MCID: PLM037
MIFTS: 73

Pulmonary Hypertension

Categories: Blood diseases, Cardiovascular diseases, Genetic diseases, Rare diseases, Respiratory diseases
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Aliases & Classifications for Pulmonary Hypertension

MalaCards integrated aliases for Pulmonary Hypertension:

Name: Pulmonary Hypertension 39 11 75 41 2 14 36 63 71 33
Primary Pulmonary Hypertension 39 11 75 28 53 5 14 31
Idiopathic Pulmonary Arterial Hypertension 71
Idiopathic Pulmonary Hypertension 71
Hypertension, Pulmonary, Primary 38
Pulmonary Htn - [hypertension] 33
Hypertension, Pulmonary 43
Hypertension Pulmonary 53

Classifications:



External Ids:

Disease Ontology 11 DOID:14557 DOID:6432
ICD9CM 34 416.0
MeSH 43 D006976
NCIt 49 C3120
SNOMED-CT 68 155328008 26174007
ICD10 31 I27.0
UMLS 71 C0020542 C0152171 C3203102

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 hypertension, primary, 1 and pulmonary venoocclusive disease, and has symptoms including hemoptysis, angina pectoris and dyspnea. An important gene associated with Pulmonary Hypertension is BMPR2 (Bone Morphogenetic Protein Receptor Type 2), and among its related pathways/superpathways are MicroRNAs in cardiomyocyte hypertrophy and miRNAs involved in DNA damage response. The drugs Bupivacaine and Ranolazine have been mentioned in the context of this disorder. Affiliated tissues include Lung, heart and endothelial, and related phenotypes are muscle and cardiovascular system

CDC: 2 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

PubMed Health : 63 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.

Disease Ontology 11 Pulmonary hypertension: A hypertension characterized by an increase of blood pressure in the pulmonary artery, pulmonary vein or pulmonary capillaries.

Primary pulmonary hypertension: 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.

Wikipedia: 75 Pulmonary hypertension (PH or PHTN) is a condition of increased blood pressure in the arteries of the... more...

Related Diseases for Pulmonary Hypertension

Diseases in the Pulmonary Hypertension family:

Pulmonary Hypertension, Primary, 1 Pulmonary Hypertension, Primary, 5
Pulmonary Hypertension, Primary, 2 Pulmonary Hypertension, Primary, 3
Pulmonary Hypertension, Primary, 4 Rare Pulmonary Hypertension

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

(show top 50) (show all 1418)
# Related Disease Score Top Affiliating Genes
1 pulmonary hypertension, primary, 1 33.9 KCNK3 BMPR2 ACVRL1
2 pulmonary venoocclusive disease 33.3 PDE5A NPPB KCNK3 EDNRA EDN1 CAV1
3 persistent fetal circulation syndrome 33.2 PDE5A MIR21 EDNRA EDN1
4 patent ductus arteriosus 1 32.9 PDE5A NPPB EDN1 BMPR2
5 diaphragmatic hernia, congenital 32.9 PDE5A EDNRA EDN1 BMPR2
6 heritable pulmonary arterial hypertension 32.8 KCNK3 CAV1 BMPR2 ACVRL1
7 hypertension, essential 32.8 PDE5A NPPB NPPA MIR22 MIR21 MIR17
8 interstitial lung disease 2 32.7 MIR21 MIR17 EDN1 CAV1 BMPR2
9 pulmonary fibrosis 32.7 MIR21 EDNRA EDN1 CAV1
10 pulmonary valve insufficiency 32.6 PDE5A NPPB KCNK3 EDN1 BMPR2
11 vascular disease 32.5 SLC6A4 PDE5A MIR21 EDN1 BMPR2 ACVRL1
12 tricuspid valve insufficiency 32.5 PDE5A NPPB EDN1 BMPR2
13 lung disease 32.4 NPPB MIR21 MIR17 EDN1 ADM
14 heart disease 32.3 SLC6A4 PDE5A NPPB NPPA MIR21 MIR17
15 heart septal defect 32.3 NPPB NPPA MIR17
16 atrial heart septal defect 32.3 NPPB NPPA EDN1 BMPR2
17 mitral valve stenosis 32.2 NPPB NPPA EDN1
18 liver cirrhosis 32.2 NPPA MALAT1 EDN1 ADM
19 pulmonary disease, chronic obstructive 32.2 PDE5A NPPB MIR21 MIR17
20 congestive heart failure 32.1 PDE5A NPPB NPPA EDNRA EDN1 ADM
21 pulmonary embolism 32.1 TNXA PDE5A NPPB
22 sleep apnea 32.0 SLC6A4 NPPB KCNK3 EDN1
23 portal hypertension 32.0 EDNRA EDN1 BMPR2
24 pulmonary edema 32.0 NPPB NPPA EDN1
25 ventricular septal defect 31.9 NPPB NPPA MIR17
26 patent foramen ovale 31.9 PDE5A NPPB NPPA EDNRA EDN1 BMPR2
27 meconium aspiration syndrome 31.9 PDE5A EDN1 CPS1
28 mitral valve disease 31.8 NPPB NPPA MIR21 EDN1
29 limited scleroderma 31.7 PDE5A EDNRA EDN1 CAV1
30 connective tissue disease 31.6 MIR22 MIR21 MIR20A MIR17 EDNRA EDN1
31 chronic kidney disease 31.6 NPPB NPPA MIR21 EDN1 ADM
32 cardiogenic shock 31.5 NPPB NPPA
33 hereditary hemorrhagic telangiectasia 31.5 KCNK3 BMPR2 ACVRL1
34 kidney disease 31.5 NPPB NPPA MIR21 EDNRA EDN1 ADM
35 hepatopulmonary syndrome 31.5 EDNRA EDN1 CAV1 BMPR2 ACVRL1
36 aortic valve disease 2 31.4 NPPB NPPA MIR22 MIR21 MIR17
37 systolic heart failure 31.4 PDE5A NPPB EDN1 ADM
38 diastolic heart failure 31.4 PDE5A NPPB EDN1
39 dilated cardiomyopathy 31.4 NPPB NPPA MIR21 MIR17 EDNRA EDN1
40 heart valve disease 31.4 NPPB NPPA MIR22 MIR21 MIR17 EDN1
41 impotence 31.3 PDE5A EDNRA EDN1
42 tetralogy of fallot 31.3 NPPB NPPA EDN1 ADM
43 oligohydramnios 31.3 NPPB EDN1 ADM
44 pre-eclampsia 31.2 PDE5A NPPA MALAT1 EDN1 ADM
45 chronic pulmonary heart disease 31.1 SLC6A4 PDE5A NPPB KCNK3 EDNRA EDN1
46 hypertrophic cardiomyopathy 31.1 NPPB NPPA MIR22 MIR21 MIR17 EDN1
47 hypertensive heart disease 31.1 NPPB NPPA EDN1
48 peripheral vascular disease 31.1 PDE5A EDN1 ACVRL1
49 lipoprotein quantitative trait locus 31.0 PDE5A NPPB NPPA MIR22 MIR21 MIR17
50 aortic valve disease 1 31.0 NPPB MIR21 MIR17 EDN1

