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CTEPH
MCID: CHR466
MIFTS: 48

Chronic Thromboembolic Pulmonary Hypertension (CTEPH)

Categories: Blood diseases, Cardiovascular diseases, Rare diseases, Respiratory diseases
Genes Tissues Related diseases Publications Pathways Symptoms & Phenotypes Drugs
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Aliases & Classifications for Chronic Thromboembolic Pulmonary Hypertension

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MalaCards integrated aliases for Chronic Thromboembolic Pulmonary Hypertension:

Name: Chronic Thromboembolic Pulmonary Hypertension 20 36 71
Cteph 20

Classifications:

MalaCards categories:
Global: Rare diseases
Anatomical: Respiratory diseases Cardiovascular diseases Blood diseases
See all MalaCards categories (disease lists)


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Summaries for Chronic Thromboembolic Pulmonary Hypertension

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KEGG : 36 Chronic thromboembolic pulmonary hypertension (CTEPH) is classified as group 4 pulmonary hypertension (PH) by the World Health Organization. It is a rare, but underdiagnosed, complication of acute pulmonary embolism (PE) resulting from occlusion of large pulmonary arteries with a fibrothrombotic material and in many cases, the development of a distal vessel arteriopathy that closely mimics pulmonary arterial hypertension. While the pathogenesis of CTEPH is not entirely understood, the primary pathophysiology appears to be related to an initial thrombotic event followed by both inadequate thrombus resolution and secondary arteriopathy. Several risk factors for development of CTEPH have been found, including the size of the initial PE, splenectomy, chronic inflammatory conditions, and indwelling catheters and cardiac pacemaker leads. There is however an increased incidence of elevated factor VIII levels in CTEPH, and this finding persists after treatment. However, none of these findings explain the development of CTEPH in the majority of patients affected by this disease.

MalaCards based summary : Chronic Thromboembolic Pulmonary Hypertension, also known as cteph, is related to pulmonary embolism and pulmonary hypertension. An important gene associated with Chronic Thromboembolic Pulmonary Hypertension is LINC01094 (Long Intergenic Non-Protein Coding RNA 1094), and among its related pathways/superpathways are Development Angiotensin activation of ERK and Platelet Aggregation Inhibitor Pathway, Pharmacodynamics. The drugs Selexipag and Treprostinil have been mentioned in the context of this disorder. Affiliated tissues include lung, endothelial and heart, and related phenotypes are congestive heart failure and pulmonary arterial hypertension

GARD : 20 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).

Wikipedia : 74 Chronic thromboembolic pulmonary hypertension (CTEPH) is a long-term disease caused by a blockage in the... more...

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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 221)
# Related Disease Score Top Affiliating Genes
1 pulmonary embolism 31.1 NPPB FGB CRP
2 pulmonary hypertension 30.8 NPPB LINC01094 FGA EDN1 CRP BMPR2
3 pulmonary edema 30.3 NPPB EDN1 CRP
4 pericardial effusion 30.2 NPPB CRP
5 eisenmenger syndrome 30.2 EDN1 BMPR2
6 pulmonary venoocclusive disease 30.2 NPPB EDN1 BMPR2
7 cardiac tamponade 30.0 NPPB CRP
8 arteriovenous malformation 30.0 EDN1 BMPR2
9 lung disease 30.0 NPPB EDN1 CRP
10 pulmonary artery disease 30.0 NPPB CRP
11 tricuspid valve insufficiency 30.0 NPPB EDN1 CRP BMPR2
12 congestive heart failure 29.9 NPPB EDN1 CRP
13 portal hypertension 29.8 EDN1 BMPR2
14 central sleep apnea 29.8 NPPB CRP
15 respiratory failure 29.8 NPPB FGA EDN1 CRP
16 sleep apnea 29.8 NPPB EDN1 CRP
17 connective tissue disease 29.8 EDN1 CRP BMPR2
18 takayasu arteritis 29.7 EDN1 CRP
19 vascular disease 29.6 NPPB FGA EDN1 CRP BMPR2
20 thrombocytosis 29.5 FGA CRP
21 lipoprotein quantitative trait locus 29.4 NPPB FGA EDN1 CRP
22 chronic kidney disease 29.2 NPPB EDN1 CRP
23 patent foramen ovale 29.1 NPPB FGA EDN1 CRP
24 cerebrovascular disease 29.1 NPPB FGB FGA CRP
25 hypertension, essential 28.9 NPPB EDN1 CRP BMPR2
26 pulmonary hypertension, chronic thromboembolic, without deep vein thrombosis 11.9
27 pulmonary hypertension, primary, 1 11.2
28 thrombophilia due to thrombin defect 10.9
29 cardiac arrest 10.9
30 antiphospholipid syndrome 10.7
31 thrombosis 10.6
32 syncope 10.6
33 thrombophilia 10.6
34 hemopericardium 10.4
35 hypothyroidism 10.4
36 vasculitis 10.4
37 sarcoidosis 1 10.4
38 pulmonary disease, chronic obstructive 10.4
39 hydrocephalus 10.4
40 sarcoma 10.4
41 heart disease 10.4
42 spindle cell sarcoma 10.4
43 mediastinitis 10.4
44 fibrosing mediastinitis 10.4
45 cardiogenic shock 10.4
46 arteries, anomalies of 10.3
47 fibrosis of extraocular muscles, congenital, 1 10.3
48 multinucleated neurons, anhydramnios, renal dysplasia, cerebellar hypoplasia, and hydranencephaly 10.3
49 hepatic veno-occlusive disease 10.3
50 thrombocytopenia 10.3

