CTEPH
MCID: CHR466
MIFTS: 45

Chronic Thromboembolic Pulmonary Hypertension (CTEPH)

Categories: Blood diseases, Rare diseases, Respiratory diseases

Aliases & Classifications for Chronic Thromboembolic Pulmonary Hypertension

Summaries for Chronic Thromboembolic Pulmonary Hypertension

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 hypertension, primary, 1 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_Leptin signaling via JAK/STAT and MAPK cascades and Cardiomyocyte Differentiation through BMP Receptors. The drugs Tissue Plasminogen Activator and Plasminogen have been mentioned in the context of this disorder. Affiliated tissues include lung, heart and endothelial, and related phenotypes are pulmonary arterial hypertension and congestive heart failure

NIH Rare Diseases : 52 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).

Related Diseases for Chronic Thromboembolic Pulmonary Hypertension

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

(show top 50) (show all 201)
# Related Disease Score Top Affiliating Genes
1 pulmonary hypertension, primary, 1 31.2 NPPB EDN1 BMPR2
2 pulmonary hypertension 31.1 NPPB EDN1 CRP BMPR2
3 pulmonary embolism 30.8 NPPB CRP CPB2
4 cardiogenic shock 30.4 NPPB CRP
5 pericardial effusion 30.3 NPPB CRP
6 pulmonary edema 30.3 NPPB EDN1 CRP
7 cardiac tamponade 30.2 NPPB CRP
8 lung disease 30.1 NPPB EDN1 CRP
9 eisenmenger syndrome 30.0 EDN1 BMPR2
10 pulmonary venoocclusive disease 30.0 NPPB EDN1 BMPR2
11 central sleep apnea 30.0 NPPB CRP
12 arteriovenous malformation 29.8 EDN1 BMPR2
13 sleep apnea 29.8 NPPB EDN1 CRP
14 congestive heart failure 29.7 NPPB EDN1 CRP
15 tricuspid valve insufficiency 29.7 NPPB EDN1 CRP BMPR2
16 respiratory failure 29.7 NPPB FGA EDN1 CRP
17 connective tissue disease 29.6 EDN1 CRP BMPR2
18 thrombocytosis 29.6 FGA CRP
19 takayasu arteritis 29.5 EDN1 CRP
20 cerebrovascular disease 29.5 NPPB FGA CRP
21 portal hypertension 29.5 EDN1 BMPR2
22 interstitial lung disease 29.4 NPPB EDN1 CRP
23 arteries, anomalies of 29.4 NPPB EDN1 CRP
24 coronary artery anomaly 29.4 NPPB EDN1 CRP
25 pulmonary artery disease 29.3 NPPB CRP CPB2
26 vascular disease 29.2 NPPB FGA EDN1 CRP BMPR2
27 heart disease 29.0 NPPB EDN1 CRP BMPR2
28 patent foramen ovale 29.0 NPPB FGA EDN1 CRP
29 hypertension, essential 28.6 NPPB EDN1 CRP BMPR2
30 pulmonary hypertension, chronic thromboembolic, without deep vein thrombosis 12.3
31 cardiac arrest 10.8
32 antiphospholipid syndrome 10.5
33 thrombophilia due to thrombin defect 10.5
34 thrombosis 10.4
35 thrombophilia 10.4
36 syncope 10.4
37 hypothyroidism 10.3
38 hydrocephalus 10.3
39 hemopericardium 10.3
40 vasculitis 10.3
41 pulmonary venoocclusive disease 1, autosomal dominant 10.2
42 pulmonary disease, chronic obstructive 10.2
43 hepatic veno-occlusive disease 10.2
44 sarcoma 10.2
45 thrombocytopenia 10.2
46 spindle cell sarcoma 10.2
47 mediastinitis 10.2
48 heritable pulmonary arterial hypertension 10.2
49 fibrosing mediastinitis 10.2
50 intracranial hypertension 10.1 NPPB 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

Human phenotypes related to Chronic Thromboembolic Pulmonary Hypertension:

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

Drugs & Therapeutics for Chronic Thromboembolic Pulmonary Hypertension

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

(show top 50) (show all 85)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1 Tissue Plasminogen Activator Phase 4
2 Plasminogen Phase 4
3
Selexipag Approved Phase 3 475086-01-2
4
Treprostinil Approved, Investigational Phase 3 81846-19-7 54786 6918140
5
Ambrisentan Approved, Investigational Phase 3 177036-94-1 6918493
6 Calamus Phase 3
7 Endothelin A Receptor Antagonists Phase 3
8 Endothelin Receptor Antagonists Phase 3
9 Antihypertensive Agents Phase 3
10
Riociguat Approved Phase 2 625115-55-1
11 Xenon Experimental Phase 2 7440-63-3
12 Fluorodeoxyglucose F18 Phase 2
13 Radiopharmaceuticals Phase 2
14 Anesthetics Phase 2
15 Central Nervous System Depressants Phase 2
16 Anesthetics, General Phase 2
17 Anesthetics, Inhalation Phase 2
18
Nitric Oxide Approved Phase 1 10102-43-9 145068
19
Heparin Approved, Investigational 9005-49-6 772 46507594
20
Phylloquinone Approved, Investigational 84-80-0
21
Edoxaban Approved 480449-70-5
22
Thrombin Approved, Investigational
23
Warfarin Approved 81-81-2 6691 54678486
24
Rivaroxaban Approved 366789-02-8
25
Acetylcysteine Approved, Investigational 616-91-1 12035
26
Macitentan Approved 441798-33-0
27
Bosentan Approved, Investigational 147536-97-8 104865
28
Iloprost Approved, Investigational 78919-13-8 6443959
29
Tadalafil Approved, Investigational 171596-29-5 110635
30
Iodine Approved, Investigational 7553-56-2 807
31
Streptokinase Approved, Investigational 9002-01-1
32
Dopamine Approved 51-61-6, 62-31-7 681
33
Methamphetamine Approved, Illicit 537-46-2 10836
34
Menadione Approved, Nutraceutical 58-27-5 4055
35
Calcium Approved, Nutraceutical 7440-70-2 271
36
Cysteine Approved, Nutraceutical 52-90-4 5862
37 Menaquinone Investigational 1182-68-9
38 Tezosentan Investigational 180384-57-0
39 Deoxyglucose
40 Antimetabolites
41 Immunoglobulins
42 Autoantibodies
43 Antibodies
44 Vitamins
45 Antithrombins
46 HIV Protease Inhibitors
47
protease inhibitors
48 Serine Proteinase Inhibitors
49 Hemostatics
50 Coagulants

Interventional clinical trials:

(show top 50) (show all 120)
# Name Status NCT ID Phase Drugs
1 Efficacy and Safety Evaluation of 12-h and 2-h Urokinase Regimes in the Treatment of Pulmonary Thromboembolism: A Multi-Center, Randomized Controlled Trial in China Completed NCT00799968 Phase 4 Urokinase;Urokinase
2 Efficacy and Safety Evaluation of Low Dosage of Recombinant Tissue Plasminogen Activator (rt-PA) in the Treatment of Pulmonary Thromboembolism: A Multi-Center, Randomized Controlled Trial in China Completed NCT00781378 Phase 4 rt-PA;rt-PA
3 Influence of Respiratory and Exercise Therapy on Oxygen Uptake, Quality of Life and Right Heart Function in Chronic Thromboembolic Pulmonary Hypertension After Thromboendarterectomy Unknown status NCT01393327 Phase 3
4 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
5 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
6 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
7 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)
8 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
9 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) Recruiting NCT03809650 Phase 3 macitentan 10 mg
10 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
11 CHANGE-MRI (CTEPH DIAGNOSIS Europe - MRI) Recruiting NCT02791282 Phase 3
12 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
13 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
14 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
15 Randomized Pilot Trial of Interventional Treatment of Residual Pulmonary Hypertension in Patients After Pulmonary Thromboendarterectomy Unknown status NCT02745106 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 Cell Proliferation in Pulmonary Hypertension. FDG-PET Comparison Between Patients and Healthy Subjects Completed NCT02886793 Phase 1, Phase 2 fludeoxyglucose
18 Evaluation of Right Ventricular Function and Metabolism Following Riociguat for the Treatment of Chronic Thromboembolic Pulmonary Hypertension- Images of Rio Completed NCT02094001 Phase 2
19 Effect of Short-term Oxygen Therapy on Cardiopulmonary Exercise Capacity in Patients With Pulmonary Arterial and Chronic Thromboembolic Pulmonary Hypertension Completed NCT01748474 Phase 2
20 A Multicenter, Non-randomized, Non-blinded, Noncontrolled Study to Investigate the Impact of Multiple Doses of BAY 63-2521 on Safety, Tolerability, Pharmacokinetics, and Pharmacodynamics in Patients With Pulmonary Hypertension in a 12-week 3 Times a Day Individual Dose Titration Scheme Completed NCT00454558 Phase 2 Riociguat (Adempas, BAY63-2521)
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 Recruiting NCT03273257 Phase 2 Riociguat;Placebo
22 Non-Invasive Diagnosis of Pulmonary Vascular Disease Using Inhaled 129Xe Magnetic Resonance Imaging Recruiting NCT03078192 Phase 2 GE-141, Hyperpolarized 129Xenon gas
23 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
24 Compassionate Use of Catheter Thrombectomy (Aspirex 11F) in Patients With Massive Pulmonary Embolism Terminated NCT00314002 Phase 1, Phase 2
25 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
26 A Phase I Study of the Safety and Feasibility of Central Intravenous Delivery of Allogeneic Human Cardiosphere-Derived Stem Cells in Patients With Pulmonary Arterial Hypertension ALPHA Trial Recruiting NCT03145298 Phase 1
27 Local Registry of Patients With Chronic Thromboembolic Pulmonary Hypertension in Novosibirsk Region Unknown status NCT03349164
28 Inspiratory Muscle Training in Patients With Chronic Thromboembolic Pulmonary Hypertension: a Randomized Controlled Trial Unknown status NCT03102294
29 Incidence of CTEPH in Dependence of Thrombus Location in Patients With Acute Pulmonary Embolism and Index Fibrinolysis Unknown status NCT01672203
30 Riociguat Versus Balloon Pulmonary Angioplasty in Non-operable Chronic thromboEmbolic Pulmonary Hypertension Unknown status NCT02634203 Riociguat
31 Prospective Registry to Evaluate the Effective Incidence of Chronic Thromboembolic Pulmonary Hypertension in Germany Unknown status NCT02660463
32 Long-term Right Ventricular Resynchronization Therapy for Chronic Thromboembolic Pulmonary Hypertension Unknown status NCT01163422
33 Assessment of Safety and Efficacy of Balloon Pulmonary Angioplasty in Non-operable CTEPH Patients Unknown status NCT02964390
34 Incidence of Latent Pulmonary Hypertension in Patients With Chronic Thromboembolic Pulmonary Hypertension After Endarterectomy and Influence of Exercise and Respiratory Therapy Unknown status NCT00477724
35 Gene Mutations in Secondary Pulmonary Hypertension Unknown status NCT00348803
36 Exhaled Breath Olfactory Signature of Pulmonary Arterial Hypertension Detected by an Artificial Nose: A Clinical Validation Study Unknown status NCT02782026
37 Epidemiological Study of Chronic Thromboembolic Pulmonary Hypertension in China Unknown status NCT01717092
38 Follow-up in Rivaroxaban Patients in Setting of Thromboembolism Unknown status NCT02248610
39 Three-dimensional Echocardiographic Assessment of Right Ventricular Function in Patients Undergoing Pulmonary Endarterectomy Unknown status NCT02426203
40 Effect of Endothelin Receptor Antagonist Bosentan in Glucose Metabolism of the Myocardium and Coronary Dependant Endothelial Vasoreactivity Measured by 18F-FDG PET / CT and 82Rb PET / CT in Patients With PAH or CTEPH Unknown status NCT02970851
41 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)
42 Identification Of The Predictive Angiographic And Computed Tomographic (CT) Criteria Of The Success Of The Thromboendarterectomy In Chronic Pulmonary Hypertension Completed NCT00657722
43 (e-MOTION PH) Electronic - Activity Level Monitoring Pilot in Pulmonary Hypertension Completed NCT02536534
44 Identification of CTEPH on Standard Computerised Tomography Pulmonary Angiography Performed in the Work-up of Suspected Pulmonary Embolism Completed NCT03083093
45 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
46 Study on the Clinical Course Of Pulmonary Embolism Completed NCT01781858
47 Smoking: a Risk Factor for Pulmonary Arterial Hypertension? A D-A-CH Pulmonary Hypertension Study (Germany- Austria - Switzerland) Completed NCT01484899
48 Evaluation of Pulmonary Perfusion Heterogeneity in Patients With Chronic Thromboembolic Pulmonary Hypertension Using Functional PET Imaging Completed NCT02114047
49 Efficacy of Medical Therapy for Pulmonary Arterial and Inoperable Chronic Thromboembolic Pulmonary Hypertension in a Real Life Setting Completed NCT03198910
50 The Effect of Adding Exercise Training to Optimal Therapy in Pulmonary Arterial Hypertension Completed NCT02961023

Search NIH Clinical Center for Chronic Thromboembolic Pulmonary Hypertension

Genetic Tests for Chronic Thromboembolic Pulmonary Hypertension

Anatomical Context for Chronic Thromboembolic Pulmonary Hypertension

MalaCards organs/tissues related to Chronic Thromboembolic Pulmonary Hypertension:

40
Lung, Heart, Endothelial, Testes, Brain, Kidney, T Cells

Publications for Chronic Thromboembolic Pulmonary Hypertension

Articles related to Chronic Thromboembolic Pulmonary Hypertension:

(show top 50) (show all 1948)
# Title Authors PMID Year
1
The Pathobiology of Chronic Thromboembolic Pulmonary Hypertension. 61 52
27571003 2016
2
Current status of long-term prognosis among all subtypes of pulmonary hypertension in Japan. 61
31813677 2020
3
Safety and efficacy of balloon pulmonary angioplasty in chronic thromboembolic pulmonary hypertension in the Netherlands. 61
31782109 2020
4
Microvascular Disease in Chronic Thromboembolic Pulmonary Hypertension: Hemodynamic Phenotyping and Histomorphometric Assessment. 61
31896275 2020
5
Effectiveness of balloon pulmonary angioplasty in patients with inoperable chronic thromboembolic pulmonary hypertension despite having lesion types suitable for surgical treatment. 61
31427133 2020
6
Long-term changes of right ventricular myocardial deformation and remodeling studied by cardiac magnetic resonance imaging in patients with chronic thromboembolic pulmonary hypertension following pulmonary thromboendarterectomy. 61
31744721 2020
7
Multicentre randomised controlled trial of balloon pulmonary angioplasty and riociguat in patients with chronic thromboembolic pulmonary hypertension: protocol for the MR BPA study. 61
32034015 2020
8
In vitro assessment of P-gp and BCRP transporter-mediated drug-drug interactions of riociguat with direct oral anticoagulants. 61
31411766 2020
9
"The post-pulmonary syndrome - results of echocardiographic driven follow up after acute pulmonary embolism". 61
31862573 2020
10
Ten-year outcome of chronic thromboembolic pulmonary hypertension patients in a tertiary center. 61
32011330 2020
11
Protective role of endothelial progenitor cells stimulated by riociguat in chronic thromboembolic pulmonary hypertension. 61
31337550 2020
12
Risk factors for chronic thromboembolic pulmonary hypertension - Importance of thyroid disease and function. 61
31734602 2020
13
Proteomic Analyses of Endarterectomized Tissues from Patients with Chronic Thromboembolic Pulmonary Hypertension. 61
31734660 2020
14
Riociguat Improves Pulmonary Hemodynamics in Patients with Inoperable Chronic Thromboembolic Pulmonary Hypertension. 61
31903010 2020
15
Chronic thromboembolic pulmonary hypertension from synchronous idiopathic upper extremity deep vein thrombosis and pulmonary embolism. 61
31953584 2020
16
Changes in Estimated Glomerular Filtration after Balloon Pulmonary Angioplasty for Chronic Thromboembolic Pulmonary Hypertension. 61
31527376 2020
17
Sex-specific differences in chronic thromboembolic pulmonary hypertension. Results from the European CTEPH registry. 61
31479557 2020
18
A multicenter study of anticoagulation in operable chronic thromboembolic pulmonary hypertension. 61
31557382 2020
19
Lung Cavities in Chronic Thromboembolic Pulmonary Hypertension. 61
31939560 2020
20
Commentary: Chronic thromboembolic pulmonary hypertension and the misguided thrombus. 61
32005578 2020
21
Tetramethylpyrazine, a Promising Drug for Treatment of Pulmonary Hypertension. 61
31976548 2020
22
Pulmonary hypertension with a low cardiac index requires a higher PaO2 level to avoid tissue hypoxia. 61
31099121 2020
23
Activated Endothelial TGFβ1 Signaling Promotes Venous Thrombus Nonresolution in Mice Via Endothelin-1: Potential Role for Chronic Thromboembolic Pulmonary Hypertension. 61
31747868 2020
24
Novel antithrombotic strategies for treatment of venous thromboembolism. 61
31917385 2020
25
Long-term treatment of venous thromboembolism. 61
31917402 2020
26
Chronic Pulmonary Thromboembolic Disease. 61
31902629 2020
27
Anesthesia Management for a Patient Undergoing Pulmonary Endarterctomy without Cardiopulmonary Bypass. 61
31793258 2020
28
The impact of riociguat on clinical parameters and quality of life in patients with chronic thromboembolic pulmonary hypertension - results of a retrospective clinical registry. 61
31974533 2020
29
Evaluation of the Incidence of Chronic Thromboembolic Pulmonary Hypertension 1 Year After First Episode of Acute Pulmonary Embolism: A Cohort Study. 61
31894412 2020
30
Preoperative C-reactive protein predicts early postoperative outcomes after pulmonary endarterectomy in patients with chronic thromboembolic pulmonary hypertension. 61
32007254 2020
31
Chronic thromboembolic pulmonary hypertension is a clot you cannot swat. 61
31660459 2019
32
2018 TSOC guideline focused update on diagnosis and treatment of pulmonary arterial hypertension. 61
30926248 2019
33
Bosentan or Macitentan Therapy in Chronic Thromboembolic Pulmonary Hypertension? 61
31583452 2019
34
Balloon pulmonary angioplasty for chronic thromboembolic pulmonary hypertension. 61
31873853 2019
35
Angioscopic findings during balloon pulmonary angioplasty in chronic thromboembolic pulmonary hypertension. 61
31838724 2019
36
Pulmonary vascular imaging characteristics after pulmonary endarterectomy for chronic thromboembolic pulmonary hypertension. 61
31866175 2019
37
[Research progress on chronic thromboembolic diseases and chronic thromboembolic pulmonary hypertension]. 61
31826536 2019
38
Usefulness of ventilatory gas analysis for the non-invasive evaluation of the severity of chronic thromboembolic pulmonary hypertension. 61
31350036 2019
39
Cardiopulmonary exercise testing with ventilatory gas analysis for evaluation of chronic thromboembolic pulmonary hypertension: Unmasking its role after a therapeuthic intervention. 61
31445674 2019
40
Catheter-directed thrombolysis for submassive pulmonary embolism in children: A case series. 61
31876109 2019
41
Evaluation of the CT imaging findings in the diagnosis of pulmonary hypertension due to chronic thromboembolism. 61
31797559 2019
42
What's next after the clot? Residual pulmonary vascular obstruction after pulmonary embolism: From imaging finding to clinical consequences. 61
31710860 2019
43
CMR-based heart deformation analysis for quantification of hemodynamics and right ventricular dysfunction in patients with CTEPH. 61
31814279 2019
44
Validation of Automated Perfusion-Weighted Phase-Resolved Functional Lung (PREFUL)-MRI in Patients With Pulmonary Diseases. 61
31872556 2019
45
Chronic thromboembolic pulmonary hypertension suspicion after pulmonary embolism in cancer patients. 61
31527015 2019
46
Hsa_circ_0046159 is involved in the development of chronic thromboembolic pulmonary hypertension. 61
31776848 2019
47
Chronic Thromboembolic Pulmonary Hypertension-Management Strategies and Outcomes. 61
31883688 2019
48
Klippel-Trenaunay syndrome as a rare cause of chronic thromboemboembolic pulmonary hypertension. 61
31557688 2019
49
Advances in targeted therapy for chronic thromboembolic pulmonary hypertension. 61
31044326 2019
50
[Clinical characteristics of patients with chronic thromboembolic pulmonary hypertension]. 61
31826562 2019

