MCID: PLM037
MIFTS: 79

Pulmonary Hypertension

Categories: Cardiovascular diseases, Genetic diseases, Rare diseases, Respiratory diseases

Aliases & Classifications for Pulmonary Hypertension

MalaCards integrated aliases for Pulmonary Hypertension:

Name: Pulmonary Hypertension 38 37 12 72 40 3 14 69 59
Primary Pulmonary Hypertension 38 12 72 36 28 51 14
Idiopathic Pulmonary Arterial Hypertension 69
Idiopathic Pulmonary Hypertension 69
Hypertension, Pulmonary 41
Hypertension Pulmonary 51

Classifications:



External Ids:

Disease Ontology 12 DOID:14557 DOID:6432
ICD10 32 I27.0 I27.2
ICD9CM 34 416.0
NCIt 46 C97119 C3120
MeSH 41 D006976
KEGG 36 H01619

Summaries for Pulmonary Hypertension

MedlinePlus : 40 Pulmonary hypertension (PH) is high blood pressure in the arteries to your lungs. It is a serious condition. If you have it, the blood vessels that carry blood from your heart to your lungs become hard and narrow. Your heart has to work harder to pump the blood through. Over time, your heart weakens and cannot do its job and you can develop heart failure. Symptoms of PH include Shortness of breath during routine activity, such as climbing two flights of stairs Tiredness Chest pain A racing heartbeat Pain on the upper right side of the abdomen Decreased appetite As PH worsens, you may find it hard to do any physical activities. There are two main kinds of PH. One runs in families or appears for no known reason. The other kind is related to another condition, usually heart or lung disease. There is no cure for PH. Treatments can control symptoms. They involve treating the heart or lung disease, medicines, oxygen, and sometimes lung transplantation. NIH: National Heart, Lung, and Blood Institute

MalaCards based summary : Pulmonary Hypertension, also known as primary pulmonary hypertension, is related to pulmonary hypertension, primary, 1 and chronic thromboembolic pulmonary hypertension, and has symptoms including snoring, hemoptysis and edema. An important gene associated with Pulmonary Hypertension is BMPR2 (Bone Morphogenetic Protein Receptor Type 2), and among its related pathways/superpathways are TGF-beta signaling pathway and Vascular smooth muscle contraction. The drugs Adcirca and Adempas have been mentioned in the context of this disorder. Affiliated tissues include Lung, heart and lung, and related phenotypes are cardiovascular system and behavior/neurological

Disease Ontology : 12 A hypertension characterized by an increase of blood pressure in the pulmonary artery, pulmonary vein or pulmonary capillaries.

CDC : 3 Pulmonary hypertension occurs when the pressure in the blood vessels leading from the heart to the lungs is too high. The heart pumps blood from the right ventricle to the lungs to get oxygen. Because the blood does not have to travel very far, the pressure in this side of the heart and in the artery taking blood from the right ventricle to the lungs is normally low—normally much lower than systolic or diastolic blood pressure. When the pressure in this artery gets too high, the arteries in the lungs can narrow and then the blood does not flow as well as it should resulting in less oxygen in the blood.1

PubMed Health : 59
About pulmonary hypertension: Pulmonary hypertension (PULL-mun-ary HI-per-TEN-shun), or PH, is increased pressure in the pulmonary arteries. These arteries carry blood from your heart to your lungs to pick up oxygen.PH causes symptoms such as shortness of breath during routine activity (for example, climbing two flights of stairs), tiredness, chest pain, and a racing heartbeat. As the condition worsens, its symptoms may limit all physical activity.

Related Diseases for Pulmonary Hypertension

Diseases in the Pulmonary Hypertension family:

Pulmonary Hypertension, Primary, 1 Pulmonary Hypertension, Primary, Autosomal Recessive
Pulmonary Hypertension, Primary, 2 Pulmonary Hypertension, Primary, 3
Pulmonary Hypertension, Primary, 4

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

(show top 50) (show all 460)
# Related Disease Score Top Affiliating Genes
1 pulmonary hypertension, primary, 1 34.9 ACVRL1 BMPR2 ENG FOXF1 KCNK3
2 chronic thromboembolic pulmonary hypertension 34.8 BMPR2 EDN1 NPPB
3 pulmonary venoocclusive disease 33.6 ACVRL1 BMPR2 EIF2AK4 KCNK3
4 persistent fetal circulation syndrome 33.5 EDN1 EDNRA NOS3 PDE5A
5 eisenmenger syndrome 33.3 BMPR2 EDN1 THBD
6 pulmonary venoocclusive disease 1, autosomal dominant 32.9 BMPR2 EIF2AK4
7 heritable pulmonary arterial hypertension 32.8 ACVRL1 BMPR2 CAV1 EIF2AK4 ENG KCNK3
8 mitral valve disease 31.1 NOS3 NPPA SLC6A4
9 heart disease 31.0 ADM BMPR2 EDN1 EDNRA EDNRB NOS3
10 hereditary hemorrhagic telangiectasia 31.0 ACVRL1 BMPR2 ENG NOS3
11 vascular disease 30.9 ACVRL1 BMPR2 EDN1 NOS3 THBD
12 sleep apnea 30.9 EDN1 NOS3 NPPB
13 pulmonary edema 30.9 EDN1 NOS3 NPPB
14 arteriovenous malformation 30.8 ACVRL1 BMPR2 ENG
15 congestive heart failure 30.8 ADM EDN1 EDNRA NPPA NPPB
16 pericardial effusion 30.8 ACVRL1 NPPA NPPB
17 central sleep apnea 30.7 KCNK3 NOS3 NPPA NPPB
18 hepatopulmonary syndrome 30.7 CAV1 EDN1 EDNRA EDNRB NOS3
19 mitral valve stenosis 30.7 ACVRL1 NPPA NPPB
20 pulmonary arteriovenous malformation 30.5 ACVRL1 ENG
21 arteries, anomalies of 30.5 ACVRL1 EDN1 NOS3 NPPB
22 coronary artery anomaly 30.5 EDN1 NOS3 NPPA NPPB
23 myocardial infarction 30.4 ADM EDN1 ENG NOS3 NPPA NPPB
24 chronic mountain sickness 30.4 EDN1 NOS3 NPPB
25 chronic pulmonary heart disease 30.3 ACVRL1 BMPR2 EDN1 SLC6A4
26 ischemic optic neuropathy 30.3 EDN1 NOS3 PDE5A
27 nonarteritic anterior ischemic optic neuropathy 30.3 EDN1 NOS3 PDE5A
28 portal hypertension 30.2 BMPR2 EDN1 EDNRA NOS3
29 dilated cardiomyopathy 30.1 EDN1 EDNRA NOS3 NPPA NPPB
30 hyperuricemia, pulmonary hypertension, renal failure, and alkalosis syndrome 12.4
31 pulmonary hypertension, primary, 4 12.3
32 pulmonary hypertension, primary, 2 12.3
33 pulmonary hypertension, primary, 3 12.3
34 pulmonary hypertension, neonatal 12.3
35 pulmonary hypertension, chronic thromboembolic, without deep vein thrombosis 12.3
36 vater-like defects with pulmonary hypertension, laryngeal webs, and growth deficiency 12.0
37 pulmonary hypertension, primary, autosomal recessive 11.9
38 alveolar capillary dysplasia with misalignment of pulmonary veins 11.6
39 pulmonary edema of mountaineers 11.6
40 lymphedema and cerebral arteriovenous anomaly 11.5
41 alveolar capillary dysplasia 11.4
42 pulmonary fibrosis, idiopathic 11.3
43 pulmonary venoocclusive disease 2, autosomal recessive 11.3
44 cantu syndrome 11.3
45 hypoxia 11.3
46 diaphragmatic hernia, congenital 11.2
47 patent ductus arteriosus 1 11.2
48 hydrops, lactic acidosis, and sideroblastic anemia 11.2
49 pulmonary vein stenosis 11.2
50 adams-oliver syndrome 1 11.2

