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 Selexipag and Treprostinil have been mentioned in the context of this disorder. Affiliated tissues include lung, heart and endothelial, and related phenotypes are congestive heart failure and pulmonary arterial hypertension

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 212)
# Related Disease Score Top Affiliating Genes
1 pulmonary hypertension, primary, 1 31.2 NPPB EDN1 BMPR2
2 pulmonary hypertension 31.2 NPPB EDN1 CRP BMPR2
3 pulmonary embolism 30.8 NPPB CRP CPB2
4 pulmonary venoocclusive disease 30.5 NPPB BMPR2
5 cardiogenic shock 30.3 NPPB CRP
6 pericardial effusion 30.3 NPPB CRP
7 pulmonary edema 30.3 NPPB EDN1 CRP
8 cardiac tamponade 30.2 NPPB CRP
9 lung disease 30.1 NPPB EDN1 CRP
10 eisenmenger syndrome 30.0 EDN1 BMPR2
11 central sleep apnea 29.9 NPPB CRP
12 arteriovenous malformation 29.8 EDN1 BMPR2
13 tricuspid valve insufficiency 29.8 NPPB EDN1 CRP BMPR2
14 sleep apnea 29.8 NPPB EDN1 CRP
15 congestive heart failure 29.7 NPPB EDN1 CRP
16 thrombocytosis 29.7 FGA CRP
17 respiratory failure 29.7 NPPB FGA EDN1 CRP
18 takayasu arteritis 29.6 EDN1 CRP
19 connective tissue disease 29.6 EDN1 CRP BMPR2
20 portal hypertension 29.6 EDN1 BMPR2
21 cerebrovascular disease 29.5 NPPB FGA CRP
22 interstitial lung disease 29.4 NPPB EDN1 CRP
23 lipoprotein quantitative trait locus 29.4 NPPB EDN1 CRP
24 pulmonary artery disease 29.3 NPPB CRP CPB2
25 vascular disease 29.2 NPPB FGA EDN1 CRP BMPR2
26 heart disease 29.1 NPPB EDN1 CRP BMPR2
27 patent foramen ovale 29.0 NPPB FGA EDN1 CRP
28 thrombocytopenia 28.7 FGA EDN1 CRP CPB2
29 hypertension, essential 28.7 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 syncope 10.5
35 thrombosis 10.4
36 thrombophilia 10.4
37 hypothyroidism 10.3
38 vasculitis 10.3
39 sarcoidosis 1 10.3
40 pulmonary disease, chronic obstructive 10.3
41 hydrocephalus 10.3
42 hemopericardium 10.3
43 mediastinitis 10.3
44 fibrosing mediastinitis 10.3
45 pulmonary venoocclusive disease 1, autosomal dominant 10.2
46 hepatic veno-occlusive disease 10.2
47 sarcoma 10.2
48 spindle cell sarcoma 10.2
49 heritable pulmonary arterial hypertension 10.2
50 autoimmune disease 10.1

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 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 edema 31 occasional (7.5%) HP:0000969
12 obesity 31 occasional (7.5%) HP:0001513
13 depressivity 31 occasional (7.5%) HP:0000716
14 syncope 31 occasional (7.5%) HP:0001279
15 right bundle branch block 31 occasional (7.5%) HP:0011712
16 elevated c-reactive protein level 31 occasional (7.5%) HP:0011227
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 left ventricular dysfunction 31 occasional (7.5%) HP:0005162
21 increased hdl cholesterol concentration 31 occasional (7.5%) HP:0012184
22 right ventricular failure 31 occasional (7.5%) HP:0001708
23 lupus anticoagulant 31 occasional (7.5%) HP:0025343
24 right ventricular dilatation 31 occasional (7.5%) HP:0005133
25 cardiac shunt 31 occasional (7.5%) HP:0001693
26 recurrent thromboembolism 31 occasional (7.5%) HP:0004831
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 62)
# 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 6918140 54786
3
Macitentan Approved Phase 3 441798-33-0
4
Ambrisentan Approved, Investigational Phase 3 177036-94-1 6918493
5 Calamus Phase 3
6 Endothelin A Receptor Antagonists Phase 3
7 Fibrinolytic Agents Phase 3
8 Tissue Plasminogen Activator Phase 3
9 Plasminogen Phase 3
10 Endothelin Receptor Antagonists Phase 3
11 Antihypertensive Agents Phase 3
12
Riociguat Approved Phase 2 625115-55-1
13 Xenon Experimental Phase 2 7440-63-3
14 Radiopharmaceuticals Phase 2
15 Anesthetics 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 46507594 772
20
Phylloquinone Approved, Investigational 84-80-0
21
Edoxaban Approved 480449-70-5
22
Warfarin Approved 81-81-2 6691 54678486
23
Rivaroxaban Approved 366789-02-8
24
Acetylcysteine Approved, Investigational 616-91-1 12035
25
Methamphetamine Approved, Illicit 537-46-2 10836
26
Iloprost Approved, Investigational 78919-13-8 6443959
27
Tadalafil Approved, Investigational 171596-29-5 110635
28
Epoprostenol Approved 35121-78-9, 61849-14-7 5282411 5280427
29
Thrombin Approved, Investigational
30
Iodine Approved, Investigational 7553-56-2 807
31
Streptokinase Approved, Investigational 9002-01-1
32
Bosentan Approved, Investigational 147536-97-8 104865
33
Menadione Approved, Nutraceutical 58-27-5 4055
34
Cysteine Approved, Nutraceutical 52-90-4 5862
35 Menaquinone Investigational 1182-68-9
36 Tezosentan Investigational 180384-57-0
37 Immunoglobulins
38 Antibodies
39 Autoantibodies
40 Vitamins
41 naphthoquinone
42 Vitamin K
43 calcium heparin
44 Hemostatics
45 Coagulants
46 Respiratory System Agents
47 Anti-Infective Agents
48 Antidotes
49 Antioxidants
50 Expectorants

