CTEPH
MCID: CHR466
MIFTS: 39

Chronic Thromboembolic Pulmonary Hypertension (CTEPH) malady

Categories: Rare diseases, Blood diseases

Aliases & Classifications for Chronic Thromboembolic Pulmonary Hypertension

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Aliases & Descriptions for Chronic Thromboembolic Pulmonary Hypertension:

Name: Chronic Thromboembolic Pulmonary Hypertension 48 68
 
Cteph 48

Classifications:



Summaries for Chronic Thromboembolic Pulmonary Hypertension

About this section
NIH Rare Diseases:48 Chronic thromboembolic pulmonary hypertension (cteph) is a unique pulmonary vascular disease caused by chronic block of the major lung arteries. signs and symptoms commonly include progressive breathing difficulties (dyspnea) on exertion, fatigue, palpitations, loss of consciousness (syncope), or swelling (edema). the disease may appear a few months or many years after the sudden blockage in a lung artery by a blood clot (acute pulmonary embolism). however, up to 60% of patients have no history of acute pulmonary embolism. some people with this disease may have clotting problems. research suggests there may be a genetic predisposition leading to abnormal vascular healing after pulmonary embolism in susceptible individuals, but no specific gene mutations have been identified in cteph. curative treatment is done with pulmonary endarterectomy (a surgery that removes the blood clots in the lungs), and more recently by pulmonary balloon angioplasty (a procedure that opens up the vessels of the lung and increases blood flow).  last updated: 10/18/2016

MalaCards based summary: Chronic Thromboembolic Pulmonary Hypertension, also known as CTEPH, is related to artery disease and pulmonary hypertension, chronic thromboembolic, without deep vein thrombosis. An important gene associated with Chronic Thromboembolic Pulmonary Hypertension is NPPB (Natriuretic Peptide B), and among its related pathways are Cardiomyocyte Differentiation through BMP Receptors and Cell adhesion_Plasmin signaling. Affiliated tissues include lung, endothelial and heart, and related mouse phenotype cardiovascular system.

Related Diseases for Chronic Thromboembolic Pulmonary Hypertension

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Diseases related to Chronic Thromboembolic Pulmonary Hypertension via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50)    (show all 87)
idRelated DiseaseScoreTop Affiliating Genes
1artery disease29.5CRP, F2
2pulmonary hypertension, chronic thromboembolic, without deep vein thrombosis11.3
3pulmonary hypertension11.1
4pulmonary embolism10.4
5unilateral multicystic dysplastic kidney10.2CRP, NPPB
6ectodermal dysplasia 9, hair/nail type10.2EDN1, NPPB
7joint disorders10.2CRP, NPPB
8eastern equine encephalitis10.2EDN1, NPPB
9secretory diarrhea myopathy and deafness10.2CRP, FGA
10toxic myocarditis10.2CRP, NPPB
11iida kannari syndrome10.2EDN1, NPPB
12petrositis10.1EDN1, NPPB
13lymphadenitis10.1EDN1, NPPB
14enamel hypoplasia cataract hydrocephaly10.1BMPR2, EDN1
15hematocele of tunica vaginalis testis10.1BMPR2, EDN1
16afibrinogenemia, congenital10.1FGA, FGB
17thrombosis10.1
18endotheliitis10.1
19pancreas lymphoma10.1CRP, NPPB
20hemorrhoid10.1CRP, EDN1
21urinary bladder posterior wall cancer10.1CRP, NPPB
22deafness, autosomal recessive 7910.1CRP, NPPB
23autonomic neuropathy10.1F2, NPPB
24acute closed-angle glaucoma10.0EDN1, NPPB
25acute infection of pinna10.0CRP, F2
26synostosis10.0CRP, F2
27midline cystocele10.0CRP, F2
28houlston ironton temple syndrome10.0CRP, F2
29myopia10.0EDN1, F2
30diabetic encephalopathy10.0CRP, EDN1, NPPB
31adult t-cell leukemia10.0CRP, EDN1, NPPB
32intestinal disaccharidase deficiency10.0CRP, F2
33retinal arterial macroaneurysm with supravalvular pulmonic stenosis10.0CRP, F2
34ceroid lipofuscinosis, neuronal, 810.0CRP, EDN1, NPPB
35sarcoma10.0
36antiphospholipid syndrome10.0
37villous adenoma10.0CRP, F2
38macular degeneration, age-related, 1210.0CRP, EDN1, NPPB
39xerophthalmia10.0CRP, F2
40alpha-2-plasmin inhibitor deficiency10.0EDN1, F2
41pregnancy loss, recurrent 19.9F2, FGA
42essential thrombocythemia9.9F2, FGB
43autoimmune disease of central nervous system9.9CRP, F2
44capillary hemangioma9.9EDN1, F2
45ischemic optic neuropathy9.9CRP, F2
46immunodeficiency 219.9F2, FGA
47atrophy of prostate9.9CRP, F2
48benign deep fibrous histiocytoma9.9F2, FGB
49dysfibrinogenemia9.9
50brill-zinsser disease9.9F2, FGA

Graphical network of the top 20 diseases related to Chronic Thromboembolic Pulmonary Hypertension:



Diseases related to chronic thromboembolic pulmonary hypertension

Symptoms & Phenotypes for Chronic Thromboembolic Pulmonary Hypertension

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MGI Mouse Phenotypes related to Chronic Thromboembolic Pulmonary Hypertension according to GeneCards Suite gene sharing:

41
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053857.1BMPR2, CRP, EDN1, F2, FGA, FGB

