MCID: PLM037
MIFTS: 77

Pulmonary Hypertension

Categories: Cardiovascular diseases, Respiratory diseases, Genetic diseases

Aliases & Classifications for Pulmonary Hypertension

MalaCards integrated aliases for Pulmonary Hypertension:

Name: Pulmonary Hypertension 39 38 12 41 3 14 69
Primary Pulmonary Hypertension 39 12 72 29 52 14
Idiopathic Pulmonary Arterial Hypertension 69
Idiopathic Pulmonary Hypertension 69
Hypertension, Pulmonary 42
Hypertension Pulmonary 52

Classifications:



External Ids:

Disease Ontology 12 DOID:14557 DOID:6432
ICD10 33 I27.0 I27.2
ICD9CM 35 416.0
NCIt 47 C97119 C3120
MeSH 42 D006976

Summaries for Pulmonary Hypertension

MedlinePlus : 41 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 venoocclusive disease 1 and antiphospholipid syndrome, and has symptoms including angina pectoris, chest pain and coughing. An important gene associated with Pulmonary Hypertension is BMPR2 (Bone Morphogenetic Protein Receptor Type 2), and among its related pathways/superpathways are Vascular smooth muscle contraction and Human Embryonic Stem Cell Pluripotency. 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 chronic pulmonary heart disease characterized by an increase of blood pressure in the pulmonary artery, pulmonary vein, or pulmonary capillaries, among others, has symptoms shortness of breath, dizziness, fainting, leg swelling.

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

Wikipedia : 72 Pulmonary hypertension (PH or PHTN) is an increase of blood pressure in the arteries of the lungs.... more...

Related Diseases for Pulmonary Hypertension

Diseases in the Pulmonary Hypertension family:

Pulmonary Hypertension, Primary, 4 Pulmonary Hypertension, Primary, 3
Pulmonary Hypertension, Primary, 2

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

(show top 50) (show all 416)
id Related Disease Score Top Affiliating Genes
1 pulmonary venoocclusive disease 1 32.5 ACVRL1 BMPR2 ENG FOXF1 KCNK3
2 antiphospholipid syndrome 30.9 EDN1 EDNRA EDNRB
3 myocardial infarction 30.8 ADM EDN1 NOS3 NPPA NPPB PDE5A
4 adult respiratory distress syndrome 30.7 EDN1 NPPA NPPB
5 pulmonary hypertension, familial primary, 1, with or without hht 12.4
6 chronic thromboembolic pulmonary hypertension 12.3
7 pulmonary hypertension, primary, 4 12.2
8 hyperuricemia, pulmonary hypertension, renal failure, and alkalosis 12.2
9 pulmonary hypertension, primary, 3 12.2
10 pulmonary hypertension, primary, 2 12.2
11 pulmonary hypertension, neonatal 12.1
12 pulmonary hypertension, chronic thromboembolic, without deep vein thrombosis 11.9
13 vater-like defects with pulmonary hypertension, laryngeal webs, and growth deficiency 11.9
14 pulmonary venoocclusive disease 11.6
15 persistent fetal circulation syndrome 11.6
16 alveolar capillary dysplasia with misalignment of pulmonary veins 11.5
17 lymphedema and cerebral arteriovenous anomaly 11.4
18 eisenmenger syndrome 11.4
19 alveolar capillary dysplasia 11.3
20 hypoxia 11.2
21 ciliary discoordination, due to random ciliary orientation 11.1 BMPR2 EDN1 NPPB
22 lethal congenital contractural syndrome 2 11.1 EDN1 NOS3 NPPB
23 elizabethkingia anophelis infection 11.1 BMPR2 EDN1 THBD
24 progressive black carbon hyperpigmentation of infancy 11.1 EDN1 NOS3
25 pulmonary vein stenosis 11.1
26 pyriform sinus cancer 11.1 KCNK3 NOS3 NPPA NPPB
27 conjunctival intraepithelial neoplasm 11.1 ACVRL1 BMPR2 EIF2AK4 KCNK3
28 autoimmune lymphoproliferative syndrome, type iib 11.1 BMPR2 EIF2AK4
29 munchausen by proxy 11.1 EDN1 NOS3 PDE5A
30 hematocele of tunica vaginalis testis 11.1 ACVRL1 BMPR2 EDN1 SLC6A4
31 adams-oliver syndrome 11.1
32 unilateral absence of a pulmonary artery 11.1
33 epithelial predominant pulmonary blastoma 11.1 CAV1 NOS3 NPPA PDE5A
34 american histoplasmosis 11.1 ACVRL1 NPPA NPPB
35 joint disorders 11.0 EDN1 NOS3 PDE5A
36 pulmonary branches stenosis 11.0 ACVRL1 ENG
37 broad ligament malignant neoplasm 11.0 ADM EDN1 NPPB
38 bernard-soulier syndrome, type a2 11.0 EDN1 NOS3 PDE5A
39 chylocele of tunica vaginalis 11.0 ADM EDN1 NPPA
40 idiopathic juxtafoveal retinal telangiectasia 11.0 ADM EDN1 NPPA NPPB
41 lymphadenitis 11.0 ADM EDN1 NOS3 NPPA
42 pyelonephritis 11.0 EDN1 NOS3 NPPB
43 urinary bladder posterior wall cancer 11.0 ACVRL1 NPPA NPPB
44 thoracic spinal canal and spinal cord meningioma 11.0 NOS3 NPPA SLC6A4
45 chronic fatigue syndrome 11.0 EDN1 NPPA THBD
46 respiratory syncytial virus infectious disease 11.0 ACVRL1 BMPR2 ENG
47 viral hepatitis 11.0 EDN1 NOS3 PDE5A
48 periampullary adenoma 11.0 EDN1 NOS3 NPPB
49 preeclampsia/eclampsia 1 11.0 EDN1 ENG NOS3 THBD
50 pituitary infarct 11.0 ACVRL1 BMPR2 ENG

