CTEPH
MCID: CHR466
MIFTS: 43

Chronic Thromboembolic Pulmonary Hypertension (CTEPH) malady

Respiratory diseases, Rare diseases, Blood diseases categories
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Summaries for Chronic Thromboembolic Pulmonary Hypertension

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47OMIM, 33MalaCards
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MalaCards: Chronic Thromboembolic Pulmonary Hypertension, also known as CTEPH, is related to pulmonary embolism and dysfibrinogenemia. An important gene associated with Chronic Thromboembolic Pulmonary Hypertension is NFKBIL1 (nuclear factor of kappa light polypeptide gene enhancer in B-cells inhibitor-like 1), and among its related pathways are Cell adhesion ECM remodeling and Response to elevated platelet cytosolic Ca2+. The compounds batroxobin and sucralfate have been mentioned in the context of this disorder. Affiliated tissues include heart, lung and endothelial.

Description from OMIM:47 612862

Aliases & Classifications for Chronic Thromboembolic Pulmonary Hypertension

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49Orphanet, 47OMIM, 62UMLS, 59SNOMED-CT via Orphanet, 63UMLS via Orphanet
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Classifications:

Malacards categories (disease lists): (See all malacards categories)
Global: Rare diseases
Anatomical: Respiratory diseases, Blood diseases


Characteristics (Orphanet epidemiological data):

49
chronic thromboembolic pulmonary hypertension:
Inheritance: Sporadic; Prevalence: 1-9/100000; Age of onset: Adulthood


Aliases & Descriptions:

chronic thromboembolic pulmonary hypertension 49 47 62
cteph 49


External Ids:

OMIM47 612862
SNOMED-CT via Orphanet59 233947005
UMLS via Orphanet63 C2363973

Related Diseases for Chronic Thromboembolic Pulmonary Hypertension

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17GeneCards, 18GeneDecks
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Graphical network of the top 20 diseases related to Chronic Thromboembolic Pulmonary Hypertension:



Diseases related to chronic thromboembolic pulmonary hypertension

Symptoms for Chronic Thromboembolic Pulmonary Hypertension

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47OMIM
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Clinical features from OMIM:

612862

Drugs & Therapeutics for Chronic Thromboembolic Pulmonary Hypertension

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42NIH Clinical Center, 6ClinicalTrials
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Drug clinical trials:

Search ClinicalTrials for Chronic Thromboembolic Pulmonary Hypertension

Search NIH Clinical Center for Chronic Thromboembolic Pulmonary Hypertension

Genetic Tests for Chronic Thromboembolic Pulmonary Hypertension

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Anatomical Context for Chronic Thromboembolic Pulmonary Hypertension

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33MalaCards
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MalaCards organs/tissues related to Chronic Thromboembolic Pulmonary Hypertension:

33
Heart, Lung, Endothelial, Brain, Lymph node, Spleen, Smooth muscle, Testes, Monocytes

Animal Models for Chronic Thromboembolic Pulmonary Hypertension or affiliated genes

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Publications for Chronic Thromboembolic Pulmonary Hypertension

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52PubMed
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Articles related to Chronic Thromboembolic Pulmonary Hypertension:

(show top 50)    (show all 377)
idTitleAuthorsYear
1
Recent advances of pulmonary endarterectomy for chronic thromboembolic pulmonary hypertension including Japanese experiences. (24068659)
2014
2
Operability Assessment and Postoperative Outcome in Chronic Thromboembolic Pulmonary Hypertension. (24401572)
2014
3
Dual-energy CT perfusion and angiography in chronic thromboembolic pulmonary hypertension: diagnostic accuracy and concordance with radionuclide scintigraphy. (23982287)
2014
4
Balloon pulmonary angioplasty in patients with inoperable chronic thromboembolic pulmonary hypertension. (23846611)
2013
5
Clinical outcomes of thromboendarterectomy for chronic thromboembolic pulmonary hypertension: 12-year experience. (23423163)
2013
6
Optical coherence tomography is superior to intravascular ultrasound for diagnosis of distal-type chronic thromboembolic pulmonary hypertension. (23386271)
2013
7
Right ventricular longitudinal deformation parameters and exercise capacity : Prognosis of patients with chronic thromboembolic pulmonary hypertension. (23740084)
2013
8
Chronic thromboembolic pulmonary hypertension due to upper-extremity deep vein thrombosis caused by thoracic outlet syndrome. (22138476)
2012
9
Evolving surgical techniques for pulmonary endarterectomy according to the changing features of chronic thromboembolic pulmonary hypertension patients during 17-year single-center experience. (22498087)
2012
10
Characterization of proximal pulmonary arterial cells from chronic thromboembolic pulmonary hypertension patients. (22452949)
2012
11
Chronic thromboembolic pulmonary hypertension with pulmonary infarction occuring in a hypertensive. (21970224)
2011
12
Clinical course of unoperated mild chronic thromboembolic pulmonary hypertension. (21594826)
2011
13
The incidence of recurrent venous thromboembolism and chronic thromboembolic pulmonary hypertension following a first episode of pulmonary embolism. (21743331)
2011
14
Surgical outcome of two difficult cases with predominant proximal pulmonary artery lesions of chronic thromboembolic pulmonary hypertension. (23555449)
2011
15
Recent advances in chronic thromboembolic pulmonary hypertension. (21620730)
2011
16
Chronic thromboembolic pulmonary hypertension (CTEPH): updated Recommendations of the Cologne Consensus Conference 2011. (22221974)
2011
17
Single photon emission computed tomography in chronic thromboembolic pulmonary hypertension. (20920137)
2011
18
Clinical and haemodynamic evaluation of chronic thromboembolic pulmonary hypertension patients scheduled for pulmonary thromboendarterectomy: Is schistosomiasis hypertension an important confounding factor? (21243290)
2010
19
Clinical features and management of chronic thromboembolic pulmonary hypertension in Thai. (20364561)
2010
20
Bosentan for chronic thromboembolic pulmonary hypertension: findings from a systematic review and meta-analysis. (20172593)
2010
21
Long-term outcome after pulmonary endarterectomy for chronic thromboembolic pulmonary hypertension: a single institution experience. (19272789)
2009
22
HLA-DPB1 and NFKBIL1 may confer the susceptibility to chronic thromboembolic pulmonary hypertension in the absence of deep vein thrombosis. (19165231)
2009
23
Role for staphylococci in misguided thrombus resolution of chronic thromboembolic pulmonary hypertension. (18239156)
2008
24
Thromboendarterectomy for severe chronic thromboembolic pulmonary hypertension. (17540993)
2007
25
Long-term bosentan in chronic thromboembolic pulmonary hypertension. (16699254)
2007
26
Chronic thromboembolic pulmonary hypertension - assessment by magnetic resonance imaging. (16838142)
2007
27
Six-minute walk distance as parameter of functional outcome after pulmonary endarterectomy for chronic thromboembolic pulmonary hypertension. (17258590)
2007
28
Effects of pulmonary thromboendarterectomy on right-sided echocardiographic parameters in patients with chronic thromboembolic pulmonary hypertension. (16770978)
2006
29
Assessment of operability in chronic thromboembolic pulmonary hypertension. (16963538)
2006
30
Angiotensin-converting enzyme gene polymorphisms and prognosis in chronic thromboembolic pulmonary hypertension. (16936432)
2006
31
Pulmonary artery-to-pulmonary artery anastomoses: angiographic demonstration in patients with chronic thromboembolic pulmonary hypertension. (16488207)
2006
32
Long-term recovery of exercise ability after pulmonary endarterectomy for chronic thromboembolic pulmonary hypertension. (16996930)
2006
33
The efficacy of bosentan in inoperable chronic thromboembolic pulmonary hypertension: a 1-year follow-up study. (16611652)
2006
34
Bosentan in inoperable chronic thromboembolic pulmonary hypertension. (16061720)
2005
35
Medical conditions increasing the risk of chronic thromboembolic pulmonary hypertension. (15735803)
2005
36
Chronic thromboembolic pulmonary hypertension. (15302364)
2004
37
Prostacyclin therapy before pulmonary thromboendarterectomy in patients with chronic thromboembolic pulmonary hypertension. (12576349)
2003
38
High prevalence of elevated clotting factor VIII in chronic thromboembolic pulmonary hypertension. (12958604)
2003
39
Thrombophilic factors in chronic thromboembolic pulmonary hypertension. (11555695)
2001
40
Chronic thromboembolic pulmonary hypertension. (11590849)
2001
41
Plasma monocyte chemoattractant protein-1 and pulmonary vascular resistance in chronic thromboembolic pulmonary hypertension. (11463608)
2001
42
Three-dimensional visualization of pulmonary thromboemboli in chronic thromboembolic pulmonary hypertension with multiple detector-row spiral computed tomography. (11413092)
2001
43
Surgical treatment of chronic thromboembolic pulmonary hypertension. (10700671)
2000
44
Chronic Thromboembolic Pulmonary Hypertension. (11096519)
2000
45
Surgical treatment for chronic thromboembolic pulmonary hypertension under profound hypothermia and circulatory arrest in 24 patients. (10875595)
1999
46
A successful case of pulmonary thromboendarterectomy for chronic thromboembolic pulmonary hypertension with a thrombus in the right ventricle. (10333425)
1999
47
Mid-term results of pulmonary thromboendarterectomy for chronic thromboembolic pulmonary hypertension. (8651785)
1996
48
Lupus anticoagulant, heparin use, and thrombocytopenia in patients with chronic thromboembolic pulmonary hypertension: a preliminary report. (7573095)
1995
49
Acute pulmonary embolism and chronic thromboembolic pulmonary hypertension: is there a relationship? (2044728)
1991
50
Chronic thromboembolic pulmonary hypertension following ventriculo-atrial shunting. (282858)
1978

