Idiopathic pulmonary arterial hypertension (IPAH) is a rare and progressive disease, which affects predominantly young women and is associated with frequent complications and high rates of premature death. We present a case of a 37-year-old woman, diagnosed with IPAH after undergoing right heart catheterisation (RHC) with registered mean pulmonary arterial pressure of 54 mmHg and other haemodynamic data suggesting precapillary disease. After a negative vasoreactive test was acquired, monotherapy with endothelin antagonist was initiated. A year later a phosphodiesterase inhibitor was added to the treatment due to worsening of the physical capacity and some other markers. For a period of four years the patient was with a stable clinical course, but since the beginning of 2024 she has started showing signs of decompensated right-sided heart failure and experienced multiple complications. As a result of rhythm and conduction pathology, an electrocardiostimulator was implanted. She was treated for jugular vein thrombosis and later on a haemotransfusion due to bleeding was performed. Treatment of patients with PAH group I is complex because of the dynamic nature of the disease and the need for individual approach, which often requires the united efforts of a multidisciplinary team.
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FDA Approved Drug Products: WINREVAIR (sotatercept-csrk) for injection, for subcutaneous use
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