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:


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

MGI Mouse Phenotypes related to Pulmonary Hypertension:

45
# Description MGI Source Accession Score Top Affiliating Genes
1 muscle MP:0005369 9.65 ACVRL1 ADM BMPR2 CAV1 EDN1 EDNRA
2 cardiovascular system MP:0005385 9.36 ACVRL1 ADM BMPR2 CAV1 EDN1 EDNRA

Drugs & Therapeutics for Pulmonary Hypertension

PubMed Health treatment related to Pulmonary Hypertension: 63

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." )

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

(show top 50) (show all 316)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Bupivacaine Approved, Investigational Phase 4 2180-92-9, 38396-39-3 2474
2
Ranolazine Approved, Investigational Phase 4 142387-99-3, 95635-55-5 56959
3
Reviparin Approved, Investigational Phase 4 9041-08-1
4
Sevoflurane Approved, Vet_approved Phase 4 28523-86-6 5206
5
Isoflurane Approved, Vet_approved Phase 4 26675-46-7 3763
6
Propofol Approved, Investigational, Vet_approved Phase 4 2078-54-8 4943
7
Silver sulfadiazine Approved, Vet_approved Phase 4 22199-08-2 441244
8
Racepinephrine Approved, Vet_approved Phase 4 51-43-4, 329-65-7 838 5816
9
Racephedrine Approved, Experimental Phase 4 299-42-3, 90-82-4, 90-81-3 5032 9294 7028
10
Phenylephrine Approved Phase 4 59-42-7 6041
11
Oxymetazoline Approved, Investigational Phase 4 1491-59-4 4636
12
Methazolamide Approved Phase 4 554-57-4 5353756 4100
13
Clevidipine Approved, Investigational Phase 4 167221-71-8 153994
14
Plasminogen Approved Phase 4 9001-91-6
15
Heparin, bovine Approved, Investigational, Withdrawn Phase 4 9005-49-6 22833565 9812414 772
16
Spironolactone Approved Phase 4 1952-01-7, 52-01-7 5833
17
Epoprostenol Approved Phase 4 61849-14-7, 35121-78-9 5282411
18
Iloprost Approved, Investigational Phase 4 78919-13-8 6443959 6435378 5311181
19
Macitentan Approved Phase 4 441798-33-0 16004692
20
Nebivolol Approved, Investigational Phase 4 152520-56-4, 99200-09-6, 118457-14-0 71301
21
Acetazolamide Approved, Vet_approved Phase 4 59-66-5, 1424-27-7 1986
22
Arginine Approved, Investigational, Nutraceutical Phase 4 74-79-3 6322
23
Beraprost Investigational Phase 4 88430-50-6 2352 5282428
24
Tezosentan Investigational Phase 4 180384-57-0
25 Analgesics, Opioid Phase 4
26 Narcotics Phase 4
27 Sodium Channel Blockers Phase 4
28 Platelet Aggregation Inhibitors Phase 4
29 Anesthetics Phase 4
30 Cardiotonic Agents Phase 4
31 Adrenergic beta-Agonists Phase 4
32 Adrenergic Agonists Phase 4
33 Adrenergic Agents Phase 4
34 Heparin, Low-Molecular-Weight Phase 4
35 Hemostatics Phase 4
36 Coagulants Phase 4
37 Arginine Vasopressin Phase 4
38 Vasopressins Phase 4
39 Vasoconstrictor Agents Phase 4
40 Adrenergic alpha-Agonists Phase 4
41 Sympathomimetics Phase 4
42 Epinephryl borate Phase 4
43 Mydriatics Phase 4
44 Nasal Decongestants Phase 4
45 Calcium heparin Phase 4
46 Tissue Plasminogen Activator Phase 4
47
Thromboplastin Phase 4
48 Hormone Antagonists Phase 4
49 Diuretics, Potassium Sparing Phase 4
50 diuretics Phase 4

Interventional clinical trials:

(show top 50) (show all 1215)
# Name Status NCT ID Phase Drugs
1 Use of the Endothelin-1 Antagonist Bosentan in Patients With Established Pulmonary Hypertension and Fibrotic Lung Disease. - A Randomised, Placebo-Controlled, Double-Blinded Study. Unknown status NCT00637065 Phase 4 Bosentan;Placebo
2 A Double Blind, Placebo Controlled Trial of Oral Riociguat for Sarcoidosis Associated Pulmonary Hypertension Unknown status NCT02625558 Phase 4 Riociguat;Placebo
3 Long Acting Phosphodiesterase 5 Inhibitors as Add-on Therapy for Patients With Pulmonary Hypertension Treated With Prostanoids. Unknown status NCT00705588 Phase 4 Tadalafil;Vardenafil
4 Comparative Effect of Combined High Spinal and General Anaesthesia With General Anaesthesia Alone On Right Ventricular Function In Patients With Mitral Valvular Disease With Pulmonary Hypertension Unknown status NCT03013075 Phase 4 Bupivacaine heavy and Morphine;General Anesthetics
5 Inhaled Iloprost for Disproportionate Pulmonary Hypertension in Chronic Obstructive Pulmonary Disease Unknown status NCT01116063 Phase 4 Inhaled iloprost
6 Vardenafil as add-on Therapy for Patients With Pulmonary Hypertension Treated With Inhaled Iloprost Unknown status NCT01649739 Phase 4 Levitra
7 Comparison of Efficacy Different Treatment Regimens in Pulmonary Hypertension Secondary to Lung Disease and or Hypoxia Unknown status NCT01449253 Phase 4 Sildenafil;Bosentan
8 Patients With Pulmonary Hypertension or Interstitial Lung Disease Travelling to Altitude - Effect of Nocturnal Oxygen Therapy on Breathing and Sleep Unknown status NCT02150616 Phase 4 Oxygen;Sham oxygen (room air)
9 Upfront Riociguat and Ambrisentan Combination Therapy for Pulmonary Arterial Hypertension: A Safety and Efficacy Pilot Study Unknown status NCT03809156 Phase 4 Riociguat Oral Product
10 Effects of Spironolactone on Collagen Metabolism in Pulmonary Arterial Hypertension Unknown status NCT01468571 Phase 4 Spironolactone;Placebo
11 Randomized Controlled Trial to Compare the Efficacy of Combination Therapy vs Monotherapy for Pulmonary Arterial Hypertension in Systemic Sclerosis Unknown status NCT03053739 Phase 4 Sildenafil 20mg and Bosentan 62.5mg;Sildenafil 20mg and Placebo
12 Hemodynamic Evaluation of Patients With Pulmonary Arterial Hypertension. Response to Sildenafil Treatment Unknown status NCT00483626 Phase 4 oral sildenafil
13 Raising the Bars in the Treatment of Pulmonary Arterial Hypertension: Goal Oriented Strategy to Preserve Ejection Fraction Trial Unknown status NCT03236818 Phase 4 ERA and PDE-5I (Sildenafil, Tadalafil, Bosentan, Macitentan)
14 Intravenous Iron Treatment In Iron Deficient Patients With Idiopathic Pulmonary Arterial Hypertension Unknown status NCT01288651 Phase 4 Ferricarboxymaltose
15 A Randomized, Double-blind, Placebo Controlled, Multi-center Study to Assess the Effect of Ranolazine on Outcomes in Subjects With Pulmonary Hypertension and Right Ventricular Dysfunction Accompanied by a Comparative Study of Cellular Metabolism in Subjects With Pulmonary Hypertension With and Without Right Ventricular Dysfunction Completed NCT01839110 Phase 4 Ranolazine;Placebo
16 Proof of Concept Study of Ranolazine in the Treatment of Pulmonary Hypertension Associated With Diastolic Left Ventricular Dysfunction Completed NCT02133352 Phase 4 Ranolazine
17 Comparison of Thrombgolytic and Anticoagulation Therapy in Submassive Pulmonary Embolism in Context of Pulmonary Hypertension, Right Heart Failure and Patient Functional Ability Completed NCT02132689 Phase 4 Actilyse (Thrombolytic therapy);Heparine (Standard anticoagulation therapy)
18 Inhaled Iloprost for Sarcoidosis Associated Pulmonary Hypertension Completed NCT00403650 Phase 4 Iloprost
19 Vasoreactivity Testing With Intravenous Sildenafil in Patients With Precapillary Pulmonary Hypertension (Treatment Optimisation Study) Completed NCT01889966 Phase 4 Sildenafil
20 Acute Effects of a Single Dose of Sildenafil (20mg/40mg) on Pulmonary Haemodynamics and Gas Exchange at Rest and During Exercise in COPD Patients With Pulmonary Hypertension Completed NCT00491803 Phase 4 Sildenafil
21 Acute Effect of Recombinant Human Brain Natriuretic Peptide in Patients With Pulmonary Hypertension Associated With Acute Exacerbation of Chronic Pulmonary Disease Completed NCT02742909 Phase 4 rhBNP;placebo
22 Patients With Pulmonary Hypertension or Interstitial Lung Disease Travelling to Altitude - Effect of Nocturnal Oxygen Therapy on Exercise Performance Completed NCT02143687 Phase 4 Oxygen;Sham oxygen (room air)
23 A Randomized, Double-blind, Placebo Controlled, Multi-center Study to Assess the Effect of Ranolazine in Subjects With Pulmonary Hypertension and Right Ventricular Dysfunction Using Cardiovascular MRI Completed NCT02829034 Phase 4 Ranolazine;Placebo
24 Phase IV Study of Chronic Infusional Epoprostenol for Severe Primary Pulmonary Hypertension Completed NCT00004754 Phase 4 epoprostenol
25 A Randomized, Placebo Controlled, Single Center Clinical Trial for Evaluation of Efficacy and Safety of Sildenafil Administration in the Cardiac ICU Following Mitral Valve Surgery in Patients With Pulmonary Hypertension Completed NCT02378649 Phase 4 Sildenafil;Placebo
26 Phase IV Study on the Effects of Sildenafil in Combination With Pulmonary Rehabilitation Program on Exercise Tolerance in Patients With COPD and Pulmonary Hypertension Completed NCT01055405 Phase 4 Sildenafil
27 Exercise Induced Pulmonary Hypertension in Systemic Sclerosis and Treatment With Ambrisentan: A Prospective Single Center, Open Label, Pilot Study Completed NCT01051960 Phase 4 Ambrisentan
