Pulmonary Hypertension

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

Aliases & Classifications for Pulmonary Hypertension

MalaCards integrated aliases for Pulmonary Hypertension:

Name: Pulmonary Hypertension 40 12 73 42 3 15 37 62 70
Primary Pulmonary Hypertension 40 12 73 36 29 54 6 15 32
Idiopathic Pulmonary Arterial Hypertension 70
Idiopathic Pulmonary Hypertension 70
Hypertension, Pulmonary 44
Hypertension Pulmonary 54


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Disease Ontology 12 DOID:14557 DOID:6432
KEGG 36 H01619
ICD9CM 34 416.0
MeSH 44 D006976
NCIt 50 C3120 C97119
SNOMED-CT 67 155328008 26174007
ICD10 32 I27.0 I27.20
UMLS 70 C0020542 C0152171 C3203102

Summaries for Pulmonary Hypertension

MedlinePlus : 42 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 angina pectoris, dyspnea and hemoptysis. An important gene associated with Pulmonary Hypertension is MIR17 (MicroRNA 17), and among its related pathways/superpathways are TGF-beta signaling pathway and Human Embryonic Stem Cell Pluripotency. The drugs Morphine and Bupivacaine have been mentioned in the context of this disorder. Affiliated tissues include Lung, heart and endothelial, and related phenotype is normal.

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

KEGG : 36 Primary pulmonary hypertension (PPH) is a progressive disease characterised by raised pulmonary vascular resistance, which results in diminished right-heart function due to increased right ventricular afterload. PPH occurs most commonly in young and middle-aged women; mean survival from onset of symptoms is 2-3 years. Mutations in the type II bone morphogenetic protein (BMP) receptor (BMPR)-II are now considered to be the genetic basis for familial PPH and ~30% of cases of sporadic PPH. BMPs are members of the transforming growth factor beta superfamily and affect intracellular signalling via Smads and mitogen-activated protein kinases. It has been shown that missense mutations of cysteine residues in the extracellular or kinase domain of BMPR2 result in intracellular localisation of the mutated receptor and negligible Smad signalling.

PubMed Health : 62 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.

Wikipedia : 73 Pulmonary hypertension (PH or PHTN) is a condition of increased blood pressure within the arteries of... more...

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 Rare Pulmonary Hypertension

