MCID: PLM037
MIFTS: 77

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 42 39 12 77 44 3 15 64 74
Primary Pulmonary Hypertension 42 12 77 38 30 56 6 15
Idiopathic Pulmonary Arterial Hypertension 74
Idiopathic Pulmonary Hypertension 74
Hypertension, Pulmonary 45
Hypertension Pulmonary 56

Classifications:



External Ids:

Disease Ontology 12 DOID:14557 DOID:6432
KEGG 38 H01619
ICD9CM 36 416.0
MeSH 45 D006976
NCIt 51 C3120 C97119
SNOMED-CT 69 26174007 70995007
ICD10 34 I27.0 I27.20

Summaries for Pulmonary Hypertension

MedlinePlus : 44 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 dyspnea, angina pectoris and edema. An important gene associated with Pulmonary Hypertension is MIR759 (MicroRNA 759), and among its related pathways/superpathways are TGF-beta signaling pathway and MicroRNAs in cancer. The drugs Adcirca and Adempas have been mentioned in the context of this disorder. Affiliated tissues include Lung, heart and endothelial, and related phenotypes are cardiovascular system and muscle

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

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

PubMed Health : 64 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 : 77 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

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

(show top 50) (show all 649)
# Related Disease Score Top Affiliating Genes
1 pulmonary hypertension, primary, 1 34.7 ACVRL1 BMPR2 EDN1 KCNK3 NPPB PDE5A
2 chronic thromboembolic pulmonary hypertension 34.7 BMPR2 NPPB
3 pulmonary venoocclusive disease 33.7 ACVRL1 BMPR2 KCNK3
4 persistent fetal circulation syndrome 33.4 EDN1 EDNRA NOS3 PDE5A
5 heritable pulmonary arterial hypertension 33.2 ACVRL1 BMPR2 CAV1 KCNK3 SMAD9
6 hypertension, essential 31.9 EDN1 EDNRA NOS3 NPPB
7 heart disease 31.6 BMPR2 EDN1 EDNRA NOS3 NPPB PDE5A
8 connective tissue disease 31.4 ACVRL1 BMPR2 EDN1 EDNRA
9 sleep apnea 31.2 EDN1 NOS3 NPPB
10 pulmonary edema 31.1 EDN1 NOS3 NPPB
11 vascular disease 31.1 ACVRL1 BMPR2 EDN1 MIR21 NOS3
12 portal hypertension 31.0 BMPR2 EDN1 EDNRA NOS3
13 congestive heart failure 30.9 EDN1 MIR21 NPPB
14 hepatopulmonary syndrome 30.8 ACVRL1 BMPR2 EDN1 NOS3
15 chronic mountain sickness 30.7 EDN1 NOS3 NPPB
16 arteries, anomalies of 30.6 ACVRL1 BMPR2 EDN1 NOS3 NPPB
17 coronary artery anomaly 30.5 EDN1 NOS3 NPPB
18 impotence 30.5 EDN1 NOS3 PDE5A
19 central sleep apnea 30.3 NOS3 NPPB
20 ischemic optic neuropathy 30.3 EDN1 NOS3 PDE5A
21 chronic pulmonary heart disease 30.3 ACVRL1 BMPR2 EDN1 SLC6A4 SMAD9
22 myocardial infarction 30.2 EDN1 MIR21 NOS3 NPPB PDE5A
23 angina pectoris 30.1 EDN1 NOS3 NPPB
24 nonarteritic anterior ischemic optic neuropathy 30.1 EDN1 NOS3 PDE5A
25 leukemia, acute lymphoblastic 28.8 MIR21 MIR22 MIR451A
26 hyperuricemia, pulmonary hypertension, renal failure, and alkalosis syndrome 12.7
27 pulmonary hypertension, neonatal 12.6
28 pulmonary hypertension, primary, 4 12.5
29 pulmonary hypertension, primary, 2 12.5
30 pulmonary hypertension, primary, 3 12.5
31 pulmonary hypertension, chronic thromboembolic, without deep vein thrombosis 12.5
32 vater-like defects with pulmonary hypertension, laryngeal webs, and growth deficiency 12.2
33 pulmonary hypertension, primary, autosomal recessive 12.1
34 eisenmenger syndrome 11.8
35 alveolar capillary dysplasia with misalignment of pulmonary veins 11.8
36 pulmonary edema of mountaineers 11.8
37 lymphedema and cerebral arteriovenous anomaly 11.7
38 alveolar capillary dysplasia 11.6
39 pulmonary fibrosis, idiopathic 11.6
40 hypoxia 11.5
41 pulmonary venoocclusive disease 2, autosomal recessive 11.5
42 cantu syndrome 11.5
43 diaphragmatic hernia, congenital 11.5
44 patent ductus arteriosus 1 11.5
45 pulmonary venoocclusive disease 1, autosomal dominant 11.4
46 pulmonary vein stenosis 11.4
47 pulmonary valve insufficiency 11.4
48 unilateral absence of a pulmonary artery 11.4
49 dehydrated hereditary stomatocytosis 1 with or without pseudohyperkalemia and/or perinatal edema 11.4
50 multiple mitochondrial dysfunctions syndrome 2 with hyperglycinemia 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:


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

MGI Mouse Phenotypes related to Pulmonary Hypertension:

47
# Description MGI Source Accession Score Top Affiliating Genes
1 cardiovascular system MP:0005385 9.97 ACVRL1 BMPR2 CAV1 EDN1 EDNRA KCNK3
2 muscle MP:0005369 9.86 ACVRL1 BMPR2 CAV1 EDN1 EDNRA NOS3
3 nervous system MP:0003631 9.85 ACVRL1 BMPR1B BMPR2 CAV1 EDN1 EDNRA
4 normal MP:0002873 9.61 ACVRL1 BMPR1B BMPR2 EDN1 EDNRA KCNK3
5 respiratory system MP:0005388 9.17 ACVRL1 BMPR2 CAV1 EDNRA KCNK3 NOS3

Drugs & Therapeutics for Pulmonary Hypertension

PubMedHealth treatment related to Pulmonary Hypertension: 64

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

FDA approved drugs:

(show all 7)
# Drug Name Active Ingredient(s) 19 Company Approval Date
1
Adcirca 19 TADALAFIL Eli Lilly May 2009
2
Adempas 19 RIOCIGUAT Bayer Healthcare Pharmaceuticals October 2013
3
Letairis 19 AMBRISENTAN Gilead June 2007
4
Opsumit 19 MACITENTAN Actelion Pharmaceuticals October 2013
5
Remodulin 19 TREPROSTINIL United Therapeutics May 2002
6
Tracleer 19 BOSENTAN Actelion November 2001
7
Tyvaso 19 TREPROSTINIL United Therapeutics July 2009

