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American Journal of Respiratory and... Aug 2023
Topics: Humans; Hypertension, Pulmonary; Lung Diseases, Interstitial
PubMed: 37343302
DOI: 10.1164/rccm.202306-1005ED -
European Respiratory Review : An... Dec 2015http://ow.ly/TAvMf
http://ow.ly/TAvMf
Topics: Congresses as Topic; Humans; Hypertension, Pulmonary; Predictive Value of Tests; Treatment Outcome
PubMed: 26621969
DOI: 10.1183/16000617.0064-2015 -
Cells Jun 2021Pulmonary hypertension (PH) is a severe and multifactorial disease characterized by a progressive elevation of pulmonary arterial resistance and pressure due to... (Review)
Review
Pulmonary hypertension (PH) is a severe and multifactorial disease characterized by a progressive elevation of pulmonary arterial resistance and pressure due to remodeling, inflammation, oxidative stress, and vasoreactive alterations of pulmonary arteries (PAs). Currently, the etiology of these pathological features is not clearly understood and, therefore, no curative treatment is available. Since the 1990s, hydrogen sulfide (HS) has been described as the third gasotransmitter with plethoric regulatory functions in cardiovascular tissues, especially in pulmonary circulation. Alteration in HS biogenesis has been associated with the hallmarks of PH. HS is also involved in pulmonary vascular cell homeostasis via the regulation of hypoxia response and mitochondrial bioenergetics, which are critical phenomena affected during the development of PH. In addition, HS modulates ATP-sensitive K channel (K) activity, and is associated with PA relaxation. In vitro or in vivo HS supplementation exerts antioxidative and anti-inflammatory properties, and reduces PA remodeling. Altogether, current findings suggest that HS promotes protective effects against PH, and could be a relevant target for a new therapeutic strategy, using attractive HS-releasing molecules. Thus, the present review discusses the involvement and dysregulation of HS metabolism in pulmonary circulation pathophysiology.
Topics: Animals; Humans; Hydrogen Sulfide; Hypertension, Pulmonary
PubMed: 34204699
DOI: 10.3390/cells10061477 -
Swiss Medical Weekly Dec 2018Chronic thromboembolic pulmonary hypertension (CTEPH) is a potentially fatal disease, which may occur as a rare complication after acute pulmonary embolism, although the... (Review)
Review
Chronic thromboembolic pulmonary hypertension (CTEPH) is a potentially fatal disease, which may occur as a rare complication after acute pulmonary embolism, although the exact epidemiology of CTEPH is unknown. The mechanisms involved in nonresolution of thrombotic material and scarring of large and/or small pulmonary arteries are unknown; some risk factors have been identified. To date, CTEPH is still underdiagnosed and undertreated. The cardinal symptom of CTEPH is dyspnoea on exertion, but diagnosis is challenging owing to nonspecific symptoms. Right heart catheterisation is mandatory for the diagnosis of pulmonary hypertension, followed by several imaging methods including besides ventilation/perfusion scan, computed tomography pulmonary angiography and conventional angiography. Operability assessment by a multidisciplinary team is crucial for the management in all CTEPH patients, as pulmonary endarterectomy (PEA) remains the only curative treatment of choice. PEA leads to substantial improvement of haemodynamics, symptoms, and life expectancy enabling many patients to lead unrestricted lives under sole anticoagulation therapy. For inoperable patients or those with disease not amenable to surgery, medical therapy or balloon angioplasty are emerging treatment options. Owing to the complexity of CTEPH, the diagnosis and treatment of CTEPH patients is reserved exclusively to experienced CTEPH centres.
Topics: Angioplasty, Balloon; Anticoagulants; Cardiac Catheterization; Endarterectomy; Humans; Hypertension, Pulmonary; Pulmonary Artery; Pulmonary Embolism; Risk Factors
PubMed: 30576568
DOI: 10.4414/smw.2018.14702 -
Arteriosclerosis, Thrombosis, and... Jun 2019
Topics: Denervation; Familial Primary Pulmonary Hypertension; Humans; Hypertension, Pulmonary; Pulmonary Arterial Hypertension; Pulmonary Artery
PubMed: 31116607
DOI: 10.1161/ATVBAHA.119.312591 -
Methodist DeBakey Cardiovascular Journal 2024Pulmonary arterial hypertension (PAH) is a rare devastating disease characterized by elevated pulmonary artery pressure and increased pulmonary vascular resistance.... (Review)
Review
Pulmonary arterial hypertension (PAH) is a rare devastating disease characterized by elevated pulmonary artery pressure and increased pulmonary vascular resistance. Females have a higher incidence of PAH, which is reflected globally across registries in the United States, Europe, and Asia. However, despite female predominance, women had better outcomes compared with male patients, a finding that has been labeled the "estrogen paradox." Special considerations should be given to women with PAH regarding sexual health, contraception, family planning, and treatment before, during, and after pregnancy. Pregnant women with PAH should be referred to a pulmonary hypertension care center; a multidisciplinary team approach is recommended, and Cesarean section is the preferred mode of delivery. While pregnancy outcomes have improved over the years with PAH-specific therapy, pregnancy portends a high-risk for those with PAH. Continued research is needed to tailor PAH treatment for women.