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

(show top 50) (show all 1137)
# Related Disease Score Top Affiliating Genes
1 pulmonary hypertension, primary, 1 33.8 KCNK3 BMPR2 ACVRL1
2 chronic thromboembolic pulmonary hypertension 33.6 NPPB EDN1 BMPR2
3 pulmonary venoocclusive disease 33.0 PDE5A NPPB KCNK3 EDN1 CAV1 BMPR2
4 eisenmenger syndrome 32.8 EDN1 BMPR2
5 heritable pulmonary arterial hypertension 32.7 KCNK3 CAV1 BMPR2 BMPR1B ACVRL1
6 diaphragmatic hernia, congenital 32.6 PDE5A EDN1 BMPR2
7 pulmonary valve insufficiency 32.5 PDE5A NPPB KCNK3 EDN1 BMPR2 ACVRL1
8 tricuspid valve insufficiency 32.4 PDE5A NPPB EDN1 BMPR2
9 vascular disease 32.3 SLC6A4 NPPB MIR21 EDN1 BMPR2 ACVRL1
10 heart disease 32.2 SMAD4 SLC6A4 PDE5A NPPB MIR21 MIR17
11 pulmonary disease, chronic obstructive 31.9 SLC6A4 PDE5A NPPB MIR21 MIR17
12 sleep apnea 31.9 SLC6A4 NPPB EDN1
13 atrial heart septal defect 31.9 NPPB EDN1 BMPR2
14 meconium aspiration syndrome 31.8 PDE5A EDN1 CPS1
15 pulmonary arterial hypertension associated with congenital heart disease 31.8 CAV1 BMPR2 BMPR1B
16 connective tissue disease 31.8 MIR22 MIR21 MIR17 EDN1 BMPR2 ACVRL1
17 telangiectasis 31.7 SMAD4 BMPR2 ACVRL1
18 arteriovenous malformation 31.6 SMAD4 EDN1 BMPR2 ACVRL1
19 hereditary hemorrhagic telangiectasia 31.6 SMAD4 BMPR2 ACVRL1
20 heart valve disease 31.4 NPPB MIR21 MIR17
21 hepatopulmonary syndrome 31.4 EDN1 BMPR2 ACVRL1
22 limited scleroderma 31.2 PDE5A EDN1 CAV1
23 ischemia 31.2 PDE5A NPPB MIR21 EDN1
24 chronic mountain sickness 31.1 NPPB EDN1
25 pulmonary arteriovenous malformation 31.1 BMPR2 ACVRL1
26 aortic valve disease 2 31.0 NPPB MIR21 MIR17
27 chronic pulmonary heart disease 31.0 SLC6A4 PDE5A NPPB EDN1 BMPR2 BMPR1B
28 aortic valve disease 1 30.9 SMAD4 MIR21 MIR17 EDN1
29 arteries, anomalies of 30.6 MIR22 MIR21 MIR17 EDN1
30 lipoprotein quantitative trait locus 30.6 PDE5A NPPB MIR22 MIR21 MIR17 EDN1
31 acute myocardial infarction 30.6 NPPB MIR22 MIR21 EDN1
32 aortic disease 30.5 SMAD4 MIR21 MIR17
33 cardiovascular system disease 30.5 PDE5A NPPB MIR21 MIR17 EDN1
34 myocardial infarction 30.5 SLC6A4 PDE5A NPPB MIR22 MIR21 MIR17
35 tricuspid valve disease 30.5 PDE5A NPPB EDN1 BMPR2
36 primary biliary cholangitis 30.4 MIR451A MIR21 MIR20A MIR17
37 hypertension, essential 30.1 SLC6A4 PDE5A NPPB MIR22 MIR21 MIR17
38 central nervous system disease 30.1 SLC6A4 MIR22 MIR21 MIR17
39 disease of mental health 29.9 SMAD4 SLC6A4 PDE5A NPPB MIR22 MIR21
40 rectum cancer 29.8 MIR21 MIR20A MIR17
41 intestinal disease 29.7 MIR22 MIR21 MIR20A MIR17
42 glioma 29.6 MIR21 MIR20A MIR17 MALAT1
43 hyperuricemia, pulmonary hypertension, renal failure, and alkalosis syndrome 11.7
44 pulmonary hypertension, chronic thromboembolic, without deep vein thrombosis 11.7
45 pulmonary hypertension, primary, 4 11.7
46 alveolar capillary dysplasia with misalignment of pulmonary veins 11.6
47 pulmonary hypertension, primary, 2 11.6
48 pulmonary hypertension, primary, 3 11.6
49 pulmonary hypertension, neonatal 11.6
50 pulmonary edema of mountaineers 11.4

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

MGI Mouse Phenotypes related to Pulmonary Hypertension:

# Description MGI Source Accession Score Top Affiliating Genes
1 normal MP:0002873 9.23 ACVRL1 BMPR1B BMPR2 EDN1 KCNK3 PDE5A

