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Cleveland Clinic Journal of Medicine Oct 2023Portopulmonary hypertension, ie, pulmonary arterial hypertension in a patient with portal hypertension, affects transplant eligibility and has a poor prognosis. The... (Review)
Review
Portopulmonary hypertension, ie, pulmonary arterial hypertension in a patient with portal hypertension, affects transplant eligibility and has a poor prognosis. The pathogenesis remains an area of active research, with various mechanisms proposed. Diagnosing it requires a detailed history, physical examination, laboratory tests, and echocardiographic evaluation, followed by a careful hemodynamic assessment.
Topics: Humans; Pulmonary Arterial Hypertension; Hypertension, Pulmonary; Hypertension, Portal; Hemodynamics; Echocardiography
PubMed: 37783493
DOI: 10.3949/ccjm.90a.23023 -
International Journal of Molecular... Jun 2022Hypoxic pulmonary hypertension (HPH) is characterized by sustained elevation of pulmonary artery pressure produced by vasoconstriction and hyperproliferative remodeling... (Review)
Review
Hypoxic pulmonary hypertension (HPH) is characterized by sustained elevation of pulmonary artery pressure produced by vasoconstriction and hyperproliferative remodeling of the pulmonary artery and subsequent right ventricular hypertrophy (RVH). The search for therapeutic targets for cardiovascular pathophysiology has extended in many directions. However, studies focused on mitigating high-altitude pulmonary hypertension (HAPH) have been rare. Because AMP-activated protein kinase (AMPK) is involved in cardiovascular and metabolic pathology, AMPK is often studied as a potential therapeutic target. AMPK is best characterized as a sensor of cellular energy that can also restore cellular metabolic homeostasis. However, AMPK has been implicated in other pathways with vasculoprotective effects. Notably, cellular metabolic stress increases the intracellular ADP/ATP or AMP/ATP ratio, and AMPK activation restores ATP levels by activating energy-producing catabolic pathways and inhibiting energy-consuming anabolic pathways, such as cell growth and proliferation pathways, promoting cardiovascular protection. Thus, AMPK activation plays an important role in antiproliferative, antihypertrophic and antioxidant pathways in the pulmonary artery in HPH. However, AMPK plays contradictory roles in promoting HPH development. This review describes the main findings related to AMPK participation in HPH and its potential as a therapeutic target. It also extrapolates known AMPK functions to discuss the less-studied HAPH context.
Topics: AMP-Activated Protein Kinases; Adenosine Triphosphate; Altitude Sickness; Humans; Hypertension, Pulmonary; Hypoxia; Pulmonary Artery
PubMed: 35682884
DOI: 10.3390/ijms23116205 -
Clinics in Chest Medicine Sep 2023The current approach for the management of pulmonary arterial hypertension (PAH) relies on data gathered from clinical trials and large registries. However, there is... (Review)
Review
The current approach for the management of pulmonary arterial hypertension (PAH) relies on data gathered from clinical trials and large registries. However, there is concern that minorities including Black, Indigenous, and People of Color are underrepresented in these trials and registries, making current data not generalizable to these groups of patients. Hence, it is important to discuss the significance of race/ethnicity and socioeconomic factors in patients with PAH. Here, we review the current knowledge on health care disparities in PAH. We also propose future steps in the global task of assuring justice and equality in access to pulmonary hypertension health care.
Topics: Humans; Pulmonary Arterial Hypertension; Healthcare Disparities; Hypertension, Pulmonary
PubMed: 37517834
DOI: 10.1016/j.ccm.2023.03.010 -
Respiratory Medicine Oct 2015Breathlessness is a common symptom in pulmonary hypertension (PH) and an important cause of morbidity. Though this has been attributed to the well described pulmonary... (Review)
Review
Breathlessness is a common symptom in pulmonary hypertension (PH) and an important cause of morbidity. Though this has been attributed to the well described pulmonary vascular abnormalities and subsequent cardiac remodelling, changes in the airways of these patients have also been reported and may contribute to symptoms. Our understanding of these airway abnormalities is poor with conflicting findings in many studies. The present review evaluates these studies for the major PH groups. In addition we describe the role of cardiopulmonary exercise testing in the assessment of pulmonary arterial hypertension (PAH) by evaluating cardiopulmonary interaction during exercise. As yet, the reasons for the abnormalities in lung function are unclear, but potential causes and the possible role of inflammation are discussed. Future research is required to provide a better understanding of this to help improve the management of these patients.
Topics: Exercise Test; Humans; Hypertension, Pulmonary; Predictive Value of Tests; Pulmonary Diffusing Capacity; Respiratory Function Tests
PubMed: 26033642
DOI: 10.1016/j.rmed.2015.05.022 -
Pneumologie (Stuttgart, Germany) Nov 2023Chronic thromboembolic pulmonary disease (CTEPD) is an important late complication of acute pulmonary embolism, in which the thrombi transform into fibrous tissue,...
