MCID: CHR466
MIFTS: 39

Chronic Thromboembolic Pulmonary Hypertension malady

Categories: Respiratory diseases, Rare diseases, Blood diseases

Aliases & Classifications for Chronic Thromboembolic Pulmonary Hypertension

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Sources:
51Orphanet, 65UMLS, 66UMLS via Orphanet
See all MalaCards sources

Aliases & Descriptions for Chronic Thromboembolic Pulmonary Hypertension:

Name: Chronic Thromboembolic Pulmonary Hypertension 51 65
 
Cteph 51

Characteristics:

Orphanet epidemiological data:

51
chronic thromboembolic pulmonary hypertension:
Inheritance: Not applicable; Prevalence: 1-9/100000 (Europe); Age of onset: Adult

Classifications:



External Ids:

Orphanet51 70591
UMLS via Orphanet66 C2363973
UMLS65 C2363973

Summaries for Chronic Thromboembolic Pulmonary Hypertension

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MalaCards based summary: Chronic Thromboembolic Pulmonary Hypertension, also known as cteph, is related to heart disease and pulmonary hypertension. An important gene associated with Chronic Thromboembolic Pulmonary Hypertension is FGA (Fibrinogen Alpha Chain), and among its related pathways are Cardiomyocyte Differentiation through BMP Receptors and MicroRNAs in cardiomyocyte hypertrophy. Affiliated tissues include heart, lung and endothelial, 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 61)
idRelated DiseaseScoreTop Affiliating Genes
1heart disease28.9CRP, EDN1, NPPB
2pulmonary hypertension11.2
3pulmonary embolism10.5
4endotheliitis10.2
5laryngeal neuroendocrine tumor10.2BMPR2, NPPB
6oral mucosa leukoplakia10.1CRP, NPPB
7sarcoma10.1
8antiphospholipid syndrome10.1
9protein-energy malnutrition10.1CRP, NPPB
10prostate squamous cell carcinoma10.1CRP, NPPB
11adams-oliver syndrome 510.1CRP, NPPB
12hemangioma10.1CRP, NPPB
13dysfibrinogenemia10.0
14postpartum depression10.0CRP, NPPB
15coxa vara, congenital10.0EDN1, NPPB
16lethal congenital contractural syndrome 29.9EDN1, NPPB
17idiopathic hypersomnia9.9EDN1, NPPB
18elastoderma9.9BMPR2, EDN1
19vitamin k deficiency hemorrhagic disease9.9CRP, FGA
20endogenous depression9.9EDN1, NPPB
21marasmus9.9BMPR2, EDN1
22hereditary hemorrhagic telangiectasia9.8
23sinusitis9.8
24thrombocytopenia9.8
25esophagitis9.8
26schistosomiasis9.8
27post-thrombotic syndrome9.8
28thoracic outlet syndrome9.8
29cerebritis9.8
30choriocarcinoma9.8
31lung disease9.8
32homocystinuria9.8
33sickle cell disease9.8
34eisenmenger syndrome9.8
35acth deficiency9.8
36aneurysm9.8
37syphilitic spinal sclerosis9.8EDN1, NPPB
38extrapyramidal and movement disease9.8EDN1, NPPB
39acute allergic mucoid otitis media9.8CRP, EDN1
40congenital chloride diarrhea9.8CRP, FGA, NPPB
41stroke, ischemic9.7CRP, FGA, PTGIS
42thrombotic thrombocytopenic purpura9.7BMPR2, EDN1
43periampullary adenoma9.7CRP, EDN1
44chronic venous insufficiency9.6CRP, EDN1
45temporal lobe epilepsy9.6CRP, EDN1
46cerebellar angioblastoma9.5BMPR2, EDN1
47neovascular glaucoma9.5BMPR2, EDN1, NPPB
48hypertension, essential9.5EDN1, NPPB, PTGIS
49mitochondrial metabolism disease9.4CRP, EDN1
50obstructive sleep apnea9.4CRP, EDN1, NPPB

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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Drugs & Therapeutics for Chronic Thromboembolic Pulmonary Hypertension

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FDA approved drugs:

id Drug Name Active Ingredient(s)15 Company Approval Date
1
Adempas15 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:15 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:15 
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 28)
idNameStatusPhaseClinical TrialsCas NumberPubChem Id
1
Bosentanapproved, investigationalPhase 3100147536-97-8104865
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
Bosentanum
C27H29N5O6S
CHEBI:51450
CHEMBL957
CID104865
D07538
 