Variations for Chronic Thromboembolic Pulmonary Hypertension

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Expression for genes affiliated with Chronic Thromboembolic Pulmonary Hypertension

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2BioGPS, 15Gene Expression Omnibus DataSets
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Expression patterns in normal tissues for genes affiliated with Chronic Thromboembolic Pulmonary Hypertension

Search GEO for disease gene expression data for Chronic Thromboembolic Pulmonary Hypertension.

Pathways for genes affiliated with Chronic Thromboembolic Pulmonary Hypertension

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50PathCards, 60Thomson Reuters, 12EMD Millipore, 55Reactome, 53QIAGEN, 30KEGG, 38NCBI BioSystems Database, 54R&D Systems, 51PharmGKB
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Compounds for genes affiliated with Chronic Thromboembolic Pulmonary Hypertension

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45Novoseek, 11DrugBank, 61Tocris Bioscience, 29IUPHAR, 51PharmGKB, 24HMDB
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Compounds related to Chronic Thromboembolic Pulmonary Hypertension according to GeneCards/GeneDecks:

(show all 20)
idCompoundScoreTop Affiliating Genes
1batroxobin459.7FGB, FGA
2sucralfate45 1110.7FGB, FGA
3rsai459.7FGB, FGA
4hirudin459.6FGB, FGA
5ptca459.5NPPB, FGA
6thallium459.5NPPB, FABP3
7mibg459.4NPPB, FABP3
8dipyridamole45 61 29 1112.4FGA, NPPB
9p00001459.4NPPB, FABP3
10aprotinin45 1110.3FGA, NPPB
11aspirin45 51 29 2412.2FGA, NPPB
12p003459.1NPPB, FABP3
13p002459.0FABP3, NPPB
14norepinephrine45 24 1110.9FABP3, NPPB, FGA
15prostacyclin458.9FGA, NPPB
16lactate458.9FABP3, NPPB, FGA
17nacl458.9NPPB, FGA, FGB
18creatinine458.9FABP3, NPPB, FGA
19heparin45 29 24 1111.7FGB, FGA, NPPB
20tyrosine458.2FABP3, NPPB, FGA, FGB

GO Terms for genes affiliated with Chronic Thromboembolic Pulmonary Hypertension

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16Gene Ontology
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Cellular components related to Chronic Thromboembolic Pulmonary Hypertension according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1fibrinogen complexGO:0055779.5FGB, FGA
2platelet alpha granuleGO:0310919.5FGA, FGB
3platelet alpha granule lumenGO:0310939.5FGA, FGB
4cell cortexGO:0059389.4FGB, FGA
5external side of plasma membraneGO:0098979.4FGA, FGB
6blood microparticleGO:0725629.3FGB, FGA
7extracellular spaceGO:0056158.3NPPB, FGA, FGB

Biological processes related to Chronic Thromboembolic Pulmonary Hypertension according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1protein polymerizationGO:0512589.4FGB, FGA
2platelet activationGO:0301689.4FGB, FGA
3response to calcium ionGO:0515929.3FGB, FGA
4platelet degranulationGO:0025769.1FGB, FGA

Molecular functions related to Chronic Thromboembolic Pulmonary Hypertension according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1protein binding, bridgingGO:0306749.4FGB, FGA
2receptor bindingGO:0051028.3FGB, FGA, NPPB

Products for genes affiliated with Chronic Thromboembolic Pulmonary Hypertension

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Sources for Chronic Thromboembolic Pulmonary Hypertension

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4CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
29IUPHAR
30KEGG
35MeSH
36MESH via Orphanet
37MGI
40NCIt
41NDF-RT
44NINDS
45Novoseek
47OMIM
48OMIM via Orphanet
52PubMed
53QIAGEN
59SNOMED-CT via Orphanet
62UMLS
63UMLS via Orphanet