28 Oral Sildenafil in Persistent Pulmonary Hypertension of Neonates Secondary to Meconium Aspiration Syndrome: A Randomized Placebo Controlled Trial Completed NCT01757782 Phase 4 Oral Sildenafil;Placebo (distilled water)
29 Randomised Cross-over Pilot Study to Determine the Effects of Isoflurane and Propofol on Pulmonary Vascular Resistance in Children With Pulmonary Hypertension. Completed NCT01212523 Phase 4 propofol and isoflurane
30 Safely Change From Bosentan to Ambrisentan in Pulmonary Hypertension Completed NCT01330108 Phase 4 ambrisentan
31 Echo Study - Characterization of LV Strain Patterns in Patients With Mildly Elevated PCWP and Pulmonary Hypertension Completed NCT01800292 Phase 4 sildenafil
32 Sildenafil for Improving Outcomes After Valvular Correction Completed NCT00862043 Phase 4 Sildenafil Citrate;Placebo
33 Rapid Switch From Intravenous Epoprostenol to Intravenous Remodulin® (Treprostinil Sodium) in Patients With Stable Pulmonary Arterial Hypertension: Safety, Efficacy and Treatment Satisfaction Completed NCT00373360 Phase 4 treprostinil sodium
34 A Multicenter, Randomized, Parallel Placebo-Controlled Study of the Safety and Efficacy of Subcutaneous Remodulin® Therapy After Transition From Flolan® in Patients With Pulmonary Arterial Hypertension Completed NCT00058929 Phase 4 treprostinil sodium
35 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
36 Natrecor® (Nesiritide) as a Nitric Oxide Sparing Agent in Patients Undergoing Lung Transplantation Completed NCT00205426 Phase 4 Natrecor
37 A Phase 4, Placebo Controlled, Single-blind, Cross-over Safety Study to Evaluate the Effect of Optison on Pulmonary Artery Systolic Pressure (PASP) and Pulmonary Vascular Resistance (PVR) as Measured by Right Heart Catheterization Completed NCT00878878 Phase 4 Optison (Perflutren Protein-Type A Microspheres Injectable Suspension);Dextrose
38 Efficacy of Beraprost in Lowering Pulmonary Arterial Pressure in Pulmonary Arterial Hypertension Children Associated With Left to Right Shunt Congenital Heart Defect Completed NCT03431649 Phase 4 Beraprost Sodium;Sildenafil Citrate
39 A Multi-center, Double-blind, Placebo-controlled Phase 4 Study in Patients With Pulmonary Arterial Hypertension to Assess the Effect of Selexipag on Daily Life Physical Activity and Patient's Self-reported Symptoms and Their Impacts Completed NCT03078907 Phase 4 Selexipag;Placebo
40 TRUST-2: An Open-label Continuation Trial of the Safety and Efficacy of Intravenous Remodulin® in Patients in India With Pulmonary Arterial Hypertension (PAH) Completed NCT03055221 Phase 4 Intravenous Treprostinil
41 COMPASS 3: An Open-label, Multi-Center Study Employing a Targeted 6-Minute Walk Test (6-MWT) Distance Threshold Approach to Guide Bosentan-Based Therapy and to Assess the Utility of Magnetic Resonance Imaging (MRI) on Cardiac Remodeling Completed NCT00433329 Phase 4 Bosentan;Sildenafil
42 An Open Label, Multi-center Study Evaluating the Safety of Long-term Inhaled Treprostinil Administration Following Transition From Inhaled Ventavis® (Iloprost) in Subjects With Pulmonary Arterial Hypertension. Completed NCT00741819 Phase 4 Inhaled treprostinil
43 An Open-label, Multicenter Study of Ambrisentan and a Phosphodiesterase Type-5 Inhibitor Combination Therapy in Subjects With Pulmonary Arterial Hypertension Who Have Demonstrated a Sub-Optimal Response to a Phosphodiesterase Type-5 Inhibitor Completed NCT00617305 Phase 4 Ambrisentan;Placebo;Sildenafil;Tadalafil
44 An Open-label Extension of Study AC-066A401 Investigating the Safety and Tolerability of ACT-385781A Compared to Flolan® in Injectable Prostanoid Treatment-naïve Patients With Pulmonary Arterial Hypertension (PAH) Completed NCT01105117 Phase 4 ACT-385781A (Actelion Epoprostenol);Flolan®
45 A Phase IV, Open-label, Randomized, Multicenter Study of the Safety, Tolerability,and Pharmacokinetics of ACT- 385781A Compared to Flolan® in Injectable Prostanoid Treatment-naïve Patients With Pulmonary Arterial Hypertension (PAH) Completed NCT01105091 Phase 4 ACT-385781A (Actelion Epoprostenol);Flolan®
46 A Prospective, Multicenter, Single-arm, Open-label, Phase 4 Study to Evaluate the Effects of Macitentan on Right vEntricular Remodeling in Pulmonary ArterIal hypeRtension Assessed by Cardiac Magnetic Resonance Imaging Completed NCT02310672 Phase 4 Macitentan
47 A Multinational, Multicentre, Randomized, Double-blind Study To Assess The Efficacy And Safety Of Oral Sildenafil 20mg Tid Or Placebo When Added To Bosentan In The Treatment Of Subjects, Aged 18 Years And Above, With Pulmonary Arterial Hypertension (Pah) Completed NCT00323297 Phase 4 Bosentan;Sildenafil Citrate
48 A Multi-center, Randomized, Double-blind, Placebo-controlled Study to Evaluate the Effects of Tracleer (Bosentan) on Oxygen Saturation and Cardiac Hemodynamics in Patients With Pulmonary Arterial Hypertension Related to Eisenmenger Physiology Completed NCT00317486 Phase 4 bosentan
49 Effects of Combination of Bosentan and Sildenafil Versus Sildenafil Monotherapy on Morbidity and Mortality in Symptomatic Patients With Pulmonary Arterial Hypertension - A Multicenter, Double-blind, Randomized, Placebo-controlled, Parallel Group, Prospective, Event Driven Phase IV Study Completed NCT00303459 Phase 4 bosentan;placebo
50 A Multi-Center, Open-Label Extension Study to Protocol AC-052-405 to Evaluate the Safety and Efficacy of Tracleer (Bosentan) in Patients With Pulmonary Arterial Hypertension Related to Eisenmenger Physiology Completed NCT00367770 Phase 4 Tracleer®