Interventional clinical trials:

(show top 50) (show all 127)
# 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 CHANGE-MRI (CTEPH DIAGNOSIS Europe - MRI) Unknown status NCT02791282 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 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
12 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 Not yet recruiting NCT04271475 Phase 3 Macitentan;Placebo
13 A Reduced Dose of Thrombolytic Treatment for Patients With Intermediate High-risk Acute Pulmonary Embolism Not yet recruiting NCT04430569 Phase 3 Alteplase;Placebo
14 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
15 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
16 Randomized Pilot Trial of Interventional Treatment of Residual Pulmonary Hypertension in Patients After Pulmonary Thromboendarterectomy Unknown status NCT02745106 Phase 2
17 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
18 Cell Proliferation in Pulmonary Hypertension. FDG-PET Comparison Between Patients and Healthy Subjects Completed NCT02886793 Phase 1, Phase 2 fludeoxyglucose
19 Evaluation of Right Ventricular Function and Metabolism Following Riociguat for the Treatment of Chronic Thromboembolic Pulmonary Hypertension- Images of Rio Completed NCT02094001 Phase 2
20 Effect of Short-term Oxygen Therapy on Cardiopulmonary Exercise Capacity in Patients With Pulmonary Arterial and Chronic Thromboembolic Pulmonary Hypertension Completed NCT01748474 Phase 2
21 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)
22 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
23 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
24 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
25 Non-Invasive Diagnosis of Pulmonary Vascular Disease Using Inhaled 129Xe Magnetic Resonance Imaging Suspended NCT03078192 Phase 2 GE-141, Hyperpolarized 129Xenon gas
26 Compassionate Use of Catheter Thrombectomy (Aspirex 11F) in Patients With Massive Pulmonary Embolism Terminated NCT00314002 Phase 1, Phase 2
27 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
28 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
29 Local Registry of Patients With Chronic Thromboembolic Pulmonary Hypertension in Novosibirsk Region Unknown status NCT03349164
30 Inspiratory Muscle Training in Patients With Chronic Thromboembolic Pulmonary Hypertension: a Randomized Controlled Trial Unknown status NCT03102294
31 Incidence of CTEPH in Dependence of Thrombus Location in Patients With Acute Pulmonary Embolism and Index Fibrinolysis Unknown status NCT01672203
32 Riociguat Versus Balloon Pulmonary Angioplasty in Non-operable Chronic thromboEmbolic Pulmonary Hypertension Unknown status NCT02634203 Riociguat
33 Prospective Registry to Evaluate the Effective Incidence of Chronic Thromboembolic Pulmonary Hypertension in Germany Unknown status NCT02660463
34 Sleep Disordered Breathing in Precapillary Pulmonary Hypertension: Effect of CPAP Treatment Unknown status NCT02583906
35 Long-term Right Ventricular Resynchronization Therapy for Chronic Thromboembolic Pulmonary Hypertension Unknown status NCT01163422
36 Assessment of Safety and Efficacy of Balloon Pulmonary Angioplasty in Non-operable CTEPH Patients Unknown status NCT02964390
37 Gene Mutations in Secondary Pulmonary Hypertension Unknown status NCT00348803
38 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
39 Exhaled Breath Olfactory Signature of Pulmonary Arterial Hypertension Detected by an Artificial Nose: A Clinical Validation Study Unknown status NCT02782026
40 Epidemiological Study of Chronic Thromboembolic Pulmonary Hypertension in China Unknown status NCT01717092
41 Follow-up in Rivaroxaban Patients in Setting of Thromboembolism Unknown status NCT02248610
42 Three-dimensional Echocardiographic Assessment of Right Ventricular Function in Patients Undergoing Pulmonary Endarterectomy Unknown status NCT02426203
43 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)
44 New International CTEPH Database Completed NCT02656238
45 Identification Of The Predictive Angiographic And Computed Tomographic (CT) Criteria Of The Success Of The Thromboendarterectomy In Chronic Pulmonary Hypertension Completed NCT00657722
46 (e-MOTION PH) Electronic - Activity Level Monitoring Pilot in Pulmonary Hypertension Completed NCT02536534
47 Identification of CTEPH on Standard Computerised Tomography Pulmonary Angiography Performed in the Work-up of Suspected Pulmonary Embolism Completed NCT03083093
48 United States CTEPH Registry Completed NCT02429284
49 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
50 Study on the Clinical Course Of Pulmonary Embolism Completed NCT01781858