Chronic thromboembolic pulmonary disease (CTEPD) is an important late complication of acute pulmonary embolism, in which the thrombi transform into fibrous tissue, become integrated into the vessel wall, and lead to chronic obstructions. CTEPD is differentiated into cases without pulmonary hypertension (PH), characterized by a mean pulmonary arterial pressure up to 20 mmHg and a form with PH. Then, it is still referred to as chronic thromboembolic pulmonary hypertension (CTEPH).When there is suspicion of CTEPH, initial diagnostic tests should include echocardiography and ventilation/perfusion scan to detect perfusion defects. Subsequently, referral to a CTEPH center is recommended, where further imaging diagnostics and right heart catheterization are performed to determine the appropriate treatment.Currently, three treatment modalities are available. The treatment of choice is pulmonary endarterectomy (PEA). For non-operable patients or patients with residual PH after PEA, PH-targeted medical therapy, and the interventional procedure of balloon pulmonary angioplasty (BPA) are available. Increasingly, PEA, BPA, and pharmacological therapy are combined in multimodal concepts.Patients require post-treatment follow-up, preferably at (CTE)PH centers. These centers are required to perform a minimum number of PEA surgeries (50/year) and BPA interventions (100/year).
Topics: Humans; Hypertension, Pulmonary; Chronic Disease; Pulmonary Embolism; Lung; Pulmonary Artery
PubMed: 37963483
DOI: 10.1055/a-2145-4807 -
Journal of the American Heart... Jul 2018
Topics: Bosentan; Cardiac Surgical Procedures; Disease Management; Endothelin Receptor Antagonists; Heart Defects, Congenital; Humans; Hypertension, Pulmonary; Vascular Malformations
PubMed: 29973393
DOI: 10.1161/JAHA.118.008587 -
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 -
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 -
Medicina (Kaunas, Lithuania) Dec 2020The interplay between coronavirus disease 2019 (COVID-19) and pulmonary hypertension (PH) in children is unknown. Adults with PH are at potential risk for severe... (Review)
Review
The interplay between coronavirus disease 2019 (COVID-19) and pulmonary hypertension (PH) in children is unknown. Adults with PH are at potential risk for severe complications and high mortality due to associated comorbidities. It is difficult to extrapolate the outcomes of COVID-19 in adults to pediatric PH patients. Overall, a small number of COVID-19 cases is reported in patients with preexisting PH. Several factors may be responsible for the low incidence of COVID-19 in children with PH. Pulmonary hypertension is a rare disease, testing is not universal, and patients may have followed more rigorously the Center for Disease Control's guidelines recommended for personal protection with mask-wearing, social distancing, and hand sanitization through ongoing health education. The small number of COVID-19 cases in patients with preexisting PH does not support that PH is protective for COVID-19. However, medications used to treat PH may have some protection against COVID-19. This review discusses the pathophysiology of PH occurring with COVID-19, differences between children and adults with COVID-19, strategies for management of preexisting PH in children during the ongoing pandemic, and its impact within the field of PH.
Topics: COVID-19; Child; Humans; Hypertension, Pulmonary; Incidence; Pandemics; SARS-CoV-2
PubMed: 33352654
DOI: 10.3390/medicina56120716 -
Journal of the American College of... Nov 2020Chronic thromboembolic pulmonary hypertension (CTEPH) is the result of pulmonary arterial obstruction by organized thrombotic material stemming from incompletely... (Review)
Review
Chronic thromboembolic pulmonary hypertension (CTEPH) is the result of pulmonary arterial obstruction by organized thrombotic material stemming from incompletely resolved acute pulmonary embolism. The exact incidence of CTEPH is unknown but appears to approximate 2.3% among survivors of acute pulmonary embolism. Although ventilation/perfusion scintigraphy has been supplanted by computed tomographic pulmonary angiography in the diagnostic approach to acute pulmonary embolism, it has a major role in the evaluation of patients with suspected CTEPH, the presence of mismatched segmental defects being consistent with the diagnosis. Diagnostic confirmation of CTEPH is provided by digital subtraction pulmonary angiography, preferably performed at a center familiar with the procedure and its interpretation. Operability assessment is then undertaken to determine if the patient is a candidate for potentially curative pulmonary endarterectomy surgery. When pulmonary endarterectomy is not an option, pulmonary arterial hypertension-targeted pharmacotherapy and balloon pulmonary angioplasty represent potential therapeutic alternatives.
Topics: Anticoagulants; Chronic Disease; Computed Tomography Angiography; Humans; Hypertension, Pulmonary; Pulmonary Embolism
PubMed: 33121723
DOI: 10.1016/j.jacc.2020.08.074