Drugs & Therapeutics for Pulmonary Hypertension

PubMed Health treatment related to Pulmonary Hypertension: 62

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 276)
# Name Status Phase Clinical Trials Cas Number PubChem Id
Morphine Approved, Investigational Phase 4 57-27-2 5288826
Bupivacaine Approved, Investigational Phase 4 38396-39-3, 2180-92-9 2474
Isoflurane Approved, Vet_approved Phase 4 26675-46-7 3763
Propofol Approved, Investigational, Vet_approved Phase 4 2078-54-8 4943
Ranolazine Approved, Investigational Phase 4 95635-55-5, 142387-99-3 56959
Silver sulfadiazine Approved, Vet_approved Phase 4 22199-08-2 441244
Clevidipine Approved, Investigational Phase 4 167221-71-8
Methazolamide Approved Phase 4 554-57-4 4100 5353756
Dalteparin Approved Phase 4 9005-49-6
Tinzaparin Approved Phase 4 9041-08-1, 9005-49-6 25244225
Milrinone Approved Phase 4 78415-72-2 4197
Heparin Approved, Investigational Phase 4 9005-49-6 772 9812414
Sevoflurane Approved, Vet_approved Phase 4 28523-86-6 5206
Nebivolol Approved, Investigational Phase 4 152520-56-4, 118457-14-0, 99200-09-6 71301
Epoprostenol Approved Phase 4 61849-14-7, 35121-78-9 5280427 5282411
Iloprost Approved, Investigational Phase 4 78919-13-8 6443959
Acetazolamide Approved, Vet_approved Phase 4 59-66-5 1986
Macitentan Approved Phase 4 441798-33-0
Tezosentan Investigational Phase 4 180384-57-0
20 Analgesics Phase 4
21 Anesthetics, Local Phase 4
22 Vardenafil Dihydrochloride Phase 4
23 Narcotics Phase 4
24 Analgesics, Opioid Phase 4
25 Anesthetics, Intravenous Phase 4
26 Heparin, Low-Molecular-Weight Phase 4
27 Cardiotonic Agents Phase 4
28 Phosphodiesterase 3 Inhibitors Phase 4
29 Fibrinolytic Agents Phase 4
30 Plasminogen Phase 4
31 Tissue Plasminogen Activator Phase 4
32 Thromboplastin Phase 4
33 calcium heparin Phase 4
34 Anesthetics, General Phase 4
35 Anesthetics Phase 4
36 Anesthetics, Inhalation Phase 4
37 Anticoagulants Phase 4
38 Chelating Agents Phase 4
39 Calcium, Dietary Phase 4
40 diuretics Phase 4
41 Anticonvulsants Phase 4
42 Carbonic Anhydrase Inhibitors Phase 4
43 Endothelin A Receptor Antagonists Phase 4
44 Natriuretic Peptide, Brain Phase 4
45 Liver Extracts Phase 4
Calcium Nutraceutical Phase 4 7440-70-2 271
Udenafil Approved, Investigational Phase 3 268203-93-6 6918523
Citalopram Approved Phase 3 59729-33-8 2771
Iodine Approved, Investigational Phase 3 7553-56-2 807
Mannitol Approved, Investigational Phase 2, Phase 3 69-65-8 453 6251

Interventional clinical trials:

(show top 50) (show all 1107)
# Name Status NCT ID Phase Drugs
1 Hemodynamic Evaluation of Patients With Pulmonary Arterial Hypertension. Response to Sildenafil Treatment Unknown status NCT00483626 Phase 4 oral sildenafil
2 Intravenous Iron Treatment In Iron Deficient Patients With Idiopathic Pulmonary Arterial Hypertension Unknown status NCT01288651 Phase 4 Ferricarboxymaltose
3 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
4 A Double Blind, Placebo Controlled Trial of Oral Riociguat for Sarcoidosis Associated Pulmonary Hypertension Unknown status NCT02625558 Phase 4 Riociguat;Placebo
5 Inhaled Iloprost for Disproportionate Pulmonary Hypertension in Chronic Obstructive Pulmonary Disease Unknown status NCT01116063 Phase 4 Inhaled iloprost
6 Comparison of Efficacy Different Treatment Regimens in Pulmonary Hypertension Secondary to Lung Disease and or Hypoxia Unknown status NCT01449253 Phase 4 Sildenafil;Bosentan
7 Effects of Spironolactone on Collagen Metabolism in Pulmonary Arterial Hypertension Unknown status NCT01468571 Phase 4 Spironolactone;Placebo
8 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)
9 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
10 Long Acting Phosphodiesterase 5 Inhibitors as Add-on Therapy for Patients With Pulmonary Hypertension Treated With Prostanoids. Unknown status NCT00705588 Phase 4 Tadalafil;Vardenafil
11 Vardenafil as add-on Therapy for Patients With Pulmonary Hypertension Treated With Inhaled Iloprost Unknown status NCT01649739 Phase 4 Levitra
12 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
13 Phase IV Study of Chronic Infusional Epoprostenol for Severe Primary Pulmonary Hypertension Completed NCT00004754 Phase 4 epoprostenol
14 Combined Inhaled Nitric Oxide and Inhaled Prostacyclin After Cardiac Surgery for Heart Transplantation and for Left Ventricular Assist Device Placement Completed NCT01717209 Phase 4 Nitric Oxide;Prostacyclin
15 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
16 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
17 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
18 Proof of Concept Study of Ranolazine in the Treatment of Pulmonary Hypertension Associated With Diastolic Left Ventricular Dysfunction Completed NCT02133352 Phase 4 Ranolazine
19 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
21 Hypoventilation and High Altitude Chronic Polycythemia: Acetazolamide as a Possible Treatment Completed NCT00424970 Phase 4 acetazolamide
22 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
23 A Phase 3, Multi-center, Open-label Study To Investigate Safety, Efficacy, And Tolerability Of Sildenafil Citrate In Pediatric Patients With Pulmonary Arterial Hypertension Completed NCT01642407 Phase 4 Sildenafil
24 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
25 Echo Study - Characterization of LV Strain Patterns in Patients With Mildly Elevated PCWP and Pulmonary Hypertension Completed NCT01800292 Phase 4 sildenafil
26 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;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 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
29 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
30 Safely Change From Bosentan to Ambrisentan in Pulmonary Hypertension Completed NCT01330108 Phase 4 ambrisentan
31 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
32 Vasoreactivity Testing With Intravenous Sildenafil in Patients With Precapillary Pulmonary Hypertension (Treatment Optimisation Study) Completed NCT01889966 Phase 4 Sildenafil
33 Inhaled Iloprost for Sarcoidosis Associated Pulmonary Hypertension Completed NCT00403650 Phase 4 Iloprost
34 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)
35 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
36 Sildenafil for Improving Outcomes After Valvular Correction Completed NCT00862043 Phase 4 Sildenafil Citrate;Placebo
37 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
38 Natrecor® (Nesiritide) as a Nitric Oxide Sparing Agent in Patients Undergoing Lung Transplantation Completed NCT00205426 Phase 4 Natrecor
39 A 16 Week, Open Label, Multi-centre, Study to Evaluate the Safety, Tolerability and Pharmacodynamic Effects of a Rapid Dose Titration Regimen of Subcutaneous Remodulin® Therapy in Subjects With Pulmonary Arterial Hypertension (PAH) Completed NCT02847260 Phase 4 Remodulin
40 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®
41 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
42 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
43 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®
44 An Open-Label Uncontrolled Study of the Safety and Efficacy of Ambrisentan in Patients With Exercise Induced Pulmonary Arterial Hypertension Completed NCT01338636 Phase 4 Ambrisentan
45 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
46 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;Bosentan;Sildenafil Citrate
47 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
48 A Prospective, Randomized, International, Multicenter, Double-arm, Controlled, Open-label Study of Riociguat in Patients With Pulmonary Arterial Hypertension (PAH) Who Are on a Stable Dose of Phosphodiesterase-5 Inhibitors (PDE-5i) With or Without Endothelin Receptor Antagonist (ERA), But Not at Treatment Goal Completed NCT02891850 Phase 4 Riociguat (Adempas, BAY63-2521);Sildenafil;Tadalafil
49 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
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 70 / NDF-RT 51 :