Search NIH Clinical Center for Pulmonary Hypertension

Inferred drug relations via UMLS 71 / NDF-RT 50 :


Alprostadil
bosentan
Epoprostenol
Epoprostenol Sodium
Hydralazine
Hydralazine Hydrochloride
Nitroglycerin
Treprostinil

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

Anatomical Context for Pulmonary Hypertension

Organs/tissues related to Pulmonary Hypertension:

MalaCards : Heart, Lung, Endothelial, Smooth Muscle, Brain, Liver, Bone Marrow
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 30748)
# Title Authors PMID Year
1
Clinical outcomes of pulmonary arterial hypertension in carriers of BMPR2 mutation. 53 62 5
18356561 2008
2
Gross BMPR2 gene rearrangements constitute a new cause for primary pulmonary hypertension. 53 62 5
15775752 2005
3
BMPR2 mutations found in Japanese patients with familial and sporadic primary pulmonary hypertension. 53 62 5
15146475 2004
4
BMPR2 haploinsufficiency as the inherited molecular mechanism for primary pulmonary hypertension. 53 62 5
11115378 2001
5
Heterozygous germline mutations in BMPR2, encoding a TGF-beta receptor, cause familial primary pulmonary hypertension. 53 62 5
10973254 2000
6
Familial primary pulmonary hypertension (gene PPH1) is caused by mutations in the bone morphogenetic protein receptor-II gene. 53 62 5
10903931 2000
7
Interleukin-6 modulates the expression of the bone morphogenic protein receptor type II through a novel STAT3-microRNA cluster 17/92 pathway. 53 62 46
19390056 2009
8
Functional analysis of bone morphogenetic protein type II receptor mutations underlying primary pulmonary hypertension. 62 5
12045205 2002
9
Clinical evaluation of nebulized verapamil in out-patients with pulmonary hypertension secondary to chronic obstructive pulmonary disease. 62 41
36336743 2022
10
The Effects of Sequential Ventilation Therapy on Blood Gas Indexes, Pulmonary Function Indexes, Clinical Efficacy, and Safety in Patients with Severe Cor Pulmonale. 62 41
36277594 2022
11
Genetic Evaluation in a Cohort of 126 Dutch Pulmonary Arterial Hypertension Patients. 5
33066286 2020
12
Novel risk genes and mechanisms implicated by exome sequencing of 2572 individuals with pulmonary arterial hypertension. 5
31727138 2019
13
Germline BMP9 mutation causes idiopathic pulmonary arterial hypertension. 5
30578397 2019
14
Genetic analyses in a cohort of 191 pulmonary arterial hypertension patients. 5
29743074 2018
15
A burden of rare variants in BMPR2 and KCNK3 contributes to a risk of familial pulmonary arterial hypertension. 5
28388887 2017
16
Molecular Analysis of BMPR2, TBX4, and KCNK3 and Genotype-Phenotype Correlations in Spanish Patients and Families With Idiopathic and Hereditary Pulmonary Arterial Hypertension. 5
27453251 2016
17
Pulmonary Arterial Hypertension: A Current Perspective on Established and Emerging Molecular Genetic Defects. 5
26387786 2015
18
Characteristics of pulmonary arterial hypertension in affected carriers of a mutation located in the cytoplasmic tail of bone morphogenetic protein receptor type 2. 5
25429696 2015
19
Defective cellular trafficking of the bone morphogenetic protein receptor type II by mutations underlying familial pulmonary arterial hypertension. 5
25688877 2015
20
Outcomes of childhood pulmonary arterial hypertension in BMPR2 and ALK1 mutation carriers. 5
22632830 2012
21
Molecular genetics and clinical features of Chinese idiopathic and heritable pulmonary arterial hypertension patients. 5
21737554 2012
22
Altered MicroRNA processing in heritable pulmonary arterial hypertension: an important role for Smad-8. 5
21920918 2011
23
Hemodynamic and clinical onset in patients with hereditary pulmonary arterial hypertension and BMPR2 mutations. 5
21801371 2011
24
Bone morphogenetic protein type II receptor mutations causing protein misfolding in heritable pulmonary arterial hypertension. 5
21030519 2010
25
Susceptibility to chronic thromboembolic pulmonary hypertension may be conferred by miR-759 via its targeted interaction with polymorphic fibrinogen alpha gene. 62 46
20677013 2010
26
Dynamic changes in lung microRNA profiles during the development of pulmonary hypertension due to chronic hypoxia and monocrotaline. 62 46
20110569 2010
27
Genetics and genomics of pulmonary arterial hypertension. 5
19555857 2009
28
Clinical implications of determining BMPR2 mutation status in a large cohort of children and adults with pulmonary arterial hypertension. 5
18503968 2008
29
High resolution structures of the bone morphogenetic protein type II receptor in two crystal forms: implications for ligand binding. 5
17094948 2006
30
High frequency of BMPR2 exonic deletions/duplications in familial pulmonary arterial hypertension. 5
16728714 2006
31
Mutations of the TGF-beta type II receptor BMPR2 in pulmonary arterial hypertension. 5
16429395 2006
32
BMPR2 gene rearrangements account for a significant proportion of mutations in familial and idiopathic pulmonary arterial hypertension. 5
16429403 2006
33
Splicing in action: assessing disease causing sequence changes. 5
16199547 2005
34
Randomized Clinical and Biochemical Study Comparing the Effect of L-arginine and Sildenafil in Beta Thalassemia Major Children With High Tricuspid Regurgitant Jet Velocity. 41
36282078 2022
35
Role of urotensin II in health and disease. 53 62
20421634 2010
36
Placenta growth factor in sickle cell disease: association with hemolysis and inflammation. 53 62
20040765 2010
37
Transcripts from a novel BMPR2 termination mutation escape nonsense mediated decay by downstream translation re-initiation: implications for treating pulmonary hypertension. 53 62
20095988 2010
38
Emerging therapies for the treatment of pulmonary hypertension. 53 62
20216170 2010
39
Sequence variants in BMPR2 and genes involved in the serotonin and nitric oxide pathways in idiopathic pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension: relation to clinical parameters and comparison with left heart disease. 53 62
19844076 2010
40
Genetic polymorphisms of the serotonin transporter, but not the 2a receptor or nitric oxide synthetase, are associated with pulmonary hypertension in chronic obstructive pulmonary disease. 53 62
19556740 2010
41
Phosphodiesterase type 5 inhibitors for high-altitude pulmonary hypertension: a meta-analysis. 53 62
20225909 2010
42
Pulmonary hemodynamic response to acute combination and monotherapy with sildenafil and brain natriuretic peptide in rats with monocrotaline-induced pulmonary hypertension. 53 62
19996941 2010
43
B-type natriuretic peptide utilization as an adjunct to management in a case of conjoined twins with pulmonary hypertension. 53 62
20085871 2010
44
GDF5 and BMP2 inhibit apoptosis via activation of BMPR2 and subsequent stabilization of XIAP. 53 62
19782107 2009
45
Brain natriuretic peptide in pulmonary arterial hypertension: biomarker and potential therapeutic agent. 53 62
20054445 2009
46
Dichloroacetate treatment partially regresses established pulmonary hypertension in mice with SM22alpha-targeted overexpression of the serotonin transporter. 53 62
19679640 2009
47
Nitric oxide synthases in infants and children with pulmonary hypertension and congenital heart disease. 53 62
19912632 2009
48
Expression of vasoactive intestinal peptide and related receptors in overcirculation-induced pulmonary hypertension in piglets. 53 62
19581838 2009
49
A novel insight into the mechanism of pulmonary hypertension involving caveolin-1 deficiency and endothelial nitric oxide synthase activation. 53 62
20382348 2009
50
Brain natriuretic peptide in chronic thromboembolic pulmonary hypertension. 53 62
19468826 2009