DB00559
EN002863
HMS2090N14
I06-1873
KS-5062
L001086
LS-31532
MolPort-003-845-300
NCGC00167440-01
P-Tert-butyl-N-(6-(2-hydroxyethoxy)-5-(O-methoxyphenoxy)-2-(2-pyrimidinyl)-4-pyrimidinyl)benzenesulfonamide
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
bosentanum
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 37781846-19-754786, 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
Orenitram
Remodulin
Remodulin (TN)
Treprostinil
Treprostinil (USAN/INN)
Treprostinil [USAN:INN]
Treprostinil sodium
Treprostinilo
Treprostinilum
Tyvaso
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
3Endothelin Receptor AntagonistsPhase 3, Phase 2189
4Antihypertensive AgentsPhase 33618
5CalamusNutraceuticalPhase 3344
6
MacitentanapprovedPhase 229441798-33-0
Synonyms:
ACT 064992
ACT-064992
 
ACT064992
Macitentanum
OPSUMIT
Opsumit
7adrenomedullinPhase 211
8Fluorodeoxyglucose F18Phase 2356
9Endothelin A Receptor AntagonistsPhase 232
10
Warfarinapproved42281-81-26691, 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 Sodium
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
11
Rivaroxabanapproved215366789-02-8
Synonyms:
BAY 59-7939
 
BAY59-7939
Xarelto
12
Iloprostapproved, investigational7478919-13-86443959
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
13
Tadalafilapproved, investigational140171596-29-5110635
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
14Serine Proteinase Inhibitors705
15Protease Inhibitors4558
16Antithrombins574
17Anticoagulants1760
18HIV Protease Inhibitors4558
19Antithrombin III563
20Factor Xa Inhibitors315
21Immunoglobulins4477
22Antibodies4477
23Tezosentan64
24Autoantibodies117
25Phosphodiesterase Inhibitors1062
26Sildenafil Citrate304171599-83-0
27Phosphodiesterase 5 Inhibitors513
28serineNutraceutical770

Interventional clinical trials:

(show top 50)    (show all 51)
idNameStatusNCT IDPhase
1Ambrisentan for Inoperable Chronic Thromboembolic Pulmonary Hypertension.CompletedNCT01884675Phase 3
2Bosentan Effects in Inoperable Forms of Chronic Thromboembolic Pulmonary HypertensionCompletedNCT00313222Phase 3
3Bosentan in Patients With Inoperable Chronic Thromboembolic Pulmonary Hypertension (CTEPH)CompletedNCT00319111Phase 3
4A Open Label Study to Assess the Long-term Safety, Tolerability and Efficacy of Ambrisentan in Subjects With Inoperable Chronic Thromboembolic Pulmonary Hypertension (CTEPH)CompletedNCT01894022Phase 3
5A Study to Evaluate Efficacy and Safety of Oral BAY63-2521 in Patients With CTEPH.CompletedNCT00855465Phase 3
6Efficacy and Tolerability of Subcutaneously Administered Treprostinil Sodium in Patients With Severe (Non-operable) Chronic Thromboembolic Pulmonary Hypertension (CTREPH)RecruitingNCT01416636Phase 3
7Effect of Long-term Oxygen Therapy on Exercise Capacity and Quality of Life in Patients With Pulmonary Arterial and Chronic Thromboembolic Pulmonary HypertensionRecruitingNCT01884012Phase 3
8Influence of Respiratory and Exercise Therapy on Oxygen Uptake and Right Heart Function in CTEPH Patients After PEARecruitingNCT01393327Phase 3
9BAY63-2521 - Long-term Extension Study in Patients With Chronic Thromboembolic Pulmonary HypertensionActive, not recruitingNCT00910429Phase 3
10An Open Multiple Dose Titration Study In Patients With Pulmonary HypertensionCompletedNCT00454558Phase 2
11Phase-II Study of the Use of PulmoBind for Molecular Imaging of Pulmonary HypertensionCompletedNCT02216279Phase 2
12Clinical Study to Assess the Safety, Tolerability and Efficacy of Macitentan in Subjects With Inoperable Chronic Thromboembolic Pulmonary HypertensionRecruitingNCT02060721Phase 2
13Effect of Short-term Oxygen During CPET in Pulmonary HypertensionRecruitingNCT01748474Phase 2
14Look at Way the Heart Functions in People With Pulmonary Hypertension (PH) Who Have Near Normal Right Ventricle (RV) Function and People With Pulmonary Hypertension Who Have Impaired RV Function. Using Imaging Studies PET Scan and Cardiac MRI.RecruitingNCT01953965Phase 2
15Clinical Study to Assess the Efficacy, Safety and Tolerability of Macitentan in Subjects With Inoperable Chronic Thromboembolic Pulmonary HypertensionActive, not recruitingNCT02021292Phase 2
16Pilot Study to Evaluate Right Ventricular Function With Riociguat in CTEPHActive, not recruitingNCT02094001Phase 2
17Lung Protective Ventilation in Pulmonary Thromboendarterectomy (PTE) PatientsCompletedNCT00747045
18Study on the Clinical Course Of Pulmonary EmbolismCompletedNCT01781858
19Identification of Criteria of the Success of the Endarterectomy in Chronic Pulmonary Post Embolic HypertensionCompletedNCT00657722
20Smoking: a Risk Factor for Pulmonary Arterial Hypertension?CompletedNCT01484899
21RF Surgical Sponge-Detecting System on the Function of Pacemakers and Implantable Cardioverter DefibrillatorsCompletedNCT02111980
22Prospective Registry to Evaluate the Effective Incidence of Chronic Thromboembolic Pulmonary Hypertension in GermanyRecruitingNCT02660463
23Registry for Pulmonary Hypertension in ChinaRecruitingNCT01417338
24Latent Pulmonary Hypertension (PH) in Chronic Thromboembolic Pulmonary Hypertension (CTEPH )After Endarterectomy and Influence of Exercise and Respiratory TherapyRecruitingNCT00477724
25Evaluation of Pulmonary Perfusion Heterogeneity in Patients With Chronic Thromboembolic Pulmonary Hypertension Using Functional PET ImagingRecruitingNCT02114047
26U.S. CTEPH RegistryRecruitingNCT02429284
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 PracticeRecruitingNCT02545465
28Prospective, Non-interventional, Multi-center Post-authorization Safety Study of Riociguat for Chronic Thromboembolic Pulmonary Hypertension (CTEPH)RecruitingNCT02117791
29A Study Investigating Routine Practice of Chronic Thromboembolic Pulmonary Hypertension Management in EMEA CountriesRecruitingNCT02637050
30Long Term Outcomes of Venous ThromboembolismRecruitingNCT02268630
31New International CTEPH DatabaseRecruitingNCT02656238
32Electronic Activity Level Monitoring Pilot in Pulmonary HypertensionRecruitingNCT02536534
33Effect of Exercise Training in Patients With Pulmonary HypertensionRecruitingNCT02558582
34Sleep Disordered Breathing in Precapillary Pulmonary Hypertension: Effect of CPAP TreatmentRecruitingNCT02583906
35Interventional Treatment of Residual Pulmonary Hypertension in Patients After Pulmonary ThromboendarterectomyRecruitingNCT02745106
36Chronic Thromboembolic Pulmonary Hypertension (CTEPH) Incidence in Patients With Central Versus Peripheral EmbolismRecruitingNCT01672203
37Epidemiological Study of Chronic Thromboembolic Pulmonary Hypertension in ChinaRecruitingNCT01717092
38Follow-up in Rivaroxaban Patients in Setting of ThromboembolismRecruitingNCT02248610
39Global Anticoagulant Registry in the FIELD- Venous Thromboembolic EventsRecruitingNCT02155491
40Gene Mutations in Secondary Pulmonary HypertensionRecruitingNCT00348803
41Zürich Pulmonary Hypertension Outcome Assessment CohortRecruitingNCT02249806
42EXPERT, EXPosurE Registry RiociguaT in Patients With Pulmonary HypertensionRecruitingNCT02092818
43VTEval Project - Prospective Cohort Studies to Evaluate and Improve Diagnostics, Management Strategies and Risk Stratification in VTERecruitingNCT02156401
44Exhaled Breath Olfactory Signature of Pulmonary Arterial HypertensionRecruitingNCT02782026
45Chronic Thrombo-embolic Pulmonary Hypertension: Classification and Long Term OutcomeActive, not recruitingNCT02565030
46Auto-immunity and Pulmonary Arterial HypertensionActive, not recruitingNCT01208792
47Right Ventricular Resynchronization TherapyEnrolling by invitationNCT01163422
48Riociguat Versus Balloon Pulmonary Angioplasty in Non-operable Chronic thromboEmbolic Pulmonary HypertensionNot yet recruitingNCT02634203
49Early Non-invasive Detection of CTEPH After Pulmonary EmbolismNot yet recruitingNCT02555137
503D Echocardiographic Assessment of RV Function in Patients Undergoing Pulmonary EndarterectomyNot yet recruitingNCT02426203