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

(show top 50) (show all 500)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Bosentan Approved, Investigational Phase 4,Phase 3,Phase 2,Phase 1,Not Applicable,Early Phase 1 147536-97-8 104865
2
Sodium Citrate Approved, Investigational Phase 4,Phase 2,Phase 3,Phase 1,Not Applicable,Early Phase 1 68-04-2
3
Iloprost Approved, Investigational Phase 4,Phase 2,Phase 3,Phase 1,Not Applicable 78919-13-8 6443959
4
Riociguat Approved Phase 4,Phase 3,Phase 2,Phase 1,Not Applicable 625115-55-1
5
Benzocaine Approved, Investigational Phase 4,Phase 3,Phase 2,Not Applicable 1994-09-7, 94-09-7 2337
6
tannic acid Approved Phase 4,Phase 3,Phase 2,Not Applicable 1401-55-4
7
Spironolactone Approved Phase 4,Phase 2,Phase 1,Not Applicable 1952-01-7, 52-01-7 5833
8
Iron Approved, Experimental Phase 4,Phase 3,Phase 2,Phase 1,Not Applicable 7439-89-6, 15438-31-0 27284 23925
9
Tadalafil Approved, Investigational Phase 4,Phase 2,Phase 3,Phase 1,Not Applicable 171596-29-5 110635
10
Epoprostenol Approved Phase 4,Phase 3,Phase 2,Not Applicable 35121-78-9, 61849-14-7 5282411 5280427
11
Treprostinil Approved, Investigational Phase 4,Phase 3,Phase 2,Phase 1,Not Applicable 81846-19-7 6918140 54786
12
Warfarin Approved Phase 4,Phase 2,Not Applicable 81-81-2 54678486 6691
13
Heparin Approved, Investigational Phase 4,Phase 3,Not Applicable 9005-49-6 46507594 772
14
Nitric Oxide Approved Phase 4,Phase 3,Phase 2,Phase 1,Not Applicable,Early Phase 1 10102-43-9 145068
15
Ambrisentan Approved, Investigational Phase 4,Phase 2,Phase 3,Phase 1,Not Applicable 177036-94-1 6918493
16
Macitentan Approved Phase 4,Phase 3,Phase 2,Phase 1,Not Applicable 441798-33-0
17
Ranolazine Approved, Investigational Phase 4,Phase 3,Phase 2,Phase 1 95635-55-5, 142387-99-3 56959
18
Dalteparin Approved Phase 4,Phase 3 9005-49-6
19
Isoflurane Approved, Vet_approved Phase 4 26675-46-7 3763
20
Propofol Approved, Investigational, Vet_approved Phase 4 2078-54-8 4943
21
Clevidipine Approved, Investigational Phase 4 167221-71-8
22
Zinc Approved, Investigational Phase 4,Not Applicable 7440-66-6 32051
23
Magnesium oxide Approved Phase 4 1309-48-4 14792
24
Silver sulfadiazine Approved, Vet_approved Phase 4 22199-08-2 441244
25
Acetazolamide Approved, Vet_approved Phase 4,Phase 2,Phase 3 59-66-5 1986
26 Poractant alfa Approved Phase 4,Phase 3 129069-19-8
27
Selexipag Approved Phase 4,Phase 3,Phase 2 475086-01-2
28
Ibuprofen Approved Phase 4,Phase 3,Not Applicable 15687-27-1 3672
29
Sevoflurane Approved, Vet_approved Phase 4 28523-86-6 5206
30
Nebivolol Approved, Investigational Phase 4 152520-56-4, 99200-09-6, 118457-14-0 71301
31
Morphine Approved, Investigational Phase 4,Phase 2 57-27-2 5288826
32
Bupivacaine Approved, Investigational Phase 4 38396-39-3, 2180-92-9 2474
33
Lidocaine Approved, Vet_approved Phase 4,Phase 1,Not Applicable 137-58-6 3676
34
Citric Acid Approved, Nutraceutical, Vet_approved Phase 4,Phase 2,Phase 3,Phase 1,Not Applicable,Early Phase 1 77-92-9 311
35
Serine Approved, Nutraceutical Phase 4,Phase 1,Not Applicable 56-45-1 5951
36
Calcium Approved, Nutraceutical Phase 4,Phase 2,Phase 3,Phase 1,Not Applicable 7440-70-2 271
37 Tezosentan Investigational Phase 4,Phase 3,Phase 2,Phase 1,Not Applicable 180384-57-0
38
Dabigatran Investigational Phase 4 211914-51-1
39 Beraprost Investigational Phase 4,Phase 3,Phase 2 88430-50-6
40
Lactitol Investigational Phase 4 585-88-6, 585-86-4 493591
41 Vasodilator Agents Phase 4,Phase 2,Phase 3,Phase 1,Not Applicable,Early Phase 1
42 Sildenafil Citrate Phase 4,Phase 2,Phase 3,Phase 1,Not Applicable,Early Phase 1 171599-83-0
43 Endothelin Receptor Antagonists Phase 4,Phase 2,Phase 3,Phase 1,Not Applicable,Early Phase 1
44 Citrate Phase 4,Phase 2,Phase 3,Phase 1,Not Applicable,Early Phase 1
45 Phosphodiesterase 5 Inhibitors Phase 4,Phase 2,Phase 3,Phase 1,Not Applicable,Early Phase 1
46 Antihypertensive Agents Phase 4,Phase 3,Phase 2,Phase 1,Not Applicable,Early Phase 1
47 Phosphodiesterase Inhibitors Phase 4,Phase 2,Phase 3,Phase 1,Not Applicable,Early Phase 1
48 Platelet Aggregation Inhibitors Phase 4,Phase 2,Phase 3,Phase 1,Not Applicable
49 Diuretics, Potassium Sparing Phase 4,Phase 3,Phase 2,Phase 1,Not Applicable
50 diuretics Phase 4,Phase 2,Phase 3,Phase 1,Not Applicable

Interventional clinical trials:

(show top 50) (show all 1251)
# 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 Inhaled Iloprost for Disproportionate Pulmonary Hypertension in Chronic Obstructive Pulmonary Disease (COPD) Unknown status NCT01116063 Phase 4 Inhaled iloprost
3 Bosentan in Pulmonary Hypertension in Interstitial Lung Disease Treatment Study Unknown status NCT00637065 Phase 4 Bosentan;Placebo
4 Riociguat for Sarcoidosis Associated Pulmonary Hypertension Unknown status NCT02625558 Phase 4 Riociguat;Placebo
5 Auto-PAP for Pulmonary Hypertension Treatment in Decompensated HF Patients With Sleep Apnea. Unknown status NCT02963597 Phase 4
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 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
11 Hemodynamic Response After Six Months of Sildenafil Unknown status NCT00483626 Phase 4 oral sildenafil
12 Evaluation of Sildenafil for the Treatment of Moderate Congestive Heart Failure Unknown status NCT00793338 Phase 4 Sildenafil
13 Hypotonic Treprostinil Subcutaneous Infusion for Control of Treprostinil Related Site Pain Unknown status NCT01615627 Phase 4 HypotonicTreprostinil Solution;Eutonic Treprostinil Solution
14 Pradaxa or Warfarin for Prevention of Recurrent VTE in Patients With Angiographically Confirmed Acute Massive Pulmonary Embolism undergoIng Endovascular Mechanical Fragmentation and Thrombolytic Therapy Unknown status NCT02979561 Phase 4 Dabigatran Etexilate;Warfarin
15 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
16 Acute Effect of Sildenafil on Exercise Tolerance and Functional Capacity in COPD, IPF and Post Pneumonectomy Patients Unknown status NCT01382368 Phase 4 Sildenafil
17 Targeting the Right Ventricle in Pulmonary Hypertension Completed NCT01839110 Phase 4 Ranolazine;Placebo
18 Targeting Right Ventricle in Pulmonary Hypertension Gilead Completed NCT02829034 Phase 4 Ranolazine;Placebo
19 PDEI Following Mitral Valve Surgery in Patients With Pulmonary Hypertension Completed NCT02378649 Phase 4 Sildenafil;Placebo
20 Study of Ranolazine in the Treatment of Pulmonary Hypertension Associated With Diastolic Left Ventricular Dysfunction Completed NCT02133352 Phase 4 Ranolazine
21 VasoREACTivity Testing With Intravenous SILdenafil in Patients With Precapillary Pulmonary Hypertension Completed NCT01889966 Phase 4 Sildenafil
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 Natrecor for Pulmonary Hypertension in Lung Transplants Completed NCT00205426 Phase 4 Natrecor
24 Exercise Induced Pulmonary Hypertension in Systemic Sclerosis and Treatment With Ambrisentan Completed NCT01051960 Phase 4 Ambrisentan
25 Oral Sildenafil in Persistent Pulmonary Hypertension Secondary to Meconium Aspiration Syndrome in Newborns Completed NCT01757782 Phase 4 Oral Sildenafil;Placebo (distilled water)
26 Sildenafil for Secondary Pulmonary Hypertension Due to Valvular Disease Completed NCT00862043 Phase 4 Sildenafil Citrate;Placebo
27 Comparison of Thrombgolytic and Anticoagulation Therapy in Submassive Pulmonary Embolism Completed NCT02132689 Phase 4 Actilyse (Thrombolytic therapy);Heparine (Standard anticoagulation therapy)
28 Safely Change From Bosentan to Ambrisentan in Pulmonary Hypertension Completed NCT01330108 Phase 4 ambrisentan
29 Sildenafil Effects on Pulmonary Haemodynamics and Gas Exchange in Chronic Obstructive Pulmonary Disease (COPD) Completed NCT00491803 Phase 4 Sildenafil;Sildenafil
30 Inhaled Iloprost for Sarcoidosis-associated Pulmonary Hypertension Completed NCT00403650 Phase 4 Iloprost
31 Measuring Outcomes In Patients With Pulmonary Arterial Hypertension Not on Active Treatment (MOTION) Completed NCT02191137 Phase 4 Riociguat (Adempas, BAY63-2521)
32 Phase IV Study of Chronic Infusional Epoprostenol for Severe Primary Pulmonary Hypertension Completed NCT00004754 Phase 4 epoprostenol
33 Efficacy of Beraprost in Lowering Pulmonary Arterial Pressure in Children Completed NCT03431649 Phase 4 Beraprost Sodium;Sildenafil Citrate
34 Characterization of LV Strain Patterns in Mildly Elevated PCWP and PAH. Completed NCT01800292 Phase 4 sildenafil
35 Inhaled Nitric Oxide and Inhaled Prostacyclin After Cardiac Surgery for Heart Transplant or LVAD Placement Completed NCT01717209 Phase 4 Nitric Oxide;Prostacyclin
36 Safety, Efficacy and Treatment Satisfaction in Patients With PAH Rapidly Switched From Epoprostenol to Remodulin Completed NCT00373360 Phase 4 treprostinil sodium
37 Effects of Propofol and Isoflurane on Pulmonary Vascular Resistance Completed NCT01212523 Phase 4 propofol and isoflurane
38 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
39 A Transition Study From Flolan® to Remodulin® in Patients With Pulmonary Arterial Hypertension Completed NCT00058929 Phase 4 treprostinil sodium
40 Efficacy Study of Riociguat and Its Effects on Exercise Performance and Pulmonary Artery Pressure at High Altitude Completed NCT02024386 Phase 4 Riociguat
41 BREATHE 5-OL: Tracleer (Bosentan) in Patients With Pulmonary Arterial Hypertension Related to Eisenmenger Physiology Completed NCT00367770 Phase 4 Tracleer®
42 Effects of Viagra on Heart Function in Patients With Heart Failure Completed NCT00781508 Phase 4 sildenafil
43 Clevidipine for Vasoreactivity Evaluation of the Pulmonary Arterial Bed Completed NCT01121458 Phase 4 Clevidipine
44 Transition From Injectable Prostacyclin Medication to Inhaled Prostacyclin Medication Completed NCT01268553 Phase 4 Treprostinil
45 Acute Hemodynamic Effects of Sildenafil in Patients With Severe Aortic Stenosis Completed NCT01060020 Phase 4 Sildenafil
46 Safety and Tolerability of Rapid Dose Titration of Subcutaneous Remodulin® Therapy in PAH Subjects (RAPID) Completed NCT02847260 Phase 4 Remodulin
47 Effect of Tadalafil on Insulin Secretion and Insulin Sensitivity in Obese Men. Completed NCT02595684 Phase 4 Tadalafil;Placebo
48 Acute Effect of Pulmonary Desufflation on Cardiac Performance in COPD Patients Completed NCT01996124 Phase 4 Indacaterol Fumarate;Placebo
49 Effects of the Combination of Bosentan and Sildenafil Versus Sildenafil Monotherapy on Pulmonary Arterial Hypertension (PAH) Completed NCT00303459 Phase 4 bosentan;placebo
50 Safety Evaluation of Inhaled Treprostinil Administration Following Transition From Inhaled Ventavis in Pulmonary Arterial Hypertension (PAH) Subjects Completed NCT00741819 Phase 4 Inhaled treprostinil