Topics: Female; Pregnancy; Humans; Male; Hypertension, Pulmonary; Cesarean Section; Pregnancy Outcome; Familial Primary Pulmonary Hypertension; Hypertension
PubMed: 38495664
DOI: 10.14797/mdcvj.1308 -
International Journal of Molecular... Jan 2024Pulmonary hypertension (PH) is recognized as a pathophysiological disorder encompassing a wide spectrum of clinical conditions related to various cardiovascular and...
Pulmonary hypertension (PH) is recognized as a pathophysiological disorder encompassing a wide spectrum of clinical conditions related to various cardiovascular and respiratory diseases [...].
Topics: Humans; Pulmonary Arterial Hypertension; Familial Primary Pulmonary Hypertension; Hypertension, Pulmonary
PubMed: 38256236
DOI: 10.3390/ijms25021166 -
Current Opinion in Pulmonary Medicine Sep 2023The purpose of this review is to provide an overview of assessment of right ventricular function in the context of pulmonary hypertension and pulmonary arterial... (Review)
Review
PURPOSE OF REVIEW
The purpose of this review is to provide an overview of assessment of right ventricular function in the context of pulmonary hypertension and pulmonary arterial hypertension (PAH). We will review unique features of right ventricular anatomy, delineation of cause of pulmonary hypertension through careful right ventricular assessment, echocardiographic and hemodynamic evaluation, and the importance of this assessment in prognosis.
RECENT FINDINGS
The importance of performance in prognosis and risk assessment in patients with pulmonary hypertension has been continually emphasized in ongoing research. Representative parameters of right ventricular function have been shown to be predictive of prognosis in patients with pulmonary hypertension. Further, the importance of serial right ventricular assessment in risk assessment and prognosis has remained an emerging theme.
SUMMARY
Careful evaluation of right ventricular function is paramount in assessing the cause of pulmonary hypertension and severity of disease. Further, it has prognostic significance, as many representative parameters of right ventricular function have been linked with mortality. In our opinion, right ventricular function should be assessed serially throughout the course of treatment in pulmonary hypertension, and baseline parameters in addition to dynamic changes should be incorporated into risk assessment. Achieving normal or near-normal right ventricular performance may serve as a principal goal in the treatment of pulmonary hypertension.
Topics: Humans; Hypertension, Pulmonary; Pulmonary Arterial Hypertension; Prognosis; Familial Primary Pulmonary Hypertension; Echocardiography; Ventricular Dysfunction, Right
PubMed: 37410491
DOI: 10.1097/MCP.0000000000000980 -
BMJ Case Reports May 2017
Topics: Adult; Female; Heart Valve Prosthesis; Heart Valve Prosthesis Implantation; Humans; Hypertension, Pulmonary; Mitral Valve; Mitral Valve Stenosis; Phonocardiography
PubMed: 28512104
DOI: 10.1136/bcr-2017-220120 -
Annals of the American Thoracic Society Aug 2021Pulmonary hypertension (PH) has been described in patients treated with leflunomide. To assess the association between leflunomide and PH. We identified incident...
Pulmonary hypertension (PH) has been described in patients treated with leflunomide. To assess the association between leflunomide and PH. We identified incident cases of PH in patients treated with leflunomide from the French PH Registry and through the pharmacoVIGIlAnce in Pulmonary ArTerial Hypertension (VIGIAPATH) program between September 1999 to December 2019. PH etiology, clinical, functional, radiologic, and hemodynamic characteristics were reviewed at baseline and follow-up. A pharmacovigilance disproportionality analysis using the World Health Organization's global database was conducted. We then investigated the effect of leflunomide on human pulmonary endothelial cells. Data are expressed as median (min-max). Twenty-eight patients treated with leflunomide before PH diagnosis was identified. A total of 21 (75%) had another risk factor for PH and 2 had two risk factors. The median time between leflunomide initiation and PH diagnosis was 32 months (1-120). Right heart catheterization confirmed precapillary PH with a cardiac index of 2.37 L⋅min ⋅m (1.19-3.1) and elevated pulmonary vascular resistance at 9.63 Wood Units (3.6-22.1) without nitric oxide reversibility. Five patients (17.9%) had no other risk factor for PH besides exposure to leflunomide. No significant hemodynamic improvement was observed after leflunomide withdrawal. The pharmacovigilance disproportionality analysis using the World Health Organization's database revealed a significant overrepresentation of leflunomide among reported pulmonary arterial hypertension-adverse drug reactions. studies showed the dose-dependent toxicity of leflunomide on human pulmonary endothelial cells. PH associated with leflunomide is rare and usually associated with other risk factors. The pharmacovigilance analysis suggests an association reinforced by experimental data.
Topics: Cardiac Catheterization; Endothelial Cells; Humans; Hypertension, Pulmonary; Leflunomide; Lung; Pharmacovigilance
PubMed: 33502958
DOI: 10.1513/AnnalsATS.202008-913OC