Plasma Lp(a) Levels Are Increased in Patients with Chronic Thromboembolic Pulmonary Hypertension

1998 ◽  
Vol 80 (08) ◽  
pp. 231-232 ◽  
Author(s):  
M. Ignatescu ◽  
G. Zorn ◽  
M. Kneussl ◽  
G. Maurer ◽  
I. M. Lang ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arteries ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Vein Thrombosis ◽  
Large Size ◽  
Number Of Patients ◽  
Thromboembolic Pulmonary Hypertension ◽  
Pulmonary Arterial ◽  
Genetically Determined ◽  
Deep Vein

SummaryChronic thromboembolic pulmonary hypertension (CTEPH) is a disease resulting from the thromboembolic obstruction of the segmental and/or large size pulmonary arteries, subsequently leading to pulmonary arterial hypertension. Incomplete resolution of acute pulmonary emboli and thrombus organization are believed to be important for the development of the disease. Primary pulmonary hypertension (PPH) is a further disease that at present is poorly understood but shows a clinical picture similar to CTEPH. Since lipoprotein(a) [Lp(a)], a genetically determined risk factor for atherosclerosis and thrombosis, has been found increased in plasma of patients with deep vein thrombosis and pulmonary embolism, we measured plasma Lp(a) levels in 40 patients with CTEPH and 50 patients with PPH and compared them to 50 matched controls. The median for Lp(a) plasma levels was significantly higher in CTEPH patients (26.6 mg/dl) than in PPH patients (9.6 mg/dl) and controls (7.2 mg/dl). Increased plasma Lp(a) could, therefore, play a significant role in the mechanisms of ongoing thrombosis and thrombus organization in CTEPH, while its possible role in PPH can be limited to a small number of patients.

scholarly journals Outpatient specialist clinics for pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension in the Nordic countries

2020 ◽  
Vol 10 (4) ◽  
pp. 204589401989749 ◽  
Author(s):  
Barbro Kjellström ◽  
Henrik Ryftenius ◽  
Lise-Lotte Landenfelt-Gestre ◽  
Bodil Ivarsson
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Complex Interventions ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Nordic Countries ◽  
Multidisciplinary Teams ◽  
Number Of Patients ◽  
Thromboembolic Pulmonary Hypertension ◽  
Pulmonary Arterial

Pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension are rare conditions that require complex interventions by multidisciplinary teams. The European Society of Cardiology (ESC)/the European Respiratory Society (ERS) 2015 guidelines included recommendations for pulmonary hypertension (PH) referral centers including minimum number of patients, staff, facilities, and network. The aim of the present study was to investigate how the PH-specialist centers in the Nordic countries are presently organized. A descriptive, questionnaire was sent to all PH-specialist centers in the Nordic countries in 2018. Sixteen of 20 PH-specialist centers completed the questionnaire. Seven centers (43%) followed less than 50 patients and three centers (19%) followed 125 patients or more. All had a physician or nurse attending or available at the clinic and eight had support staff such as physiotherapists, counsellors, dieticians, or psychologists directly connected to the center. Twelve centers were available by telephone five days or more per week. Nine centers offered a nurse-led outpatient clinic and of those, six had nurses delegated to make protocol-led changes in pulmonary arterial hypertension-specific treatment. Half of the centers had cooperation with a patient organization. All centers except one used international guidelines to guide care and treatment. More than half of the Nordic PH-specialist centers adhered to the ESC/ERS 2015 guidelines recommendations for volumes and staff in 2018, but there is potential for improvement. However, when formulating recommendations of patient volumes in guidelines, the situation for the geographical large but sparsely populated Nordic countries needs to be considered.

NF-κB pathway is involved in CRP-induced effects on pulmonary arterial endothelial cells in chronic thromboembolic pulmonary hypertension

2013 ◽  
Vol 305 (12) ◽  
pp. L934-L942 ◽  
Author(s):  
Marijke Wynants ◽  
Leanda Vengethasamy ◽  
Alicja Ronisz ◽  
Bart Meyns ◽  
Marion Delcroix ◽  
...  
Keyword(s):  
Endothelial Cells ◽  
Pulmonary Hypertension ◽  
Serotonin Receptor ◽  
Molecular Mechanisms ◽  
Inflammatory Marker ◽  
Pulmonary Arteries ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Thromboembolic Pulmonary Hypertension ◽  
Pulmonary Arterial ◽  
Arterial Endothelial Cells

