapy have been introduced for the treatment of PAH. Data are presented on efficacy and safety of newer approved and investigational agents: prostacyclin analogues, oral endothelin antagonists, and phosphodiesterase 5 inhibitors.
FDA Approves Combination Therapy for Pulmonary Arterial Hypertension
March 25th 2024J&J’s Opsynvi is single-tablet combination of macitentan, an endothelin receptor antagonist, and tadalafil, a PDE5 inhibitor. It will be priced on parity with Opsumit, which is also a J&J product to treat patients with PAH.