Drugs & Therapeutics for Chronic Thromboembolic Pulmonary Hypertension

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FDA approved drugs:

id Drug Name Active Ingredient(s)16 Company Approval Date
1
Adempas16 RIOCIGUAT Bayer Healthcare Pharmaceuticals October 2013
FDA Label: Adempas
Disease/s that Drug Treats:Chronic Thromboembolic Pulmonary Hypertension and Pulmonary Arterial Hypertension
Indications and Usage:16 Adempas is a soluble guanylate cyclase (sGC) stimulator indicated for the treatment of adults with: * Persistent/recurrent Chronic Thromboembolic Pulmonary Hypertension (CTEPH) (WHO Group 4) after surgical treatment or inoperable CTEPH to improve exercise capacity and WHO functional class. (1.1) * Pulmonary Arterial Hypertension (PAH) (WHO Group 1) to improve exercise capacity, improve WHO functional class and to delay clinical worsening. (1.2)
DrugBank Targets: -
Mechanism of Action:16 
Target: soluble guanylate cyclase (sGC)
Action: stimulator
FDA: Riociguat is a stimulator of soluble guanylate cyclase (sGC), an enzyme in the cardiopulmonary system and the receptor for nitric oxide (NO). When NO binds to sGC, the enzyme catalyzes synthesis of the signaling molecule cyclic guanosine monophosphate (cGMP). Intracellular cGMP plays an important role in regulating processes that influence vascular tone, proliferation, fibrosis and inflammation.Pulmonary hypertension is associated with endothelial dysfunction, impaired synthesis of nitric oxide and insufficient stimulation of the NO-sGC-cGMP pathway. Riociguat has a dual mode of action. It sensitizes sGC to endogenous NO by stabilizing the NO-sGC binding. Riociguat also directly stimulates sGC via a different binding site, independently of NO. Riociguat stimulates the NO-sGC-cGMP pathway and leads to increased generation of cGMP with subsequent vasodilation. The active metabolite (M1) of riociguat is 1/3 to 1/10 as potent as riociguat.

Drugs for Chronic Thromboembolic Pulmonary Hypertension (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 40)
idNameStatusPhaseClinical TrialsCas NumberPubChem Id
1
Bosentanapproved, investigationalPhase 3, Phase 1, Phase 2104147536-97-8104865
Synonyms:
-5-(o-methoxyphenoxy)-2-(2-pyrimidinyl)-4-pyrimidinyl]benzenesulfonamide
147536-97-8
174227-18-0
4-(1,1-Dimethylethyl)-N-(6-(2-hydroxyethoxy)-5-(2-methoxyphenoxy)-(2,2'-bipyrimidin)-4-yl) benzenesulfornamide
4-(1,1-Dimethylethyl)-N-(6-(2-hydroxyethoxy)-5-(2-methoxyphenoxy)-(2,2'-bipyrimidin)-4-yl)benzenesulfornamide
4-t-butyl-N-(6-(2-hydroxyethoxy)-5-(2-methoxyphenoxy)-2,2'-bipyrimidin-4-yl)benzenesulfonamide
4-tert-butyl-N-[6-(2-hydroxyethoxy)-5-(2-methoxyphenoxy)-2,2'-bipyrimidin-4-yl]benzenesulfonamide
4-tert-butyl-N-[6-(2-hydroxyethoxy)-5-(2-methoxyphenoxy)-2-pyrimidin-2-ylpyrimidin-4-yl]benzenesulfonamide
AC-148
AC1L2XN2
Actelion
Bosentan
Bosentan (INN)
Bosentan [USAN:INN:BAN]
Bosentan hydrate
C27H29N5O6S
CHEBI:51450
CHEMBL957
CID104865
D07538
DB00559
 
EN002863
HMS2090N14
I06-1873
KS-5062
L001086
LS-31532
MolPort-003-845-300
NCGC00167440-01
PDSP1_001731
PDSP2_001714
Ro 47-0203
Ro 47-0203/039
Ro-47-0203
Ro-47-0203/029
Ro-47-0203/039
TL8001039
Tracleer
UNII-XUL93R30K2
bosentan
bosentanum
bosentán
p-tert-Butyl-N-(6-(2-hydroxyethoxy)-5-(o-methoxyphenoxy)-2-(2-pyrimidinyl)-4-pyrimidinyl)benzenesulfonamide
p-tert-Butyl-N-[6-(2-hydroxyethoxy)
2
Treprostinilapproved, investigationalPhase 39181846-19-754786, 6918140
Synonyms:
((1R,2R,3aS,9aS)-2-hydroxy-1-((3S)-3-hydroxyoctyl)-2,3,3a,4,9,9a-hexahydro-1H-cylopent(b)naphthalen-5-yl)oxy)acetate
(1R,2R,3aS,9aS)-[[2,3,3a,4,9,9a-hexahydro-2-hydroxy- 1-[(3S)-3-hydroxyoctyl]-1H-benz[f]inden-5-yl] oxy]acetic acid
({(1R,2R,3aS,9aS)-2-hydroxy-1-[(3S)-3-hydroxyoctyl]-2,3,3a,4,9,9a-hexahydro-1H-cyclopenta[b]naphthalen-5-yl}oxy)acetic acid
15 AU81
15AU81
2-[[(1R,2R,3aS,9aS)-2-hydroxy-1-[(3S)-3-hydroxyoctyl]-2,3,3a,4,9,9a-hexahydro-1H-cyclopenta[g]naphthalen-5-yl]oxy]acetic acid
2-[[(1S,2S,3aR,9aR)-2-hydroxy-1-[(3R)-3-hydroxyoctyl]-2,3,3a,4,9,9a-hexahydro-1H-cyclopenta[g]naphthalen-5-yl]oxy]acetic acid
289480-64-4 (Sodium salt)
81846-19-7
AC1L1HPX
AC1OCF2J
CHEBI:50861
CID11246284
CID54786
CID6918140
CID9800580
D06213
DB00374
L001079
 