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:


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

MGI Mouse Phenotypes related to Pulmonary Hypertension:

44 (show all 13)
id Description MGI Source Accession Score Top Affiliating Genes
1 cardiovascular system MP:0005385 10.39 ACVRL1 ADM BMPR2 CAV1 EDN1 EDNRA
2 behavior/neurological MP:0005386 10.3 CAV1 EDNRA EDNRB EIF2AK4 ENG KCNK3
3 homeostasis/metabolism MP:0005376 10.28 ACVRL1 ADM BMPR2 CAV1 CPS1 EDN1
4 mortality/aging MP:0010768 10.24 CPS1 EDN1 EDNRA EDNRB EIF2AK4 ENG
5 growth/size/body region MP:0005378 10.23 SMAD9 THBD ACVRL1 BMPR2 CAV1 EDN1
6 embryo MP:0005380 10.18 EDNRA EDNRB ENG FOXF1 SMAD9 THBD
7 muscle MP:0005369 10.17 SMAD9 ACVRL1 ADM BMPR2 CAV1 EDN1
8 digestive/alimentary MP:0005381 10.15 ACVRL1 CAV1 EDN1 EDNRA EDNRB ENG
9 hematopoietic system MP:0005397 10.13 ACVRL1 ADM BMPR2 CAV1 EDNRA EIF2AK4
10 nervous system MP:0003631 10.07 ACVRL1 BMPR2 CAV1 EDN1 EDNRA EDNRB
11 normal MP:0002873 9.85 ACVRL1 BMPR2 EDN1 EDNRA ENG KCNK3
12 renal/urinary system MP:0005367 9.65 ADM BMPR2 CAV1 EDN1 EDNRA EDNRB
13 respiratory system MP:0005388 9.4 ACVRL1 ADM BMPR2 CAV1 EDNRA EDNRB

Drugs & Therapeutics for Pulmonary Hypertension

FDA approved drugs:

(show all 7)
id 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 408)
id 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 9005-49-6 772 46507594
5
Spironolactone Approved Phase 4,Phase 1,Phase 2 1952-01-7, 52-01-7 5833
6
Iron Approved Phase 4,Phase 2,Phase 1 7439-89-6 23925
7
Epoprostenol Approved Phase 4,Phase 3,Phase 2 61849-14-7, 35121-78-9 5280427 5282411
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 54786 6918140
10
Nitric Oxide Approved Phase 4,Phase 3,Phase 2,Phase 1,Early Phase 1 10102-43-9 145068
11
Benzocaine Approved Phase 4,Phase 3,Phase 2 1994-09-7, 94-09-7 2337
12
Warfarin Approved Phase 4,Phase 2 81-81-2 6691 54678486
13
Ranolazine Approved, Investigational Phase 4,Phase 3,Phase 2,Phase 1 142387-99-3, 95635-55-5 56959
14
Isoflurane Approved, Vet_approved Phase 4 26675-46-7 3763
15
Propofol Approved, Investigational, Vet_approved Phase 4 2078-54-8 4943
16
Clevidipine Approved Phase 4 167221-71-8
17
Magnesium oxide Approved Phase 4 1309-48-4 14792
18
Zinc Approved Phase 4 7440-66-6 32051 23994
19
Silver sulfadiazine Approved, Vet_approved Phase 4 22199-08-2 441244
20
Acetazolamide Approved, Vet_approved Phase 4,Phase 2,Phase 3 59-66-5 1986
21
Miconazole Approved, Investigational, Vet_approved Phase 4,Phase 1 22916-47-8 4189
22
Nitroglycerin Approved, Investigational Phase 4,Phase 1 55-63-0 4510
23
Methazolamide Approved Phase 4 554-57-4 4100
24
Macitentan Approved Phase 4,Phase 3,Phase 2,Phase 1 441798-33-0
25 Selexipag Approved Phase 4,Phase 3,Phase 2 475086-01-2
26
Ibuprofen Approved Phase 4,Phase 3 15687-27-1 3672
27
Sevoflurane Approved, Vet_approved Phase 4 28523-86-6 5206
28
Nebivolol Approved, Investigational Phase 4,Phase 2,Phase 3 152520-56-4, 99200-09-6, 118457-14-0 71301
29
Bupivacaine Approved, Investigational Phase 4 2180-92-9, 38396-39-3 2474
30
Morphine Approved, Investigational Phase 4 57-27-2 5288826
31
Lidocaine Approved, Vet_approved Phase 4,Phase 1 137-58-6 3676
32 tannic acid Approved, Nutraceutical Phase 4,Phase 3,Phase 2
33
Lactitol Investigational Phase 4 585-86-4 3871
34 Antihypertensive Agents Phase 4,Phase 3,Phase 2,Phase 1,Early Phase 1
35 Endothelin Receptor Antagonists Phase 4,Phase 2,Phase 3,Phase 1,Early Phase 1
36 Phosphodiesterase 5 Inhibitors Phase 4,Phase 3,Phase 2,Phase 1
37 Phosphodiesterase Inhibitors Phase 4,Phase 3,Phase 2,Phase 1
38 Sildenafil Citrate Phase 4,Phase 2,Phase 3,Phase 1 171599-83-0
39 Vasodilator Agents Phase 4,Phase 2,Phase 3,Phase 1,Early Phase 1
40 Platelet Aggregation Inhibitors Phase 4,Phase 2,Phase 3,Phase 1
41 Anticoagulants Phase 4,Phase 2,Phase 3
42 calcium heparin Phase 4,Phase 3
43 Fibrinolytic Agents Phase 4,Phase 3,Phase 2
44 Heparin, Low-Molecular-Weight Phase 4,Phase 3
45 Plasminogen Phase 4,Phase 3
46 Tissue Plasminogen Activator Phase 4,Phase 3
47 diuretics Phase 4,Phase 2,Phase 3,Phase 1
48 Diuretics, Potassium Sparing Phase 4,Phase 3,Phase 1,Phase 2
49 Hormone Antagonists Phase 4,Phase 3,Phase 2,Phase 1,Early Phase 1
50 Hormones Phase 4,Phase 3,Phase 2,Phase 1,Early Phase 1

Interventional clinical trials:

(show top 50) (show all 1065)