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



Diseases related to Pulmonary Hypertension

Symptoms & Phenotypes for Pulmonary Hypertension

UMLS symptoms related to Pulmonary Hypertension:


snoring, hemoptysis, edema, coughing, chest pain, angina pectoris, dyspnea

MGI Mouse Phenotypes related to Pulmonary Hypertension:

43 (show all 13)
# Description MGI Source Accession Score Top Affiliating Genes
1 cardiovascular system MP:0005385 10.39 KCNK3 EDNRA ENG EDNRB FOXF1 SMAD9
2 behavior/neurological MP:0005386 10.3 EDNRB EIF2AK4 EDNRA ENG SLC6A4 KCNK3
3 homeostasis/metabolism MP:0005376 10.28 KCNK3 EIF2AK4 EDNRA ENG EDNRB SLC6A4
4 mortality/aging MP:0010768 10.24 EDNRB FOXF1 EIF2AK4 EDNRA ENG SLC6A4
5 growth/size/body region MP:0005378 10.23 EDNRB EDN1 EIF2AK4 EDNRA ENG SLC6A4
6 embryo MP:0005380 10.18 FOXF1 EDNRB EDNRA ENG SMAD9 THBD
7 hematopoietic system MP:0005397 10.17 EDNRB EIF2AK4 EDNRA ENG NOS3 THBD
8 muscle MP:0005369 10.17 EIF2AK4 EDNRA ENG EDNRB FOXF1 SMAD9
9 digestive/alimentary MP:0005381 10.15 FOXF1 EDNRB EDNRA ENG NOS3 THBD
10 nervous system MP:0003631 10.07 EDNRB KCNK3 EIF2AK4 EDNRA ENG SLC6A4
11 normal MP:0002873 9.85 KCNK3 EDNRA ENG SLC6A4 SMAD9 NOS3
12 renal/urinary system MP:0005367 9.65 EDNRA EDNRB NOS3 THBD NPPA CAV1
13 respiratory system MP:0005388 9.4 FOXF1 KCNK3 EDNRA ENG EDNRB SMAD9

Drugs & Therapeutics for Pulmonary Hypertension

PubMedHealth treatment related to Pulmonary Hypertension: 59

Pulmonary hypertension (PH) has no cure. However, treatment may help relieve symptoms and slow the progress of the disease.PH is treated with medicines, procedures, and other therapies. Treatment will depend on what type of PH you have and its severity. (For more information, go to "Types of Pulmonary Hypertension.")

FDA approved drugs:

(show all 7)
# Drug Name Active Ingredient(s) 17 Company Approval Date
1
Adcirca 17 TADALAFIL Eli Lilly May 2009
2
Adempas 17 RIOCIGUAT Bayer Healthcare Pharmaceuticals October 2013
3
Letairis 17 AMBRISENTAN Gilead June 2007
4
Opsumit 17 MACITENTAN Actelion Pharmaceuticals October 2013
5
Remodulin 17 TREPROSTINIL United Therapeutics May 2002
6
Tracleer 17 BOSENTAN Actelion November 2001
7
Tyvaso 17 TREPROSTINIL United Therapeutics July 2009

