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Circulation Research Apr 2022Pulmonary hypertension is a complex disease with multiple causes, corresponding to phenotypic heterogeneity and variable therapeutic responses. Advancing understanding... (Review)
Review
Pulmonary hypertension is a complex disease with multiple causes, corresponding to phenotypic heterogeneity and variable therapeutic responses. Advancing understanding of pulmonary hypertension pathogenesis is likely to hinge on integrated methods that leverage data from health records, imaging, novel molecular -omics profiling, and other modalities. In this review, we summarize key data sets generated thus far in the field and describe analytical methods that hold promise for deciphering the molecular mechanisms that underpin pulmonary vascular remodeling, including machine learning, network medicine, and functional genetics. We also detail how genetic and subphenotyping approaches enable earlier diagnosis, refined prognostication, and optimized treatment prediction. We propose strategies that identify functionally important molecular pathways, bolstered by findings across multi-omics platforms, which are well-positioned to individualize drug therapy selection and advance precision medicine in this highly morbid disease.
Topics: Big Data; Humans; Hypertension, Pulmonary; Machine Learning; Precision Medicine
PubMed: 35482840
DOI: 10.1161/CIRCRESAHA.121.319969 -
Hematology. American Society of... Dec 2021Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare complication in pulmonary embolism (PE) survivors, characterized by chronic vascular occlusion and...
Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare complication in pulmonary embolism (PE) survivors, characterized by chronic vascular occlusion and pulmonary hypertension. The identification and diagnosis of CTEPH requires a stepwise approach, starting with symptom evaluation, functional evaluation, screening imaging, and progressing to interventional hemodynamic assessment. On the backbone of anticoagulation, CTEPH management necessitates a multidisciplinary approach. Surgical pulmonary thromboendarterectomy (PTE) is the only potentially curative option. In nonoperable disease or residual disease after PTE, interventional balloon pulmonary angioplasty and/or pulmonary-vasodilator therapies can be offered, in collaboration with interventional and vascular pulmonary colleagues. As it is a disease that can cause high morbidity and mortality, CTEPH requires a high index of suspicion to diagnose and treat in patients following PE.
Topics: Angioplasty, Balloon; Anticoagulants; Chronic Disease; Disease Management; Endarterectomy; Humans; Hypertension, Pulmonary; Male; Middle Aged; Pulmonary Embolism
PubMed: 34889396
DOI: 10.1182/hematology.2021000282 -
Praxis Jan 2023Pulmonary Endarterectomy and Treatment for Chronic Thromboembolic Pulmonary Hypertension Chronic thromboembolic pulmonary hypertension is a relatively rare disease...
Pulmonary Endarterectomy and Treatment for Chronic Thromboembolic Pulmonary Hypertension Chronic thromboembolic pulmonary hypertension is a relatively rare disease which mostly evolves as a complication of acute pulmonary embolism resulting from the fibrotic organization of residual thrombotic material despite adequate anticoagulation leading to precapillary pulmonary hypertension and persistence of its symptoms. The elevated pulmonary vascular resistance leads to right ventricular heart failure, its symptoms and reduced prognosis. The therapy of choice is the pulmonary endarterectomy, which leads to a reduction of symptoms, optimization of the hemodynamics and improved prognosis. Misdiagnosis and delayed referral often lead to disease progression along with poor surgical outcome. In case of more distal, surgically non-accessible disease, treatment consists of balloon pulmonary angioplasty and pulmonary vasodilator drugs.
Topics: Humans; Hypertension, Pulmonary; Pulmonary Artery; Chronic Disease; Pulmonary Embolism; Endarterectomy
PubMed: 36597683
DOI: 10.1024/1661-8157/a003964 -
Physiological Research 2012Hypoxic pulmonary hypertension (HPH) is a syndrome characterized by the increase of pulmonary vascular tone and the structural remodeling of peripheral pulmonary... (Review)
Review
Hypoxic pulmonary hypertension (HPH) is a syndrome characterized by the increase of pulmonary vascular tone and the structural remodeling of peripheral pulmonary arteries. Mast cells have an important role in many inflammatory diseases and they are also involved in tissue remodeling. Tissue hypoxia is associated with mast cell activation and the release of proteolytic enzymes, angiogenic and growth factors which mediate tissue destruction and remodeling in a variety of physiological and pathological conditions. Here we focused on the role of mast cells in the pathogenesis of hypoxic pulmonary hypertension from the past to the present.