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, Smooth Muscle, Neutrophil

Publications for Chronic Thromboembolic Pulmonary Hypertension

Articles related to Chronic Thromboembolic Pulmonary Hypertension:

(show top 50) (show all 2036)
# Title Authors PMID Year
1
The Pathobiology of Chronic Thromboembolic Pulmonary Hypertension. 61 52
27571003 2016
2
The potential for early diagnosis of pulmonary arterial hypertension using lung iodine-123-metaiodobenzylguanidine (123I-MIBG) uptake: A case report. 61
32528606 2020
3
Does combination therapy work in chronic thromboembolic pulmonary hypertension? 61
32518816 2020
4
Post-Intensive Care Unit Follow-up of Pulmonary Embolism. 61
32473699 2020
5
A systematic literature review and meta-analysis of spectral CT compared to scintigraphy in the diagnosis of acute and chronic pulmonary embolisms. 61
32112117 2020
6
Validation of Automated Perfusion-Weighted Phase-Resolved Functional Lung (PREFUL)-MRI in Patients With Pulmonary Diseases. 61
31872556 2020
7
Effects of balloon pulmonary angioplasty for chronic thromboembolic pulmonary hypertension on remodeling in right-sided heart. 61
32086654 2020
8
Long-term real world clinical outcomes of macitentan therapy in chronic thromboembolic pulmonary hypertension. 61
32421542 2020
9
Balloon Pulmonary Angioplasty with Stent Implantation as a Treatment of Proximal Chronic Thromboembolic Pulmonary Hypertension. 61
32492944 2020
10
Editorial for "Chronic Thromboembolic Pulmonary Hypertension Perioperative Monitoring Using Phase-Resolved Functional Lung (PREFUL)-MRI". 61
32506708 2020
11
CAMPHOR score: patient-reported outcomes are improved by pulmonary endarterectomy in chronic thromboembolic pulmonary hypertension (CTEPH). 61
32513780 2020
12
Long-term changes of exercise hemodynamics and physical capacity in chronic thromboembolic pulmonary hypertension after pulmonary thromboendarterectomy. 61
32497568 2020
13
Comparison of Balloon Pulmonary Angioplasty and Pulmonary Vasodilators for Inoperable Chronic Thromboembolic Pulmonary Hypertension: A Systematic Review and Meta-Analysis. 61
32483219 2020
14
Virtual Histology to Evaluate Mechanisms of Pulmonary Artery Lumen Enlargement in Response to Balloon Pulmonary Angioplasty in Chronic Thromboembolic Pulmonary Hypertension. 61
32492788 2020
15
Persistent exercise intolerance after pulmonary endarterectomy for chronic thromboembolic pulmonary hypertension. 61
32366481 2020
16
Egg and Banana sign - A Radiological sign of Severe Pulmonary Arterial Hypertension. 61
32539095 2020
17
Quantifying the Influence of Wedge Pressure, Age, and Heart Rate on the Systolic Thresholds for Detection of Pulmonary Hypertension. 61
32419583 2020
18
Novel Insight into How Nurses Working at PH Specialist Clinics in Sweden Perceive Their Work. 61
32575509 2020
19
Gadolinium contrast balloon pulmonary angioplasty for a patient with chronic thromboembolic pulmonary hypertension and severe iodine allergy. 61
32478464 2020
20
Tetramethylpyrazine: A promising drug for the treatment of pulmonary hypertension. 61
31976548 2020
21
Assessment of ventilation-perfusion scans in patients with chronic thromboembolic pulmonary hypertension before and after surgery and correlation with clinical parameters. 61
32531708 2020
22
Dual-Energy Computed Tomography as an Alternative Noninvasive Study for Evaluation of Chronic Thromboembolic Pulmonary Hypertension Postoperatively. 61
32397817 2020
23
Speckle tracking for predicting outcomes of balloon pulmonary angioplasty in patients with chronic thromboembolic pulmonary hypertension. 61
32447819 2020
24
The Factor V Leiden variant and risk of chronic thromboembolic pulmonary hypertension. 61