Search NIH Clinical Center for Pulmonary Hypertension

Inferred drug relations via UMLS 74 / NDF-RT 52 :


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 30 BMPR2

Anatomical Context for Pulmonary Hypertension

MalaCards organs/tissues related to Pulmonary Hypertension:

42
Heart, Endothelial, Testes, Lung, Smooth Muscle, Brain, Bone
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 7483)
# Title Authors Year
1
Traumatic Pulmonary Hypertension Secondary to Arteriovenous Fistula and Remote Gunshot Wound. ( 30760433 )
2019
2
Pulmonary Hypertension that Developed During Treatment for Hepatopulmonary Syndrome and Pulmonary Arteriovenous Malformation. ( 30799344 )
2019
3
Outcome of atrial fibrillation ablation in pulmonary hypertension: Is pulmonary hypertension a modifiable risk factor? ( 31016225 )
2019
4
Impact of pulmonary hypertension in patients undergoing atrial fibrillation ablation: A nationwide study. ( 30976653 )
2019
5
Risk Factors for Mitral Valve Surgery: Atrial Fibrillation and Pulmonary Hypertension. ( 30608218 )
2019
6
Case 2 / 2019 - Complete Atrioventricular Septal Defect, with Down Syndrome, without Pulmonary Hypertension and Natural History at 33 Years of Age. ( 30916196 )
2019
7
The Growth Differentiation Factor-15 (GDF-15) levels are increased in patients with compound heterozygous sickle cell and beta-thalassemia (HbS/βthal), correlate with markers of hemolysis, iron burden, coagulation, endothelial dysfunction and pulmonary hypertension. ( 31071550 )
2019
8
Pulmonary hypertension during respiratory syncytial virus bronchiolitis: a risk factor for severity of illness. ( 31104634 )
2019
9
EXPRESS: Sildenafil for bronchopulmonary dysplasia and pulmonary hypertension: a meta-analysis. ( 30803328 )
2019
10
Acute Vasoreactivity Testing during Cardiac Catheterization of Neonates with Bronchopulmonary Dysplasia-Associated Pulmonary Hypertension. ( 30871795 )
2019
11
Screening Echocardiography and Brain Natriuretic Peptide Levels Predict Late Pulmonary Hypertension in Infants with Bronchopulmonary Dysplasia. ( 30937503 )
2019
12
Accuracy of Brain Natriuretic Peptide for Diagnosing Pulmonary Hypertension in Severe Bronchopulmonary Dysplasia. ( 31096210 )
2019
13
Right ventricular function in infants with bronchopulmonary dysplasia and pulmonary hypertension: a pilot study. ( 30419798 )
2019
14
Agreement of an echocardiogram-based diagnosis of pulmonary hypertension in infants at risk for bronchopulmonary dysplasia among masked reviewers. ( 30464221 )
2019
15
Somatic growth and the risks of bronchopulmonary dysplasia and pulmonary hypertension: connecting epidemiology and physiology 1. ( 30512966 )
2019
16
Death or resolution: the "natural history" of pulmonary hypertension in bronchopulmonary dysplasia. ( 30617286 )
2019
17
Developmental outcomes of preterm infants with bronchopulmonary dysplasia-associated pulmonary hypertension at 18-24 months of corrected age. ( 30654786 )
2019
18
Digital Subtraction Pulmonary Angiography in Children with Pulmonary Hypertension due to Bronchopulmonary Dysplasia. ( 30743999 )
2019
19
Inhaled nitric oxide might be a contributing tool for successful resuscitation of cardiac arrest related to pulmonary hypertension. ( 30795771 )
2019
20
Cardiac arrest and pulmonary hypertension in scurvy: a case report. ( 30351242 )
2019
21
Pathophysiology, incidence, management, and consequences of cardiac arrhythmia in pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension. ( 30747032 )
2019
22
Angioscopic Evaluation During Balloon Pulmonary Angioplasty in Chronic Thromboembolic Pulmonary Hypertension. ( 30224170 )
2019
23
Growing number of incident pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension patients in Latvia: a shifting epidemiological landscape? Data from a national pulmonary hypertension registry. ( 30279036 )
2019
24
Exercise Training After Pulmonary Endarterectomy for Patients with Chronic Thromboembolic Pulmonary Hypertension. ( 30293071 )
2019
25