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



Diseases related to Chronic Thromboembolic Pulmonary Hypertension
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Symptoms & Phenotypes for Chronic Thromboembolic Pulmonary Hypertension

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Human phenotypes related to Chronic Thromboembolic Pulmonary Hypertension:

31 (show all 34)
# Description HPO Frequency HPO Source Accession
1 congestive heart failure 31 hallmark (90%) HP:0001635
2 pulmonary arterial hypertension 31 hallmark (90%) HP:0002092
3 increased pulmonary vascular resistance 31 hallmark (90%) HP:0005317
4 fatigue 31 frequent (33%) HP:0012378
5 pulmonary embolism 31 frequent (33%) HP:0002204
6 reduced vital capacity 31 frequent (33%) HP:0002792
7 exertional dyspnea 31 frequent (33%) HP:0002875
8 deep venous thrombosis 31 frequent (33%) HP:0002625
9 palpitations 31 frequent (33%) HP:0001962
10 reduced fev1/fvc ratio 31 frequent (33%) HP:0030877
11 depressivity 31 occasional (7.5%) HP:0000716
12 obesity 31 occasional (7.5%) HP:0001513
13 syncope 31 occasional (7.5%) HP:0001279
14 right bundle branch block 31 occasional (7.5%) HP:0011712
15 elevated c-reactive protein level 31 occasional (7.5%) HP:0011227
16 edema 31 occasional (7.5%) HP:0000969
17 abnormal t-wave 31 occasional (7.5%) HP:0005135
18 antiphospholipid antibody positivity 31 occasional (7.5%) HP:0003613
19 central sleep apnea 31 occasional (7.5%) HP:0010536
20 increased hdl cholesterol concentration 31 occasional (7.5%) HP:0012184
21 right ventricular failure 31 occasional (7.5%) HP:0001708
22 lupus anticoagulant 31 occasional (7.5%) HP:0025343
23 right ventricular dilatation 31 occasional (7.5%) HP:0005133
24 cardiac shunt 31 occasional (7.5%) HP:0001693
25 recurrent thromboembolism 31 occasional (7.5%) HP:0004831
26 dysfibrinogenemia 31 occasional (7.5%) HP:0011901
27 abnormality of von willebrand factor 31 occasional (7.5%) HP:0012146
28 hypocapnia 31 occasional (7.5%) HP:0012417
29 right atrial enlargement 31 occasional (7.5%) HP:0030718
30 increased factor viii activity 31 occasional (7.5%) HP:0030977
31 abnormal left ventricular function 31 occasional (7.5%) HP:0005162
32 inflammation of the large intestine 31 very rare (1%) HP:0002037
33 osteomyelitis 31 very rare (1%) HP:0002754
34 myeloproliferative disorder 31 very rare (1%) HP:0005547
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Drugs & Therapeutics for Chronic Thromboembolic Pulmonary Hypertension