Variations for Pulmonary Hypertension

ClinVar genetic disease variations for Pulmonary Hypertension:

5 (show top 50) (show all 180)
# Gene Name Type Significance ClinVarId dbSNP ID Position
1 BMPR2 NM_001204.7(BMPR2):c.120T>A (p.Tyr40Ter) SNV Pathogenic
1387800 GRCh37: 2:203329575-203329575
GRCh38: 2:202464852-202464852
2 BMPR2 NM_001204.7(BMPR2):c.1069del (p.Arg357fs) DEL Pathogenic
1379840 GRCh37: 2:203395618-203395618
GRCh38: 2:202530895-202530895
3 BMPR2 NM_001204.7(BMPR2):c.1332dup (p.Thr445fs) DUP Pathogenic
1424050 GRCh37: 2:203407086-203407087
GRCh38: 2:202542363-202542364
4 BMPR2 NM_001204.7(BMPR2):c.409dup (p.Thr137fs) DUP Pathogenic
1423038 GRCh37: 2:203332401-203332402
GRCh38: 2:202467678-202467679
5 BMPR2 NM_001204.7(BMPR2):c.1175del (p.Val392fs) DEL Pathogenic
1456366 GRCh37: 2:203397354-203397354
GRCh38: 2:202532631-202532631
6 BMPR2 NM_001204.7(BMPR2):c.1028dup (p.Asn343fs) DUP Pathogenic
1459305 GRCh37: 2:203395572-203395573
GRCh38: 2:202530849-202530850
7 BMPR2 NC_000002.11:g.(?_203241529)_(203242283_?)del DEL Pathogenic
1071169 GRCh37: 2:203241529-203242283
GRCh38:
8 BMPR2 NC_000002.11:g.(?_203329512)_(203332432_?)del DEL Pathogenic
1075069 GRCh37: 2:203329512-203332432
GRCh38:
9 BMPR2 NC_000002.11:g.(?_203378432)_(203379712_?)del DEL Pathogenic
1075070 GRCh37: 2:203378432-203379712
GRCh38:
10 BMPR2 NM_001204.7(BMPR2):c.346del (p.Cys116fs) DEL Pathogenic
1443330 GRCh37: 2:203332340-203332340
GRCh38: 2:202467617-202467617
11 BMPR2 NM_001204.7(BMPR2):c.135del (p.Gly45_Ile46insTer) DEL Pathogenic
1452839 GRCh37: 2:203329588-203329588
GRCh38: 2:202464865-202464865
12 BMPR2 NM_001204.7(BMPR2):c.443del (p.Asp148fs) DEL Pathogenic
836469 rs1687663690 GRCh37: 2:203378466-203378466
GRCh38: 2:202513743-202513743
13 BMPR2 NM_001204.7(BMPR2):c.142dup (p.Glu48fs) DUP Pathogenic
838701 rs1692288591 GRCh37: 2:203329596-203329597
GRCh38: 2:202464873-202464874
14 BMPR2 NM_001204.7(BMPR2):c.2202del (p.Pro735fs) DEL Pathogenic
845712 rs1688559190 GRCh37: 2:203420589-203420589
GRCh38: 2:202555866-202555866
15 BMPR2 NM_001204.7(BMPR2):c.1233_1236dup (p.Tyr413fs) DUP Pathogenic
967816 rs1688050712 GRCh37: 2:203397411-203397412
GRCh38: 2:202532688-202532689
16 BMPR2 NM_001204.7(BMPR2):c.897_903del (p.Ser300fs) DEL Pathogenic
1069902 GRCh37: 2:203384853-203384859
GRCh38: 2:202520130-202520136
17 BMPR2 NM_001204.7(BMPR2):c.1206del (p.Lys402fs) DEL Pathogenic
1073354 GRCh37: 2:203397383-203397383
GRCh38: 2:202532660-202532660
18 BMPR2 NM_001204.7(BMPR2):c.610A>T (p.Lys204Ter) SNV Pathogenic
1072447 GRCh37: 2:203379691-203379691
GRCh38: 2:202514968-202514968
19 BMPR2 NC_000002.12:g.(?_202467509)_(202467699_?)del DEL Pathogenic
830694 GRCh37: 2:203332232-203332422
GRCh38:
20 BMPR2 NC_000002.12:g.(?_202514864)_(202514989_?)del DEL Pathogenic
833519 GRCh37: 2:203379587-203379712
GRCh38:
21 BMPR2 NM_001204.7(BMPR2):c.200A>G (p.Tyr67Cys) SNV Pathogenic
425719 rs1085307177 GRCh37: 2:203329655-203329655
GRCh38: 2:202464932-202464932
22 BMPR2 NC_000002.12:g.(?_202513699)_(202542467_?)del DEL Pathogenic
583625 GRCh37: 2:203378422-203407190
GRCh38: 2:202513699-202542467
23 BMPR2 NC_000002.12:g.(?_202513709)_(202520211_?)del DEL Pathogenic
660143 GRCh37: 2:203378432-203384934
GRCh38: 2:202513709-202520211
24 BMPR2 NC_000002.12:g.(?_202513699)_(202530974_?)del DEL Pathogenic
658435 GRCh37: 2:203378422-203395697
GRCh38: 2:202513699-202530974
25 BMPR2 NC_000002.12:g.(?_202530784)_(202542457_?)del DEL Pathogenic
832606 GRCh37: 2:203395507-203407180
GRCh38:
26 BMPR2 NC_000002.12:g.(?_202467499)_(202514999_?)del DEL Pathogenic
458622 GRCh37: 2:203332222-203379722
GRCh38: 2:202467499-202514999
27 BMPR2 NM_001204.7(BMPR2):c.354T>G (p.Cys118Trp) SNV Pathogenic