Topics: Animals; Cell Hypoxia; Humans; Hypertension, Pulmonary; Mast Cells
PubMed: 22188111
DOI: 10.33549/physiolres.932221 -
The European Respiratory Journal Nov 2010
Topics: Algorithms; Familial Primary Pulmonary Hypertension; Humans; Hypertension, Pulmonary; Terminology as Topic
PubMed: 21037366
DOI: 10.1183/09031936.00038410 -
European Respiratory Review : An... Mar 20132011 to 2012 has seen an explosion in published research in the field of pulmonary vascular disease, especially pulmonary hypertension. In conjunction with this research... (Review)
Review
2011 to 2012 has seen an explosion in published research in the field of pulmonary vascular disease, especially pulmonary hypertension. In conjunction with this research has been an explosion in clinical interest in treating pulmonary hypertension. This is possible because we now have three different generic classes of drug therapy: endothelin receptor antagonists, phosphodiesterase-5 inhibitors and prostacyclins. Clearly, however, we need to be careful that we are treating the correct disease with the correct drug and measuring the correct response. Herein, I will review the papers published over the last year that offer the most insight into the pathobiology, but also those that give us the clinical information we need in epidemiology, treatment and end-points so that we can treat this devastating group of disease.
Topics: Antihypertensive Agents; Blood Pressure; Evidence-Based Medicine; Humans; Hypertension, Pulmonary; Registries; Risk Factors; Treatment Outcome
PubMed: 23457160
DOI: 10.1183/09059180.00006912 -
Current Rheumatology Reports Feb 2010Patients with systemic sclerosis (SSc) can develop pulmonary hypertension (PH; mean pulmonary artery pressure >/= 25 mm Hg) caused by pulmonary arterial hypertension... (Review)
Review
Patients with systemic sclerosis (SSc) can develop pulmonary hypertension (PH; mean pulmonary artery pressure >/= 25 mm Hg) caused by pulmonary arterial hypertension (PAH), left ventricular disease, or pulmonary fibrosis. PAH is a pulmonary vascular disease, the diagnosis of which requires pulmonary capillary wedge pressure less than 15 mm Hg, pulmonary vascular resistance greater than 3 Wood Units, and exclusion of thromboembolism and parenchymal lung disease. Molecular mechanisms underlying PAH-SSc include activation of inflammatory and fibrogenic pathways in the vasculature and right ventricle. Circulating autoantibodies trigger endothelial damage and fibroblast activation. PAH most commonly occurs as a late complication in patients with limited cutaneous disease and anticentromere antibodies. Although echocardiography is a useful screening tool, heart catheterization is required to diagnose PAH before initiating therapy. Prognosis and therapeutic response are worse in PAH-SSc than in other PAH categories (median survival, 1-3 y). Approved therapies include prostacyclins, endothelin antagonists, and phosphodiesterase type 5 inhibitors. Research is needed to define disease mechanisms and develop effective therapies.
Topics: Antihypertensive Agents; Exercise Test; Humans; Hypertension, Pulmonary; Scleroderma, Systemic; Ultrasonography
PubMed: 20425528
DOI: 10.1007/s11926-009-0078-1 -
Annals of the Academy of Medicine,... May 2020Chronic thromboembolic pulmonary hypertension (CTEPH) is a known sequela after acute pulmonary embolism (PE). It is a debilitating disease, and potentially fatal if left... (Review)
Review
INTRODUCTION
Chronic thromboembolic pulmonary hypertension (CTEPH) is a known sequela after acute pulmonary embolism (PE). It is a debilitating disease, and potentially fatal if left untreated. This review provides a clinically relevant overview of the disease and discusses the usefulness and limitations of the various investigational and treatment options.