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

(show all 45)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Selexipag Approved Phase 3 475086-01-2
2
Treprostinil Approved, Investigational Phase 3 81846-19-7 54786 6918140
3
Edoxaban Approved Phase 3 480449-70-5
4
Warfarin Approved Phase 3 81-81-2 6691 54678486
5
Macitentan Approved Phase 3 441798-33-0
6
Ambrisentan Approved, Investigational Phase 3 177036-94-1 6918493
7 Calamus Phase 3
8 Anticoagulants Phase 3
9 Antithrombins Phase 3
10 Serine Proteinase Inhibitors Phase 3
11 HIV Protease Inhibitors Phase 3
12 Factor Xa Inhibitors Phase 3
13
protease inhibitors Phase 3
14 Antithrombin III Phase 3
15 Endothelin A Receptor Antagonists Phase 3
16 Endothelin Receptor Antagonists Phase 3
17 Antihypertensive Agents Phase 3
18
Serine Investigational, Nutraceutical Phase 3 56-45-1 5951
19
Riociguat Approved Phase 2 625115-55-1
20 Radiopharmaceuticals Phase 2
21
Nitric Oxide Approved Phase 1 10102-43-9 145068
22
Iodine Approved, Investigational 7553-56-2 807
23
Acetylcysteine Approved, Investigational 616-91-1 12035
24
Thrombin Approved, Investigational
25
Iloprost Approved, Investigational 78919-13-8 6443959
26
Epoprostenol Approved 61849-14-7, 35121-78-9 5280427 5282411
27
Tadalafil Approved, Investigational 171596-29-5 110635
28
Bosentan Approved, Investigational 147536-97-8 104865
29
Cysteine Approved, Nutraceutical 52-90-4 5862
30
Cadexomer iodine Experimental 94820-09-4
31 Tezosentan Investigational 180384-57-0
32 Protective Agents
33 Respiratory System Agents
34 Antioxidants
35 Anti-Infective Agents
36 Antiviral Agents
37 Expectorants
38 N-monoacetylcystine
39 Antidotes
40 Hemostatics
41 Coagulants
42 Phosphodiesterase 5 Inhibitors
43 Sildenafil Citrate 171599-83-0
44 Fluorodeoxyglucose F18
45 Deoxyglucose

Interventional clinical trials:

(show top 50) (show all 74)
# Name Status NCT ID Phase Drugs
1 CHANGE-MRI (CTEPH DIAGNOSIS Europe - MRI) Unknown status NCT02791282 Phase 3
2 Prospective, Randomized, Placebo-controlled, Double-blind, Multicenter, Parallel Group Study to Assess the Efficacy, Safety and Tolerability of Bosentan in Patients With Inoperable Chronic Thromboembolic Pulmonary Hypertension (CTEPH) Completed NCT00313222 Phase 3 bosentan
3 Long-term Open-label Extension Study in Patients With Inoperable Chronic Thromboembolic Pulmonary Hypertension (CTEPH) Who Completed Protocol AC-052-366 (BENEFIT, NCT00313222) Completed NCT00319111 Phase 3 bosentan
4 Randomized, Double-blind, Placebo-controlled, Multi-centre, Multi-national Study to Evaluate the Efficacy and Safety of Oral BAY63-2521 (1 mg, 1.5 mg, 2 mg, or 2.5 mg Tid) in Patients With Chronic Thromboembolic Pulmonary Hypertension (CTEPH) Completed NCT00855465 Phase 3 Riociguat (Adempas, BAY63-2521);Placebo
5 Long-term Extension, Multicentre, Multi-international Study to Evaluate the Safety and Tolerability of Oral BAY63-2521 (1mg, 1.5 mg, 2.0 mg, 2.5 mg Tid) in Patients With Chronic Thromboembolic Pulmonary Hypertension (CTEPH). Completed NCT00910429 Phase 3 Riociguat (Adempas, BAY63-2521)
6 Effect of Long-term Oxygen Therapy on Exercise Capacity and Quality of Life in Patients With Pulmonary Arterial and Chronic Thromboembolic Pulmonary Hypertension Completed NCT01884012 Phase 3
7 A Prospective, Randomized, Double-blind, Multicenter, Placebo-controlled, Parallel Group, Adaptive Phase 3 Study With Open-label Extension to Evaluate Efficacy and Safety of Macitentan 75 mg in Inoperable or Persistent/Recurrent Chronic Thromboembolic Pulmonary Hypertension Recruiting NCT04271475 Phase 3 Macitentan;Placebo
8 A Multicenter, Randomized, Double-blind, Placebo-controlled, Parallel-group, Group-sequential, Adaptive, Phase 3 Study With Open-label Extension Period to Assess the Efficacy and Safety of Selexipag as an add-on to Standard of Care Therapy in Subjects With Inoperable or Persistent/Recurrent After Surgical and/or Interventional Treatment Chronic Thromboembolic Pulmonary Hypertension Recruiting NCT03689244 Phase 3 Selexipag;Placebo
9 A Double Blind Controlled Clinical Study to Investigate the Efficacy and Tolerability of Subcutaneous Treprostinil Sodium in Patients With Severe Non-operable Chronic Thromboembolic Pulmonary Hypertension (CTREPH) Active, not recruiting NCT01416636 Phase 3 Treprostinil sodium
10 An Investigator-initiated, Phase III Trial, Randomized, Single-blind, Double-dummy, Parallel-group, Multi-center Study for the Evaluation of Efficacy and Safety of Warfarin Versus Edoxaban in Subjects With CTEPH Taking Warfarin: KABUKI Not yet recruiting NCT04730037 Phase 3 Edoxaban;Warfarin Potassium;Warfarin Potassium placebo;Edoxaban placebo
11 A Prospective, Multicenter, Open-label, Single Arm, Phase III Study to Assess the Efficacy and Safety of Macitentan (ACT-064992) in Subjects With Chronic Thromboembolic Pulmonary Hypertension (CTEPH) Terminated NCT03809650 Phase 3 macitentan 10 mg
12 A Randomised, Multicentre, Double-Blind, Placebo-Controlled Study Of Ambrisentan In Subjects With Inoperable Chronic Thromboembolic Pulmonary Hypertension (CTEPH). Terminated NCT01884675 Phase 3 Ambrisentan 5 mg;Placebo
13 An Open-label Extension Study of the Long-term Safety, Tolerability and Efficacy of Ambrisentan in Subjects With Inoperable Chronic Thromboembolic Pulmonary Hypertension (CTEPH) Terminated NCT01894022 Phase 3 Ambrisentan 5 mg
14 Randomized Pilot Trial of Interventional Treatment of Residual Pulmonary Hypertension in Patients After Pulmonary Thromboendarterectomy Unknown status NCT02745106 Phase 2
15 Effect of Short-term Oxygen Therapy on Cardiopulmonary Exercise Capacity in Patients With Pulmonary Arterial and Chronic Thromboembolic Pulmonary Hypertension Completed NCT01748474 Phase 2
16 Prospective, Randomized, Placebo-controlled, Double-blind, Multicenter, Parallel-group, 24-week Study to Assess the Efficacy, Safety and Tolerability of Macitentan in Subjects With Inoperable Chronic Thromboembolic Pulmonary Hypertension Completed NCT02021292 Phase 2 Macitentan;Placebo
17 Evaluation of Right Ventricular Function and Metabolism Following Riociguat for the Treatment of Chronic Thromboembolic Pulmonary Hypertension- Images of Rio Completed NCT02094001 Phase 2
18 The Effect of Riociguat for Peak Cardiac Index on Cardiopulmonary Exercise Test in CTEPH Patients After Normalization of Pulmonary Artery Pressure by Combination Treatment of Riociguat and Balloon Pulmonary Angioplasty Recruiting NCT04600492 Phase 2 Riociguat Oral Tablet
19 Prospective, Randomized, Controlled Trial of the Effect of Long-term Oxygen Therapy on 6-minute Walking Distance, Clinical Parameters and Hemodynamics in Patients With PAH and CTEPH Recruiting NCT04207593 Phase 2 Oxygen
20 MERIT-2 : Long Term, Multicenter, Single-arm, Open-label Extension Study of the MERIT-1 Study, to Assess the Safety, Tolerabilty and Efficacy of Macitentan in Subjects With Inoperable Chronic Thromboembolic Pulmonary Hypertension (CTEPH) Active, not recruiting NCT02060721 Phase 2 Macitentan