Variations for Chronic Thromboembolic Pulmonary Hypertension

Expression for Chronic Thromboembolic Pulmonary Hypertension

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

Pathways for Chronic Thromboembolic Pulmonary Hypertension

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

# Super pathways Score Top Affiliating Genes
1
Show member pathways
10.43 EDN1 CRP
2 9.97 NPPB BMPR2

GO Terms for Chronic Thromboembolic Pulmonary Hypertension

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 9.35 NPPB FGA EDN1 CRP CPB2
2 extracellular space GO:0005615 9.1 NPPB FGA EDN1 CRP CPB2 BMPR2

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

# Name GO ID Score Top Affiliating Genes
1 regulation of blood pressure GO:0008217 9.43 NPPB EDN1
2 positive regulation of endothelial cell migration GO:0010595 9.4 EDN1 BMPR2
3 hemostasis GO:0007599 9.37 FGA CPB2
4 fibrinolysis GO:0042730 9.32 FGA CPB2
5 positive regulation of renal sodium excretion GO:0035815 9.26 NPPB EDN1
6 positive regulation of urine volume GO:0035810 9.16 NPPB EDN1
7 negative regulation of systemic arterial blood pressure GO:0003085 8.96 NPPB BMPR2
8 body fluid secretion GO:0007589 8.62 NPPB EDN1

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

# Name GO ID Score Top Affiliating Genes
1 signaling receptor binding GO:0005102 8.8 NPPB FGA EDN1

Sources for Chronic Thromboembolic Pulmonary Hypertension

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
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
43 MeSH
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45 MGI
48 NCI
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50 NDF-RT
53 NINDS
54 Novoseek
56 OMIM
57 OMIM via Orphanet
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
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