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European Respiratory Review : An... Dec 2021Pulmonary arterial hypertension (PAH) is a rare disease characterised by pulmonary vascular remodelling and elevated pulmonary pressure, which eventually leads to right... (Review)
Review
Pulmonary arterial hypertension (PAH) is a rare disease characterised by pulmonary vascular remodelling and elevated pulmonary pressure, which eventually leads to right heart failure and death. Registries worldwide have noted a female predominance of the disease, spurring particular interest in hormonal involvement in the disease pathobiology. Several experimental models have shown both protective and deleterious effects of oestrogens, suggesting that complex mechanisms participate in PAH pathogenesis. In fact, oestrogen metabolites as well as receptors and enzymes implicated in oestrogen signalling pathways and associated conditions such as mutation contribute to PAH penetrance more specifically in women. Conversely, females have better right ventricular function, translating to a better prognosis. Along with right ventricular adaptation, women tend to respond to PAH treatment differently from men. As some young women suffer from PAH, contraception is of particular importance, considering that pregnancy in patients with PAH is strongly discouraged due to high risk of death. When contraception measures fail, pregnant women need a multidisciplinary team-based approach. This article aims to review epidemiology, mechanisms underlying the higher female predominance, but better prognosis and the intricacies in management of women affected by PAH.
Topics: Familial Primary Pulmonary Hypertension; Female; Humans; Hypertension, Pulmonary; Lung; Male; Pregnancy; Pulmonary Arterial Hypertension; Ventricular Function, Right
PubMed: 34750113
DOI: 10.1183/16000617.0330-2020 -
Journal of the American College of... May 2017Pulmonary hypertension (PH) in neonates, infants, children, adolescents, and young adults is a complex condition that can be associated with several cardiac, pulmonary,... (Review)
Review
Pulmonary hypertension (PH) in neonates, infants, children, adolescents, and young adults is a complex condition that can be associated with several cardiac, pulmonary, and systemic diseases contributing to morbidity and mortality. The underlying pulmonary hypertensive vascular disease (PHVD) is characterized by inflammation, pulmonary vascular remodeling, and angio-obliteration leading to elevated pulmonary arterial pressure and resistance, right ventricular dysfunction, left ventricular compression, and subsequent heart failure. Recent advancements in PH-targeted therapies and interventional-surgical procedures have contributed to the improvement in quality of life and survival in PH/PHVD. This paper gives an update on recent developments in the diagnosis and treatment of children and young adults with PH. The focus is on the heterogeneous etiology/pathophysiology of PH in the young, and particularly on PHVD associated with congenital heart disease. Moreover, new pharmacological, surgical, and interventional therapies and their practical application in progressive/severe pulmonary arterial hypertension with inadequate response to conventional pharmacotherapy are discussed.
Topics: Child; Disease Management; Disease Progression; Heart Defects, Congenital; Heart Failure; Humans; Hypertension, Pulmonary; Infant; Quality of Life; Young Adult
PubMed: 28521893
DOI: 10.1016/j.jacc.2017.03.575 -
European Respiratory Review : An... Dec 2017http://ow.ly/7y3l30gN0hq
http://ow.ly/7y3l30gN0hq
Topics: Animals; Congresses as Topic; Humans; Hypertension, Pulmonary
PubMed: 29263177
DOI: 10.1183/16000617.0120-2017 -
Revista Medica de Chile Jul 2016
Topics: Humans; Hypertension, Pulmonary
PubMed: 27661543
DOI: 10.4067/S0034-98872016000700001 -
Anatolian Journal of Cardiology Jun 2023Cancer therapy-related pulmonary hypertension is a rare yet potentially fatal cardiotoxicity. However, it is a reversible cause of pulmonary hypertension if detected in... (Review)
Review
Cancer therapy-related pulmonary hypertension is a rare yet potentially fatal cardiotoxicity. However, it is a reversible cause of pulmonary hypertension if detected in its early stages. Cancer therapy-related pulmonary hypertension has been encountered in patients using tyrosine kinase inhibitors, particularly dasatinib. However, it is also well known that many agents used in cancer treatment such as alkylating agents, proteasome inhibitors, thoracic radiation exposure, and immune checkpoint inhibitors are particularly associated with pulmonary hypertension evolution. In case that history, symptoms, and clinical findings suggest a potential cancer therapy-related pulmonary hypertension, echocardiography is considered as the initial tool to detect pulmonary hypertension. If the possibility of pulmonary hypertension is high based on echocardiographic data, cancer treatment, as the initial step, should be discontinued due to its potential risks and other causes for pulmonary hypertension should be investigated thoroughly. Right heart catheterization should be the next step to establish the final diagnosis, and medical management, where appropriate, should be started without delay in these patients according to their pulmonary hypertension subgroup. There exists limited information regarding the diagnostic and management strategies of cancer therapy-related pulmonary hypertension in the current guidelines. In this review article, we aim to present current literature data on the mechanisms and management of cancer therapy-related pulmonary hypertension along with its follow-up algorithm in the setting of cardio-oncology practice.