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

(show top 50) (show all 392)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Bosentan Approved, Investigational Phase 4,Phase 3,Phase 2,Phase 1,Early Phase 1 147536-97-8 104865
2
Iloprost Approved, Investigational Phase 4,Phase 2,Phase 3,Phase 1 78919-13-8 6443959
3
Dalteparin Approved Phase 4,Phase 3 9005-49-6
4
Heparin Approved, Investigational Phase 4,Phase 3 9005-49-6 46507594 772
5
Spironolactone Approved Phase 4,Phase 1,Phase 2 1952-01-7, 52-01-7 5833
6
Iron Approved Phase 4,Phase 3,Phase 2,Phase 1 7439-89-6 23925
7
Epoprostenol Approved Phase 4,Phase 3,Phase 2 61849-14-7, 35121-78-9 5282411 5280427
8
Tadalafil Approved, Investigational Phase 4,Phase 2,Phase 3,Phase 1 171596-29-5 110635
9
Treprostinil Approved, Investigational Phase 4,Phase 3,Phase 2,Phase 1 81846-19-7 6918140 54786
10
Nitric Oxide Approved Phase 4,Phase 3,Phase 2,Phase 1,Early Phase 1 10102-43-9 145068
11
Benzocaine Approved, Investigational Phase 4,Phase 3,Phase 2 1994-09-7, 94-09-7 2337
12
Warfarin Approved Phase 4,Phase 2 81-81-2 54678486 6691
13
Lidocaine Approved, Vet_approved Phase 4,Phase 1 137-58-6 3676
14
Ranolazine Approved, Investigational Phase 4,Phase 3,Phase 2,Phase 1 95635-55-5, 142387-99-3 56959
15
Isoflurane Approved, Vet_approved Phase 4 26675-46-7 3763
16
Propofol Approved, Investigational, Vet_approved Phase 4 2078-54-8 4943
17
Clevidipine Approved, Investigational Phase 4 167221-71-8
18
Magnesium oxide Approved Phase 4 1309-48-4 14792
19
Zinc Approved, Investigational Phase 4 7440-66-6 32051 23994
20
Silver sulfadiazine Approved, Vet_approved Phase 4 22199-08-2 441244
21
Acetazolamide Approved, Vet_approved Phase 4,Phase 2,Phase 3 59-66-5 1986
22 Selexipag Approved Phase 4,Phase 3,Phase 2 475086-01-2
23
Macitentan Approved Phase 4,Phase 3,Phase 2,Phase 1 441798-33-0
24
Ibuprofen Approved Phase 4,Phase 3 15687-27-1 3672
25
Sevoflurane Approved, Vet_approved Phase 4 28523-86-6 5206
26
Nebivolol Approved, Investigational Phase 4 152520-56-4, 118457-14-0, 99200-09-6 71301
27
Bupivacaine Approved, Investigational Phase 4 38396-39-3, 2180-92-9 2474
28
Morphine Approved, Investigational Phase 4,Phase 2 57-27-2 5288826
29
Citric Acid Approved, Nutraceutical, Vet_approved Phase 4,Phase 2,Phase 3,Phase 1 77-92-9 311
30 tannic acid Approved, Nutraceutical Phase 4,Phase 3,Phase 2
31
Lactitol Investigational Phase 4 585-86-4 3871
32 Antihypertensive Agents Phase 4,Phase 2,Phase 3,Phase 1,Early Phase 1
33 Endothelin Receptor Antagonists Phase 4,Phase 2,Phase 3,Phase 1,Early Phase 1
34 Phosphodiesterase 5 Inhibitors Phase 4,Phase 3,Phase 2,Phase 1
35 Phosphodiesterase Inhibitors Phase 4,Phase 3,Phase 2,Phase 1
36 Sildenafil Citrate Phase 4,Phase 2,Phase 3,Phase 1 171599-83-0
37 Vasodilator Agents Phase 4,Phase 2,Phase 3,Phase 1,Early Phase 1
38 Platelet Aggregation Inhibitors Phase 4,Phase 2,Phase 3,Phase 1
39 Anticoagulants Phase 4,Phase 2,Phase 3
40 calcium heparin Phase 4,Phase 3
41 Fibrinolytic Agents Phase 4,Phase 3,Phase 2
42 Heparin, Low-Molecular-Weight Phase 4,Phase 3
43 Plasminogen Phase 4,Phase 3
44 Tissue Plasminogen Activator Phase 4,Phase 3
45 diuretics Phase 4,Phase 2,Phase 3,Phase 1
46 Diuretics, Potassium Sparing Phase 4,Phase 3,Phase 1,Phase 2
47 Hormone Antagonists Phase 4,Phase 3,Phase 2,Phase 1,Early Phase 1
48 Hormones Phase 4,Phase 3,Phase 2,Phase 1,Early Phase 1
49 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 4,Phase 3,Phase 2,Phase 1,Early Phase 1
50 Mineralocorticoid Receptor Antagonists Phase 4,Phase 1,Phase 2

Interventional clinical trials:

(show top 50) (show all 1023)