8799 rs137852743 GRCh37: 2:203332348-203332348
GRCh38: 2:202467625-202467625
28 BMPR2 NM_001204.7(BMPR2):c.1398G>A (p.Trp466Ter) SNV Pathogenic
409813 rs1060502576 GRCh37: 2:203407155-203407155
GRCh38: 2:202542432-202542432
29 BMPR2 NM_001204.7(BMPR2):c.48G>A (p.Trp16Ter) SNV Pathogenic
425685 rs1085307154 GRCh37: 2:203242245-203242245
GRCh38: 2:202377522-202377522
30 BMPR2 NM_001204.7(BMPR2):c.1125_1128+16del DEL Pathogenic
238628 rs878854272 GRCh37: 2:203395674-203395693
GRCh38: 2:202530951-202530970
31 BMPR2 NM_001204.7(BMPR2):c.2450_2451del (p.Asn817fs) DEL Pathogenic
1069537 GRCh37: 2:203420838-203420839
GRCh38: 2:202556115-202556116
32 BMPR2 NM_001204.7(BMPR2):c.1748dup (p.Asn583fs) DUP Pathogenic
1069660 GRCh37: 2:203420129-203420130
GRCh38: 2:202555406-202555407
33 BMPR2 NM_001204.7(BMPR2):c.1102G>T (p.Glu368Ter) SNV Pathogenic
1073527 GRCh37: 2:203395651-203395651
GRCh38: 2:202530928-202530928
34 BMPR2 NM_001204.7(BMPR2):c.1097del (p.Pro366fs) DEL Pathogenic
425871 rs1085307296 GRCh37: 2:203395644-203395644
GRCh38: 2:202530921-202530921
35 BMPR2 NM_001204.7(BMPR2):c.1141dup (p.Arg381fs) DUP Pathogenic
425880 rs1085307303 GRCh37: 2:203397319-203397320
GRCh38: 2:202532596-202532597
36 BMPR2 NM_001204.7(BMPR2):c.524del (p.Met174_Leu175insTer) DEL Pathogenic
1076573 GRCh37: 2:203378546-203378546
GRCh38: 2:202513823-202513823
37 BMPR2 NM_001204.7(BMPR2):c.2617C>T (p.Arg873Ter) SNV Pathogenic
8805 rs137852748 GRCh37: 2:203421005-203421005
GRCh38: 2:202556282-202556282
38 BMPR2 NM_001204.7(BMPR2):c.439C>T (p.Arg147Ter) SNV Pathogenic
222513 rs869025366 GRCh37: 2:203378462-203378462
GRCh38: 2:202513739-202513739
39 BMPR2 NM_001204.7(BMPR2):c.201T>G (p.Tyr67Ter) SNV Pathogenic
425721 rs1085307179 GRCh37: 2:203329656-203329656
GRCh38: 2:202464933-202464933
40 BMPR2 NM_001204.7(BMPR2):c.296G>A (p.Cys99Tyr) SNV Pathogenic
425754 rs1085307205 GRCh37: 2:203332290-203332290
GRCh38: 2:202467567-202467567
41 BMPR2 NM_001204.7(BMPR2):c.1259G>A (p.Cys420Tyr) SNV Pathogenic
425907 rs1085307325 GRCh37: 2:203397438-203397438
GRCh38: 2:202532715-202532715
42 BMPR2 NM_001204.7(BMPR2):c.969dup (p.His324fs) DUP Pathogenic
425858 rs1085307283 GRCh37: 2:203395517-203395518
GRCh38: 2:202530794-202530795
43 BMPR2 NM_001204.7(BMPR2):c.260dup (p.His87fs) DUP Pathogenic
425746 rs1085307199 GRCh37: 2:203332253-203332254
GRCh38: 2:202467530-202467531
44 BMPR2 NM_001204.7(BMPR2):c.961del (p.Arg321fs) DEL Pathogenic
425847 rs1085307276 GRCh37: 2:203384918-203384918
GRCh38: 2:202520195-202520195
45 BMPR2 NM_001204.7(BMPR2):c.1451G>A (p.Trp484Ter) SNV Pathogenic
425947 rs1085307355 GRCh37: 2:203417476-203417476
GRCh38: 2:202552753-202552753
46 BMPR2 NM_001204.7(BMPR2):c.894G>A (p.Trp298Ter) SNV Pathogenic
1372691 GRCh37: 2:203384851-203384851
GRCh38: 2:202520128-202520128
47 BMPR2 NC_000002.11:g.(?_203407024)_(203407180_?)del DEL Pathogenic
1419785 GRCh37: 2:203407024-203407180
GRCh38:
48 BMPR2 NC_000002.11:g.(?_203329522)_(203379712_?)del DEL Pathogenic
1409534 GRCh37: 2:203329522-203379712
GRCh38:
49 BMPR2 NM_001204.7(BMPR2):c.320C>A (p.Ser107Ter) SNV Pathogenic
1453966 GRCh37: 2:203332314-203332314
GRCh38: 2:202467591-202467591
50 overlap with 6 genes NC_000002.11:g.(?_202566574)_(203424669_?)del DEL Pathogenic
1455397 GRCh37: 2:202566574-203424669
GRCh38:

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 GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1
Show member pathways
11.62 NPPB NPPA MIR21 EDN1
2 11.26 MIR21 MIR20A MIR17
3
Show member pathways
10.53 CAV1 BMPR2 ACVRL1
4 10.14 NPPB NPPA BMPR2

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 9.86 TNXA NPPB NPPA MIR451A MIR22 MIR21
2 extracellular vesicle GO:1903561 9.02 MIR451A MIR22 MIR21 MIR20A MIR17