Chronic thromboembolic pulmonary hypertension (CTEPH) is characterized by thrombofibrotic obstruction of proximal pulmonary arteries. The cellular and molecular mechanisms underlying the pathogenesis remain incompletely understood, although we recently evidenced the potential involvement of the inflammatory marker C-reactive protein (CRP). We aimed to investigate the intracellular mechanisms induced by CRP in proximal pulmonary arterial endothelial cells (PAEC). PAEC were isolated from vascular material obtained during pulmonary endarterectomy. RNA was extracted from CRP-stimulated PAEC, and first-stand cDNA was generated. A RT2 profiler PCR Array was used to evaluate the expression of 84 key genes related to NF-κB-mediated signal transduction. CRP-induced NF-κB activation was studied. The effects of pyrrolidine-dithio-carbamate ammonium (PDTC), an inhibitor of the NF-κB pathway, were investigated on CRP-induced adhesion of monocytes to PAEC, adhesion molecule expression, endothelin-1 (ET-1), interleukin-6 (IL-6), and von Willebrand factor (vWF) secretion. Compared with nonstimulated PAEC, serotonin receptor 2B was downregulated by 25%, inhibitor of NF-κB kinase subunit epsilon (IKBKE) by 30%, and toll-like receptor-4 and -6 by 18 and 39%, respectively, in CRP-stimulated PAEC. The transcription factor FOS was threefold upregulated. CRP induced RelA/NF-κBp65 phosphorylation. PDTC dose dependently inhibited the adhesion of monocytes to CRP-stimulated PAEC. PDTC also inhibited the CRP-induced expression of ICAM-1 at the surface of PAEC. PDTC impaired the secretion of ET-1 by 18% and tended to inhibit the secretion of IL-6 by CRP-stimulated PAEC by 46%. PDTC did not inhibit the CRP-induced secretion of vWF. These results suggest an involvement of the NF-κB pathway in mediating different effects of CRP on proximal CTEPH-PAEC.

scholarly journals Medical management of chronic thromboembolic pulmonary hypertension

2017 ◽  
Vol 26 (143) ◽  
pp. 160107 ◽  
Author(s):  
Joanna Pepke-Zaba ◽  
Hossein-Ardeschir Ghofrani ◽  
Marius M. Hoeper
Keyword(s):  
Pulmonary Hypertension ◽  
Medical Therapy ◽  
Soluble Guanylate Cyclase ◽  
Pulmonary Arteries ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Patient Characteristics ◽  
Term Survival ◽  
Thromboembolic Pulmonary Hypertension ◽  
Soluble Guanylate Cyclase Stimulator ◽  
Pulmonary Arterial

Chronic thromboembolic pulmonary hypertension (CTEPH) results from incomplete resolution of acute pulmonary emboli, organised into fibrotic material that obstructs large pulmonary arteries, and distal small-vessel arteriopathy. Pulmonary endarterectomy (PEA) is the treatment of choice for eligible patients with CTEPH; in expert centres, PEA has low in-hospital mortality rates and excellent long-term survival. Supportive medical therapy consists of lifelong anticoagulation plus diuretics and oxygen, as needed.An important recent advance in medical therapy for CTEPH is the arrival of medical therapies for patients with inoperable disease or persistent/recurrent pulmonary hypertension after PEA. The soluble guanylate cyclase stimulator riociguat is licensed for the treatment of CTEPH in patients with inoperable disease or with recurrent/persistent pulmonary hypertension after PEA. Clinical trials of this agent have shown improvements in patients' haemodynamics and exercise capacity. Phosphodiesterase-5 inhibitors, endothelin receptor antagonists and prostanoids have been used in the treatment of CTEPH, but evidence of benefit is limited. Challenges in the future development of medical therapy for CTEPH include better understanding of the underlying pathology, end-points to monitor the condition's progress, and the optimisation of pulmonary arterial hypertension therapies in relation to diverse patient characteristics and emerging options such as balloon pulmonary angioplasty.

Chronic Thromboembolic Pulmonary Hypertension Due to Upper-Extremity Deep Vein Thrombosis Caused Thoracic Outlet Syndrome

2012 ◽  
Vol 48 (2) ◽  
pp. 61-63
Author(s):  
Marta Ferrer Galván ◽  
Luis Jara Palomares ◽  
Candela Caballero Eraso ◽  
José Luis López Villalobos ◽  
Teresa Elías Hernández ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Deep Vein Thrombosis ◽  
Upper Extremity ◽  
Thoracic Outlet Syndrome ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Vein Thrombosis ◽  
Thromboembolic Pulmonary Hypertension ◽  
Deep Vein
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