# Name Status NCT ID Phase Drugs
1 Comparison of Efficacy Different Treatment Regimens in Pulmonary Hypertension Secondary to Lung Disease and or Hypoxia Unknown status NCT01449253 Phase 4 Sildenafil;Bosentan
2 Exercise Induced Pulmonary Hypertension in Systemic Sclerosis and Treatment With Ambrisentan Unknown status NCT01051960 Phase 4 Ambrisentan
3 Inhaled Iloprost for Disproportionate Pulmonary Hypertension in Chronic Obstructive Pulmonary Disease (COPD) Unknown status NCT01116063 Phase 4 Inhaled iloprost
4 Bosentan in Pulmonary Hypertension in Interstitial Lung Disease Treatment Study Unknown status NCT00637065 Phase 4 Bosentan;Placebo
5 Comparison of Thrombgolytic and Anticoagulation Therapy in Submassive Pulmonary Embolism Unknown status NCT02132689 Phase 4 Actilyse (Thrombolytic therapy);Heparine (Standard anticoagulation therapy)
6 Effects of Spironolactone on Collagen Metabolism in Patients With Pulmonary Arterial Hypertension Unknown status NCT01468571 Phase 4 Spironolactone;Placebo
7 Iron Deficiency In Pulmonary Hypertension Unknown status NCT01288651 Phase 4 Ferricarboxymaltose
8 Vardenafil as add-on Therapy for Patients With Pulmonary Hypertension Treated With Inhaled Iloprost Unknown status NCT01649739 Phase 4 Levitra
9 Long Acting Phosphodiesterase 5 Inhibitors as Add-on Therapy for Patients With Pulmonary Hypertension Treated With Prostanoids. Unknown status NCT00705588 Phase 4 Tadalafil;Vardenafil
10 Pulmonary Arterial Hypertension Secondary to Idiopathic Pulmonary Fibrosis and Treatment With Bosentan Unknown status NCT00625469 Phase 4 bosentan
11 Pulmonary Arterial Hypertension Secondary to Idiopathic Pulmonary Fibrosis And Treatment With Sildenafil Unknown status NCT00625079 Phase 4 sildenafil;sildenafil
12 Low Dose Prolonged Infusion of Tissue Type Plasminogen Activator Therapy in Massive Pulmonary Embolism Unknown status NCT02029456 Phase 4 25 mg Actilyse ( Boehringer Ingelheim, Germany) infusion in 6 hours
13 Hemodynamic Response After Six Months of Sildenafil Unknown status NCT00483626 Phase 4 oral sildenafil
14 Effect of BMPR-2 Gene Mutations on Hemodynamic Response by Iloprost Inhalation in Pulmonary Arterial Hypertension Unknown status NCT01054105 Phase 4 Iloprost
15 Effects of Iloprost Treatment in Adult Patients With Pulmonary Arterial Hypertension Related to Congenital Heart Disease Unknown status NCT01383083 Phase 4
16 Evaluation of Sildenafil for the Treatment of Moderate Congestive Heart Failure Unknown status NCT00793338 Phase 4 Sildenafil
17 Hypotonic Treprostinil Subcutaneous Infusion for Control of Treprostinil Related Site Pain Unknown status NCT01615627 Phase 4 HypotonicTreprostinil Solution;Eutonic Treprostinil Solution
18 Study of Ranolazine in the Treatment of Pulmonary Hypertension Associated With Diastolic Left Ventricular Dysfunction Completed NCT02133352 Phase 4 Ranolazine
19 VasoREACTivity Testing With Intravenous SILdenafil in Patients With Precapillary Pulmonary Hypertension Completed NCT01889966 Phase 4 Sildenafil
20 Study of Sildenafil Effects in Combination With Rehabilitation in Patients With Chronic Obstructive Pulmonary Disease (COPD) and Associated Pulmonary Hypertension Completed NCT01055405 Phase 4 Sildenafil
21 Natrecor for Pulmonary Hypertension in Lung Transplants Completed NCT00205426 Phase 4 Natrecor
22 Post-Marketing Surveillance of Ventavis® in Chinese Patients With Primary Pulmonary Hypertension (PPH) Completed NCT00882947 Phase 4 Ventavis (Iloprost, BAYQ6256)
23 Oral Sildenafil in Persistent Pulmonary Hypertension Secondary to Meconium Aspiration Syndrome in Newborns Completed NCT01757782 Phase 4 Oral Sildenafil;Placebo (distilled water)
24 Sildenafil for Secondary Pulmonary Hypertension Due to Valvular Disease Completed NCT00862043 Phase 4 Sildenafil Citrate;Placebo
25 Safely Change From Bosentan to Ambrisentan in Pulmonary Hypertension Completed NCT01330108 Phase 4 ambrisentan
26 Sildenafil Effects on Pulmonary Haemodynamics and Gas Exchange in Chronic Obstructive Pulmonary Disease (COPD) Completed NCT00491803 Phase 4 Sildenafil;Sildenafil