LRX 15
LS-177654
LS-187014
LS-187645
Remodulin (TN)
Treprostinil
Treprostinil (USAN/INN)
Treprostinil [USAN:INN]
Treprostinil sodium
Treprostinilo
Treprostinilum
U 62840
U-62,840
UNII-RUM6K67ESG
UT-15
Uniprost
Viveta
[[(1R,2R,3aS,9aS)-2-Hydroxy-1-[(3S)-3-hydroxyoctyl]-2,3,3a,4,9,9a-hexahydro-1H-cyclopenta[b]naphtalen-5-yl]oxy]acetic acid
treprostinilo
treprostinilum
3Antihypertensive AgentsPhase 3, Phase 2, Phase 14207
4Endothelin Receptor AntagonistsPhase 3, Phase 2, Phase 1218
5CalamusNutraceuticalPhase 3459
6
MacitentanapprovedPhase 236441798-33-0
Synonyms:
 
Macitentanum
7Protective AgentsPhase 27443
8Peripheral Nervous System AgentsPhase 223689
9Vasodilator AgentsPhase 23543
10Bronchodilator AgentsPhase 22965
11Respiratory System AgentsPhase 24997
12RadiopharmaceuticalsPhase 2, Phase 1514
13Endothelin A Receptor AntagonistsPhase 240
14Fluorodeoxyglucose F18Phase 2, Phase 1412
15adrenomedullinPhase 217
16Autonomic AgentsPhase 210150
17Anti-Asthmatic AgentsPhase 23488
18DeoxyglucosePhase 1, Phase 2123
19Antiviral AgentsPhase 1, Phase 29967
20AntimetabolitesPhase 1, Phase 212054
21Anti-Infective AgentsPhase 1, Phase 222062
22
Rivaroxabanapproved259366789-02-8
Synonyms:
 
Xarelto
bay 59-7939
23
Warfarinapproved47081-81-26691, 54678486
Synonyms:
(-)-Warfarin
(S)-4-hydroxy-3-(3-oxo-1-phenylbutyl)-2-benzopyrone
(S)-Warfarin
(phenyl-1 acetyl-2 ethyl) 3-hydroxy-4 coumarin
(phenyl-1 acetyl-2 ethyl) 3-hydroxy-4 coumarine
1-(4'-Hydroxy-3'-coumarinyl)-1-phenyl-3-butanone
200 Coumarin
200 coumarin
3-(1'-Phenyl-2'-acetylethyl)-4-hydroxycoumarin
3-(Acetonylbenzyl)-4-hydroxycoumarin
3-(alpha-Acetonylbenzyl)-4-hydroxycoumarin
3-(alpha-Phenyl-beta-acetylaethyl)-4-hydroxycumarin
3-(alpha-Phenyl-beta-acetylethyl)-4-hydroxycoumarin
4-Hydroxy-3- (3-oxo-1-fenyl-butyl) cumarine
4-Hydroxy-3- (3-oxo-1-phenyl-butyl)-cumarin
4-Hydroxy-3-(3-oxo-1-fenyl-butyl) cumarine
4-Hydroxy-3-(3-oxo-1-phenyl-butyl)-cumarin
4-Hydroxy-3-(3-oxo-1-phenylbutyl)-2H-1-benzopyran-2-one
4-Hydroxy-3-(3-oxo-1-phenylbutyl)-2H-chromen-2-one
4-Hydroxy-3-(3-oxo-1-phenylbutyl)coumarin
4-Idrossi-3- (3-oxo-)-fenil-butil)-cumarine
4-Idrossi-3-(3-oxo-)-fenil-butil)-cumarine
4-Idrossi-3-(3-oxo-1-fenil-butil)-cumarine
45706_FLUKA
45706_RIEDEL
4oh-coumarin deriv.
5543-56-6
5543-57-7
81-81-2
A2250_FLUKA
A2250_SIGMA
AC-15978
AC1L1N37
AC1Q6AN3
AR-1E2310
Arab Rat Death
Arab rat death
Arab rat deth
Athrombin
Athrombin-K
Athrombin-k
Athrombine-K
Athrombine-k
BRD-A24514565-001-02-2
BRD-A24514565-236-01-6
BRN 1293536
BSPBio_002026
Bio-0586
Brumolin
C01541
C19H16O4
CBDivE_002127
CBDivE_013236
CBKinase1_000192
CBKinase1_012592
CHEBI:10033
CHEMBL1464
CID6691
CO-Rax
Caswell No. 903
Choice
Choice (TN)
Co-Rax
Co-rax
Compound 42
Coumadin
Coumadin Tabs
Coumafen
Coumafene
Coumafene [French]
Coumaphen
Coumaphene
Coumarins
Coumefene
Cov-R-Tox
Cov-R-tox
D-Con
D08682
DB00682
DL-3-(alpha-acetonylbenzyl)-4-hydroxycoumarin
Dethmor
Dethnel
Dicusat E
Dicusat e
DivK1c_000444
EINECS 201-377-6
EINECS 226-907-3
EPA Pesticide Chemical Code 086002
Eastern states duocide
Fasco fascrat powder
Frass-Ratron
Frass-ratron
HMS1921K07
HMS2092C20
HMS501G06
HSDB 1786
I14-0958
IDI1_000444
Jantoven
KBio1_000444
KBio2_000674
KBio2_003242
KBio2_005810
KBio3_001526
KBioGR_000901
KBioSS_000674
Killgerm sewarin p
Kumader
Kumadu
Kumatox
Kypfarin
LS-55083
Latka 42
Latka 42 [Czech]
Liqua-tox
MLS001304042
Maag Rattentod Cum
Maag rattentod cum
Mar-Frin
Mar-frin
Marevan
Martin'S mar-frin
Martin's mar-frin
 