Epoprostenol Sodium
Hydralazine Hydrochloride

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 29

Anatomical Context for Pulmonary Hypertension

MalaCards organs/tissues related to Pulmonary Hypertension:

Heart, Endothelial, Smooth Muscle, Lung, Liver, Bone, Kidney
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 30478)
# Title Authors PMID Year
Interleukin-6 modulates the expression of the bone morphogenic protein receptor type II through a novel STAT3-microRNA cluster 17/92 pathway. 54 47 61
19390056 2009
Long-Term Clinical and Hemodynamic Outcomes after Heart Transplantation in Patients Pre-Treated with Sildenafil. 61 42
33656068 2021
Risk assessment in precapillary pulmonary hypertension: a comparative analysis. 61 42
33478493 2021
Susceptibility to chronic thromboembolic pulmonary hypertension may be conferred by miR-759 via its targeted interaction with polymorphic fibrinogen alpha gene. 61 47
20677013 2010
Dynamic changes in lung microRNA profiles during the development of pulmonary hypertension due to chronic hypoxia and monocrotaline. 61 47
20110569 2010
Recent advances in the approach to hepatopulmonary syndrome and portopulmonary hypertension. 42
33639700 2021
[Prognostic value of assay of brain natriuretic peptide in patients with acute respiratory distress syndrome]. 61 54
20519078 2010
Effectiveness of human atrial natriuretic peptide supplementation in pulmonary edema patients using the pulse contour cardiac output system. 54 61
20376887 2010
Role of urotensin II in health and disease. 61 54
20421634 2010
Transcripts from a novel BMPR2 termination mutation escape nonsense mediated decay by downstream translation re-initiation: implications for treating pulmonary hypertension. 54 61
20095988 2010
Emerging therapies for the treatment of pulmonary hypertension. 54 61
20216170 2010
Placenta growth factor in sickle cell disease: association with hemolysis and inflammation. 61 54
20040765 2010
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. 54 61
19844076 2010
Phosphodiesterase type 5 inhibitors for high-altitude pulmonary hypertension: a meta-analysis. 61 54
20225909 2010
Pulmonary hemodynamic response to acute combination and monotherapy with sildenafil and brain natriuretic peptide in rats with monocrotaline-induced pulmonary hypertension. 54 61
19996941 2010
B-type natriuretic peptide utilization as an adjunct to management in a case of conjoined twins with pulmonary hypertension. 61 54
20085871 2010
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. 54 61
19556740 2010
Brain natriuretic peptide in pulmonary arterial hypertension: biomarker and potential therapeutic agent. 54 61
20054445 2009
Dichloroacetate treatment partially regresses established pulmonary hypertension in mice with SM22alpha-targeted overexpression of the serotonin transporter. 61 54
19679640 2009
GDF5 and BMP2 inhibit apoptosis via activation of BMPR2 and subsequent stabilization of XIAP. 61 54
19782107 2009
Interaction between bone morphogenetic proteins and endothelin-1 in human pulmonary artery smooth muscle. 61 54
19786120 2009
Nitric oxide synthases in infants and children with pulmonary hypertension and congenital heart disease. 54 61
19912632 2009
A novel insight into the mechanism of pulmonary hypertension involving caveolin-1 deficiency and endothelial nitric oxide synthase activation. 61 54
20382348 2009
Expression of vasoactive intestinal peptide and related receptors in overcirculation-induced pulmonary hypertension in piglets. 61 54
19581838 2009
Brain natriuretic peptide in chronic thromboembolic pulmonary hypertension. 61 54