Discordance between Imaging Modalities in the Evaluation of Chronic Thromboembolic Pulmonary Hypertension: A Combined Experience from Two Academic Medical Centers. ( 30359536 )
2019
26
Intraoperative 3-Dimensional Echocardiography-Derived Right Ventricular Volumetric Analysis in Chronic Thromboembolic Pulmonary Hypertension Patients Before and After Pulmonary Thromboendarterectomy. ( 30385197 )
2019
27
Increasing mixed venous oxygen saturation is a predictor of improved renal function after balloon pulmonary angioplasty in patients with chronic thromboembolic pulmonary hypertension. ( 30386916 )
2019
28
Response to letter from dr Altmayer regarding publication "Sequential treatment with sildenafil and riociguat in patients with persistent or inoperable chronic thromboembolic pulmonary hypertension improves functional class and pulmonary hemodynamics". ( 30414750 )
2019
29
Validation of a claims-based algorithm to identify patients with chronic thromboembolic pulmonary hypertension using electronic health record data. ( 30419792 )
2019
30
The added value of right ventricular longitudinal strain in patients with chronic thromboembolic pulmonary hypertension. ( 30421353 )
2019
31
Subcutaneous treprostinil for the treatment of severe non-operable chronic thromboembolic pulmonary hypertension (CTREPH): a double-blind, phase 3, randomised controlled trial. ( 30477763 )
2019
32
Subcutaneous treprostinil: a new treatment for chronic thromboembolic pulmonary hypertension? ( 30477764 )
2019
33
Magnetic resonance direct thrombus imaging for pre-operative assessment of acute thrombosis in chronic thromboembolic pulmonary hypertension. ( 30496427 )
2019
34
Efficacy and Safety of Bronchial Artery Embolization on Hemoptysis in Chronic Thromboembolic Pulmonary Hypertension: A Pilot Prospective Cohort Study. ( 30531186 )
2019
35
Three-Dimensional Echocardiographic Evaluation of Right Ventricular Function in Chronic Thromboembolic Pulmonary Hypertension: Finding Meaning in the "Forgotten". ( 30538062 )
2019
36
Prevalence of antiphospholipid (aPL) antibodies among patients with chronic thromboembolic pulmonary hypertension: a systematic review and meta-analysis. ( 30603858 )
2019
37
Multimodality treatment for chronic thromboembolic pulmonary hypertension. ( 30611397 )
2019
38
The ADAMTS13-VWF axis is dysregulated in chronic thromboembolic pulmonary hypertension. ( 30655285 )
2019
39
Switching from sildenafil to riociguat in patients with persistent or inoperable chronic thromboembolic pulmonary hypertension. ( 30661547 )
2019
40
Traditional screening tool in the era of modern treatment for chronic thromboembolic pulmonary hypertension. ( 30661845 )
2019
41
Autologous endothelial progenitor cell therapy improves right ventricular function in a model of chronic thromboembolic pulmonary hypertension. ( 30669226 )
2019
42
Chronic thromboembolic pulmonary hypertension versus fibrosing mediastinitis. ( 30694812 )
2019
43
Chronic thromboembolic pulmonary hypertension (CTEPH): what do we know about it? A comprehensive review of the literature. ( 30720636 )
2019
44
Working backward: Retrograde balloon angioplasty of atretic arteries in chronic thromboembolic pulmonary hypertension. ( 30723998 )
2019
45
Chronic Thromboembolic Pulmonary Hypertension Cases Cluster in Families. ( 30732691 )
2019
46
Pathophysiology of right ventricular failure in acute pulmonary embolism and chronic thromboembolic pulmonary hypertension: a pictorial essay for the interventional radiologist. ( 30758687 )
2019
47
Elevated levels of autoantibodies against EXD2 and PHAX in the sera of patients with chronic thromboembolic pulmonary hypertension. ( 30759165 )
2019
48
Molecular Research in Chronic Thromboembolic Pulmonary Hypertension. ( 30759794 )
2019
49
Shortening Hospital Stay Is Feasible and Safe in Patients With Chronic Thromboembolic Pulmonary Hypertension Treated With Balloon Pulmonary Angioplasty. ( 30760426 )
2019
50
Right Ventricular Load and Function in CTEPH: Differences between Proximal and Distal CTEPH. ( 30768356 )
2019

