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 41 38 12 76 43 3 15 63 73
Primary Pulmonary Hypertension 41 12 76 37 29 55 6 15
Idiopathic Pulmonary Arterial Hypertension 73
Idiopathic Pulmonary Hypertension 73
Hypertension, Pulmonary 44
Hypertension Pulmonary 55

Classifications:



External Ids:

Disease Ontology 12 DOID:14557 DOID:6432
ICD10 33 I27.0 I27.20
ICD9CM 35 416.0
NCIt 50 C97119 C3120
SNOMED-CT 68 26174007 70995007
MeSH 44 D006976
KEGG 37 H01619

Summaries for Pulmonary Hypertension

MedlinePlus : 43 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 lung, 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 : 63 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 : 76 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 564)
# Related Disease Score Top Affiliating Genes
1 pulmonary hypertension, primary, 1 34.5 ACVRL1 BMPR2 EDN1 KCNK3 NPPB PDE5A
2 chronic thromboembolic pulmonary hypertension 34.3 BMPR2 EDN1 NPPB
3 pulmonary venoocclusive disease 33.3 ACVRL1 BMPR2 KCNK3
4 persistent fetal circulation syndrome 33.1 EDN1 EDNRA NOS3 PDE5A
5 heritable pulmonary arterial hypertension 32.9 ACVRL1 BMPR2 CAV1 KCNK3 SMAD9
6 heart disease 31.2 BMPR2 EDN1 EDNRA NOS3 NPPB PDE5A
7 connective tissue disease 31.1 ACVRL1 BMPR2 EDN1 EDNRA
8 vascular disease 30.8 ACVRL1 BMPR2 EDN1 MIR21 NOS3
9 pulmonary edema 30.7 EDN1 NOS3 NPPB
10 sleep apnea 30.7 EDN1 NOS3 NPPB
11 congestive heart failure 30.5 EDN1 MIR21 NPPB
12 hepatopulmonary syndrome 30.5 ACVRL1 BMPR2 EDN1 NOS3
13 portal hypertension 30.5 BMPR2 EDN1 EDNRA NOS3
14 chronic mountain sickness 30.4 EDN1 NOS3 NPPB
15 arteries, anomalies of 30.1 ACVRL1 BMPR2 EDN1 NOS3 NPPB
16 coronary artery anomaly 30.1 EDN1 NOS3 NPPB
17 central sleep apnea 30.1 NOS3 NPPB
18 ischemic optic neuropathy 30.0 EDN1 NOS3 PDE5A
19 chronic pulmonary heart disease 30.0 ACVRL1 BMPR2 EDN1 SLC6A4 SMAD9
20 myocardial infarction 29.9 EDN1 MIR21 NOS3 NPPB PDE5A
21 angina pectoris 29.8 EDN1 NOS3 NPPB
22 impotence 29.8 EDN1 NOS3 PDE5A
23 nonarteritic anterior ischemic optic neuropathy 29.8 EDN1 NOS3 PDE5A
24 hypertension, essential 29.7 EDN1 EDNRA NOS3 NPPB
25 hyperuricemia, pulmonary hypertension, renal failure, and alkalosis syndrome 12.6
26 pulmonary hypertension, primary, 4 12.5
27 pulmonary hypertension, primary, 2 12.4
28 pulmonary hypertension, primary, 3 12.4
29 pulmonary hypertension, neonatal 12.4
30 pulmonary hypertension, chronic thromboembolic, without deep vein thrombosis 12.4
31 vater-like defects with pulmonary hypertension, laryngeal webs, and growth deficiency 12.1
32 pulmonary hypertension, primary, autosomal recessive 12.1
33 alveolar capillary dysplasia with misalignment of pulmonary veins 11.8
34 pulmonary edema of mountaineers 11.7
35 eisenmenger syndrome 11.7
36 lymphedema and cerebral arteriovenous anomaly 11.6
37 alveolar capillary dysplasia 11.6
38 pulmonary fibrosis, idiopathic 11.5
39 hypoxia 11.5
40 pulmonary venoocclusive disease 2, autosomal recessive 11.5
41 cantu syndrome 11.5
42 diaphragmatic hernia, congenital 11.4
43 patent ductus arteriosus 1 11.4
44 pulmonary venoocclusive disease 1, autosomal dominant 11.4
45 pulmonary vein stenosis 11.3
46 pulmonary valve insufficiency 11.3
47 unilateral absence of a pulmonary artery 11.3
48 adams-oliver syndrome 1 11.3
49 dehydrated hereditary stomatocytosis 1 with or without pseudohyperkalemia and/or perinatal edema 11.3
50 adams-oliver syndrome 11.3

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:

46
# 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: 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.")