19468826 2009
Soluble guanylate cyclase agonists inhibit expression and procoagulant activity of tissue factor. 61 54
19592462 2009
Endothelin-1 across the lung circulation in patients with pulmonary arterial hypertension and influence of epoprostenol infusion. 54 61
19632577 2009
Chronic treatment with sildenafil stimulates Leydig cell and testosterone secretion. 61 54
19659904 2009
Persistent eNOS activation secondary to caveolin-1 deficiency induces pulmonary hypertension in mice and humans through PKG nitration. 61 54
19487814 2009
Effects of roflumilast, a phosphodiesterase-4 inhibitor, on hypoxia- and monocrotaline-induced pulmonary hypertension in rats. 61 54
19386793 2009
Intratracheal gene transfer of adrenomedullin using polyplex nanomicelles attenuates monocrotaline-induced pulmonary hypertension in rats. 61 54
19337232 2009
Right atrium contractility and right ventricular diastolic function assessed by pulsed tissue Doppler imaging can predict brain natriuretic peptide in adults with acquired pulmonary hypertension. 54 61
18793807 2009
RhoA and Rho kinase activation in human pulmonary hypertension: role of 5-HT signaling. 54 61
19299501 2009
Prognostic value of B-type natriuretic peptide in children with pulmonary hypertension. 61 54
18599134 2009
Erectile dysfunction and heart failure: the role of phosphodiesterase type 5 inhibitors. 61 54
19387454 2009
Discovery of riociguat (BAY 63-2521): a potent, oral stimulator of soluble guanylate cyclase for the treatment of pulmonary hypertension. 54 61
19263460 2009
Plasma endothelin-1 and nitrate levels in Down's syndrome with complete atrioventricular septal defect-associated pulmonary hypertension: a comparison with non-Down's syndrome children. 61 54
18682983 2009
Inhibition of cGMP phosphodiesterase 5 suppresses serotonin signalling in pulmonary artery smooth muscles cells. 61 54
19416631 2009
Caveolin-1 Expression and Hemodynamics in COPD Patients. 54 61
19572028 2009
Brain natriuretic peptide as a preclinical marker of chronic pulmonary hypertension in patients with pulmonary embolism. 61 54
19247590 2009
Combined use of PDE5 inhibitors and nitrates in the treatment of pulmonary arterial hypertension in patients with heart failure. 54 61
19181291 2009
Endothelin-1 inhibition by ambrisentan as a potential treatment adjunct after debulking surgery in epithelial ovarian cancer. 61 54
19544975 2009
Hypoxic upregulation of preproendothelin-1 gene expression is associated with protein tyrosine kinase-PI3K signaling in cultured lung vascular endothelial cells. 61 54
19436835 2009
NO-independent, haem-dependent soluble guanylate cyclase stimulators. 61 54
19089334 2009
[Estimation of plasmic concentration of the brain natriuretic peptide in interstitial pulmonary diseases with pulmonary hypertension: clinical role]. 54 61
19459423 2009
[Endothelin-1 and brain natriuretic peptide in the development of pulmonary hypertension in interstitial lung diseases]. 61 54
20481052 2009
Superoxide dismutase restores eNOS expression and function in resistance pulmonary arteries from neonatal lambs with persistent pulmonary hypertension. 54 61
18790993 2008
Inhalation of vasoactive intestinal peptide in pulmonary hypertension. 54 61
18978135 2008
Differential roles of endothelin-1 ETA and ETB receptors and vasoactive intestinal polypeptide in regulation of the airways and the pulmonary vasculature in isolated rat lung. 61 54
18567602 2008
["Pulmonary hypertension" of the gastrointestinal tract in patients with systemic sclerosis: thoughts on a not (yet) existing disease entity]. 54 61
18814097 2008