Variations for Pulmonary Hypertension

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:

38
# Name Kegg Source Accession
1 TGF-beta signaling pathway hsa04350

GO Terms for Pulmonary Hypertension

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

# Name GO ID Score Top Affiliating Genes
1 integral component of plasma membrane GO:0005887 9.7 ACVRL1 BMPR1B BMPR2 CAV1 EDNRA KCNK3
2 extracellular space GO:0005615 9.5 BMPR2 EDN1 MIR20A MIR21 MIR22 MIR451A
3 caveola GO:0005901 8.8 BMPR2 CAV1 NOS3

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

(show top 50) (show all 52)
# Name GO ID Score Top Affiliating Genes
1 negative regulation of gene expression GO:0010629 9.97 ACVRL1 EDN1 MIR20A MIR21
2 in utero embryonic development GO:0001701 9.93 ACVRL1 EDN1 EDNRA NOS3
3 negative regulation of cell growth GO:0030308 9.88 ACVRL1 BMPR2 NPPB
4 positive regulation of angiogenesis GO:0045766 9.87 ACVRL1 MIR20A MIR21 NOS3
5 response to hypoxia GO:0001666 9.83 ACVRL1 CAV1 EDN1 EDNRA SLC6A4
6 pattern specification process GO:0007389 9.82 ACVRL1 BMPR1B BMPR2
7 cellular response to transforming growth factor beta stimulus GO:0071560 9.8 ACVRL1 CAV1 EDN1
8 positive regulation of osteoblast differentiation GO:0045669 9.79 BMPR1B BMPR2 MIR21
9 positive regulation of endothelial cell migration GO:0010595 9.76 BMPR2 EDN1 MIR21
10 positive regulation of vascular smooth muscle cell proliferation GO:1904707 9.75 EDN1 MIR20A MIR21
11 transforming growth factor beta receptor signaling pathway GO:0007179 9.72 ACVRL1 BMPR1B BMPR2 MIR21 SMAD9
12 membrane depolarization GO:0051899 9.71 CAV1 EDN1
13 negative regulation of endothelial cell migration GO:0010596 9.71 ACVRL1 MIR21
14 positive regulation of chondrocyte differentiation GO:0032332 9.71 ACVRL1 BMPR1B
15 blood vessel remodeling GO:0001974 9.71 ACVRL1 BMPR2 NOS3
16 negative regulation of endothelial cell proliferation GO:0001937 9.71 ACVRL1 CAV1 MIR21 MIR22
17 cellular response to peptide hormone stimulus GO:0071375 9.7 CAV1 EDN1
18 regulation of nitric-oxide synthase activity GO:0050999 9.7 CAV1 NOS3
19 vasodilation GO:0042311 9.7 CPS1 NOS3
20 positive regulation of endothelial cell differentiation GO:0045603 9.69 ACVRL1 MIR21
21 activin receptor signaling pathway GO:0032924 9.69 ACVRL1 BMPR2
22 chondrocyte development GO:0002063 9.69 BMPR1B BMPR2
23 positive regulation of cardiac muscle hypertrophy GO:0010613 9.69 EDN1 MIR21 PDE5A
24 endocardial cushion morphogenesis GO:0003203 9.68 ACVRL1 NOS3
25 positive regulation of cartilage development GO:0061036 9.68 BMPR1B BMPR2
26 positive regulation of renal sodium excretion GO:0035815 9.68 EDN1 NPPB
27 positive regulation of urine volume GO:0035810 9.68 EDN1 NPPB
28 proteoglycan biosynthetic process GO:0030166 9.67 BMPR1B BMPR2