FDA approved drugs:

(show all 7)
# Drug Name Active Ingredient(s) 18 Company Approval Date
1
Adcirca 18 TADALAFIL Eli Lilly May 2009
2
Adempas 18 RIOCIGUAT Bayer Healthcare Pharmaceuticals October 2013
3
Letairis 18 AMBRISENTAN Gilead June 2007
4
Opsumit 18 MACITENTAN Actelion Pharmaceuticals October 2013
5
Remodulin 18 TREPROSTINIL United Therapeutics May 2002
6
Tracleer 18 BOSENTAN Actelion November 2001
7
Tyvaso 18 TREPROSTINIL United Therapeutics July 2009

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

(show top 50) (show all 435)
# 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
Iloprost Approved, Investigational Phase 4,Phase 2,Phase 3,Phase 1,Not Applicable 78919-13-8 6443959
3
Benzocaine Approved, Investigational Phase 4,Phase 3,Phase 2,Not Applicable 94-09-7, 1994-09-7 2337
4 tannic acid Approved Phase 4,Phase 3,Phase 2,Not Applicable
5
Spironolactone Approved Phase 4,Phase 2,Phase 1,Not Applicable 52-01-7, 1952-01-7 5833
6
Iron Approved Phase 4,Phase 3,Phase 2,Phase 1,Not Applicable 7439-89-6 23925
7
Tadalafil Approved, Investigational Phase 4,Phase 2,Phase 3,Phase 1,Not Applicable 171596-29-5 110635
8
Epoprostenol Approved Phase 4,Phase 3,Phase 2,Not Applicable 61849-14-7, 35121-78-9 5280427 5282411
9
Treprostinil Approved, Investigational Phase 4,Phase 3,Phase 2,Phase 1,Not Applicable 81846-19-7 54786 6918140
10
Warfarin Approved Phase 4,Phase 2 81-81-2 6691 54678486
11
Heparin Approved, Investigational Phase 4,Phase 3 9005-49-6 772 46507594
12
Nitric Oxide Approved Phase 4,Phase 3,Phase 2,Phase 1,Not Applicable,Early Phase 1 10102-43-9 145068
13
Ambrisentan Approved, Investigational Phase 4,Phase 2,Phase 3,Phase 1,Not Applicable 177036-94-1 6918493
14
Macitentan Approved Phase 4,Phase 3,Phase 2,Phase 1,Not Applicable 441798-33-0
15
Lidocaine Approved, Vet_approved Phase 4,Phase 1,Not Applicable 137-58-6 3676
16
Ranolazine Approved, Investigational Phase 4,Phase 3,Phase 2,Phase 1,Not Applicable 142387-99-3, 95635-55-5 56959
17
Dalteparin Approved Phase 4,Phase 3 9005-49-6
18
Isoflurane Approved, Vet_approved Phase 4 26675-46-7 3763
19
Propofol Approved, Investigational, Vet_approved Phase 4 2078-54-8 4943
20
Clevidipine Approved, Investigational Phase 4 167221-71-8
21
Zinc Approved, Investigational Phase 4,Not Applicable 7440-66-6
22
Magnesium oxide Approved Phase 4 1309-48-4 14792
23
Silver sulfadiazine Approved, Vet_approved Phase 4 22199-08-2 441244
24
Acetazolamide Approved, Vet_approved Phase 4,Phase 2,Phase 3 59-66-5 1986
25 Poractant alfa Approved Phase 4,Phase 3 129069-19-8
26 Selexipag Approved Phase 4,Phase 3,Phase 2 475086-01-2
27
Ibuprofen Approved Phase 4,Phase 3,Not Applicable 15687-27-1 3672
28
Sevoflurane Approved, Vet_approved Phase 4 28523-86-6 5206
29
Nebivolol Approved, Investigational Phase 4 99200-09-6, 152520-56-4, 118457-14-0 71301
30
Bupivacaine Approved, Investigational Phase 4 2180-92-9, 38396-39-3 2474
31
Morphine Approved, Investigational Phase 4,Phase 2 57-27-2 5288826
32
Citric Acid Approved, Nutraceutical, Vet_approved Phase 4,Phase 2,Phase 3,Phase 1,Not Applicable 77-92-9 311
33
Serine Approved, Nutraceutical Phase 4,Phase 1 56-45-1 5951
34 Tezosentan Investigational Phase 4,Phase 3,Phase 2,Not Applicable 180384-57-0
35 Beraprost Investigational Phase 4,Phase 3,Phase 2 88430-50-6
36
Lactitol Investigational Phase 4 585-86-4 3871
37 Vasodilator Agents Phase 4,Phase 2,Phase 3,Phase 1,Not Applicable,Early Phase 1
38 Sildenafil Citrate Phase 4,Phase 2,Phase 3,Phase 1,Not Applicable 171599-83-0
39 Endothelin Receptor Antagonists Phase 4,Phase 2,Phase 3,Phase 1,Not Applicable,Early Phase 1
40 Citrate Phase 4,Phase 2,Phase 3,Phase 1,Not Applicable
41 Phosphodiesterase 5 Inhibitors Phase 4,Phase 2,Phase 3,Phase 1,Not Applicable
42 Antihypertensive Agents Phase 4,Phase 3,Phase 2,Phase 1,Not Applicable,Early Phase 1
43 Phosphodiesterase Inhibitors Phase 4,Phase 2,Phase 3,Phase 1,Not Applicable
44 Platelet Aggregation Inhibitors Phase 4,Phase 2,Phase 3,Phase 1,Not Applicable
45 Diuretics, Potassium Sparing Phase 4,Phase 3,Phase 2,Phase 1,Not Applicable
46 diuretics Phase 4,Phase 2,Phase 3,Phase 1,Not Applicable
47 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 4,Phase 3,Phase 2,Phase 1,Early Phase 1,Not Applicable
48 Hormone Antagonists Phase 4,Phase 3,Phase 2,Phase 1,Early Phase 1,Not Applicable
49 Mineralocorticoids Phase 4,Phase 2,Phase 1,Not Applicable
50 Hormones Phase 4,Phase 3,Phase 2,Phase 1,Early Phase 1,Not Applicable