27 Inhaled Iloprost for Sarcoidosis-associated Pulmonary Hypertension Completed NCT00403650 Phase 4 Iloprost
28 Phase IV Study of Chronic Infusional Epoprostenol for Severe Primary Pulmonary Hypertension Completed NCT00004754 Phase 4 epoprostenol
29 Efficacy of Beraprost in Lowering Pulmonary Arterial Pressure in Children Completed NCT03431649 Phase 4 Beraprost Sodium;Sildenafil Citrate
30 Measuring Outcomes In Patients With Pulmonary Arterial Hypertension Not on Active Treatment (MOTION) Completed NCT02191137 Phase 4 Riociguat (Adempas, BAY63-2521)
31 Characterization of LV Strain Patterns in Mildly Elevated PCWP and PAH. Completed NCT01800292 Phase 4 sildenafil
32 Safety, Efficacy and Treatment Satisfaction in Patients With PAH Rapidly Switched From Epoprostenol to Remodulin Completed NCT00373360 Phase 4 treprostinil sodium
33 Effects of Propofol and Isoflurane on Pulmonary Vascular Resistance Completed NCT01212523 Phase 4 propofol and isoflurane
34 Acute Hemodynamic Effects of Sildenafil in Patients With Severe Aortic Stenosis Completed NCT01060020 Phase 4 Sildenafil
35 Evaluate Effect of Optison on Pulmonary Artery Systolic Pressure (PASP) and Pulmonary Vascular Resistance (PVR). Completed NCT00878878 Phase 4 Optison (Perflutren Protein-Type A Microspheres Injectable Suspension);Dextrose
36 A Transition Study From Flolan® to Remodulin® in Patients With Pulmonary Arterial Hypertension Completed NCT00058929 Phase 4 treprostinil sodium
37 Inhaled Nitric Oxide and Inhaled Prostacyclin After Cardiac Surgery for Heart Transplant or LVAD Placement Completed NCT01717209 Phase 4 Nitric Oxide;Prostacyclin
38 Efficacy Study of Riociguat and Its Effects on Exercise Performance and Pulmonary Artery Pressure at High Altitude Completed NCT02024386 Phase 4 Riociguat
39 BREATHE 5-OL: Tracleer (Bosentan) in Patients With Pulmonary Arterial Hypertension Related to Eisenmenger Physiology Completed NCT00367770 Phase 4 Tracleer®
40 Effects of Viagra on Heart Function in Patients With Heart Failure Completed NCT00781508 Phase 4 sildenafil
41 Clevidipine for Vasoreactivity Evaluation of the Pulmonary Arterial Bed Completed NCT01121458 Phase 4 Clevidipine
42 Effect of Tadalafil on Insulin Secretion and Insulin Sensitivity in Obese Men. Completed NCT02595684 Phase 4 Tadalafil;Placebo
43 Nebulized Heparin and Salbutamol in Mechanically Ventilated Patients With AECOPD Completed NCT03333395 Phase 4 Nebulized heparin and salbutamol;Nebulized salbutamol
44 Acute Effect of Pulmonary Desufflation on Cardiac Performance in COPD Patients Completed NCT01996124 Phase 4 Indacaterol Fumarate;Placebo
45 Effects of the Combination of Bosentan and Sildenafil Versus Sildenafil Monotherapy on Pulmonary Arterial Hypertension (PAH) Completed NCT00303459 Phase 4 bosentan;placebo
46 Transition From Injectable Prostacyclin Medication to Inhaled Prostacyclin Medication Completed NCT01268553 Phase 4 Treprostinil
47 Safety and Tolerability of Rapid Dose Titration of Subcutaneous Remodulin® Therapy in PAH Subjects (RAPID) Completed NCT02847260 Phase 4 Remodulin
48 Safety Evaluation of Inhaled Treprostinil Administration Following Transition From Inhaled Ventavis in Pulmonary Arterial Hypertension (PAH) Subjects Completed NCT00741819 Phase 4 Inhaled treprostinil
49 Study of Add-on Ambrisentan Therapy to Background Phosphodiesterase Type-5 Inhibitor (PDE5i) Therapy in Pulmonary Arterial Hypertension (ATHENA-1) Completed NCT00617305 Phase 4 Ambrisentan;Placebo;Sildenafil;Tadalafil
50 A Clinical Trial of Ambrisentan and Tadalafil in Pulmonary Arterial Hypertension Associated With Systemic Sclerosis Completed NCT01042158 Phase 4 tadalafil and ambrisentan upfront combination therapy

Search NIH Clinical Center for Pulmonary Hypertension

Inferred drug relations via UMLS 69 / NDF-RT 47 :


Cell-based therapeutics:


LifeMap Discovery
Data from LifeMap, the Embryonic Development and Stem Cells Database
Read about Pulmonary Hypertension cell therapies at LifeMap Discovery.