Topics: Humans; Hypertension, Pulmonary; Dasatinib; Echocardiography; Cardiotoxicity; Heart; Neoplasms
PubMed: 37257013
DOI: 10.14744/AnatolJCardiol.2023.3013 -
International Journal of Molecular... May 2023This Special Issue, "Molecular Research on Pulmonary Hypertension 3 [...].
This Special Issue, "Molecular Research on Pulmonary Hypertension 3 [...].
Topics: Humans; Hypertension, Pulmonary; Translational Research, Biomedical; Research
PubMed: 37239809
DOI: 10.3390/ijms24108462 -
Nagoya Journal of Medical Science Feb 2019Pulmonary hypertension (PH) is a hemodynamic state that is characterized by a resting mean pulmonary artery pressure ≧ 25 mmHg. The common forms of PH are pulmonary... (Review)
Review
Pulmonary hypertension (PH) is a hemodynamic state that is characterized by a resting mean pulmonary artery pressure ≧ 25 mmHg. The common forms of PH are pulmonary arterial hypertension (PAH), chronic thromboembolic pulmonary hypertension (CTEPH), PH caused by left-heart disease, and PH due to lung disease. Previously regarded as untreatable, the treatment of PAH has dramatically advanced since the introduction of the drug epoprostenol in 1999, with three-year survival rates improving from 30%-40% to over 85%. Drugs available for the specific treatment of PAH include endothelin-receptor antagonists, phosphodiesterase type 5 inhibitors, soluble guanylate cyclase stimulators, prostacyclin analogs, and prostacyclin-receptor agonists. In the past decade, management and treatment of CTEPH have also improved. While pulmonary endarterectomy used to be the only option for the treatment of CTEPH, newer treatments include a soluble guanylate cyclase stimulator, which has proven to be an efficacious targeted therapy. Other cases benefit from balloon pulmonary angioplasty.
Topics: Animals; Endarterectomy; Endothelin Receptor Antagonists; Humans; Hypertension, Pulmonary; Pulmonary Artery; Pulmonary Embolism
PubMed: 30962652
DOI: 10.18999/nagjms.81.1.19 -
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 -
British Journal of Hospital Medicine... Aug 2023Post-thrombotic syndrome and chronic thromboembolic pulmonary hypertension are two distinct clinical syndromes associated with adverse patient outcomes following a... (Review)
Review
Post-thrombotic syndrome and chronic thromboembolic pulmonary hypertension are two distinct clinical syndromes associated with adverse patient outcomes following a venous thromboembolism. Clinical manifestations of post-thrombotic syndrome include persistent pain, swelling and ultimately venous ulceration following a deep venous thrombosis. Patients experiencing chronic thromboembolic pulmonary hypertension may have symptoms ranging from exertional dyspnoea to overt right heart failure. From a physician's perspective, the most effective preventative strategy is good quality anticoagulation for prophylaxis of primary and secondary venous thromboembolism. The treatment of post-thrombotic syndrome mainly involves lifestyle modifications alongside the use of elastic compression stockings while patients with chronic thromboembolic pulmonary hypertension should be offered targeted surgical and medical treatment options available at expert centres. Further research is warranted for both conditions to determine the role of direct oral anticoagulants when used with a preventive or therapeutic intent.
Topics: Humans; Hypertension, Pulmonary; Venous Thromboembolism; Dyspnea; Heart Failure; Life Style
PubMed: 37646549
DOI: 10.12968/hmed.2023.0114