Interventional clinical trials:

(show top 50) (show all 1155)
# 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 Targeting the Right Ventricle in Pulmonary Hypertension Completed NCT01839110 Phase 4 Ranolazine;Placebo
16 PDEI Following Mitral Valve Surgery in Patients With Pulmonary Hypertension Completed NCT02378649 Phase 4 Sildenafil;Placebo
17 Study of Ranolazine in the Treatment of Pulmonary Hypertension Associated With Diastolic Left Ventricular Dysfunction Completed NCT02133352 Phase 4 Ranolazine
18 VasoREACTivity Testing With Intravenous SILdenafil in Patients With Precapillary Pulmonary Hypertension Completed NCT01889966 Phase 4 Sildenafil
19 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
20 Natrecor for Pulmonary Hypertension in Lung Transplants Completed NCT00205426 Phase 4 Natrecor
21 Exercise Induced Pulmonary Hypertension in Systemic Sclerosis and Treatment With Ambrisentan Completed NCT01051960 Phase 4 Ambrisentan
22 Oral Sildenafil in Persistent Pulmonary Hypertension Secondary to Meconium Aspiration Syndrome in Newborns Completed NCT01757782 Phase 4 Oral Sildenafil;Placebo (distilled water)
23 Sildenafil for Secondary Pulmonary Hypertension Due to Valvular Disease Completed NCT00862043 Phase 4 Sildenafil Citrate;Placebo
24 Comparison of Thrombgolytic and Anticoagulation Therapy in Submassive Pulmonary Embolism Completed NCT02132689 Phase 4 Actilyse (Thrombolytic therapy);Heparine (Standard anticoagulation therapy)
25 Safely Change From Bosentan to Ambrisentan in Pulmonary Hypertension Completed NCT01330108 Phase 4 ambrisentan
26 Sildenafil Effects on Pulmonary Haemodynamics and Gas Exchange in Chronic Obstructive Pulmonary Disease (COPD) Completed NCT00491803 Phase 4 Sildenafil;Sildenafil
27 Inhaled Iloprost for Sarcoidosis-associated Pulmonary Hypertension Completed NCT00403650 Phase 4 Iloprost
28 Measuring Outcomes In Patients With Pulmonary Arterial Hypertension Not on Active Treatment (MOTION) Completed NCT02191137 Phase 4 Riociguat (Adempas, BAY63-2521)
29 Phase IV Study of Chronic Infusional Epoprostenol for Severe Primary Pulmonary Hypertension Completed NCT00004754 Phase 4 epoprostenol
30 Efficacy of Beraprost in Lowering Pulmonary Arterial Pressure in Children Completed NCT03431649 Phase 4 Beraprost Sodium;Sildenafil Citrate
31 Characterization of LV Strain Patterns in Mildly Elevated PCWP and PAH. Completed NCT01800292 Phase 4 sildenafil
32 Inhaled Nitric Oxide and Inhaled Prostacyclin After Cardiac Surgery for Heart Transplant or LVAD Placement Completed NCT01717209 Phase 4 Nitric Oxide;Prostacyclin
33 Safety, Efficacy and Treatment Satisfaction in Patients With PAH Rapidly Switched From Epoprostenol to Remodulin Completed NCT00373360 Phase 4 treprostinil sodium
