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Lack of efficacy: case report An approximately 18-year-old man exhibited lack of efficacy during treatment with epoprostenol and levosimendan for idiopathic pulmonary arterial hypertension [not all dosages stated]. The man was admitted due to severe drug-resistant idiopathic pulmonary arterial hypertension (IPAH) at the age of 20 years. The drug-resistant IPAH had been diagnosed two years before this admission (at approximately 18 years of age). Upon anamnesis, it was noted that he was initially treated with IV epoprostenol [initial dosage not stated]. Despite this treatment, his condition deteriorated eventually requiring several hospitalisations. Since February 2019, he had significant impairment in the mobility to WHO functional class-IV. He also developed severe right heart failure (RHF). His epoprostenol dose reached to 91.5 ng/kg/min. Additionally, IV infusions of levosimendan 3 times every few months were also initiated. However, his condition worsened thereafter. Due to the progressive deterioration despite ongoing medicines and due to lack of alternative medical therapy, he underwent atrial flow regulator (AFR) transplantation for the drug-resistant IPAH. Janus M, et al. Atrial flow regulator as a bridge to lung transplant in a young patient with drug-resistant idiopathic pulmonary arterial hypertension. Kardiologia Polska 78: 803505068 461-462, No. 5, 25 May 2020. Available from: URL: http://doi.org/10.33963/KP.15178

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Reactions 3 Oct 2020 No. 1824