29 negative regulation of DNA biosynthetic process GO:2000279 9.67 ACVRL1 BMPR2
30 positive regulation of vascular associated smooth muscle cell migration GO:1904754 9.67 MIR20A MIR21 MIR451A
31 cellular response to BMP stimulus GO:0071773 9.67 ACVRL1 BMPR1B BMPR2 SMAD9
32 lymphangiogenesis GO:0001946 9.66 ACVRL1 BMPR2
33 response to dexamethasone GO:0071548 9.66 CPS1 EDN1
34 artery smooth muscle contraction GO:0014824 9.65 EDN1 EDNRA
35 body fluid secretion GO:0007589 9.65 EDN1 NPPB
36 negative regulation of smooth muscle cell apoptotic process GO:0034392 9.65 EDN1 MIR21
37 transmembrane receptor protein serine/threonine kinase signaling pathway GO:0007178 9.65 ACVRL1 BMPR1B BMPR2
38 BMP signaling pathway GO:0030509 9.65 ACVRL1 BMPR1B BMPR2 MIR21 SMAD9
39 artery development GO:0060840 9.64 ACVRL1 BMPR2
40 retina vasculature development in camera-type eye GO:0061298 9.63 ACVRL1 BMPR2
41 regulation of blood vessel size GO:0050880 9.63 EDN1 NOS3 NPPB
42 endothelial tube morphogenesis GO:0061154 9.61 ACVRL1 MIR21
43 regulation of glucose transmembrane transport GO:0010827 9.61 EDN1 EDNRA
44 lymphatic endothelial cell differentiation GO:0060836 9.6 ACVRL1 BMPR2
45 regulation of systemic arterial blood pressure by endothelin GO:0003100 9.59 EDN1 NOS3
46 endochondral bone morphogenesis GO:0060350 9.56 BMPR1B BMPR2
47 venous blood vessel development GO:0060841 9.55 ACVRL1 BMPR2
48 regulation of the force of heart contraction by chemical signal GO:0003057 9.52 CAV1 NOS3
49 regulation of blood pressure GO:0008217 9.35 ACVRL1 EDN1 EDNRA NOS3 NPPB
50 negative regulation of chondrocyte proliferation GO:1902731 9.33 BMPR1B BMPR2 MIR21

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

# Name GO ID Score Top Affiliating Genes
1 SMAD binding GO:0046332 9.43 ACVRL1 BMPR1B BMPR2
2 nitric-oxide synthase binding GO:0050998 9.4 CAV1 SLC6A4
3 BMP receptor activity GO:0098821 9.37 ACVRL1 BMPR2
4 growth factor binding GO:0019838 9.33 ACVRL1 BMPR1B BMPR2
5 transforming growth factor beta receptor activity, type I GO:0005025 9.32 ACVRL1 BMPR1B
6 transforming growth factor beta-activated receptor activity GO:0005024 9.13 ACVRL1 BMPR1B BMPR2
7 transmembrane receptor protein serine/threonine kinase activity GO:0004675 8.8 ACVRL1 BMPR1B BMPR2

Sources for Pulmonary Hypertension

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
20 FMA
29 GO
30 GTR
31 HGMD
32 HMDB
33 HPO
34 ICD10
35 ICD10 via Orphanet
36 ICD9CM
37 IUPHAR
38 KEGG
39 LifeMap
41 LOVD
43 MedGen
45 MeSH
46 MESH via Orphanet
47 MGI
50 NCI
51 NCIt
52 NDF-RT
55 NINDS
56 Novoseek
58 OMIM
59 OMIM via Orphanet
63 PubMed
65 QIAGEN
70 SNOMED-CT via HPO
71 SNOMED-CT via Orphanet
72 TGDB
73 Tocris
74 UMLS
75 UMLS via Orphanet
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