Maveran
MolPort-001-787-822
Mouse pak
NCGC00094810-01
NCGC00094810-02
NCGC00159398-03
NCGC00178855-01
NINDS_000444
NSC 59813
NSC59813
PS104_SUPELCO
Panwarfin
Place-Pax
Place-pax
Prothromadin
RAX
RCR grey squirrel killer concentrate
RCRA waste no. P001
Rat & mice bait
Rat and mice bait
Rat-B-gon
Rat-Gard
Rat-Kill
Rat-Mix
Rat-O-cide #2
Rat-O-cide no. 2
Rat-Ola
Rat-Trol
Rat-a-way
Rat-alpha-way
Rat-b-gon
Rat-beta-gon
Rat-gard
Rat-kill
Rat-mix
Rat-o-cide #2
Rat-ola
Rat-trol
Ratorex
Ratox
Ratoxin
Ratron
Ratron G
Ratron g
Rats-no-more
Ratten-Koederrohr
Ratten-koederrohr
Rattenstreupulver Neu Schacht
Rattenstreupulver neu schacht
Rattenstreupulver new schacht
Rattentraenke
Rattunal
Rcra waste number P001
Ro-Deth
Ro-deth
Rodafarin
Rodafarin C
Rodafarin c
Rodex
Rodex blox
Rosex
Rough & ready mouse mix
Rough and ready mouse mix
SMP1_000316
SMR000718754
SPBio_000870
SPECTRUM1500613
STK364490
Sakarat
Sewarin
Sofarin
Solfarin
Sorexa Plus
Sorexa plus
Spectrum2_000935
Spectrum3_000583
Spectrum4_000381
Spectrum5_001751
Spectrum_000194
Spray-trol brand roden-trol
Temus W
Temus w
Tintorane
Tox-Hid
Tox-hid
Twin light rat away
UNII-5Q7ZVV76EI
Vampirinip II
Vampirinip III
Vampirinip iii
W.A.R.F. 42
WARF compound 42
WLN: T66 BOVJ DYR&1V1 EQ
Waran
Warf 10
Warf 42
Warfarat
Warfarin (INN)
Warfarin (and salts of)
Warfarin Plus
Warfarin Q
Warfarin [BSI:ISO]
Warfarin [INN:BAN]
Warfarin plus
Warfarin plus [discontinued]
Warfarin sodium
Warfarin titrated to an INR of 2.5-3.0
Warfarin(R)
Warfarina
Warfarina [INN-Spanish]
Warfarine
Warfarine [INN-French]
Warfarine [ISO-French]
Warfarinum
Warfarinum [INN-Latin]
Warficide
Warfilone
Zoocoumarin
Zoocoumarin [Netherlands and USSR]
Zoocoumarin [Russian]
delta-Con
nchembio.307-comp19
rac-Warfarin
warfarin
24
Iloprostapproved, investigational7978919-13-86443959
Synonyms:
(16R,S)-Methyl-18,18,19,19-tetradehydro-6a-carbaprostaglandin I(sub 2)
(5E)-5-[(3aS,4R,5R,6aS)-5-hydroxy-4-[(E,3S)-3-hydroxy-4-methyloct-1-en-6-ynyl]-3,3a,4,5,6,6a-hexahydro-1H-pentalen-2-ylidene]pentanoic acid
(5E)-5-[(3aS,5R,6aS)-5-hydroxy-4-[(1E,3S)-3-hydroxy-4-methyloct-1-en-6-yn-1-yl]hexahydropentalen-2(1H)-ylidene]pentanoic acid
(5E)-5-[(3aS,5R,6aS)-5-hydroxy-4-[(E,3S)-3-hydroxy-4-methyloct-1-en-6-ynyl]-3,3a,4,5,6,6a-hexahydro-1H-pentalen-2-ylidene]pentanoic acid
(5Z)-5-[(3aS,5R,6aS)-5-hydroxy-4-[(E,3R)-3-hydroxy-4-methyloct-1-en-6-ynyl]-3,3a,4,5,6,6a-hexahydro-1H-pentalen-2-ylidene]pentanoic acid
(E)-(3aS,4R,5R,6aS)-Hexahydro-5-hydroxy-4-((E)-(3S,4RS)-3-hydroxy-4-methyl-1-octen-6-ynyl)-delta(sup 2(1H),delta)-pentalenevaleric acid
(E)-5-(3aS,4R,5R,6aS)-5-Hydroxy-4((E)-(3S,4RS)-3-hydroxy-4-methyl-1-octen-6-inyl)perhydropentalen-2-yliden)valeriansaeure
73873-87-7
78919-13-8
AC1NSK5T
AC1O5JIJ
AC1O5JP3
AC1Q1J28
ACT-213105
C22H32O4
CHEMBL138694
CHEMBL236025
CHEMBL494
CID10937300
CID11639089
CID5311181
CID6435293
CID6435378
CID9885245
 
