The pathophysiology of pulmonary arterial hypertension (PAH) is underlined by cell proliferation and vasoconstriction of pulmonary arterioles this involves multiple molecular factors or proteins, but it is not clear what the exact roles of these factors/proteins are. In addition, there may be other factors/proteins that have not been identified that contribute to PAH pathophysiology. Therefore, research has focused on investigating novel role players, in order to facilitate a better understanding of how PAH develop. Evidence suggest that mitochondrial regulators are key role players in PAH pathophysiology, but regulators that have not received sufficient attention in PAH are metallothioneins (MTs). In PAH patients, MT expression is elevated compared to healthy individuals, suggesting that MTs may be possible biomarkers. In other disease-models, MTs have been shown to regulate cell proliferation and vasoconstriction, processes that are instrumental in PAH pathophysiology. Due to the involvement of these processes in PAH pathophysiology and the ability of MTs to modulate them, this paper propose that cellular MTs may also play a role in PAH development. This paper suggests that PAH-research should perhaps begin to investigate the involvement of cellular MTs in the development of PAH.
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http://dx.doi.org/10.1016/j.lfs.2018.10.039 | DOI Listing |
J Manag Care Spec Pharm
January 2025
PRIME Education, New York City, NY.
Pulmonary hypertension associated with interstitial lung disease (PH-ILD) is a complex condition in which 2 consequential diseases interact and increase negative outcomes. Although the pathophysiologic mechanisms of PH-ILD are not yet well understood, the pronounced effect on functional status, supplemental oxygen requirements, health care resource utilization, and mortality that frequently accompany this diagnosis are well documented. A critical feature that complicates pathophysiologic understanding of PH-ILD is that progression of the pulmonary vascular disease does not always appear to be driven by the underlying lung disease.
View Article and Find Full Text PDFEchocardiography
January 2025
Department of Cardiology, Nanjing First Hospital, Nanjing Medical University, Nanjing, Jiangsu, China.
Objectives: Numerous studies have demonstrated impaired right ventricular (RV) synchronicity in pulmonary arterial hypertension (PAH). However, few studies have focused on connective tissue disease (CTD)-associated PAH. This study evaluates RV dyssynchrony and its prognostic value in CTD-associated PAH.
View Article and Find Full Text PDFInt J Cardiol Congenit Heart Dis
September 2024
VPD Heart and Lung Research Institute, University of Cambridge, United Kingdom.
Pulmonary hypertension (PH) encompasses a group of conditions which ultimately lead to elevated pulmonary arterial pressure. PH is classified into five subgroups, of which Group 1 pulmonary arterial hypertension (PAH), is the most extensively studied. Numerous causal genes have been identified in PAH, most notably germline mutations in bone morphogenetic protein receptor type 2 () and the wider BMP pathway.
View Article and Find Full Text PDFInt J Cardiol Congenit Heart Dis
September 2024
Royal Brompton Hospital, Part of Guys St Thomas NHS Trust, and National Heart and Lung Institute, Imperial College London, London, United Kingdom.
Pulmonary arterial hypertension (PAH) is defined as increase in mean pulmonary arterial pressure and pulmonary vascular resistance (PVR). It can be associated with congenital heart disease (CHD) with the following subtypes: 1) uncorrected left-to-right (L-R) intracardiac shunt leading to overload of the pulmonary circulation and a progressive increase of PVR; 2) Eisenmenger syndrome, appearing when a large post-tricuspid shunt is left uncorrected and pulmonary vascular disease (PVD) is severe, so the shunt becomes bidirectional or right-to-left, causing cyanosis; 3) PAH after shunt closure, when PVR arises after a defect correction; and 4) PAH associated with small or coincidental defects. While the treatment of patients with Eisenmenger syndrome is well established, the treatment of patients with PAH in whom there is a L-R shunt (with no cyanosis) remains unclear and requires expertise.
View Article and Find Full Text PDFBMC 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).
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