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Background: Liver transplantation (LTx) is vital in patients with end-stage liver disease, with metabolic dysfunction-associated steatotic liver disease being the most common indication. Primary sclerosing cholangitis (PSC) is an important indication. Portopulmonary hypertension, associated with portal hypertension, poses a significant perioperative risk, making pretransplant screening essential.

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Article Synopsis
  • * She had a splenectomy to address her thrombocytopenia caused by an enlarged spleen (hypersplenism), but nine months later, she experienced exertional dyspnea and was diagnosed with portopulmonary hypertension (PoPH).
  • * Treatment with the medication macitentan improved her symptoms and right heart pressure readings, marking a rare case of PoPH arising after splenectomy.
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Objective: Improving understanding of actual pulmonary hypertension (PH) treatment adherence patterns is crucial to properly treating these patients. We aimed to primarily assess adherence to treatments used for pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) specific therapies, identify potential factors related to it and secondly describe its treatment patterns.

Methods: A 6-month observational cross-sectional study in a tertiary care hospital was conducted.

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Macitentan is a dual endothelin receptor antagonist (ERA) approved for treating pulmonary arterial hypertension (PAH). SOPRANO evaluated the efficacy and safety of macitentan versus placebo in pulmonary hypertension (PH) patients after left ventricular assist device (LVAD) implantation. SOPRANO was a phase 2, multicenter, double-blind, randomized, placebo-controlled, parallel-group study.

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Background: Adverse drug events (ADEs) for endothelin receptor antagonists (ERAs) and prostacyclin-related drugs (PRDs) have been reported in clinical trials, but large-scale, real-world evaluations for respiratory, thoracic, and mediastinal disorders (RTMD) remain scarce.

Methods: A pharmacovigilance analysis of the FAERS database (Q1 2004~Q2 2024) used the reporting odds ratio (ROR) method for disproportionality analysis to assess the adverse drug events (ADEs) of ERAs and PRDs in pulmonary arterial hypertension, focusing on risks related to RTMD.

Results: Reports of ADEs for ERAs (bosentan, ambrisentan, and macitentan) were 15,286, 36795, and 17,497, respectively, and for PRDs (epoprostenol, treprostinil, iloprost, and selexipag) were 5,477, 57265, 3,247, and 5,504.

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