21 A Phase 2, Randomised, Double-Blind, Placebo-Controlled, Multicentre, Prospective Study to Assess Efficacy of Riociguat in Patients With Operable CTEPH Prior to Pulmonary Endarterectomy With High Preoperative Pulmonary Vascular Resistance Terminated NCT03273257 Phase 2 Riociguat;Placebo
22 A Non-randomized Two Part Multi-Center, Open-label, Single Dose Trial With an Escalation Part in Untreated Pulmonary Arterial Hypertension (PAH) and Chronic Thromboembolic Pulmonary Hypertension (CTEPH) Patients (Part A), Followed by a Parallel Group Part in Untreated and Pre-treated Patients With PAH and CTEPH (Part B) to Investigate the Safety, Tolerability and Pharmacodynamics of Inhaled BAY1237592 Recruiting NCT03754660 Phase 1 BAY1237592;PH-monotherapy;PH-combination therapy
23 Prospective Registry to Evaluate the Effective Incidence of Chronic Thromboembolic Pulmonary Hypertension in Germany Unknown status NCT02660463
24 Assessment of Safety and Efficacy of Balloon Pulmonary Angioplasty in Non-operable CTEPH Patients Unknown status NCT02964390
25 Lung Perfusion Measured With Dual-Energy CT (DECT) in Patients With Chronic Thromboembolic Pulmonary Hypertension (CTEPH): Comparative Study With Right Heart Catheterization and Ventilation/Perfusion Scintigraphy (V/Q) Unknown status NCT03450304
26 IodiNe Subtraction maPpIng in the Diagnosis of chRonic Pulmonary thromboEmbolic Disease (INSPIRE): An Observational Diagnostic Study Unknown status NCT03806907
27 Gene Mutations in Secondary Pulmonary Hypertension Unknown status NCT00348803
28 Three-dimensional Echocardiographic Assessment of Right Ventricular Function in Patients Undergoing Pulmonary Endarterectomy Unknown status NCT02426203
29 Inspiratory Muscle Training in Patients With Chronic Thromboembolic Pulmonary Hypertension: a Randomized Controlled Trial Unknown status NCT03102294
30 Local Registry of Patients With Chronic Thromboembolic Pulmonary Hypertension in Novosibirsk Region Unknown status NCT03349164
31 Long-term Right Ventricular Resynchronization Therapy for Chronic Thromboembolic Pulmonary Hypertension Unknown status NCT01163422
32 National, Prospective, Multicenter,Observational Registry Study on Pulmonary Arterial Hypertension or Chronic Thromboembolic Pulmonary Hypertension in China Unknown status NCT01417338
33 Epidemiological Study of Chronic Thromboembolic Pulmonary Hypertension in China Unknown status NCT01717092
34 Russian National Registry of Patients With Pulmonary Arterial Hypertension and Chronic Thromboembolic Pulmonary Hypertension Unknown status NCT03707561
35 Incidence of CTEPH in Dependence of Thrombus Location in Patients With Acute Pulmonary Embolism and Index Fibrinolysis Unknown status NCT01672203
36 Early Non-invasive Detection of CTEPH After Pulmonary Embolism - The InShape-2 Study Completed NCT02555137
37 United States CTEPH Registry Completed NCT02429284
38 New International CTEPH Database Completed NCT02656238
39 Riociguat Versus Balloon Pulmonary Angioplasty in Non-operable Chronic thromboEmbolic Pulmonary Hypertension Completed NCT02634203 Riociguat
40 Evaluation of Pulmonary Perfusion Heterogeneity in Patients With Chronic Thromboembolic Pulmonary Hypertension Using Functional PET Imaging Completed NCT02114047
41 A Prospective Single Blind Controlled Study to Assess the Efficacy of a Lung Protective Ventilation Strategy in the Prevention of Reperfusion Lung Injury Following PTE Completed NCT00747045
42 Identification Of The Predictive Angiographic And Computed Tomographic (CT) Criteria Of The Success Of The Thromboendarterectomy In Chronic Pulmonary Hypertension Completed NCT00657722
43 The Right Ventricle in Chronic Pressure Overload: Identifying Novel Molecular Targets for Functional Imaging Completed NCT03199131
44 Efficacy of Medical Therapy for Pulmonary Arterial and Inoperable Chronic Thromboembolic Pulmonary Hypertension in a Real Life Setting Completed NCT03198910
45 Role of Inflammation and Angiogenesis in Chronic Thromboembolic Pulmonary Hypertension Completed NCT04472533
46 EMEA CTEPH Registry: An International Prospective Registry Investigating the Epidemiology, Diagnosis and Treatment of CTEPH Patients in EMEA Countries Completed NCT02637050
47 Identification of CTEPH on Standard Computerised Tomography Pulmonary Angiography Performed in the Work-up of Suspected Pulmonary Embolism Completed NCT03083093
48 Retrospective Chart Review of Patients With PAH or Inoperable/Persistent/Recurrent CTEPH Who Transition Their PH Treatment to Adempas Completed NCT02545465 Riociguat (Adempas, BAY63-2521)
49 The Precise Surgical Treatment of Chronic Thromboembolic Pulmonary Hypertension: Pulmonary Endarterectomy Guided by Three-dimensional Pulmonary Angiography Recruiting NCT03795740
50 Sleep-Disordered Breathing in Chronic Thromboembolic Pulmonary Hypertension - a Prospective, Descriptive Cohort Study Recruiting NCT03074539 Riociguat

