scholarly journals Pulmonary hypertension in patients with chronic myeloproliferative disorders

2015 ◽  
Vol 24 (137) ◽  
pp. 400-410 ◽  
Author(s):  
Yochai Adir ◽  
Davide Elia ◽  
Sergio Harari
Keyword(s):  
Pulmonary Hypertension ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Major Complication ◽  
Myeloproliferative Disorders ◽  
Clinical Classification ◽  
Drug Induced ◽  
Myeloproliferative Diseases ◽  
Thromboembolic Pulmonary Hypertension ◽  
Haematological Disorders ◽  
Chronic Myeloproliferative Diseases

Pulmonary hypertension (PH) is a major complication of several haematological disorders. Chronic myeloproliferative diseases (CMPDs) associated with pulmonary hypertension have been included in group five of the clinical classification for pulmonary hypertension, corresponding to pulmonary hypertension for which the aetiology is unclear and/or multifactorial. The aim of this review is to discuss the epidemiology, pathogenic mechanism and treatment approaches of the more common forms of pulmonary hypertension in the context of CMPD's: chronic thromboembolic pulmonary hypertension, precapillary pulmonary hypertension and drug-induced PH.

scholarly journals Myeloproliferative Diseases as Possible Risk Factor for Development of Chronic Thromboembolic Pulmonary Hypertension—A Genetic Study

2020 ◽  
Vol 21 (9) ◽  
pp. 3339
Author(s):  
Christina A. Eichstaedt ◽  
Jeremias Verweyen ◽  
Michael Halank ◽  
Nicola Benjamin ◽  
Christine Fischer ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Risk Factor ◽  
Genetic Predisposition ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Janus Kinase ◽  
Additional Risk Factor ◽  
Myeloproliferative Disease ◽  
Pathogenic Variants ◽  
Myeloproliferative Diseases ◽  
Thromboembolic Pulmonary Hypertension

Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare disease which is often caused by recurrent emboli. These are also frequently found in patients with myeloproliferative diseases. While myeloproliferative diseases can be caused by gene defects, the genetic predisposition to CTEPH is largely unexplored. Therefore, the objective of this study was to analyse these genes and further genes involved in pulmonary hypertension in CTEPH patients. A systematic screening was conducted for pathogenic variants using a gene panel based on next generation sequencing. CTEPH was diagnosed according to current guidelines. In this study, out of 40 CTEPH patients 4 (10%) carried pathogenic variants. One patient had a nonsense variant (c.2071A>T p.Lys691*) in the BMPR2 gene and three further patients carried the same pathogenic variant (missense variant, c.1849G>T p.Val617Phe) in the Janus kinase 2 (JAK2) gene. The latter led to a myeloproliferative disease in each patient. The prevalence of this JAK2 variant was significantly higher than expected (p < 0.0001). CTEPH patients may have a genetic predisposition more often than previously thought. The predisposition for myeloproliferative diseases could be an additional risk factor for CTEPH development. Thus, clinical screening for myeloproliferative diseases and genetic testing may be considered also for CTEPH patients.

Pulmonary hypertension: clinical classification

ESC CardioMed ◽  
2018 ◽  
pp. 2500-2502
Author(s):  
Francisca A. Gavilanes Oleas ◽  
Caio J. C. Fernandes ◽  
Rogério Souza
Keyword(s):  
Pulmonary Hypertension ◽  
Management Strategies ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Pulmonary Vascular Disease ◽  
Left Heart ◽  
Clinical Classification ◽  
Current Classification ◽  
Thromboembolic Pulmonary Hypertension ◽  
Pulmonary Arterial ◽  
Clinical Conditions

Pulmonary hypertension is a clinical and haemodynamic condition associated with several baseline clinical conditions or existing as a pure pulmonary vascular disease. The current classification groups aetiologies of pulmonary hypertension that share similarities in terms of clinical presentation, pathological findings, and haemodynamic profiles, in an attempt to guide management strategies in these distinct conditions. As a consequence, pulmonary hypertension is classified into five different groups: pulmonary arterial hypertension, pulmonary hypertension due to left heart disease, pulmonary hypertension due to lung disease and/or hypoxia, chronic thromboembolic pulmonary hypertension, and pulmonary hypertension with unclear multifactorial mechanisms. This chapter reviews the rational supporting the current classification system alluding also to the existing evidence for specific therapies in each one of the groups.

IP-10 and IL-6 as biomarkers in Chronic Thromboembolic Pulmonary Hypertension

Pneumologie ◽  
2013 ◽  
Vol 67 (05) ◽  
Author(s):  
D Zabini ◽  
Z Bálint ◽  
C Nagarai ◽  
V Foris ◽  
G Kwapiszewska ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Thromboembolic Pulmonary Hypertension

Pulmonary Endarterectomy: Assessment of Operability, Surgical Description, and Post-op Care

2014 ◽  
Vol 12 (4) ◽  
pp. 186-192 ◽  
Author(s):  
David Poch ◽  
Victor Pretorius
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Artery ◽  
Pulmonary Artery Pressure ◽  
Pulmonary Arteries ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Pulmonary Artery Wedge Pressure ◽  
Pulmonary Endarterectomy ◽  
Pulmonary Thromboendarterectomy ◽  
Thromboembolic Pulmonary Hypertension ◽  
Wedge Pressure

Chronic thromboembolic pulmonary hypertension (CTEPH) is defined as a mean pulmonary artery pressure ≥25 mm Hg and pulmonary artery wedge pressure ≤15 mm Hg in the presence of occlusive thrombi within the pulmonary arteries. Surgical pulmonary thromboendarterectomy (PTE) is considered the best treatment option for CTEPH.

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