Cochrane evidence based reviews: hypertension, pulmonary

Genetic Tests for Pulmonary Hypertension

Genetic tests related to Pulmonary Hypertension:

# Genetic test Affiliating Genes
1 Primary Pulmonary Hypertension 28 BMPR2

Anatomical Context for Pulmonary Hypertension

MalaCards organs/tissues related to Pulmonary Hypertension:

38
Heart, Lung, Endothelial, Testes, Smooth Muscle, Brain, Liver
LifeMap Discovery
Data from LifeMap, the Embryonic Development and Stem Cells Database

Cells/anatomical compartments in embryo or adult related to Pulmonary Hypertension:
# Tissue Anatomical CompartmentCell Relevance
1 Lung Pulmonary Capillary Plexus Affected by disease

Publications for Pulmonary Hypertension

Articles related to Pulmonary Hypertension:

(show top 50) (show all 2585)
# Title Authors Year
1
Screening for pulmonary hypertension in interstitial lung disease: Many reasons to ECHO! ( 29415370 )
2018
2
Fibrosing mediastinitis with pulmonary hypertension as a complication of pulmonary vein stenosis: A case report and review of the literature. ( 29369193 )
2018
3
Adherence and medication belief in patients with pulmonary arterial hypertension or chronic thromboembolic pulmonary hypertension: A nationwide population-based cohort survey. ( 29356440 )
2018
4
Chronic Thromboembolic Pulmonary Hypertension. ( 29446688 )
2018
5
Multiparametric Magnetic Resonance Imaging in the Assessment of Pulmonary Hypertension: Initial Experience of a One-Stop Study. ( 29435739 )
2018
6
Epidemiology and risk factors for chronic thromboembolic pulmonary hypertension. ( 29338883 )
2018
7
H2-receptor Antagonist Use and Mortality in Pulmonary Hypertension: Insight from the VA-CART Program. ( 29437490 )
2018
8
Dual pathology causing severe pulmonary hypertension following surgical repair of total anomalous pulmonary venous connection: Successful outcome following serial transcatheter interventions. ( 29440835 )
2018
9
Increasing Incidence and Prevalence of World Health Organization Groups 1 to 4 Pulmonary Hypertension: A Population-Based Cohort Study in Ontario, Canada. ( 29444925 )
2018
10
RV Fractional Area Change and TAPSE as Predictors of Severe Right Ventricular Dysfunction in Pulmonary Hypertension: A CMR Study. ( 29435740 )
2018
11
Macitentan in pulmonary hypertension due to left ventricular dysfunction. ( 29437943 )
2018
12
Determination of Cardiac Output in Pulmonary Hypertension Using Impedance Cardiography. ( 29428946 )
2018
13
Coping, social support and information in patients with pulmonary arterial hypertension or chronic thromboembolic pulmonary hypertension: A 2-year retrospective cohort study. ( 29326818 )
2018
14
Pulmonary Vascular Disease: Hemodynamic Assessment and Treatment Selection-Focus on Group II Pulmonary Hypertension. ( 29417467 )
2018
15
Incidence and characteristics of chronic thromboembolic pulmonary hypertension in Germany. ( 29450722 )
2018
16
Bosentan Therapy for Pulmonary Arterial Hypertension and Chronic Thromboembolic Pulmonary Hypertension: A Systemic Review and Meta-Analysis. ( 29393580 )
2018
17
Persistent Pulmonary Hypertension Without Underlying Cardiac Disease as a Presentation of Pulmonary Interstitial Glycogenosis. ( 29336713 )
2018
18
The Growing Burden of Pulmonary Hypertension in the Modern Era: A Zebra No More? ( 29444928 )
2018
19
Treatment of pulmonary hypertension with inhaled agents in the pediatric intensive care unit. ( 29436464 )
2018
20
Severe Compression of the Left Main Coronary Artery in a Patient with Chronic Thromboembolic Pulmonary Hypertension. ( 29321385 )
2018
21
Prognostic significance of pulmonary hypertension in patients with cystic fibrosis: A systematic review and meta-analysis. ( 29443734 )
2018
22
Sex-specific cardiopulmonary exercise testing indices to estimate the severity of inoperable chronic thromboembolic pulmonary hypertension. ( 29416329 )
2018
23
Haemodynamics to predict outcome in pulmonary hypertension due to left heart disease: a meta-analysis. ( 29439019 )
2018
24
Impact of Balloon Pulmonary Angioplasty on Hemodynamics and Clinical Outcomes in Patients with Chronic Thromboembolic Pulmonary Hypertension: the Initial Korean Experience. ( 29318791 )
2018
25
Approach to Pulmonary Hypertension: From CT to Clinical Diagnosis. ( 29432063 )
2018
26
Chronic thromboembolic pulmonary hypertension and homocysteinaemia. ( 29294117 )
2018
27
Surrogate Endpoints for Pulmonary Hypertension Management and Trial Design: Moving in the Right Direction. ( 29447738 )
2018
28
Will we be singing a different tune on combined post- and pre-capillary pulmonary hypertension? ( 29437950 )
2018
29
Pulmonary Hypertension, Mortality, and Cardiovascular Disease in CKD and ESRD Patients: A Systematic Review and Meta-analysis. ( 29429751 )
2018
30
New NO Donor NCX 1443: Therapeutic Effects on Pulmonary Hypertension in the SAD Mouse Model of Sickle Cell Disease. ( 29438213 )
2018
31
Balloon Pulmonary Angioplasty Relieves Hemodynamic Stress towards Untreated-Side Pulmonary Vasculature and Ameliorates Its Resistance in Patients with Chronic Thromboembolic Pulmonary Hypertension. ( 29360065 )
2018
32
Granzyme B deficiency promotes osteoblastic differentiation and calcification of vascular smooth muscle cells in hypoxic pulmonary hypertension. ( 29445095 )
2018
33
Management of pulmonary hypertension from left heart disease in candidates for orthotopic heart transplantation. ( 28932571 )
2017
34
Pulmonary Hypertension Complicating Connective Tissue Disease. ( 29032565 )
2017
35
Efficacy of inhaled nitric oxide in neonates with hypoxic respiratory failure and pulmonary hypertension: the Japanese experience. ( 28926340 )
2017
36
Haemodynamically proven pulmonary hypertension in a patient with GATA2 deficiency-associated pulmonary alveolarA proteinosis and fibrosis. ( 28495697 )
2017
37
Apoptosis Signal-regulating Kinase 1 Inhibition in Pulmonary Hypertension: Too Much to ASK? ( 28930481 )
2017
38
Pulmonary Hypertension Associated with Pulmonary Veno-occlusive Disease in Patients with Polycythemia Vera. ( 28824072 )
2017
39
Pulmonary Hypertension Prevalence and Prognosis in a Cohort of Patients with Hereditary Hemorrhagic Telangiectasia Undergoing Embolization of pAVMs. ( 28375020 )
2017
40
Balloon pulmonary angioplasty is effective for treating peripheral-type chronic thromboembolic pulmonary hypertension in elderly patients. ( 29278287 )
2017
41
Loss of smooth muscle cell hypoxia inducible factor-1I+ underlies increased vascular contractility in pulmonary hypertension. ( 27811062 )
2017
42
N-terminal pro-B-type natriuretic peptide for monitoring after balloon pulmonary angioplasty for chronic thromboembolic pulmonary hypertension. ( 29329761 )
2017
43
Role of Stromelysin 2 (Matrix Metalloproteinase 10) as a Novel Mediator of Vascular Remodeling Underlying Pulmonary Hypertension Associated With Systemic Sclerosis. ( 28805015 )
2017
44
Combination therapy with riociquat and inhaled treprostinil in inoperable and progressive chronic thromboembolic pulmonary hypertension. ( 27942445 )
2017
45
Contralateral Pulmonary Hypertension Following Resuscitation of Unilateral Ductal Origin of a Pulmonary Artery: A Multi-institutional Review. ( 28948321 )
2017
46
Pulmonary hypertension associated with combined fibrosing mediastinitis and bronchial anthracofibrosis: A retrospective analysis in a single Chinese hospital. ( 28419740 )
2017
47
Reply: Tidal Carbon Dioxide as a Prognostic Feature in Inoperable Chronic Thromboembolic Pulmonary Hypertension. ( 28806530 )
2017
48
Images in COPD: Combined Pulmonary Emphysema and Fibrosis with Pulmonary Hypertension. ( 28848914 )
2017
49
Impact of High-Priority Allocation on Lung and Heart-Lung Transplantation for Pulmonary Hypertension. ( 28527964 )
2017
50
The changing landscape of chronic thromboembolic pulmonary hypertension management. ( 29263176 )
2017