Search NIH Clinical Center for Chronic Thromboembolic Pulmonary Hypertension

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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:

40
Lung, Endothelial, Heart, Smooth Muscle, Neutrophil, Lymph Node, Spleen
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Publications for Chronic Thromboembolic Pulmonary Hypertension

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Articles related to Chronic Thromboembolic Pulmonary Hypertension:

(show top 50) (show all 2193)
# Title Authors PMID Year
1
The Pathobiology of Chronic Thromboembolic Pulmonary Hypertension. 20 61
27571003 2016
2
Chronic Thromboembolic Disease and Chronic Thromboembolic Pulmonary Hypertension. 61
33541619 2021
3
The role of balloon pulmonary angioplasty and pulmonary endarterectomy: Is chronic thromboembolic pulmonary hypertension still a life-threatening disease? 61
33086125 2021
4
Three treatment options and the combination for patients with chronic thromboembolic pulmonary hypertension - established or not. 61
33221350 2021
5
Pulmonary angioplasty: A step further in the continuously changing landscape of chronic thromboembolic pulmonary hypertension management. 61
33524919 2021
6
Systematic Staged Percutaneous Balloon Pulmonary Angioplasty in Severe Inoperable Chronic Thromboembolic Pulmonary Hypertension. 61
33566998 2021
7
Clinical characteristics and aetiological analysis of 133 patients for pulmonary embolism combined with haemoptysis. 61
32975914 2021
8
Determinants and Management of the Post-Pulmonary Embolism Syndrome. 61
33548930 2021
9
Effect of EZH2 on pulmonary artery smooth muscle cell migration in pulmonary hypertension. 61
33313943 2021
10
NEDD9 is a Novel and Modifiable Mediator of Platelet-Endothelial Adhesion in the Pulmonary Circulation. 61
33523764 2021
11
Evaluating the Right Ventricle in Acute and Chronic Pulmonary Embolism: Current and Future Considerations. 61
33548932 2021
12
Effect of Normobaric Hypoxia on Exercise Performance in Pulmonary Hypertension: Randomized Trial. 61
32918899 2021
13
'Surgery first' if cure is your aim in CTEPH. 61
33073458 2021
14
Characteristics and long-term survival of patients with chronic thromboembolic pulmonary hypertension in China. 61
32954622 2021
15
Balloon pulmonary angioplasty vs. pulmonary endarterectomy in patients with chronic thromboembolic pulmonary hypertension: a systematic review and meta-analysis. 61
33544306 2021
16
Evolution of patients with chronic thromboembolic pulmonary hypertension treated by balloon pulmonary angioplasty, according to their anticoagulant regimens. 61
33582861 2021
17
Treatment effect prediction using CT after balloon pulmonary angioplasty in chronic thromboembolic pulmonary hypertension. 61
33569619 2021
18
Correlation Between Perfusion Abnormalities Extent in Ventilation/Perfusion SPECT/CT with Hemodynamic Parameters in Patients with Chronic Thromboembolic Pulmonary Hypertension 61
33586404 2021
19
Possible immune regulation mechanisms for the progression of chronic thromboembolic pulmonary hypertension. 61
33316641 2021
20
Pulmonary atherosclerosis in a patient with chronic thromboembolic pulmonary hypertension. 61
33316138 2021
21
Risk stratification in chronic thromboembolic pulmonary hypertension predicts survival. 61
32586166 2021
22
Chronic thromboembolic pulmonary hypertension and totally implantable central venous access systems. 61
32675214 2021
23
Recurrent chronic thromboembolic disease despite optimal anticoagulation in setting of recent COVID-19 infection. 61
33509878 2021
24
Pulmonary manifestations of antiphospholipid syndrome: a retrospective analysis of 67 patients. 61