CID9975807
Ciloprost
D02721
E-1030
Endoprost
HMS2090A19
ILOPROST
Ilomedin
Ilomedine
Iloprost
Iloprost (INN)
Iloprost [BAN:INN]
Iloprostum
Iloprostum [Latin]
L000996
LS-101845
NCGC00181784-01
PDSP2_001536
SH-401
UNII-JED5K35YGL
Ventavis
Ventavis (TN)
ZK 36374
ZK-36374
ZK-363775
[3H]-Iloprost
25
Tadalafilapproved, investigational155171596-29-5110635
Synonyms:
(6R,12AR)-2,3,6,7,12,12a-hexahydro-2-methyl-6-(3,4-(methylenedioxy)phenyl) pyrazino(1',2':1,6)pyrido(3,4-b)indole-1,4-dione
(6R,12aR)-2,3,6,7,12,12a-Hexahydro-2-methyl-6-(3,4-(methylenedioxy)phenyl) pyrazino(1',2':1,6)pyrido(3,4-b)indole-1,4-dione
(6R,12aR)-2,3,6,7,12,12a-Hexahydro-2-methyl-6-(3,4-methylenedioxyphenyl)pyrazino(1',2':1,6)pyrido(3,4-b)indole-1,4-dione
(6R-trans)-6-(1,3-Benzodioxol-5-yl)-2,3,6,7,12,12a-hexahydro-2-methyl-pyrazino(1',2':1,6)pyrido(3,4-b)indole-1,4-dione
171596-29-5
1xoz
6-BENZO[1,3]dioxol-5-yl-2-methyl-2,3,6,7,12,12a-hexahydro-pyrazino[1',2':1,6]pyrido[3,4-b]indole-1,4-dione
AC1L380B
ADCIRCA
Acdirca
Adcirca
C429886
CHEBI:41488
CHEMBL779
CID110635
CPD000466321
Cialis
Cialis (TN)
Cialis, GF 196960, IC 351, ICOS 351, Tadalafil
D02008
DB00820
FT-0080116
GF 196960
GF-196960
 
HMS2051N17
HSDB 7303
IC 351
IC-351
ICOS 351
Ic351
KS-1117
LS-186558
LS-187015
LS-187770
Lilly brand of tadalafil
MLS000759426
MLS001165782
MLS001195644
MLS001424132
MolPort-002-885-864
S1512_Selleck
SAM001246586
SMR000466321
Tadalafil
Tadalafil (JAN/USAN/INN)
Tadalafil [USAN]
Tadanafil
UNII-742SXX0ICT
ZINC03993855
26Thrombin304
27Serine Proteinase Inhibitors878
28
protease inhibitors5471
Synonyms:
 
protease inhibitors
29Anticoagulants2623
30Antithrombin III690
31Antithrombins697
32Factor Xa Inhibitors411
33HIV Protease Inhibitors5470
34Immunoglobulins6394
35Autoantibodies130
36Antibodies6394
37Phosphodiesterase 5 Inhibitors589
38Tezosentan64
39Sildenafil Citrate331171599-83-0
40serineNutraceutical954

Interventional clinical trials:

(show top 50)    (show all 60)
idNameStatusNCT IDPhase
1Influence of Respiratory and Exercise Therapy on Oxygen Uptake and Right Heart Function in CTEPH Patients After PEAUnknown statusNCT01393327Phase 3
2Bosentan Effects in Inoperable Forms of Chronic Thromboembolic Pulmonary HypertensionCompletedNCT00313222Phase 3
3Effect of Long-term Oxygen Therapy on Exercise Capacity and Quality of Life in Patients With Pulmonary Arterial and Chronic Thromboembolic Pulmonary HypertensionCompletedNCT01884012Phase 3
4Bosentan in Patients With Inoperable Chronic Thromboembolic Pulmonary Hypertension (CTEPH)CompletedNCT00319111Phase 3
5A Study to Evaluate Efficacy and Safety of Oral BAY63-2521 in Patients With CTEPH.CompletedNCT00855465Phase 3
6CTEPH DIAGNOSIS Europe - MRIRecruitingNCT02791282Phase 3
7Efficacy and Tolerability of Subcutaneously Administered Treprostinil Sodium in Patients With Severe (Non-operable) Chronic Thromboembolic Pulmonary Hypertension (CTREPH)Active, not recruitingNCT01416636Phase 3
8BAY63-2521 - Long-term Extension Study in Patients With Chronic Thromboembolic Pulmonary HypertensionActive, not recruitingNCT00910429Phase 3
9Ambrisentan for Inoperable Chronic Thromboembolic Pulmonary Hypertension.TerminatedNCT01884675Phase 3
10A Open Label Study to Assess the Long-term Safety, Tolerability and Efficacy of Ambrisentan in Subjects With Inoperable Chronic Thromboembolic Pulmonary Hypertension (CTEPH)TerminatedNCT01894022Phase 3
11Clinical Study to Assess the Efficacy, Safety and Tolerability of Macitentan in Subjects With Inoperable Chronic Thromboembolic Pulmonary HypertensionCompletedNCT02021292Phase 2
12Pilot Study to Evaluate Right Ventricular Function With Riociguat in CTEPHCompletedNCT02094001Phase 2
13Effect of Short-term Oxygen During CPET in Pulmonary HypertensionCompletedNCT01748474Phase 2
14An Open Multiple Dose Titration Study In Patients With Pulmonary HypertensionCompletedNCT00454558Phase 2
15Phase-II Study of the Use of PulmoBind for Molecular Imaging of Pulmonary HypertensionCompletedNCT02216279Phase 2
16Interventional Treatment of Residual Pulmonary Hypertension in Patients After Pulmonary ThromboendarterectomyRecruitingNCT02745106Phase 2
17Bosentan in Myocardium Metabolism and Perfusion Measured by 18F-2-fluoro-2-deoxy-D-glucose (18F-FDG) and Rubidium-82 (82Rb) Positron Emission Tomography/Computed Tomography (PET/CTs) on Pulmonary Arterial Hypertension (PAH) or Chronic Thromboembolic PulmoRecruitingNCT02970851Phase 1, Phase 2
18Clinical Study to Assess the Safety, Tolerability and Efficacy of Macitentan in Subjects With Inoperable Chronic Thromboembolic Pulmonary HypertensionActive, not recruitingNCT02060721Phase 2
19Cell Proliferation in Pulmonary Hypertension. FDG-PET Comparison Between Patients and Healthy SubjectsNot yet recruitingNCT02886793Phase 1, Phase 2
20Latent Pulmonary Hypertension (PH) in Chronic Thromboembolic Pulmonary Hypertension (CTEPH )After Endarterectomy and Influence of Exercise and Respiratory TherapyUnknown statusNCT00477724
21Right Ventricular Resynchronization TherapyUnknown statusNCT01163422
223D Echocardiographic Assessment of RV Function in Patients Undergoing Pulmonary EndarterectomyUnknown statusNCT02426203
23Chronic Thromboembolic Pulmonary Hypertension (CTEPH) Incidence in Patients With Central Versus Peripheral EmbolismUnknown statusNCT01672203
24Epidemiological Study of Chronic Thromboembolic Pulmonary Hypertension in ChinaUnknown statusNCT01717092
25Gene Mutations in Secondary Pulmonary HypertensionUnknown statusNCT00348803
26Pulmonary Perfusion Heterogeneity in Patients With CTEPH Using Functional PET ImagingCompletedNCT02114047
27A Study to Understand the Treatment Patterns in Patients With Pulmonary Arterial Hypertension or Chronic Thromboembolic Pulmonary Hypertension During a Switch of Treatment to Adempas in Real-life Clinical PracticeCompletedNCT02545465
28Electronic Activity Level Monitoring Pilot in Pulmonary HypertensionCompletedNCT02536534
29Lung Protective Ventilation in Pulmonary Thromboendarterectomy (PTE) PatientsCompletedNCT00747045
30Study on the Clinical Course Of Pulmonary EmbolismCompletedNCT01781858
31Identification of Criteria of the Success of the Endarterectomy in Chronic Pulmonary Post Embolic HypertensionCompletedNCT00657722
32Smoking: a Risk Factor for Pulmonary Arterial Hypertension?CompletedNCT01484899
33RF Surgical Sponge-Detecting System on the Function of Pacemakers and Implantable Cardioverter DefibrillatorsCompletedNCT02111980
34Riociguat Versus Balloon Pulmonary Angioplasty in Non-operable Chronic thromboEmbolic Pulmonary HypertensionRecruitingNCT02634203
35Sleep Disorder Breathing in Chronic Thromboembolic Pulmonary HypertensionRecruitingNCT03074539
36Prospective Registry to Evaluate the Effective Incidence of Chronic Thromboembolic Pulmonary Hypertension in GermanyRecruitingNCT02660463
37Registry for Pulmonary Hypertension in ChinaRecruitingNCT01417338
38A Study Investigating Routine Practice of Chronic Thromboembolic Pulmonary Hypertension Management in EMEA CountriesRecruitingNCT02637050
39U.S. CTEPH RegistryRecruitingNCT02429284
40Prospective, Non-interventional, Multi-center Post-authorization Safety Study of Riociguat for Chronic Thromboembolic Pulmonary Hypertension (CTEPH )RecruitingNCT02117791
41Balloon Pulmonary Angioplasty in Non-operable CTEPH PatientsRecruitingNCT02964390
42Long Term Outcomes of Venous ThromboembolismRecruitingNCT02268630
43Effect of Exercise Training in Patients With Pulmonary HypertensionRecruitingNCT02558582
44Early Non-invasive Detection of CTEPH After Pulmonary EmbolismRecruitingNCT02555137
45Sleep Disordered Breathing in Precapillary Pulmonary Hypertension: Effect of CPAP TreatmentRecruitingNCT02583906
46Six Years of Follow-up After Idiopathic Venous ThrobmoembolismRecruitingNCT02884934
47Follow-up in Rivaroxaban Patients in Setting of ThromboembolismRecruitingNCT02248610
48Global Anticoagulant Registry in the FIELD- Venous Thromboembolic EventsRecruitingNCT02155491
49CTEPH Identification an Standard Computerised Tomography Pulmonary Angiography in Pulmonary Embolism PatientsRecruitingNCT03083093
50Zürich Pulmonary Hypertension Outcome Assessment CohortRecruitingNCT02249806

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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MalaCards organs/tissues related to Chronic Thromboembolic Pulmonary Hypertension:

36
Lung, Endothelial, Heart, Brain, Neutrophil, Monocytes, Smooth muscle

Publications for Chronic Thromboembolic Pulmonary Hypertension

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Articles related to Chronic Thromboembolic Pulmonary Hypertension:

(show top 50)    (show all 497)
idTitleAuthorsYear
1
Activated TAFI Promotes the Development of Chronic Thromboembolic Pulmonary Hypertension: A Possible Novel Therapeutic Target. (28289017)
2017
2
Transition of Intravenous Treprostinil to Oral Therapy in a Patient with Functional Class IV Chronic Thromboembolic Pulmonary Hypertension. (28513869)
2017
3
Depressive Status in Patients With Chronic Thromboembolic Pulmonary Hypertension. (28529263)
2017
4
Diagnostic accuracy of lung subtraction iodine mapping CT for the evaluation of pulmonary perfusion in patients with chronic thromboembolic pulmonary hypertension: Correlation with perfusion SPECT/CT. (28526539)
2017
5
Epidemiology and management of chronic thromboembolic pulmonary hypertension: Experience from two expert centers. (28528258)
2017
6
To screen or not to screen for chronic thromboembolic pulmonary hypertension after acute pulmonary embolism. (28073030)
2017
7
An Update on the Management of Chronic Thromboembolic Pulmonary Hypertension. (27989311)
2017
8
Imaging in Chronic Thromboembolic Pulmonary Hypertension. (28060193)
2017
9
Combination therapy with riociquat and inhaled treprostinil in inoperable and progressive chronic thromboembolic pulmonary hypertension. (27942445)
2017
10
A chronic thromboembolic pulmonary hypertension catch-22 situation: inferior vena caval filter plays a pivotal role in an unlikely situation. (28523175)
2017
11
Energy efficiency and pulmonary artery flow after balloon pulmonary angioplasty for inoperable, chronic thromboembolic pulmonary hypertension: Analysis by phase-contrast MRI. (28065382)
2017
12
A Review of Clinical Trial Endpoints of Patients with Pulmonary Arterial Hypertension and Chronic Thromboembolic Pulmonary Hypertension and How They Relate to Patient Outcomes in the United States. (28025931)
2017
13
Percutaneous treatment of chronic thromboembolic pulmonary hypertension (CTEPH). (27174110)
2016
14
Clinical usefulness of end-tidal CO2 profiles during incremental exercise in patients with chronic thromboembolic pulmonary hypertension. (27817818)
2016
15
Familial chronic thromboembolic pulmonary hypertension in aA mother and aA son: successful treatment with refined balloon pulmonary angioplasty. (28010001)
2016
16
Thrombin-Activatable Fibrinolysis Inhibitor in Chronic Thromboembolic Pulmonary Hypertension. (27102961)
2016
17
Information, social support and coping in patients with pulmonary arterial hypertension or chronic thromboembolic pulmonary hypertension-A nationwide population-based study. (28041647)
2016
18
Quality of life in patients with chronic thromboembolic pulmonary hypertension. (27076580)
2016
19
Pulmonary arteriovenous malformation in chronic thromboembolic pulmonary hypertension. (27413264)
2016
20
Balloon Pulmonary Angioplasty Improves Biventricular Functions and Pulmonary Flow in Chronic Thromboembolic Pulmonary Hypertension. (27097557)
2016
21
An international physician survey of chronic thromboembolic pulmonary hypertension management. (28090289)
2016
22
Predictors of long-term outcomes in patients treated with riociguat for chronic thromboembolic pulmonary hypertension: data from the CHEST-2 open-label, randomised, long-term extension trial. (27067478)
2016
23
TCT-91 Efficacy of aggressive balloon pulmonary angioplasty on chronic thromboembolic pulmonary hypertension beyond normalized mean pulmonary arterial pressure. (27970256)
2016
24
Population pharmacokinetics and the pharmacokinetic/pharmacodynamic relationship of riociguat in patients with pulmonary arterial hypertension or chronic thromboembolic pulmonary hypertension. (27162632)
2016
25
Native T1 mapping and extracellular volume fraction measurement for assessment of right ventricular insertion point and septal fibrosis in chronic thromboembolic pulmonary hypertension. (27651142)
2016
26
Efficacy and safety of balloon pulmonary angioplasty for chronic thromboembolic pulmonary hypertension guided by cone-beam computed tomography and electrocardiogram-gated area detector computed tomography. (28034568)
2016
27
TCT-90 Efficacy and safety of sequential hybrid therapy with pulmonary endarterectomy and additional balloon pulmonary angioplasty for chronic thromboembolic pulmonary hypertension. (27970252)
2016
28
Long-Term Outcomes After Percutaneous Transluminal Pulmonary Angioplasty for Chronic Thromboembolic Pulmonary Hypertension. (27956405)
2016
29
TCT-92 Short- and Long-Term Remodeling of Pulmonary Arteries After Balloon Angioplasty in Patients with Inoperable Chronic Thromboembolic Pulmonary Hypertension. (27970255)
2016
30
The role of mononuclear cell tissue factor and inflammatory cytokines in patients with chronic thromboembolic pulmonary hypertension. (26667361)
2016
31
TCT-771 Clinical and Hemodynamic Efficacy of Balloon Pulmonary Angioplasty forA Inoperable Chronic Thromboembolic Pulmonary Hypertension: AA Meta-Analysis. (27970157)
2016
32
Choriocarcinoma mimicking chronic thromboembolic pulmonary hypertension. (26916795)
2016
33
Spontaneous enlargement of pulmonary artery after successful balloon pulmonary angioplasty in a patient with chronic thromboembolic pulmonary hypertension. (27934612)
2016
34
Risk factors detection in chronic thromboembolic pulmonary hypertension, a tool for risk quantification? (27826972)
2016
35
Re: Outcome after surgical treatment of chronic thromboembolic pulmonary hypertension: dealing with different patient subsets. A single-centre experience. (27147627)
2016
36
Successful balloon pulmonary angioplasty with gadolinium contrast media for a patient with chronic thromboembolic pulmonary hypertension and iodine allergy. (27141436)
2016
37
Haemodynamic effects of riociguat in inoperable/recurrent chronic thromboembolic pulmonary hypertension. (28011757)
2016
38
Fractional Flow Reserve-guided Pulmonary Angioplasty in Chronic Thromboembolic Pulmonary Hypertension. (27052125)
2016
39
Chronic thromboembolic pulmonary hypertension - a diagnostic and therapeutic update. (27128884)
2016
40
REDUCED FORCE OF DIAPHRAGM MUSCLE FIBERS IN PATIENTS WITH CHRONIC THROMBOEMBOLIC PULMONARY HYPERTENSION. (27190061)
2016
41
Review of the Association between Splenectomy and Chronic Thromboembolic Pulmonary Hypertension. (27058013)
2016
42
Insights into ventilation-gas exchange coupling in chronic thromboembolic pulmonary hypertension. (27076589)
2016
43
Pulmonary endarterectomy for the treatment of chronic thromboembolic pulmonary hypertension. (27070482)
2016
44
Beneficial Therapeutic Effects of Balloon Pulmonary Angioplasty on Biventricular Function in Patients With Chronic Thromboembolic Pulmonary Hypertension. (27170202)
2016
45
CXCL13 in idiopathic pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension. (26927848)
2016
46
Development and Characterization of an Inducible Rat Model of Chronic Thromboembolic Pulmonary Hypertension. (27045032)
2016
47
High prevalence of severe coronary artery disease in elderly patients with non-operable chronic thromboembolic pulmonary hypertension referred for balloon pulmonary angioplasty. (27980550)
2016
48
Endothelin ETA receptors predominate in chronic thromboembolic pulmonary hypertension. (26874031)
2016
49
Pulmonary vascular morphology as an imaging biomarker in chronic thromboembolic pulmonary hypertension. (27162616)
2016
50
Outcome after surgical treatment of chronic thromboembolic pulmonary hypertension: dealing with different patient subsets. A single-centre experience. (27072006)
2016