id 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 Hemodynamic Response After Six Months of Sildenafil Unknown status NCT00483626 Phase 4 oral sildenafil
13 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
14 Effects of Iloprost Treatment in Adult Patients With Pulmonary Arterial Hypertension Related to Congenital Heart Disease Unknown status NCT01383083 Phase 4
15 Evaluation of Sildenafil for the Treatment of Moderate Congestive Heart Failure Unknown status NCT00793338 Phase 4 Sildenafil
16 Hypotonic Treprostinil Subcutaneous Infusion for Control of Treprostinil Related Site Pain Unknown status NCT01615627 Phase 4 HypotonicTreprostinil Solution;Eutonic Treprostinil Solution
17 The Study of Using Esophageal Pressure to Guide the PEEP Setting in Abdominal Hypertension Patients Who Undergoing Mechanical Ventilation Unknown status NCT01825304 Phase 4
18 Acute Effect of Sildenafil on Exercise Tolerance and Functional Capacity in COPD, IPF and Post Pneumonectomy Patients Unknown status NCT01382368 Phase 4 Sildenafil
19 Study of Ranolazine in the Treatment of Pulmonary Hypertension Associated With Diastolic Left Ventricular Dysfunction Completed NCT02133352 Phase 4 Ranolazine
20 VasoREACTivity Testing With Intravenous SILdenafil in Patients With Precapillary Pulmonary Hypertension Completed NCT01889966 Phase 4 Sildenafil
21 Natrecor for Pulmonary Hypertension in Lung Transplants Completed NCT00205426 Phase 4 Natrecor
22 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
23 Post-Marketing Surveillance of Ventavis® in Chinese Patients With Primary Pulmonary Hypertension (PPH) Completed NCT00882947 Phase 4 Ventavis (Iloprost, BAYQ6256)
24 Oral Sildenafil in Persistent Pulmonary Hypertension Secondary to Meconium Aspiration Syndrome in Newborns Completed NCT01757782 Phase 4 Oral Sildenafil;Placebo (distilled water)
25 Sildenafil for Secondary Pulmonary Hypertension Due to Valvular Disease Completed NCT00862043 Phase 4 Sildenafil Citrate;Placebo
26 Safely Change From Bosentan to Ambrisentan in Pulmonary Hypertension Completed NCT01330108 Phase 4 ambrisentan
27 Sildenafil Effects on Pulmonary Haemodynamics and Gas Exchange in Chronic Obstructive Pulmonary Disease (COPD) Completed NCT00491803 Phase 4 Sildenafil;Sildenafil
28 Inhaled Iloprost for Sarcoidosis-associated Pulmonary Hypertension Completed NCT00403650 Phase 4 Iloprost
29 Phase IV Study of Chronic Infusional Epoprostenol for Severe Primary Pulmonary Hypertension Completed NCT00004754 Phase 4 epoprostenol
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 BREATHE 5-OL: Tracleer (Bosentan) in Patients With Pulmonary Arterial Hypertension Related to Eisenmenger Physiology Completed NCT00367770 Phase 4 Tracleer®
39 Efficacy Study of Riociguat and Its Effects on Exercise Performance and Pulmonary Artery Pressure at High Altitude Completed NCT02024386 Phase 4 Riociguat
40 Clevidipine for Vasoreactivity Evaluation of the Pulmonary Arterial Bed Completed NCT01121458 Phase 4 Clevidipine
41 Effects of Viagra on Heart Function in Patients With Heart Failure Completed NCT00781508 Phase 4 sildenafil
42 Effects of the Combination of Bosentan and Sildenafil Versus Sildenafil Monotherapy on Pulmonary Arterial Hypertension (PAH) Completed NCT00303459 Phase 4 bosentan;placebo
43 Transition From Injectable Prostacyclin Medication to Inhaled Prostacyclin Medication Completed NCT01268553 Phase 4 Treprostinil
44 Effect of Tadalafil on Insulin Secretion and Insulin Sensitivity in Obese Men. Completed NCT02595684 Phase 4 Tadalafil;Placebo
45 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
46 Acute Effect of Pulmonary Desufflation on Cardiac Performance in COPD Patients Completed NCT01996124 Phase 4 Indacaterol Fumarate;Placebo
47 Safety Evaluation of Inhaled Treprostinil Administration Following Transition From Inhaled Ventavis in Pulmonary Arterial Hypertension (PAH) Subjects Completed NCT00741819 Phase 4 Inhaled treprostinil
48 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
49 Safety and Tolerability of Rapid Dose Titration of Subcutaneous Remodulin® Therapy in PAH Subjects (RAPID) Completed NCT02847260 Phase 4 Remodulin
50 Assess the Efficacy and Safety of Sildenafil When Added to Bosentan in the Treatment of Pulmonary Arterial Hypertension Completed NCT00323297 Phase 4 Bosentan;Bosentan;Sildenafil Citrate

Search NIH Clinical Center for Pulmonary Hypertension

Inferred drug relations via UMLS 69 / NDF-RT 48 :


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:

id Genetic test Affiliating Genes
1 Primary Pulmonary Hypertension 29

Anatomical Context for Pulmonary Hypertension

MalaCards organs/tissues related to Pulmonary Hypertension:

39
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:
id 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 2529)
id Title Authors Year
1
Chronic Thromboembolic Pulmonary Hypertension: Epidemiology, Diagnosis, and Management. ( 28832374 )
2017
2
Activated TAFI Promotes the Development of Chronic Thromboembolic Pulmonary Hypertension: A Possible Novel Therapeutic Target. ( 28289017 )
2017
3
Imaging in Chronic Thromboembolic Pulmonary Hypertension. ( 28060193 )
2017
4
Bronchopulmonary dysplasia and pulmonary hypertension: a meta-analysis. ( 28079864 )
2017
5
Novel Predictor of Lung Injury After Balloon Pulmonary Angioplasty in Patients With Chronic Thromboembolic Pulmonary Hypertension. ( 28717116 )
2017
6
Impact of High-Priority Allocation on Lung and Heart-Lung Transplantation for Pulmonary Hypertension. ( 28527964 )
2017
7
Contralateral Pulmonary Hypertension Following Resuscitation of Unilateral Ductal Origin of a Pulmonary Artery: A Multi-institutional Review. ( 28948321 )
2017
8
High Prevalence of Pulmonary Hypertension Complicates the Care of Infants with Omphalocele. ( 28704835 )
2017
9
Aquaporin 1 controls the functional phenotype of pulmonary smooth muscle cells in hypoxia-induced pulmonary hypertension. ( 28409279 )
2017
10
An Update on the Management of Chronic Thromboembolic Pulmonary Hypertension. ( 27989311 )
2017
11
Cobalamin disorder CblC presenting with hemolytic uremic syndrome and pulmonary hypertension. ( 28610805 )
2017
12
Statins may be beneficial for patients with pulmonary hypertension secondary to lung diseases. ( 28932549 )
2017
13
Epidemiology and management of chronic thromboembolic pulmonary hypertension: Experience from two expert centers. ( 28528258 )
2017
14
Management of pulmonary hypertension from left heart disease in candidates for orthotopic heart transplantation. ( 28932571 )
2017
15
Stepwise optimisation of balloon pulmonary angioplasty in a patient with severe non-operable chronic thromboembolic pulmonary hypertension. ( 28691912 )
2017
16
Osteoprotegerin Disruption Attenuates HySu-Induced Pulmonary Hypertension Through Integrin I+vI^3/FAK/AKT Pathway Suppression. ( 28077433 )
2017
17
Associations of Exercise Tolerance With Hemodynamic Parameters for Pulmonary Arterial Hypertension and for Chronic Thromboembolic Pulmonary Hypertension. ( 28671932 )
2017
18
Predictors of Exercise-Induced Pulmonary Hypertension in Patients with Asymptomatic Degenerative Mitral Regurgitation: Mechanistic Insights from 2D Speckle-Tracking Echocardiography. ( 28071674 )
2017
19
Microarray expression profile of circular RNAs in chronic thromboembolic pulmonary hypertension. ( 28682884 )
2017
20
Plexiform Vasculopathy in Chronic Thromboembolic Pulmonary Hypertension. ( 28892403 )
2017
21
Obstructive Sleep Apnea and Pulmonary Hypertension in Children. ( 28185814 )
2017
22
Comparison of echocardiographic parameters to assess right ventricular function in pulmonary hypertension. ( 28527022 )
2017
23
Pulmonary Hypertension in Infants, Children, and Young Adults. ( 28521893 )
2017
24
Understanding the Significance of Aerosolized Vasodilator Use in Pulmonary Hypertension: What Is Numerically, Statistically, and Clinically Meaningful? ( 28953496 )
2017
25
Evaluation of criteria for exercise-induced pulmonary hypertension in patients with resting pulmonary hypertension. ( 28931665 )
2017
26