33386561 2021
25
Does kidney function matter in pulmonary thromboembolism management? 61
33470418 2021
26
Sleep-related breathing disorders and pulmonary hypertension. 61
32747397 2021
27
Clinical Significance of Guanylate Cyclase Stimulator, Riociguat, on Right Ventricular Functional Improvement in Patients with Pulmonary Hypertension. 61
33238268 2021
28
Peripheral microangiopathy in precapillary pulmonary hypertension: a nailfold video capillaroscopy prospective study. 61
33478514 2021
29
An evaluation of selexipag for the treatment of pulmonary hypertension. 61
32867545 2021
30
Balloon pulmonary angioplasty in the treatment of chronic thromboembolic pulmonary hypertension: recent advances and future perspectives. 61
33463996 2021
31
Comparison of 68Ga-FAPI imaging and cardiac magnetic resonance in detection of myocardial fibrosis in a patient with chronic thromboembolic pulmonary hypertension. 61
33474696 2021
32
Epidemiology of pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension: identification of the most accurate estimates from a systematic literature review. 61
33456755 2021
33
Direct oral anticoagulant therapy in patients with morbid obesity after intermediate- or high-risk pulmonary emboli. 61
33569503 2021
34
Evaluation of microvasculopathy using dual-energy computed tomography in patients with chronic thromboembolic pulmonary hypertension. 61
33532057 2021
35
Chronic thromboembolic pulmonary hypertension in patients with persistent chest symptoms after acute pulmonary embolism. 61
33382052 2021
36
Patient Journey and Disease-Related Burden in Japanese Patients With Chronic Thromboembolic Pulmonary Hypertension: A Mixed Methods Study. 61
33476859 2021
37
Chronic thromboembolic pulmonary hypertension: evaluation of V/Q SPECT/CT and V/Q Quotient SPECT findings with postoperative results of pulmonary endarterectomy. 61
33443395 2021
38
Soluble ST2 as a Biomarker for Early Complications in Patients with Chronic Thromboembolic Pulmonary Hypertension Treated with Balloon Pulmonary Angioplasty. 61
33467121 2021
39
When Imaging in Chronic Thromboembolic Pulmonary Hypertension Is Not Enough. 61
33026826 2021
40
Model of Chronic Thromboembolic Pulmonary Hypertension in Rats Caused by Repeated Intravenous Administration of Partially Biodegradable Sodium Alginate Microspheres. 61
33498971 2021
41
Right Ventricular Extracellular Volume with Dual-Layer Spectral Detector CT: Value in Chronic Thromboembolic Pulmonary Hypertension. 61
33497315 2021
42
Radiological differences between chronic thromboembolic pulmonary disease (CTEPD) and chronic thromboembolic pulmonary hypertension (CTEPH). 61
33507354 2021
43
Chronic thromboembolic pulmonary hypertension due to an implantable cardioverter-defibrillator's lead thrombosis. 61
33524614 2021
44
Interventional and pharmacological management of chronic thromboembolic pulmonary hypertension. 61
33465538 2021
45
Early return of reflected waves increases right ventricular wall stress in chronic thromboembolic pulmonary hypertension. 61
33035435 2020
46
A Prospective, Comparative Study of Ventilation-Perfusion Planar Imaging and Ventilation-Perfusion SPECT for Chronic Thromboembolic Pulmonary Hypertension. 61
32198309 2020
47
ERS Statement on Chronic Thromboembolic Pulmonary Hypertension. 61
33334946 2020
48
Neutrophil extracellular traps promote fibrous vascular occlusions in chronic thrombosis. 61
33512471 2020
49
Prediction Score for persisting perfusion defects after pulmonary embolism. 61
31551608 2020
50
Application of DynaCT angiographic reconstruction in balloon pulmonary angioplasty. 61
32621239 2020
Sources