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

(show all 47)
# Name GO ID Score Top Affiliating Genes
1 positive regulation of angiogenesis GO:0045766 10.15 MIR21 MIR20A ADM ACVRL1
2 cellular response to hypoxia GO:0071456 10.09 MIR17 MALAT1 KCNK3 EDN1
3 vasodilation GO:0042311 10.09 NPPB NPPA CPS1
4 blood vessel remodeling GO:0001974 10.08 EDNRA BMPR2 ACVRL1
5 positive regulation of BMP signaling pathway GO:0030513 10.04 MIR20A BMPR2 ACVRL1
6 negative regulation of gene expression GO:0010629 10.03 MIR451A MIR21 MIR20A MIR17 EDN1 ACVRL1
7 positive regulation of heart rate GO:0010460 10.01 ADM EDN1 NPPA
8 endothelin receptor signaling pathway GO:0086100 10 EDNRA EDN1
9 positive regulation of cation channel activity GO:2001259 10 EDNRA EDN1
10 retina vasculature development in camera-type eye GO:0061298 9.99 BMPR2 ACVRL1
11 axonogenesis involved in innervation GO:0060385 9.99 EDNRA EDN1
12 regulation of glucose transmembrane transport GO:0010827 9.98 EDNRA EDN1
13 noradrenergic neuron differentiation GO:0003357 9.97 EDNRA EDN1
14 cardiac neural crest cell migration involved in outflow tract morphogenesis GO:0003253 9.97 EDNRA EDN1
15 outflow tract morphogenesis GO:0003151 9.96 MIR20A MIR17 BMPR2
16 body fluid secretion GO:0007589 9.96 EDN1 NPPB
17 lymphatic endothelial cell differentiation GO:0060836 9.96 ACVRL1 BMPR2
18 cellular response to luteinizing hormone stimulus GO:0071373 9.95 EDN1 EDNRA
19 negative regulation of vasoconstriction GO:0045906 9.93 ADM BMPR2
20 venous blood vessel development GO:0060841 9.93 ACVRL1 BMPR2
21 positive regulation of cardiac muscle hypertrophy GO:0010613 9.92 EDN1 MIR21 PDE5A
22 regulation of blood pressure GO:0008217 9.91 NPPB NPPA EDNRA EDN1 ACVRL1
23 response to hypoxia GO:0001666 9.9 ACVRL1 ADM CAV1 EDN1 EDNRA SLC6A4
24 neural crest cell fate commitment GO:0014034 9.89 EDN1 EDNRA
25 sympathetic neuron axon guidance GO:0097492 9.88 EDN1 EDNRA
26 negative regulation of smooth muscle cell apoptotic process GO:0034392 9.87 MIR21 EDN1
27 regulation of calcium ion transmembrane transport via high voltage-gated calcium channel GO:1902514 9.87 NPPA MIR21
28 blood vessel diameter maintenance GO:0097746 9.87 NPPB NPPA EDN1
29 endothelial tube morphogenesis GO:0061154 9.86 MIR21 ACVRL1
30 endothelin receptor signaling pathway involved in heart process GO:0086101 9.86 EDNRA EDN1
31 miRNA-mediated gene silencing by inhibition of translation GO:0035278 9.85 MIR17 MIR20A MIR21
32 response to organic substance GO:0010033 9.85 SLC6A4 EDNRA ADM
33 cellular response to organic substance GO:0071310 9.85 SLC6A4 EDNRA EDN1
34 pharyngeal arch artery morphogenesis GO:0061626 9.85 EDNRA EDN1 BMPR2
35 negative regulation of chondrocyte proliferation GO:1902731 9.82 MIR21 BMPR2
36 meiotic cell cycle process involved in oocyte maturation GO:1903537 9.8 EDNRA EDN1
37 positive regulation of vascular associated smooth muscle cell proliferation GO:1904707 9.8 EDN1 MIR17 MIR20A MIR21
38 negative regulation of endothelial cell proliferation GO:0001937 9.8 MIR22 MIR21 CAV1 ACVRL1
39 cellular response to human chorionic gonadotropin stimulus GO:0044751 9.78 EDNRA EDN1
40 positive regulation of potassium ion export across plasma membrane GO:1903766 9.75 NPPA MIR21
41 positive regulation of cardiac muscle hypertrophy in response to stress GO:1903244 9.65 MIR20A MIR17
42 positive regulation of vascular associated smooth muscle cell migration GO:1904754 9.63 MIR451A MIR21 MIR20A
43 negative regulation of vascular associated smooth muscle cell apoptotic process GO:1905460 9.62 MIR17 MIR21
44 positive regulation of metalloendopeptidase activity GO:1904685 9.61 MIR21 MIR17
45 vasoconstriction GO:0042310 9.56 SLC6A4 EDNRA EDN1 CAV1
46 positive regulation of pulmonary blood vessel remodeling GO:1905111 9.51 MIR20A MIR17
47 negative regulation of systemic arterial blood pressure GO:0003085 9.17 NPPB NPPA MIR17 BMPR2

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

# Name GO ID Score Top Affiliating Genes
1 transforming growth factor beta receptor activity GO:0005024 9.67 BMPR2 ACVRL1
2 mRNA 3'-UTR binding GO:0003730 9.62 MIR451A MIR21 MIR20A MIR17
3 transmembrane receptor protein serine/threonine kinase activity GO:0004675 9.58 BMPR2 ACVRL1
4 BMP receptor activity GO:0098821 9.56 BMPR2 ACVRL1
5 hormone activity GO:0005179 9.56 NPPB NPPA EDN1 ADM
6 hormone receptor binding GO:0051427 9.46 NPPB NPPA
7 mRNA base-pairing translational repressor activity GO:1903231 9.02 MIR451A MIR22 MIR21 MIR20A MIR17

Sources for Pulmonary Hypertension

2 CDC
6 CNVD
8 Cosmic
9 dbSNP
10 DGIdb
16 EFO
17 ExPASy
18 FMA
19 GARD
27 GO
28 GTR
29 HMDB
30 HPO
31 ICD10
32 ICD10 via Orphanet
33 ICD11
34 ICD9CM
35 IUPHAR
36 LifeMap
38 LOVD
40 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
52 NINDS
53 Novoseek
55 ODiseA
56 OMIM via Orphanet
57 OMIM® (Updated 08-Dec-2022)
61 PubChem
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 Tocris
71 UMLS
72 UMLS via Orphanet
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