Riociguat for patients with chronic thromboembolic pulmonary hypertension: Usefulness of transitioning from phosphodiesterase type 5 inhibitor. ( 28705306 )
2017
27
Loss of smooth muscle cell hypoxia inducible factor-1I+ underlies increased vascular contractility in pulmonary hypertension. ( 27811062 )
2017
28
Pulmonary Hypertension Associated with Pulmonary Veno-occlusive Disease in Patients with Polycythemia Vera. ( 28824072 )
2017
29
An Infant with Abernethy Malformation Associated with Heterotaxy and Pulmonary Hypertension. ( 28875963 )
2017
30
Beyond the inhaled nitric oxide in persistent pulmonary hypertension of the newborn. ( 28923474 )
2017
31
A Review of Clinical Trial Endpoints of Patients with Pulmonary Arterial Hypertension and Chronic Thromboembolic Pulmonary Hypertension and How They Relate to Patient Outcomes in the United States. ( 28025931 )
2017
32
Extensive revascularization by balloon pulmonary angioplasty for chronic thromboembolic pulmonary hypertension beyond hemodynamic normalization. ( 28804055 )
2017
33
Mosaic attenuation pattern in non-contrast computed tomography for the assessment of pulmonary perfusion in chronic thromboembolic pulmonary hypertension. ( 28942885 )
2017
34
Haemodynamically proven pulmonary hypertension in a patient with GATA2 deficiency-associated pulmonary alveolar proteinosis and fibrosis. ( 28495695 )
2017
35
Apoptosis Signal-regulating Kinase 1 Inhibition in Pulmonary Hypertension: Too Much to ASK? ( 28930481 )
2017
36
Plexogenic pulmonary hypertension associated with POEMS syndrome. ( 28856089 )
2017
37
Safety and tolerability of subcutaneous treprostinil in newborns with congenital diaphragmatic hernia and life-threatening pulmonary hypertension. ( 28389079 )
2017
38
Protective effects of aloperin on monocroline-induced pulmonary hypertension via regulation of Rho A/Rho kinsase pathway in rats. ( 28926926 )
2017
39
Modern diagnosis of chronic thromboembolic pulmonary hypertension. ( 28918335 )
2017
40
Extracorporeal Membrane Oxygenation for End-Stage Interstitial Lung Disease With Secondary Pulmonary Hypertension at Rest and Exercise: Insights From Simulation Modeling. ( 28863039 )
2017
41
Management of combined pre- and post-capillary pulmonary hypertension in advanced heart failure with reduced ejection fraction. ( 28947049 )
2017
42
Pulmonary endarterectomy in chronic thromboembolic pulmonary hypertension. ( 28750932 )
2017
43
Pulmonary Hypertension: Molecular Aspects of Current Therapeutic Intervention and Future Direction. ( 28926093 )
2017
44
Images in COPD: Combined Pulmonary Emphysema and Fibrosis with Pulmonary Hypertension. ( 28848914 )
2017
45
Patient-Specific iPSC-Derived Endothelial Cells Uncover Pathways that Protect against Pulmonary Hypertension in BMPR2 Mutation Carriers. ( 28017794 )
2017
46
Early Development of Right Ventricular Ischemic Lesions in a Novel Large Animal Model of Acute Right Heart Failure in Chronic Thromboembolic Pulmonary Hypertension. ( 28801076 )
2017
47
Bronchopulmonary dysplasia-associated pulmonary hypertension: clues from placental pathology. ( 28880261 )
2017
48
Pulmonary hypertension in Nigerian adults with sickle cell anemia. ( 28507438 )
2017
49
Hypoxia inhibits expression and function of mitochondrial thioredoxin 2 to promote pulmonary hypertension. ( 28130258 )
2017
50
The Y Chromosome Plays a Protective Role in Experimental Hypoxic Pulmonary Hypertension. ( 28934553 )
2017