METHODS
A PubMed search on articles relevant to PE, pulmonary hypertension, CTEPH, pulmonary endarterectomy, and balloon pulmonary angioplasty were performed. A total of 68 articles were found to be relevant and were reviewed.
RESULTS
CTEPH occurs as a result of non-resolution of thrombotic material, with subsequent fibrosis and scarring of the pulmonary arteries. Risk factors have been identified, but the underlying mechanisms have yet to be fully elucidated. The cardinal symptom of CTEPH is dyspnoea on exertion, but the diagnosis is often challenging due to lack of awareness. The ventilation/perfusion scan is recommended for screening for CTEPH, with other modalities (eg. dual energy computed tomography pulmonary angiography) also being utilised in expert centres. Conventional pulmonary angiography with right heart catheterisation is important in the final diagnosis of CTEPH.
CONCLUSION
Operability assessment by a multidisciplinary team is crucial for the management of CTEPH, as pulmonary endarterectomy (PEA) remains the guideline recommended treatment and has the best chance of cure. For inoperable patients or those with residual disease post-PEA, medical therapy or balloon pulmonary angioplasty are potential treatment options.
Topics: Chronic Disease; Endarterectomy; Humans; Hypertension, Pulmonary; Pulmonary Artery; Pulmonary Embolism
PubMed: 32582909
DOI: No ID Found -
Autoimmunity Reviews Apr 2024Pre-capillary pulmonary arterial hypertension (PAH) is hemodynamically characterized by a mean pulmonary arterial pressure (mPAP) ≥ 20 mmHg, pulmonary capillary... (Review)
Review
Pre-capillary pulmonary arterial hypertension (PAH) is hemodynamically characterized by a mean pulmonary arterial pressure (mPAP) ≥ 20 mmHg, pulmonary capillary wedge pressure (PAWP) ≤15 mmHg and pulmonary vascular resistance (PVR) > 2. PAH is classified in six clinical subgroups, including idiopathic PAH (IPAH) and PAH associated to connective tissue diseases (CTD-PAH), that will be the main object of this review. The aim is to compare these two PAH subgroups in terms of epidemiology, histological and pathogenic findings in an attempt to define disease-specific features, including autoimmunity, that may explain the heterogeneity of response to therapy between IPAH and CTD-PAH.
Topics: Humans; Connective Tissue Diseases; Autoimmunity; Pulmonary Arterial Hypertension; Hypertension, Pulmonary; Familial Primary Pulmonary Hypertension
PubMed: 38181859
DOI: 10.1016/j.autrev.2024.103514 -
Journal of the American College of... Dec 2013Since the last World Symposium on Pulmonary Hypertension in 2008, we have witnessed numerous and exciting developments in chronic thromboembolic pulmonary hypertension... (Review)
Review
Since the last World Symposium on Pulmonary Hypertension in 2008, we have witnessed numerous and exciting developments in chronic thromboembolic pulmonary hypertension (CTEPH). Emerging clinical data and advances in technology have led to reinforcing and updated guidance on diagnostic approaches to pulmonary hypertension, guidelines that we hope will lead to better recognition and more timely diagnosis of CTEPH. We have new data on treatment practices across international boundaries as well as long-term outcomes for CTEPH patients treated with or without pulmonary endarterectomy. Furthermore, we have expanded data on alternative treatment options for select CTEPH patients, including data from multiple clinical trials of medical therapy, including 1 recent pivotal trial, and compelling case series of percutaneous pulmonary angioplasty. Lastly, we have garnered more experience, and on a larger international scale, with pulmonary endarterectomy, which is the treatment of choice for operable CTEPH. This report overviews and highlights these important interval developments as deliberated among our task force of CTEPH experts and presented at the 2013 World Symposium on Pulmonary Hypertension in Nice, France.
Topics: Animals; Cardiac Catheterization; Chronic Disease; Humans; Hypertension, Pulmonary; Pulmonary Embolism
PubMed: 24355646
DOI: 10.1016/j.jacc.2013.10.024