Background: Pulmonary hypertension (PH) is a rare and complex disease with poor prognosis, which requires lifelong treatment.
Objective: To describe 3-year follow-up real-life data on treatment with soluble guanylate cyclase stimulators (Riociguat) of patients with PH, measuring current risk assessment parameters.
Methods: This study retrospectively collected clinical and epidemiological data of patients with PH of group 1 (pulmonary arterial hypertension) and group 4 (chronic thromboembolic PH). Non-invasive and invasive parameters corresponding to the risk assessment were analyzed at baseline and follow-up. Statistical analyses were performed using the SPSS 18.0 software, and p-values < 0.050 were considered statistically significant.
Results: In total, 41 patients receiving riociguat were included in the study. Of them, 31 had already completed 3 years of treatment and were selected for the following analysis. At baseline, 70.7% of patients were in WHO functional class III or IV. After 3 years of treatment, the WHO functional class significantly improved in all patients. In addition, the median of the 6-minute walk test (6MWT) significantly increased from 394 ± 91 m at baseline to 458 ± 100 m after 3 years of follow-up (p= 0.014). The three-year survival rate was 96.7%.
Conclusion: In our real-life cohort, most patients with PH treated with riociguat showed stable or improved risk parameters, especially in the 6MWT, at 3 years of follow-up.
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http://dx.doi.org/10.36660/abc.20210492 | DOI Listing |
The main treatment of patients with chronic thromboembolic pulmonary hypertension (CTEPH) is radical surgery, pulmonary thromboendarterectomy (PEA). However, about 40% of patients with CTEPH are inoperable due to distal pulmonary vascular lesions or the severity of hemodynamic disorders. Almost 30% of patients with CTEPH experience persistent or recurrent pulmonary hypertension after surgery, that requires a drug treatment with PAH-specific drugs.
View Article and Find Full Text PDFBMJ Open
December 2024
Clinical Research Department, Ignacio Chávez National Heart Institute, Mexico City, Mexico
Objectives: To investigate clinical characteristics, symptom profile, testing practices, treatment patterns and quality of life (QoL) among patients with pulmonary arterial hypertension (PAH) in Latin America.
Design: Data from the Adelphi Real World PAH Disease Specific Programme, a cross-sectional survey with retrospective data collection.
Setting: University/teaching hospital, regional centres, private practices and government institutions in Argentina, Brazil, Colombia and Mexico.
J Cachexia Sarcopenia Muscle
February 2025
Mitodicure GmbH, Kriftel, Germany.
Background: Recent studies provide strong evidence for a key role of skeletal muscle pathophysiology in myalgic encephalomyelitis/chronic fatigue syndrome (ME/CFS). In a 2021 review article on the pathophysiology of ME/CFS, we postulated that hypoperfusion and ischemia can result in excessive sodium and calcium overload in skeletal muscles of ME/CFS patients to cause mitochondrial damage. Since then, experimental evidence has been provided that supports this concept.
View Article and Find Full Text PDFJ Pharmacol Sci
January 2025
Department of Pathological and Molecular Pharmacology, Faculty of Pharmacy, Osaka Medical and Pharmaceutical University, Takatsuki, Osaka, 569-1094, Japan.
Nitric oxide (NO)-donor drugs, which stimulate reduced form of soluble guanylate cyclase (sGC), have different efficacy to the arteries and veins. This study examined whether sGC activators, which activate oxidized/apo sGC, also have arteriovenous selectivity similar to that of NO-donor drugs. The mechanical responses of the isolated blood vessels were assessed using the organ chamber technique and protein expression was verified using western blotting.
View Article and Find Full Text PDFNephrol Dial Transplant
December 2024
Department of Nephrology and Hypertension, University Hospital Erlangen, Friedrich Alexander University, Erlangen, Germany.
Background And Hypothesis: In chronic kidney disease (CKD) the nitric oxide (NO)-soluble guanylate cyclase (sGC)-cyclic guanosine monophosphate (cGMP) pathway is impaired. Runcaciguat, an sGC activator, activates heme-free sGC, restoring cGMP production. This phase 2a trial studied the efficacy, safety, and tolerability of runcaciguat in CKD patients with or without sodium-glucose co-transporter-2 inhibitor (SGLT2i).
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