Pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) are progressive diseases that can lead to right heart failure and death. Right ventricular dysfunction, hypertrophy and maladaptive remodelling are consequences of increased right ventricular (RV) afterload in PAH and CTEPH and are indicative of long-term outcomes. Because RV failure is the main cause of morbidity and mortality in PAH and CTEPH, successful treatments should lead to improvements in RV parameters. Riociguat is a soluble guanylate cyclase stimulator approved for the treatment of PAH and inoperable or persistent/recurrent CTEPH after pulmonary endarterectomy. This review examines the current evidence showing the effect of riociguat on the right ventricle, with particular focus on remodelling, function and structural parameters in preclinical models and patients with PAH or CTEPH.
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http://dx.doi.org/10.1183/16000617.0061-2022 | DOI Listing |
Quant Imaging Med Surg
December 2024
Department of Positron Emission Tomography/Computed Tomography, The First Affiliated Hospital of Xi'an Jiaotong University, Xi'an, China.
Background: All patients with pulmonary hypertension (PH), without left heart disease or lung diseases, need further distinguishing between pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH). This study aimed to investigate the value of quantitative assessment of pulmonary blood flow redistribution in further classification.
Methods: Forty-six patients who underwent echocardiography and computed tomography pulmonary angiography at The First Affiliated Hospital of Xi'an Jiaotong University were included in this cross-sectional study, and all patients were categorized into a non-PH group (18 cases), a PAH group (12 cases), and a CTEPH group (16 cases).
Eur Heart J Open
November 2024
Department of Pulmonology, University Hospital Zurich, Rämistrasse 100, 8091 Zurich, Switzerland.
Aims: More than 220 Mio people live at altitudes above 2000 m, many of whom have pre-existing chronic diseases, including pulmonary vascular diseases (PVDs) such as pulmonary arterial hypertension (PAH) or chronic thromboembolic pulmonary hypertension (CTEPH). We investigated the acute effects of high-dose supplemental oxygen on pulmonary haemodynamics assessed by echocardiography in patients with PVD permanently living at 2850 m.
Methods And Results: In a randomized, single-blind, placebo-controlled crossover trial, patients with PVD diagnosed with PAH or CTEPH were allocated to receive 10 L/min supplemental oxygen (FiO ≈ 95%) and placebo air administered via a facial mask with reservoir near their living altitude in Quito at 2850 m (FiO0.
BMC Pulm Med
December 2024
Actelion Pharmaceuticals Ltd, Allschwil, Switzerland.
Background: Pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) are severe, progressive diseases characterized by key symptoms such as dyspnea and fatigue. These symptoms impair physical functioning, with patients struggling to perform their daily activities. One traditional measure of physical functioning and exercise capacity is the 6-minute walk test (6MWT).
View Article and Find Full Text PDFThe right ventricular stroke work index (RVSWI) reflects the active work of the right ventricle (RV), but its clinical usefulness is not yet fully known in pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH). We aimed to evaluate the correlation of RVSWI to clinical parameters, the presence of comorbidities and response to therapy. We performed a retrospective observational study of 54 patients (PAH: = 30, CTEPH: = 24) and control patients ( = 11), and collected clinical data including RVSWI and comorbidities at baseline.
View Article and Find Full Text PDFEur J Pharmacol
January 2025
Center for Experimental Medicine, Institute for Clinical and Experimental Medicine, Prague, Czech Republic; First Department of Internal Medicine, Cardiology, Olomouc University Hospital and Palacký University, Olomouc, Czech Republic.
Nitric oxide (NO)-stimulated cyclic guanosine monophosphate (cGMP) is a key regulator of cardiovascular health, as NO-cGMP signalling is impaired in diseases like pulmonary hypertension, heart failure and chronic kidney disease. The development of NO-independent sGC stimulators and activators provide a novel therapeutic option to restore altered NO signalling. sGC stimulators have been already approved for the treatment of pulmonary arterial hypertension (PAH), chronic thromboembolic pulmonary hypertension (CTEPH), and chronic heart failure (HFrEF), while sGC activators are currently in phase-2 clinical trials for CKD.
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