Variations for Chronic Thromboembolic Pulmonary Hypertension

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Expression for genes affiliated with Chronic Thromboembolic Pulmonary Hypertension

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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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GO Terms for genes affiliated with Chronic Thromboembolic Pulmonary Hypertension

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Cellular components related to Chronic Thromboembolic Pulmonary Hypertension according to GeneCards Suite gene sharing:

idNameGO IDScoreTop Affiliating Genes
1extracellular vesicleGO:190356110.0FGA, FGB
2fibrinogen complexGO:00055779.9FGA, FGB
3platelet alpha granuleGO:00310919.9FGA, FGB
4platelet alpha granule lumenGO:00310939.4FGA, FGB
5blood microparticleGO:00725629.4F2, FGA, FGB
6external side of plasma membraneGO:00098979.4F2, FGA, FGB
7extracellular regionGO:00055767.9CRP, EDN1, F2, FGA, FGB, NPPB
8extracellular spaceGO:00056157.3BMPR2, CRP, EDN1, F2, FGA, FGB

Biological processes related to Chronic Thromboembolic Pulmonary Hypertension according to GeneCards Suite gene sharing:

(show all 29)
idNameGO IDScoreTop Affiliating Genes
1body fluid secretionGO:000758910.3EDN1, NPPB
2positive regulation of renal sodium excretionGO:003581510.2EDN1, NPPB
3positive regulation of urine volumeGO:003581010.2EDN1, NPPB
4blood coagulation, fibrin clot formationGO:007237810.2FGA, FGB
5positive regulation of endothelial cell migrationGO:001059510.1BMPR2, EDN1
6cellular protein complex assemblyGO:004362310.1FGA, FGB
7regulation of blood pressureGO:000821710.1EDN1, NPPB
8induction of bacterial agglutinationGO:004315210.1FGA, FGB
9negative regulation of endothelial cell apoptotic processGO:200035210.1FGA, FGB
10regulation of blood vessel sizeGO:005088010.1EDN1, NPPB
11negative regulation of extrinsic apoptotic signaling pathway via death domain receptorsGO:190204210.1FGA, FGB
12plasminogen activationGO:003163910.1FGA, FGB
13platelet aggregationGO:007052710.1FGA, FGB
14positive regulation of exocytosisGO:004592110.1FGA, FGB
15positive regulation of heterotypic cell-cell adhesionGO:003411610.1FGA, FGB
16positive regulation of peptide hormone secretionGO:009027710.1FGA, FGB
17positive regulation of protein secretionGO:005071410.1FGA, FGB
18positive regulation of substrate adhesion-dependent cell spreadingGO:190002610.0FGA, FGB
19positive regulation of vasoconstrictionGO:004590710.0FGA, FGB
20protein polymerizationGO:005125810.0FGA, FGB
21cellular response to interleukin-1GO:007134710.0EDN1, FGB
22response to calcium ionGO:00515929.9FGA, FGB
23acute-phase responseGO:00069539.8CRP, F2
24cell surface receptor signaling pathwayGO:00071669.4EDN1, F2, NPPB
25toll-like receptor signaling pathwayGO:00022249.4FGA, FGB
26blood coagulationGO:00075969.3F2, FGA, FGB
27fibrinolysisGO:00427309.3F2, FGA, FGB
28hemostasisGO:00075999.3F2, FGA, FGB
29platelet activationGO:00301689.3F2, FGA, FGB

Molecular functions related to Chronic Thromboembolic Pulmonary Hypertension according to GeneCards Suite gene sharing:

idNameGO IDScoreTop Affiliating Genes
1cell adhesion molecule bindingGO:005083910.1FGA, FGB
2receptor bindingGO:00051027.7EDN1, F2, FGA, FGB, NPPB

Sources for Chronic Thromboembolic Pulmonary Hypertension

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2CDC
6CNVD
10DGIdb
15ExPASy
16FDA
17FMA
27GTR
28HGMD
29HMDB
30ICD10
31ICD10 via Orphanet
32ICD9CM
33IUPHAR
34KEGG
37MedGen
39MeSH
40MESH via Orphanet
41MGI
44NCI
45NCIt
46NDF-RT
49NINDS
50Novoseek
52OMIM
53OMIM via Orphanet
57PubMed
58QIAGEN
63SNOMED-CT via Orphanet
67Tumor Gene Family of Databases
68UMLS
69UMLS via Orphanet