Variations for Pulmonary Hypertension

ClinVar genetic disease variations for Pulmonary Hypertension:

6 (show top 50) (show all 381)
id 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.797G> C (p.Arg266Thr) single nucleotide variant Pathogenic rs374694591 GRCh37 Chromosome 2, 203383720: 203383720
28 BMPR2 NM_001204.6(BMPR2): c.853-2A> G single nucleotide variant Pathogenic rs863223424 GRCh38 Chromosome 2, 202520085: 202520085
29 BMPR2 NM_001204.6(BMPR2): c.1128+1G> A single nucleotide variant Pathogenic rs863223420 GRCh38 Chromosome 2, 202530955: 202530955
30 BMPR2 NM_001204.6(BMPR2): c.1426_1450del25 (p.Leu476Glyfs) deletion Pathogenic rs863223422 GRCh38 Chromosome 2, 202552728: 202552752
31 ACVRL1 NM_000020.2(ACVRL1): c.430C> T (p.Arg144Ter) single nucleotide variant Pathogenic rs758683062 GRCh37 Chromosome 12, 52307459: 52307459
32 BMPR2 NM_001204.6(BMPR2): c.439C> T (p.Arg147Ter) single nucleotide variant Pathogenic rs869025366 GRCh37 Chromosome 2, 203378462: 203378462
33 BMPR2 NM_001204.6(BMPR2): c.1276G> C (p.Gly426Arg) single nucleotide variant Pathogenic/Likely pathogenic rs869025367 GRCh37 Chromosome 2, 203397455: 203397455
34 FOXF1 NC_000016.10: g.(?_86510584)_(86513143_?)del deletion Pathogenic GRCh38 Chromosome 16, 86510584: 86513143
35 BMPR2 NM_001204.6(BMPR2): c.1125_1128+16del deletion Pathogenic rs878854272 GRCh37 Chromosome 2, 203395674: 203395693
36 BMPR2 NM_001204.6(BMPR2): c.16C> T (p.Gln6Ter) single nucleotide variant Pathogenic rs886039219 GRCh38 Chromosome 2, 202377490: 202377490
37 BMPR2 NM_001204.6(BMPR2): c.174_175dupAT (p.Leu59Tyrfs) duplication Pathogenic rs886039223 GRCh38 Chromosome 2, 202464906: 202464907
38 BMPR2 NM_001204.6(BMPR2): c.1277-10_1277-9insGGG insertion Pathogenic rs886039220 GRCh38 Chromosome 2, 202542301: 202542302
39 BMPR2 NM_001204.6(BMPR2): c.1443_1445delAGA (p.Glu481del) deletion Pathogenic rs886039221 GRCh37 Chromosome 2, 203417468: 203417470
40 BMPR2 NM_001204.6(BMPR2): c.1469C> T (p.Ala490Val) single nucleotide variant Pathogenic rs886039222 GRCh38 Chromosome 2, 202552771: 202552771
41 BMPR2 NM_001204.6(BMPR2): c.1789C> T (p.Arg597Ter) single nucleotide variant Pathogenic rs886039673 GRCh37 Chromosome 2, 203420177: 203420177
42 BMPR2 NM_001204.6(BMPR2): c.637C> T (p.Arg213Ter) single nucleotide variant Pathogenic rs886041324 GRCh37 Chromosome 2, 203383560: 203383560
43 BMPR2 NM_001204.6(BMPR2): c.77-?_418+?del deletion Pathogenic GRCh37 Chromosome 2, 203329532: 203332412
44 BMPR2 NM_001204.6(BMPR2): c.1398G> A (p.Trp466Ter) single nucleotide variant Pathogenic rs1060502576 GRCh38 Chromosome 2, 202542432: 202542432
45 BMPR2 NM_001204.6(BMPR2): c.961C> T (p.Arg321Ter) single nucleotide variant Pathogenic rs1060502581 GRCh38 Chromosome 2, 202520195: 202520195
46 BMPR2 NG_009363.1: g.(6225_93482)_(188621_?)del deletion Pathogenic GRCh38 Chromosome 2, 202377551: 202559947
47 BMPR2 NG_009363.1: g.(96364_142392)_(188621_?)del deletion Pathogenic GRCh38 Chromosome 2, 202467690: 202559947
48 BMPR2 NG_009363.1: g.(171122_181389)_(188621_?)del deletion Pathogenic GRCh38 Chromosome 2, 202542448: 202559947
49 BMPR2 NG_009363.1: g.(?_5609)_(6225_93482)del deletion Pathogenic GRCh38 Chromosome 2, 202376935: 202464808
50 BMPR2 NG_009363.1: g.(?_5609)_(188621_?)del deletion Pathogenic GRCh38 Chromosome 2, 202376935: 202559947

Expression for Pulmonary Hypertension

Search GEO for disease gene expression data for Pulmonary Hypertension.

