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A rare case of idiopathic portal hypertension with portopulmonary hypertension occurred following splenectomy with a change in portal hemodynamics.
Clin J Gastroenterol
December 2024
Department of Gastroenterology and Hepatology, Mie University Graduate School of Medicine, 2-174 Edobashi, Tsu, 514-8507, Japan.
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.
Treatment patterns and factors associated with adherence in pulmonary arterial hypertension.
Farm Hosp
December 2024
Servicio de Farmacia, División de Medicamentos, Hospital Clínico de Barcelona, Universidad sw Barcelona, Barcelona, España.
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.
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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- The OPUS/OrPHeUS studies focused on patients with pulmonary arterial hypertension (PAH) related to congenital heart disease (CHD), collecting data on their real-world clinical practices and treatment outcomes while starting on macitentan.
- A total of 272 patients (6.1% of the population) were identified with CHD-PAH, where most were in moderate functional health categories, and many began macitentan as part of combination therapy; a high percentage experienced adverse events, but survival rates were relatively positive over one and two years.
- The findings indicate that macitentan is commonly used in CHD-PAH patients, with its safety profile matching existing knowledge,
Long-Term Safety and Efficacy of Macitentan in Inoperable Chronic Thromboembolic Pulmonary Hypertension: Results from MERIT and its Open-Label Extension.
Pulm Ther
November 2024
German Center for Lung Research (DZL), Giessen, Germany and University of Giessen and Marburg Lung Center (UGMLC), Giessen, Germany.
Introduction: Evidence for use of pulmonary arterial hypertension targeted-therapies in patients with chronic thromboembolic pulmonary hypertension (CTEPH) is limited. In MERIT-1, the endothelin receptor antagonist macitentan improved hemodynamic and functional parameters versus placebo in patients with inoperable CTEPH over a 24-week double-blind (DB) period. Its open-label (OL) extension study (MERIT-2) provides long-term safety/efficacy data.
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