Variations for Pulmonary Hypertension

ClinVar genetic disease variations for Pulmonary Hypertension:

6 (show top 50) (show all 381)
# Gene Variation Type Significance SNP ID Assembly Location
1 BMPR2 BMPR2, 1-BP DEL, 2579T deletion Pathogenic
2 BMPR2 NM_001204.6(BMPR2): c.2695C> T (p.Arg899Ter) single nucleotide variant Pathogenic rs137852741 GRCh37 Chromosome 2, 203421083: 203421083
3 BMPR2 NM_001204.6(BMPR2): c.218C> G (p.Ser73Ter) single nucleotide variant Pathogenic rs137852742 GRCh37 Chromosome 2, 203329673: 203329673
4 BMPR2 BMPR2, 1-BP DEL, 355A deletion Pathogenic
5 BMPR2 NM_001204.6(BMPR2): c.354T> G (p.Cys118Trp) single nucleotide variant Pathogenic rs137852743 GRCh37 Chromosome 2, 203332348: 203332348
6 BMPR2 NM_001204.6(BMPR2): c.1040G> A (p.Cys347Tyr) single nucleotide variant Pathogenic rs137852744 GRCh37 Chromosome 2, 203395589: 203395589
7 BMPR2 NM_001204.6(BMPR2): c.1454A> G (p.Asp485Gly) single nucleotide variant Pathogenic rs137852745 GRCh37 Chromosome 2, 203417479: 203417479
8 BMPR2 NM_001204.6(BMPR2): c.1471C> T (p.Arg491Trp) single nucleotide variant Pathogenic rs137852746 GRCh37 Chromosome 2, 203417496: 203417496
9 BMPR2 BMPR2, 5-BP DEL, NT1099 deletion Pathogenic
10 BMPR2 NM_001204.6(BMPR2): c.507C> A (p.Cys169Ter) single nucleotide variant Pathogenic rs137852747 GRCh37 Chromosome 2, 203378530: 203378530
11 BMPR2 NM_001204.6(BMPR2): c.2617C> T (p.Arg873Ter) single nucleotide variant Pathogenic rs137852748 GRCh37 Chromosome 2, 203421005: 203421005
12 BMPR2 NM_001204.6(BMPR2): c.1472G> A (p.Arg491Gln) single nucleotide variant Pathogenic rs137852749 GRCh37 Chromosome 2, 203417497: 203417497
13 BMPR2 BMPR2, 2-BP DEL, 1-BP INS, NT690 indel Pathogenic
14 BMPR2 NM_001204.6(BMPR2): c.367T> C (p.Cys123Arg) single nucleotide variant Pathogenic rs137852750 GRCh37 Chromosome 2, 203332361: 203332361
15 BMPR2 NM_001204.6(BMPR2): c.367T> A (p.Cys123Ser) single nucleotide variant Pathogenic rs137852750 GRCh37 Chromosome 2, 203332361: 203332361
16 BMPR2 NM_001204.6(BMPR2): c.994C> T (p.Arg332Ter) single nucleotide variant Pathogenic rs137852751 GRCh37 Chromosome 2, 203395543: 203395543
17 BMPR2 NM_001204.6(BMPR2): c.2696G> C (p.Arg899Pro) single nucleotide variant Pathogenic rs137852752 GRCh37 Chromosome 2, 203421084: 203421084
18 BMPR2 NM_001204.6(BMPR2): c.631C> T (p.Arg211Ter) single nucleotide variant Pathogenic rs137852753 GRCh37 Chromosome 2, 203383554: 203383554
19 BMPR2 NM_001204.6(BMPR2): c.44delC (p.Pro15Hisfs) deletion Pathogenic rs483352902 GRCh37 Chromosome 2, 203242241: 203242241
20 BMPR2 NM_001204.6(BMPR2): c.77-?_247+?del (p.A26_Q82del) deletion Pathogenic GRCh37 Chromosome 2, 203329532: 203329702
21 BMPR2 BMPR2, EX10DEL deletion Pathogenic
22 BMPR2 BMPR2, EX1-13DEL deletion Pathogenic
23 BMPR2 NM_001204.6(BMPR2): c.1297C> T (p.Gln433Ter) single nucleotide variant Pathogenic rs137852756 GRCh37 Chromosome 2, 203407054: 203407054
24 KCNK3 NM_002246.2(KCNK3): c.608G> A (p.Gly203Asp) single nucleotide variant Pathogenic rs398123039 GRCh37 Chromosome 2, 26950859: 26950859
25 BMPR2 NM_001204.6(BMPR2): c.295T> C (p.Cys99Arg) single nucleotide variant Pathogenic rs863223425 GRCh37 Chromosome 2, 203332289: 203332289
26 BMPR2 NM_001204.6(BMPR2): c.377A> G (p.Asn126Ser) single nucleotide variant Pathogenic rs863223426 GRCh37 Chromosome 2, 203332371: 203332371
27 BMPR2 NM_001204.6(BMPR2): c.853-2A> G single nucleotide variant Pathogenic rs863223424 GRCh38 Chromosome 2, 202520085: 202520085
28 BMPR2 NM_001204.6(BMPR2): c.1128+1G> A single nucleotide variant Pathogenic rs863223420 GRCh38 Chromosome 2, 202530955: 202530955
29 BMPR2 NM_001204.6(BMPR2): c.1426_1450del25 (p.Leu476Glyfs) deletion Pathogenic rs863223422 GRCh38 Chromosome 2, 202552728: 202552752
30 ACVRL1 NM_000020.2(ACVRL1): c.430C> T (p.Arg144Ter) single nucleotide variant Pathogenic rs758683062 GRCh37 Chromosome 12, 52307459: 52307459
31 BMPR2 NM_001204.6(BMPR2): c.439C> T (p.Arg147Ter) single nucleotide variant Pathogenic rs869025366 GRCh37 Chromosome 2, 203378462: 203378462
32 BMPR2 NM_001204.6(BMPR2): c.1276G> C (p.Gly426Arg) single nucleotide variant Pathogenic/Likely pathogenic rs869025367 GRCh37 Chromosome 2, 203397455: 203397455
33 FOXF1 NC_000016.10: g.(?_86510584)_(86513143_?)del deletion Pathogenic GRCh38 Chromosome 16, 86510584: 86513143
34 BMPR2 NM_001204.6(BMPR2): c.1125_1128+16del deletion Pathogenic rs878854272 GRCh37 Chromosome 2, 203395674: 203395693
35 BMPR2 NM_001204.6(BMPR2): c.16C> T (p.Gln6Ter) single nucleotide variant Pathogenic rs886039219 GRCh38 Chromosome 2, 202377490: 202377490
36 BMPR2 NM_001204.6(BMPR2): c.174_175dupAT (p.Leu59Tyrfs) duplication Pathogenic rs886039223 GRCh38 Chromosome 2, 202464906: 202464907
37 BMPR2 NM_001204.6(BMPR2): c.1277-10_1277-9insGGG insertion Pathogenic rs886039220 GRCh38 Chromosome 2, 202542301: 202542302
38 BMPR2 NM_001204.6(BMPR2): c.1443_1445delAGA (p.Glu481del) deletion Pathogenic rs886039221 GRCh37 Chromosome 2, 203417468: 203417470
39 BMPR2 NM_001204.6(BMPR2): c.1469C> T (p.Ala490Val) single nucleotide variant Pathogenic rs886039222 GRCh38 Chromosome 2, 202552771: 202552771
40 BMPR2 NM_001204.6(BMPR2): c.1789C> T (p.Arg597Ter) single nucleotide variant Pathogenic rs886039673 GRCh38 Chromosome 2, 202555454: 202555454
41 BMPR2 NM_001204.6(BMPR2): c.637C> T (p.Arg213Ter) single nucleotide variant Pathogenic rs886041324 GRCh37 Chromosome 2, 203383560: 203383560
42 BMPR2 NM_001204.6(BMPR2): c.77-?_418+?del deletion Pathogenic GRCh37 Chromosome 2, 203329532: 203332412
43 BMPR2 NM_001204.6(BMPR2): c.1398G> A (p.Trp466Ter) single nucleotide variant Pathogenic rs1060502576 GRCh38 Chromosome 2, 202542432: 202542432
44 BMPR2 NM_001204.6(BMPR2): c.961C> T (p.Arg321Ter) single nucleotide variant Pathogenic rs1060502581 GRCh38 Chromosome 2, 202520195: 202520195
45 BMPR2 NM_001204.6(BMPR2): c.(76+1_77-1)_(*1_?)del deletion Pathogenic GRCh38 Chromosome 2, 202377551: 202559947
46 BMPR2 NM_001204.6(BMPR2): c.(418+1_419-1)_(*1_?)del deletion Pathogenic GRCh38 Chromosome 2, 202467690: 202559947
47 BMPR2 NM_001204.6(BMPR2): c.(1413+1_1414-1)_(*1_?)del deletion Pathogenic GRCh38 Chromosome 2, 202542448: 202559947
48 BMPR2 NM_001204.6(BMPR2): c.(?_-540)_(76+1_77-1)del deletion Pathogenic GRCh38 Chromosome 2, 202376935: 202464808
49 BMPR2 NM_001204.6(BMPR2): c.(?_-540)_(*1_?)del deletion Pathogenic GRCh38 Chromosome 2, 202376935: 202559947
50 BMPR2 NM_001204.6(BMPR2): c.(?_-1)_(76+1_77-1)del deletion Pathogenic GRCh38 Chromosome 2, 202377474: 202464808

Expression for Pulmonary Hypertension

Search GEO for disease gene expression data for Pulmonary Hypertension.