Pathways for Pulmonary Hypertension

GO Terms for Pulmonary Hypertension

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

id 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 58)
id Name GO ID Score Top Affiliating Genes
1 positive regulation of cytosolic calcium ion concentration GO:0007204 9.94 ADM EDN1 EDNRA EDNRB
2 in utero embryonic development GO:0001701 9.92 ACVRL1 EDN1 EDNRA FOXF1 NOS3
3 negative regulation of cell growth GO:0030308 9.91 ACVRL1 BMPR2 NPPA NPPB
4 response to drug GO:0042493 9.91 CPS1 EDN1 ENG KCNK3 SLC6A4
5 positive regulation of angiogenesis GO:0045766 9.9 ACVRL1 ADM ENG NOS3
6 female pregnancy GO:0007565 9.89 ADM NPPA THBD
7 BMP signaling pathway GO:0030509 9.85 ACVRL1 BMPR2 ENG SMAD9
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 9.82 ACVRL1 BMPR2 ENG
10 cellular response to BMP stimulus GO:0071773 9.8 ACVRL1 BMPR2 SMAD9
11 heart development GO:0007507 9.8 ADM EDN1 EDNRA ENG FOXF1
12 positive regulation of BMP signaling pathway GO:0030513 9.79 ACVRL1 BMPR2 ENG
13 blood vessel remodeling GO:0001974 9.77 ACVRL1 BMPR2 NOS3
14 response to lipopolysaccharide GO:0032496 9.77 ADM CPS1 EDN1 EDNRB THBD
15 branching involved in blood vessel morphogenesis GO:0001569 9.76 EDN1 EDNRA ENG
16 vasculogenesis GO:0001570 9.76 ADM CAV1 ENG FOXF1
17 vasodilation GO:0042311 9.73 CPS1 EDNRB NOS3
18 activin receptor signaling pathway GO:0032924 9.72 ACVRL1 BMPR2
19 response to muscle stretch GO:0035994 9.72 EDN1 NPPA
20 negative regulation of cAMP biosynthetic process GO:0030818 9.72 EDN1 EDNRA
21 endocardial cushion morphogenesis GO:0003203 9.72 ACVRL1 ENG
22 negative regulation of blood coagulation GO:0030195 9.71 EDN1 THBD
23 negative regulation of DNA biosynthetic process GO:2000279 9.71 ACVRL1 BMPR2
24 response to dexamethasone GO:0071548 9.71 CPS1 EDN1
25 endocardial cushion development GO:0003197 9.71 BMPR2 FOXF1
26 positive regulation of heart rate GO:0010460 9.71 ADM EDN1 NPPA
27 lymphangiogenesis GO:0001946 9.7 ACVRL1 BMPR2
28 cellular response to mechanical stimulus GO:0071260 9.7 ENG NPPA
29 enteric nervous system development GO:0048484 9.7 EDNRA EDNRB
30 receptor guanylyl cyclase signaling pathway GO:0007168 9.69 NPPA NPPB
31 response to transforming growth factor beta GO:0071559 9.69 EDN1 ENG
32 body fluid secretion GO:0007589 9.69 EDN1 NPPB
33 artery smooth muscle contraction GO:0014824 9.68 EDN1 EDNRA
34 negative regulation of platelet activation GO:0010544 9.68 NOS3 THBD
35 midgut development GO:0007494 9.68 CPS1 FOXF1
36 artery development GO:0060840 9.68 ACVRL1 BMPR2
37 retina vasculature development in camera-type eye GO:0061298 9.67 ACVRL1 BMPR2
38 dorsal aorta morphogenesis GO:0035912 9.67 ACVRL1 ENG
39 positive regulation of renal sodium excretion GO:0035815 9.67 EDN1 EDNRB NPPB
40 epithelial fluid transport GO:0042045 9.66 EDN1 EDNRB
41 negative regulation of cellular protein metabolic process GO:0032269 9.66 EDN1 EDNRB
42 lymphatic endothelial cell differentiation GO:0060836 9.65 ACVRL1 BMPR2
43 vascular smooth muscle cell development GO:0097084 9.65 ADM ENG
44 positive regulation of urine volume GO:0035810 9.65 EDN1 EDNRB NPPB
45 regulation of systemic arterial blood pressure by endothelin GO:0003100 9.64 EDN1 NOS3
46 vein smooth muscle contraction GO:0014826 9.63 EDN1 EDNRB
47 negative regulation of vasoconstriction GO:0045906 9.63 ADM BMPR2
48 endocardial cushion to mesenchymal transition GO:0090500 9.61 ACVRL1 ENG
49 endothelin receptor signaling pathway GO:0086100 9.58 EDNRA EDNRB
50 regulation of the force of heart contraction by chemical signal GO:0003057 9.57 CAV1 NOS3

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

id Name GO ID Score Top Affiliating Genes
1 receptor binding GO:0005102 9.65 ADM CAV1 EDN1 NPPA NPPB
2 signal transducer, downstream of receptor, with serine/threonine kinase activity GO:0004702 9.48 ACVRL1 BMPR2
3 nitric-oxide synthase binding GO:0050998 9.46 CAV1 SLC6A4
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 9.32 BMPR2 ENG
7 hormone activity GO:0005179 9.26 ADM EDN1 NPPA NPPB
8 BMP receptor activity GO:0098821 9.16 ACVRL1 BMPR2
9 peptide hormone receptor binding GO:0051428 9.07 NPPA
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
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 MedGen
42 MeSH
43 MESH via Orphanet
44 MGI
46 NCI
47 NCIt
48 NDF-RT
51 NINDS
52 Novoseek
54 OMIM
55 OMIM via Orphanet
59 PubMed
60 QIAGEN
65 SNOMED-CT via HPO
66 SNOMED-CT via Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
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