CTEPH
MCID: CHR466
MIFTS: 43

Chronic Thromboembolic Pulmonary Hypertension (CTEPH) malady

Respiratory diseases, Rare diseases, Blood diseases categories

Summaries for Chronic Thromboembolic Pulmonary Hypertension

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48OMIM, 34MalaCards
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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:48 612862

Aliases & Classifications for Chronic Thromboembolic Pulmonary Hypertension

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50Orphanet, 48OMIM, 63UMLS, 60SNOMED-CT via Orphanet, 64UMLS 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):

50
chronic thromboembolic pulmonary hypertension:
Inheritance: Sporadic; Prevalence: 1-9/100000; Age of onset: Adulthood


Aliases & Descriptions:

chronic thromboembolic pulmonary hypertension 50 48 63
cteph 50


External Ids:

OMIM48 612862
SNOMED-CT via Orphanet60 233947005
UMLS via Orphanet64 C2363973

Related Diseases for Chronic Thromboembolic Pulmonary Hypertension

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18GeneCards, 19GeneDecks
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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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48OMIM
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Clinical features from OMIM:

612862

Drugs & Therapeutics for Chronic Thromboembolic Pulmonary Hypertension

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6CenterWatch, 43NIH Clinical Center, 7ClinicalTrials
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Approved drugs:

Search CenterWatch for Chronic Thromboembolic Pulmonary Hypertension

Drug clinical trials:

Search ClinicalTrials for Chronic Thromboembolic Pulmonary Hypertension

Search NIH Clinical Center for Chronic Thromboembolic Pulmonary Hypertension

Search CenterWatch 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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34MalaCards
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MalaCards organs/tissues related to Chronic Thromboembolic Pulmonary Hypertension:

34
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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53PubMed
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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, 16Gene 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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Sources:
51PathCards, 61Thomson Reuters, 13EMD Millipore, 56Reactome, 54QIAGEN, 31KEGG, 39NCBI BioSystems Database, 55R&D Systems, 52PharmGKB
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Compounds for genes affiliated with Chronic Thromboembolic Pulmonary Hypertension

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46Novoseek, 12DrugBank, 62Tocris Bioscience, 30IUPHAR, 52PharmGKB, 25HMDB
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Compounds related to Chronic Thromboembolic Pulmonary Hypertension according to GeneCards/GeneDecks:

(show all 20)
idCompoundScoreTop Affiliating Genes
1batroxobin469.7FGB, FGA
2sucralfate46 1210.7FGB, FGA
3rsai469.7FGB, FGA
4hirudin469.6FGB, FGA
5ptca469.5NPPB, FGA
6thallium469.5NPPB, FABP3
7mibg469.4NPPB, FABP3
8dipyridamole46 62 30 1212.4FGA, NPPB
9p00001469.4NPPB, FABP3
10aprotinin46 1210.3FGA, NPPB
11aspirin46 52 30 2512.2FGA, NPPB
12p003469.1NPPB, FABP3
13p002469.0FABP3, NPPB
14norepinephrine46 25 1210.9FABP3, NPPB, FGA
15prostacyclin468.9FGA, NPPB
16lactate468.9FABP3, NPPB, FGA
17nacl468.9NPPB, FGA, FGB
18creatinine468.9FABP3, NPPB, FGA
19heparin46 30 25 1211.7FGB, FGA, NPPB
20tyrosine468.2FABP3, NPPB, FGA, FGB

GO Terms for genes affiliated with Chronic Thromboembolic Pulmonary Hypertension

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17Gene 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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  • Antibodies
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  • Lysates
  • Antibodies

Sources for Chronic Thromboembolic Pulmonary Hypertension

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4CDC
14ExPASy
15FMA
23GTR
24HGMD
25HMDB
26ICD10
27ICD10 via Orphanet
28ICD9CM
30IUPHAR
31KEGG
36MeSH
37MESH via Orphanet
38MGI
41NCIt
42NDF-RT
45NINDS
46Novoseek
48OMIM
49OMIM via Orphanet
53PubMed
54QIAGEN
60SNOMED-CT via Orphanet
63UMLS
64UMLS via Orphanet