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:

33
Heart, Lung, Endothelial, Prostate, Breast, Liver, B cells

Animal Models for Chronic Thromboembolic Pulmonary Hypertension or affiliated genes

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MGI Mouse Phenotypes related to Chronic Thromboembolic Pulmonary Hypertension:

38
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053857.6BMPR2, CRP, EDN1, FGA, PTGIS

Publications for Chronic Thromboembolic Pulmonary Hypertension

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Articles related to Chronic Thromboembolic Pulmonary Hypertension:

(show top 50)    (show all 466)
idTitleAuthorsYear
1
Pulmonary vascular morphology as an imaging biomarker in chronic thromboembolic pulmonary hypertension. (27162616)
2016
2
Noninvasive assessment of pulmonary vascular resistance by echocardiography in chronic thromboembolic pulmonary hypertension. (26344610)
2015
3
Balloon pulmonary angioplasty using contrast agents improves impaired renal function in patients with chronic thromboembolic pulmonary hypertension. (25880583)
2015
4
Recent advances of pulmonary endarterectomy for chronic thromboembolic pulmonary hypertension including Japanese experiences. (24068659)
2014
5
Operability Assessment and Postoperative Outcome in Chronic Thromboembolic Pulmonary Hypertension. (24401572)
2014
6
Differentially expressed plasma microRNAs and the potential regulatory function of Let-7b in chronic thromboembolic pulmonary hypertension. (24978044)
2014
7
Dual-energy CT perfusion and angiography in chronic thromboembolic pulmonary hypertension: diagnostic accuracy and concordance with radionuclide scintigraphy. (23982287)
2014
8
Balloon pulmonary angioplasty in patients with inoperable chronic thromboembolic pulmonary hypertension. (23846611)
2013
9
Clinical outcomes of thromboendarterectomy for chronic thromboembolic pulmonary hypertension: 12-year experience. (23423163)
2013
10
Optical coherence tomography is superior to intravascular ultrasound for diagnosis of distal-type chronic thromboembolic pulmonary hypertension. (23386271)
2013
11
Right ventricular longitudinal deformation parameters and exercise capacity : Prognosis of patients with chronic thromboembolic pulmonary hypertension. (23740084)
2013
12
Chronic thromboembolic pulmonary hypertension due to upper-extremity deep vein thrombosis caused by thoracic outlet syndrome. (22138476)
2012
13
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
14
Chronic thromboembolic pulmonary hypertension with pulmonary infarction occuring in a hypertensive. (21970224)
2011
15
Clinical course of unoperated mild chronic thromboembolic pulmonary hypertension. (21594826)
2011
16
The incidence of recurrent venous thromboembolism and chronic thromboembolic pulmonary hypertension following a first episode of pulmonary embolism. (21743331)
2011
17
Surgical outcome of two difficult cases with predominant proximal pulmonary artery lesions of chronic thromboembolic pulmonary hypertension. (23555449)
2011
18
Recent advances in chronic thromboembolic pulmonary hypertension. (21620730)
2011
19
Chronic thromboembolic pulmonary hypertension (CTEPH): updated Recommendations of the Cologne Consensus Conference 2011. (22221974)
2011
20
Single photon emission computed tomography in chronic thromboembolic pulmonary hypertension. (20920137)
2011
21
Clinical and haemodynamic evaluation of chronic thromboembolic pulmonary hypertension patients scheduled for pulmonary thromboendarterectomy: Is schistosomiasis hypertension an important confounding factor? (21243290)
2010
22
Clinical features and management of chronic thromboembolic pulmonary hypertension in Thai. (20364561)
2010
23
Bosentan for chronic thromboembolic pulmonary hypertension: findings from a systematic review and meta-analysis. (20172593)
2010
24
Long-term outcome after pulmonary endarterectomy for chronic thromboembolic pulmonary hypertension: a single institution experience. (19272789)
2009
25
HLA-DPB1 and NFKBIL1 may confer the susceptibility to chronic thromboembolic pulmonary hypertension in the absence of deep vein thrombosis. (19165231)
2009
26
Role for staphylococci in misguided thrombus resolution of chronic thromboembolic pulmonary hypertension. (18239156)
2008
27
Thromboendarterectomy for severe chronic thromboembolic pulmonary hypertension. (17540993)
2007
28
Role of NT-proBNP and 6MWD in chronic thromboembolic pulmonary hypertension. (17706409)
2007
29
Long-term bosentan in chronic thromboembolic pulmonary hypertension. (16699254)
2007
30
Chronic thromboembolic pulmonary hypertension - assessment by magnetic resonance imaging. (16838142)
2007
31
Effects of pulmonary thromboendarterectomy on right-sided echocardiographic parameters in patients with chronic thromboembolic pulmonary hypertension. (16770978)
2006
32
Assessment of operability in chronic thromboembolic pulmonary hypertension. (16963538)
2006
33
Angiotensin-converting enzyme gene polymorphisms and prognosis in chronic thromboembolic pulmonary hypertension. (16936432)
2006
34
Pulmonary artery-to-pulmonary artery anastomoses: angiographic demonstration in patients with chronic thromboembolic pulmonary hypertension. (16488207)
2006
35
Long-term recovery of exercise ability after pulmonary endarterectomy for chronic thromboembolic pulmonary hypertension. (16996930)
2006
36
Bosentan in inoperable chronic thromboembolic pulmonary hypertension. (16061720)
2005
37
Medical conditions increasing the risk of chronic thromboembolic pulmonary hypertension. (15735803)
2005
38
Chronic thromboembolic pulmonary hypertension. (15302364)
2004
39
Prostacyclin therapy before pulmonary thromboendarterectomy in patients with chronic thromboembolic pulmonary hypertension. (12576349)
2003
40
Thrombophilic factors in chronic thromboembolic pulmonary hypertension. (11555695)
2001
41
Chronic thromboembolic pulmonary hypertension. (11590849)
2001
42
Plasma monocyte chemoattractant protein-1 and pulmonary vascular resistance in chronic thromboembolic pulmonary hypertension. (11463608)
2001
43
Three-dimensional visualization of pulmonary thromboemboli in chronic thromboembolic pulmonary hypertension with multiple detector-row spiral computed tomography. (11413092)
2001
44
Surgical treatment of chronic thromboembolic pulmonary hypertension. (10700671)
2000
45
Chronic Thromboembolic Pulmonary Hypertension. (11096519)
2000
46
Surgical treatment for chronic thromboembolic pulmonary hypertension under profound hypothermia and circulatory arrest in 24 patients. (10875595)
1999
47
A successful case of pulmonary thromboendarterectomy for chronic thromboembolic pulmonary hypertension with a thrombus in the right ventricle. (10333425)
1999
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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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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Pathways related to Chronic Thromboembolic Pulmonary Hypertension according to GeneCards Suite gene sharing:

idSuper pathwaysScoreTop Affiliating Genes
19.5BMPR2, NPPB
29.2EDN1, NPPB
39.1EDN1, PTGIS
4
Show member pathways
9.0CRP, EDN1

GO Terms for genes affiliated with Chronic Thromboembolic Pulmonary Hypertension

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

idNameGO IDScoreTop Affiliating Genes
1cellular response to interleukin-6GO:00713549.9FGA, PTGIS
2prostaglandin biosynthetic processGO:00015169.6EDN1, PTGIS
3negative regulation of cell growthGO:00303089.5BMPR2, NPPB
4positive regulation of urine volumeGO:00358109.4EDN1, NPPB

Sources for Chronic Thromboembolic Pulmonary Hypertension

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2CDC
14ExPASy
15FDA
16FMA
24GTR
25HGMD
26HMDB
27ICD10
28ICD10 via Orphanet
29ICD9CM
30IUPHAR
31KEGG
34MedGen
36MeSH
37MESH via Orphanet
38MGI
41NCI
42NCIt
43NDF-RT
46NINDS
47Novoseek
49OMIM
50OMIM via Orphanet
54PubMed
55QIAGEN
60SNOMED-CT via Orphanet
64Tumor Gene Family of Databases
65UMLS
66UMLS via Orphanet