Variations for Chronic Thromboembolic Pulmonary Hypertension

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Expression for Chronic Thromboembolic Pulmonary Hypertension

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

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

# Super pathways Score Top Affiliating Genes
1
Development Angiotensin activation of ERK 23
Show member pathways
 12.26 FGB FGA EDN1
2
Platelet Aggregation Inhibitor Pathway, Pharmacodynamics 60
10.96 FGB FGA
3
Cell adhesion_Plasmin signaling 23
10.85 FGB FGA
4
Development_Leptin signaling via JAK/STAT and MAPK cascades 23
Show member pathways
 10.43 EDN1 CRP
5
Cardiomyocyte Differentiation through BMP Receptors 63
9.97 NPPB BMPR2
Sources

GO Terms for Chronic Thromboembolic Pulmonary Hypertension

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

# Name GO ID Score Top Affiliating Genes
1 extracellular region GO:0005576 9.72 NPPB FGB FGA EDN1 CRP
2 platelet alpha granule lumen GO:0031093 9.26 FGB FGA
3 platelet alpha granule GO:0031091 9.16 FGB FGA
4 extracellular space GO:0005615 9.1 NPPB FGB FGA EDN1 CRP BMPR2
5 fibrinogen complex GO:0005577 8.96 FGB FGA

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

(show all 25)
# Name GO ID Score Top Affiliating Genes
1 regulation of blood pressure GO:0008217 9.62 NPPB EDN1
2 positive regulation of endothelial cell migration GO:0010595 9.62 EDN1 BMPR2
3 response to calcium ion GO:0051592 9.61 FGB FGA
4 hemostasis GO:0007599 9.61 FGB FGA
5 positive regulation of protein secretion GO:0050714 9.6 FGB FGA
6 toll-like receptor signaling pathway GO:0002224 9.59 FGB FGA
7 platelet aggregation GO:0070527 9.58 FGB FGA
8 positive regulation of substrate adhesion-dependent cell spreading GO:1900026 9.58 FGB FGA
9 negative regulation of extrinsic apoptotic signaling pathway via death domain receptors GO:1902042 9.57 FGB FGA
10 negative regulation of endothelial cell apoptotic process GO:2000352 9.56 FGB FGA
11 positive regulation of vasoconstriction GO:0045907 9.55 FGB FGA
12 positive regulation of exocytosis GO:0045921 9.54 FGB FGA
13 cellular protein-containing complex assembly GO:0034622 9.52 FGB FGA
14 fibrinolysis GO:0042730 9.51 FGB FGA
15 vasoconstriction GO:0042310 9.49 EDN1 CRP
16 positive regulation of heterotypic cell-cell adhesion GO:0034116 9.48 FGB FGA
17 protein polymerization GO:0051258 9.46 FGB FGA
18 positive regulation of renal sodium excretion GO:0035815 9.43 NPPB EDN1
19 positive regulation of urine volume GO:0035810 9.4 NPPB EDN1
20 negative regulation of systemic arterial blood pressure GO:0003085 9.37 NPPB BMPR2
21 plasminogen activation GO:0031639 9.32 FGB FGA
22 positive regulation of peptide hormone secretion GO:0090277 9.26 FGB FGA
23 body fluid secretion GO:0007589 9.16 NPPB EDN1
24 blood coagulation, fibrin clot formation GO:0072378 8.96 FGB FGA
25 induction of bacterial agglutination GO:0043152 8.62 FGB FGA

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

# Name GO ID Score Top Affiliating Genes
1 cell adhesion molecule binding GO:0050839 8.96 FGB FGA
2 signaling receptor binding GO:0005102 8.92 NPPB FGB FGA EDN1
Sources

Sources for Chronic Thromboembolic Pulmonary Hypertension

About this section
3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
20 GARD
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
53 NINDS
54 Novoseek
56 OMIM via Orphanet
57 OMIM® (Updated 05-Mar-2021)
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
The MalaCards human disease database index: 1-9 A B C D E F G H I J K L M N O P Q R S T U V W X Y Z
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