Pathways for Pulmonary Hypertension

Pathways related to Pulmonary Hypertension according to KEGG:

36
# Name Kegg Source Accession
1 TGF-beta signaling pathway hsa04350

GO Terms for Pulmonary Hypertension

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

# Name GO ID Score Top Affiliating Genes
1 extracellular space GO:0005615 9.76 ADM BMPR2 EDN1 ENG KCNK3 NPPA
2 integral component of plasma membrane GO:0005887 9.23 ACVRL1 BMPR2 CAV1 EDNRA EDNRB KCNK3
3 caveola GO:0005901 9.13 BMPR2 CAV1 NOS3

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

(show top 50) (show all 57)
# Name GO ID Score Top Affiliating Genes
1 heart development GO:0007507 9.93 ADM EDN1 EDNRA ENG FOXF1
2 positive regulation of cytosolic calcium ion concentration GO:0007204 9.92 ADM EDN1 EDNRA EDNRB
3 positive regulation of angiogenesis GO:0045766 9.91 ACVRL1 ADM ENG NOS3
4 female pregnancy GO:0007565 9.89 ADM NPPA THBD
5 negative regulation of cell growth GO:0030308 9.88 ACVRL1 BMPR2 NPPA NPPB
6 in utero embryonic development GO:0001701 9.88 ACVRL1 EDN1 EDNRA FOXF1 NOS3
7 response to lipopolysaccharide GO:0032496 9.85 ADM CPS1 EDN1 EDNRB THBD
8 cellular response to transforming growth factor beta stimulus GO:0071560 9.84 ACVRL1 CAV1 EDN1
9 positive regulation of pathway-restricted SMAD protein phosphorylation GO:0010862 9.81 ACVRL1 BMPR2 ENG
10 BMP signaling pathway GO:0030509 9.81 ACVRL1 BMPR2 ENG SMAD9
11 cellular response to BMP stimulus GO:0071773 9.78 ACVRL1 BMPR2 SMAD9
12 positive regulation of BMP signaling pathway GO:0030513 9.77 ACVRL1 BMPR2 ENG
13 blood vessel remodeling GO:0001974 9.75 ACVRL1 BMPR2 NOS3
14 branching involved in blood vessel morphogenesis GO:0001569 9.74 EDN1 EDNRA ENG
15 vasculogenesis GO:0001570 9.73 ADM CAV1 ENG FOXF1
16 activin receptor signaling pathway GO:0032924 9.72 ACVRL1 BMPR2
17 negative regulation of cAMP biosynthetic process GO:0030818 9.72 EDN1 EDNRA
18 response to muscle stretch GO:0035994 9.72 EDN1 NPPA
19 endocardial cushion morphogenesis GO:0003203 9.72 ACVRL1 ENG
20 negative regulation of blood coagulation GO:0030195 9.71 EDN1 THBD
21 negative regulation of DNA biosynthetic process GO:2000279 9.71 ACVRL1 BMPR2
22 response to dexamethasone GO:0071548 9.71 CPS1 EDN1
23 endocardial cushion development GO:0003197 9.71 BMPR2 FOXF1
24 lymphangiogenesis GO:0001946 9.7 ACVRL1 BMPR2
25 enteric nervous system development GO:0048484 9.7 EDNRA EDNRB
26 receptor guanylyl cyclase signaling pathway GO:0007168 9.7 NPPA NPPB
27 vasodilation GO:0042311 9.7 CPS1 EDNRB NOS3
28 response to hypoxia GO:0001666 9.7 ACVRL1 ADM CAV1 EDN1 EDNRA NPPA
29 response to transforming growth factor beta GO:0071559 9.69 EDN1 ENG
30 retina vasculature development in camera-type eye GO:0061298 9.69 ACVRL1 BMPR2
31 artery smooth muscle contraction GO:0014824 9.68 EDN1 EDNRA
32 body fluid secretion GO:0007589 9.68 EDN1 NPPB
33 negative regulation of platelet activation GO:0010544 9.68 NOS3 THBD
34 negative regulation of systemic arterial blood pressure GO:0003085 9.68 BMPR2 NPPA
35 midgut development GO:0007494 9.67 CPS1 FOXF1
36 artery development GO:0060840 9.67 ACVRL1 BMPR2
37 dorsal aorta morphogenesis GO:0035912 9.67 ACVRL1 ENG
38 positive regulation of heart rate GO:0010460 9.67 ADM EDN1 NPPA
39 regulation of systemic arterial blood pressure by endothelin GO:0003100 9.66 EDN1 NOS3
40 negative regulation of cellular protein metabolic process GO:0032269 9.65 EDN1 EDNRB
41 epithelial fluid transport GO:0042045 9.65 EDN1 EDNRB
42 lymphatic endothelial cell differentiation GO:0060836 9.65 ACVRL1 BMPR2
43 vascular smooth muscle cell development GO:0097084 9.64 ADM ENG
44 vein smooth muscle contraction GO:0014826 9.63 EDN1 EDNRB
45 negative regulation of vasoconstriction GO:0045906 9.62 ADM BMPR2
46 endocardial cushion to mesenchymal transition GO:0090500 9.59 ACVRL1 ENG
47 positive regulation of renal sodium excretion GO:0035815 9.58 EDN1 EDNRB NPPB
48 endothelin receptor signaling pathway GO:0086100 9.56 EDNRA EDNRB
49 regulation of blood vessel size GO:0050880 9.56 EDN1 NOS3 NPPA NPPB
50 regulation of the force of heart contraction by chemical signal GO:0003057 9.55 CAV1 NOS3

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

# Name GO ID Score Top Affiliating Genes
1 receptor binding GO:0005102 9.72 ADM CAV1 EDN1 NPPA NPPB
2 nitric-oxide synthase binding GO:0050998 9.48 CAV1 SLC6A4
3 signal transducer, downstream of receptor, with serine/threonine kinase activity GO:0004702 9.46 ACVRL1 BMPR2
4 activin binding GO:0048185 9.43 ACVRL1 ENG
5 transmembrane receptor protein serine/threonine kinase activity GO:0004675 9.4 ACVRL1 BMPR2
6 BMP binding GO:0036122 9.37 BMPR2 ENG
7 BMP receptor activity GO:0098821 9.26 ACVRL1 BMPR2
8 hormone activity GO:0005179 9.26 ADM EDN1 NPPA NPPB
9 hormone receptor binding GO:0051427 9.16 NPPA NPPB
10 endothelin receptor activity GO:0004962 8.62 EDNRA EDNRB

Sources for Pulmonary Hypertension

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
16 ExPASy
18 FMA
27 GO
28 GTR
29 HGMD
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 MedGen
41 MeSH
42 MESH via Orphanet
43 MGI
45 NCI
46 NCIt
47 NDF-RT
50 NINDS
51 Novoseek
53 OMIM
54 OMIM via Orphanet
58 PubMed
60 QIAGEN
65 SNOMED-CT via HPO
66 SNOMED-CT via Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
Content
Loading form....