32444403 2020
25
Myeloproliferative Diseases as Possible Risk Factor for Development of Chronic Thromboembolic Pulmonary Hypertension-A Genetic Study. 61
32397294 2020
26
Balloon pulmonary angioplasty improves right atrial reservoir and conduit functions in chronic thromboembolic pulmonary hypertension. 61
32359071 2020
27
A rare childhood case of Behcet's disease and chronic thromboembolic pulmonary hypertension. 61
32419255 2020
28
Balloon pulmonary angioplasty improves quality of life in Japanese patients with chronic thromboembolic pulmonary hypertension. 61
32402668 2020
29
Comparison of V/Q SPECT and CT Angiography for the Diagnosis of Chronic Thromboembolic Pulmonary Hypertension. 61
32427559 2020
30
Chronic Pulmonary Thromboembolic Disease. 61
31902629 2020
31
Ocular hypotonia following pulmonary endarterectomy. 61
31580413 2020
32
Chronic thromboembolic pulmonary hypertension secondary to implantable cardioverter defibrillator lead thrombus in a patient with Brugada syndrome: a rare complication requiring a multidisciplinary approach. 61
32434878 2020
33
Machine Learning and Deep Neural Network Applications in the Thorax: Pulmonary Embolism, Chronic Thromboembolic Pulmonary Hypertension, Aorta, and Chronic Obstructive Pulmonary Disease. 61
32271281 2020
34
Surgical and Percutaneous Interventions for Chronic Thromboembolic Pulmonary Hypertension. 61
32284102 2020
35
Chronic thromboembolic pulmonary hypertension: interventional approaches. 61
32404404 2020
36
EPC modulation, a new mechanism for CTEPH therapy? 61
32354406 2020
37
Catheter-directed thrombolysis for submassive pulmonary embolism in children: A case series. 61
31876109 2020
38
Intrapulmonary Activated Factor VII for Hemoptysis Complicating Pulmonary Thromboendarterectomy. 61
31470015 2020
39
Imaging of Chronic Thromboembolic Disease. 61
32166427 2020
40
The role of inflammation in a rat model of chronic thromboembolic pulmonary hypertension induced by carrageenan. 61
32395536 2020
41
Life expectancy after pulmonary endarterectomy for chronic thromboembolic pulmonary hypertension: a Swedish single-center study. 61
32313643 2020
42
Hsa_circ_0046159 is involved in the development of chronic thromboembolic pulmonary hypertension. 61
31776848 2020
43
Chronic thromboembolic pulmonary hypertension in Saudi Arabia: preliminary results from the SAUDIPH registry. 61
32420315 2020
44
Targeted Angiogenesis Gene Expression Profiling of Patients with Chronic Thromboembolic Pulmonary Hypertension. 61
32465370 2020
45
Tricuspid Surgery and Pulmonary Thromboendarterectomy in Patients with Chronic Thromboembolic Pulmonary Hypertension. 61
32465947 2020
46
Pregnancy in a Patient With Chronic Thromboembolic Pulmonary Hypertension After Successful Treatment with Balloon Pulmonary Angioplasty. 61
32115306 2020
47
Cyclophilin A as a biomarker for the therapeutic effect of balloon angioplasty in chronic thromboembolic pulmonary hypertension. 61
31615744 2020
48
Real-world experience with riociguat as potential bridging therapy in patients with chronic thromboembolic pulmonary hypertension: a case series. 61
32328237 2020
49
Outcomes of Patients with Chronic Thromboembolic Pulmonary Hypertension Risk Stratified by a Dedicated Heart Team to Receive Pulmonary Endarectomy or Balloon Pulmonary Angiogiplasty. 61
32464975 2020
50
Exercise intolerance in chronic thromboembolic pulmonary hypertension after pulmonary angioplasty. 61
32312861 2020

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
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
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
Content
Loading form....