Variations for Pulmonary Hypertension

ClinVar genetic disease variations for Pulmonary Hypertension:

# Gene Name Type Significance ClinVarId dbSNP ID Position
1 BMPR2 NM_001204.7(BMPR2):c.668A>G (p.Asp223Gly) SNV Uncertain significance 1000593 GRCh37: 2:203383591-203383591
GRCh38: 2:202518868-202518868
2 BMPR2 NC_000002.11:g.(?_203378432)_(203397465_?)dup Duplication Uncertain significance 1022721 GRCh37: 2:203378432-203397465
3 BMPR2 NM_001204.7(BMPR2):c.1286_1309del (p.Val429_Gln437delinsGlu) Deletion Uncertain significance 1027322 GRCh37: 2:203407043-203407066
GRCh38: 2:202542320-202542343
4 BMPR2 NM_001204.7(BMPR2):c.1927A>G (p.Thr643Ala) SNV Uncertain significance 1036452 GRCh37: 2:203420315-203420315
GRCh38: 2:202555592-202555592
5 BMPR2 NM_001204.7(BMPR2):c.529+4A>G SNV Uncertain significance 1047237 GRCh37: 2:203378556-203378556
GRCh38: 2:202513833-202513833
6 BMPR2 NM_001204.7(BMPR2):c.1039T>C (p.Cys347Arg) SNV Uncertain significance 425865 rs1085307290 GRCh37: 2:203395588-203395588
GRCh38: 2:202530865-202530865
7 BMPR2 NM_001204.7(BMPR2):c.335C>A (p.Thr112Lys) SNV Uncertain significance 1056659 GRCh37: 2:203332329-203332329
GRCh38: 2:202467606-202467606

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:

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

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

# Super pathways Score Top Affiliating Genes
Show member pathways
2 12.05 MIR451A MIR21 MIR20A MIR17 BMPR2
4 11.55 SMAD4 BMPR2 BMPR1B
Show member pathways
Show member pathways
7 10.41 SMAD4 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 9.56 NPPB MIR451A MIR22 MIR21 MIR20A MIR17
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 gene silencing by miRNA GO:0035195 10.09 MIR451A MIR22 MIR21 MIR20A MIR17
2 response to drug GO:0042493 9.99 SLC6A4 KCNK3 EDN1 CPS1
3 negative regulation of gene expression GO:0010629 9.88 MIR451A MIR21 MIR20A MIR17 EDN1 ACVRL1
4 transforming growth factor beta receptor signaling pathway GO:0007179 9.87 SMAD4 MIR21 ACVRL1
5 miRNA mediated inhibition of translation GO:0035278 9.87 MIR21 MIR20A MIR17
6 cellular response to hypoxia GO:0071456 9.85 MIR17 MALAT1 KCNK3 EDN1
7 response to hypoxia GO:0001666 9.85 SMAD4 SLC6A4 EDN1 CAV1 ACVRL1
8 negative regulation of cell growth GO:0030308 9.84 SMAD4 NPPB BMPR2 ACVRL1
9 regulation of blood pressure GO:0008217 9.82 NPPB EDN1 ACVRL1
10 positive regulation of endothelial cell migration GO:0010595 9.81 MIR21 EDN1 BMPR2
11 cellular response to growth factor stimulus GO:0071363 9.81 BMPR2 BMPR1B ACVRL1
12 cellular response to transforming growth factor beta stimulus GO:0071560 9.79 EDN1 CAV1 ACVRL1
13 positive regulation of osteoblast differentiation GO:0045669 9.78 MIR21 MIR20A BMPR2 BMPR1B
14 outflow tract morphogenesis GO:0003151 9.77 MIR20A MIR17 BMPR2
15 positive regulation of pathway-restricted SMAD protein phosphorylation GO:0010862 9.76 SMAD4 BMPR2 ACVRL1
16 dorsal/ventral pattern formation GO:0009953 9.73 EDN1 BMPR1B ACVRL1
17 positive regulation of chondrocyte differentiation GO:0032332 9.7 BMPR1B ACVRL1
18 positive regulation of cartilage development GO:0061036 9.7 BMPR2 BMPR1B
19 positive regulation of vascular associated smooth muscle cell migration GO:1904754 9.7 MIR451A MIR21 MIR20A
20 vasoconstriction GO:0042310 9.69 SLC6A4 EDN1 CAV1
21 negative regulation of DNA biosynthetic process GO:2000279 9.68 BMPR2 ACVRL1
22 positive regulation of renal sodium excretion GO:0035815 9.68 NPPB EDN1
23 positive regulation of urine volume GO:0035810 9.68 NPPB EDN1
24 negative regulation of cytokine-mediated signaling pathway GO:0001960 9.68 MIR21 CAV1
25 lymphangiogenesis GO:0001946 9.67 BMPR2 ACVRL1
26 positive regulation of cardiac muscle hypertrophy in response to stress GO:1903244 9.67 MIR20A MIR17
27 response to transforming growth factor beta GO:0071559 9.67 SMAD4 EDN1
28 transmembrane receptor protein serine/threonine kinase signaling pathway GO:0007178 9.67 BMPR2 BMPR1B ACVRL1
29 positive regulation of vascular smooth muscle cell proliferation GO:1904707 9.67 MIR21 MIR20A MIR17 EDN1
30 response to dexamethasone GO:0071548 9.66 EDN1 CPS1
31 positive regulation of metalloendopeptidase activity GO:1904685 9.66 MIR21 MIR17
32 endothelial tube morphogenesis GO:0061154 9.65 MIR21 ACVRL1
33 artery development GO:0060840 9.65 BMPR2 ACVRL1
34 negative regulation of smooth muscle cell apoptotic process GO:0034392 9.65 MIR21 EDN1
35 retina vasculature development in camera-type eye GO:0061298 9.63 BMPR2 ACVRL1
36 body fluid secretion GO:0007589 9.62 NPPB EDN1
37 negative regulation of vascular associated smooth muscle cell apoptotic process GO:1905460 9.62 MIR21 MIR17
38 negative regulation of endothelial cell proliferation GO:0001937 9.62 MIR22 MIR21 CAV1 ACVRL1
39 lymphatic endothelial cell differentiation GO:0060836 9.61 BMPR2 ACVRL1
40 negative regulation of systemic arterial blood pressure GO:0003085 9.61 NPPB MIR17 BMPR2
41 endochondral bone morphogenesis GO:0060350 9.59 BMPR2 BMPR1B
42 positive regulation of pulmonary blood vessel remodeling GO:1905111 9.58 MIR20A MIR17
43 venous blood vessel development GO:0060841 9.57 BMPR2 ACVRL1
44 cellular response to BMP stimulus GO:0071773 9.56 SMAD4 BMPR2 BMPR1B ACVRL1
45 positive regulation of BMP signaling pathway GO:0030513 9.46 SMAD4 MIR20A BMPR2 ACVRL1
46 negative regulation of chondrocyte proliferation GO:1902731 9.13 MIR21 BMPR2 BMPR1B
47 BMP signaling pathway GO:0030509 9.02 SMAD4 MIR21 BMPR2 BMPR1B ACVRL1

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

# Name GO ID Score Top Affiliating Genes
1 mRNA binding involved in posttranscriptional gene silencing GO:1903231 9.67 MIR22 MIR21 MIR20A MIR17
2 nitric-oxide synthase binding GO:0050998 9.32 SLC6A4 CAV1
3 transforming growth factor beta-activated receptor activity GO:0005024 9.26 BMPR2 ACVRL1
4 BMP receptor activity GO:0098821 9.16 BMPR2 ACVRL1
5 transforming growth factor beta receptor activity, type I GO:0005025 8.96 BMPR1B ACVRL1
6 transmembrane receptor protein serine/threonine kinase activity GO:0004675 8.8 BMPR2 BMPR1B ACVRL1

Sources for Pulmonary Hypertension

9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
28 GO
29 GTR
31 HPO
32 ICD10
33 ICD10 via Orphanet
37 LifeMap
41 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
54 Novoseek
56 OMIM via Orphanet
57 OMIM® (Updated 20-May-2021)
61 PubMed
69 Tocris
71 UMLS via Orphanet
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