34 Effects of Propofol and Isoflurane on Pulmonary Vascular Resistance Completed NCT01212523 Phase 4 propofol and isoflurane
35 Evaluate Effect of Optison on Pulmonary Artery Systolic Pressure (PASP) and Pulmonary Vascular Resistance (PVR). Completed NCT00878878 Phase 4 Optison (Perflutren Protein-Type A Microspheres Injectable Suspension);Dextrose
36 A Transition Study From Flolan® to Remodulin® in Patients With Pulmonary Arterial Hypertension Completed NCT00058929 Phase 4 treprostinil sodium
37 Efficacy Study of Riociguat and Its Effects on Exercise Performance and Pulmonary Artery Pressure at High Altitude Completed NCT02024386 Phase 4 Riociguat
38 BREATHE 5-OL: Tracleer (Bosentan) in Patients With Pulmonary Arterial Hypertension Related to Eisenmenger Physiology Completed NCT00367770 Phase 4 Tracleer®
39 Effects of Viagra on Heart Function in Patients With Heart Failure Completed NCT00781508 Phase 4 sildenafil
40 Clevidipine for Vasoreactivity Evaluation of the Pulmonary Arterial Bed Completed NCT01121458 Phase 4 Clevidipine
41 Transition From Injectable Prostacyclin Medication to Inhaled Prostacyclin Medication Completed NCT01268553 Phase 4 Treprostinil
42 Acute Hemodynamic Effects of Sildenafil in Patients With Severe Aortic Stenosis Completed NCT01060020 Phase 4 Sildenafil
43 Safety and Tolerability of Rapid Dose Titration of Subcutaneous Remodulin® Therapy in PAH Subjects (RAPID) Completed NCT02847260 Phase 4 Remodulin
44 Effect of Tadalafil on Insulin Secretion and Insulin Sensitivity in Obese Men. Completed NCT02595684 Phase 4 Tadalafil;Placebo
45 Acute Effect of Pulmonary Desufflation on Cardiac Performance in COPD Patients Completed NCT01996124 Phase 4 Indacaterol Fumarate;Placebo
46 Effects of the Combination of Bosentan and Sildenafil Versus Sildenafil Monotherapy on Pulmonary Arterial Hypertension (PAH) Completed NCT00303459 Phase 4 bosentan;placebo
47 Safety Evaluation of Inhaled Treprostinil Administration Following Transition From Inhaled Ventavis in Pulmonary Arterial Hypertension (PAH) Subjects Completed NCT00741819 Phase 4 Inhaled treprostinil
48 Study of Add-on Ambrisentan Therapy to Background Phosphodiesterase Type-5 Inhibitor (PDE5i) Therapy in Pulmonary Arterial Hypertension (ATHENA-1) Completed NCT00617305 Phase 4 Ambrisentan;Placebo;Sildenafil;Tadalafil
49 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
50 Assess the Efficacy and Safety of Sildenafil When Added to Bosentan in the Treatment of Pulmonary Arterial Hypertension Completed NCT00323297 Phase 4 Bosentan;Bosentan;Sildenafil Citrate

Search NIH Clinical Center for Pulmonary Hypertension

Inferred drug relations via UMLS 73 / NDF-RT 51 :


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 BMPR2

Anatomical Context for Pulmonary Hypertension

MalaCards organs/tissues related to Pulmonary Hypertension:

41
Heart, Lung, Endothelial, Testes, Smooth Muscle, Liver, Brain
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 5480)
# Title Authors Year
1
Right ventricular function in infants with bronchopulmonary dysplasia and pulmonary hypertension: a pilot study. ( 30419798 )
2019
2
Health-related quality of life, treatment adherence and psychosocial support in patients with pulmonary arterial hypertension or chronic thromboembolic pulmonary hypertension. ( 30011997 )
2019
3
Validation of a claims-based algorithm to identify patients with chronic thromboembolic pulmonary hypertension using electronic health record data. ( 30419792 )
2019
4
Hemodynamics of the diastolic pressure gradients in acute heart failure: implications for the diagnosis of pre-capillary pulmonary hypertension in left heart disease. ( 30419797 )
2019
5
MicroRNA-150 relieves vascular remodeling and fibrosis in hypoxia-induced pulmonary hypertension. ( 30551428 )
2019
6
Stabilization of p22phox by Hypoxia Promotes Pulmonary Hypertension. ( 30044141 )
2019
7
Tricuspid annular plane systolic excursion (TAPSE) in pediatric pulmonary hypertension: Integrating right ventricular ejection efficiency (RVEe) into advanced multi-parametric imaging. ( 30454722 )
2019
8
Target oxygen saturation and development of pulmonary hypertension and increased pulmonary vascular resistance in preterm infants. ( 30461218 )
2019
9
Treprostinil treatment decreases circulating platelet microvesicles and their procoagulant activity in pediatric pulmonary hypertension. ( 30485728 )
2019
10
AOS ameliorates monocrotaline-induced pulmonary hypertension by restraining the activation of P-selectin/p38MAPK/NF-κB pathway in rats. ( 30551382 )
2019
11
Genipin attenuates hyperoxia-induced lung injury and pulmonary hypertension via targeting glycogen synthase kinase-3 β in neonatal rats. ( 30196116 )
2019
12
Anesthetic management of an infant with Jeune syndrome and severe pulmonary hypertension for tracheostomy. ( 30218885 )
2019
13
Oxymatrine prevents the development of monocrotaline-induced pulmonary hypertension via regulation of the NG, NG-dimethyl-L-arginine metabolism pathways in rats. ( 30419238 )
2019
14
Hemodynamic changes after acute fluid loading in patients with systemic sclerosis without pulmonary hypertension. ( 30419796 )
2019
15
Proximal pulmonary arterial wall disease in patients with persistent pulmonary hypertension after successful left-sided valve replacement according to the hemodynamic phenotype. ( 30430894 )
2019
16
Pulmonary Hypertension in Women. ( 30449376 )
2019
17
Pulmonary hypertension in an adolescent with end-stage-renal disease-a diagnostic challenge: Questions. ( 29532234 )
2019
18
Pulmonary hypertension in an adolescent with end-stage-renal disease-a diagnostic challenge: Answers. ( 29532236 )
2019
19
Balloon Pulmonary Angioplasty for Inoperable Patients With Chronic Thromboembolic Pulmonary Hypertension. Observational Study in a Referral Unit. ( 29857972 )
2018
20
Sequential Hybrid Therapy With Pulmonary Endarterectomy and Additional Balloon Pulmonary Angioplasty for Chronic Thromboembolic Pulmonary Hypertension. ( 29929993 )
2018
21
Prevalence and impact of WHO group 3 pulmonary hypertension in advanced idiopathic nonspecific interstitial pneumonia. ( 29773690 )
2018
22
Impact of Balloon Pulmonary Angioplasty on Hemodynamics and Clinical Outcomes in Patients with Chronic Thromboembolic Pulmonary Hypertension: the Initial Korean Experience. ( 29318791 )
2018
23
Approach to Pulmonary Hypertension: From CT to Clinical Diagnosis. ( 29432063 )
2018
24
Balloon Pulmonary Angioplasty Relieves Hemodynamic Stress towards Untreated-Side Pulmonary Vasculature and Ameliorates Its Resistance in Patients with Chronic Thromboembolic Pulmonary Hypertension. ( 29360065 )
2018
25
Neopterin as a Biomarker in Patients with Pulmonary Arterial Hypertension and Chronic Thromboembolic Pulmonary Hypertension. ( 29909420 )
2018
26
Vascular endothelial growth factor and pulmonary hypertension in children with beta thalassemia major. ( 29859904 )
2018
27
Pulmonary Hypertension Related to Chronic Obstructive Pulmonary Disease and Diffuse Parenchymal Lung Disease: A Focus on Right Ventricular (Dys)Function. ( 29966637 )
2018
28
Epidemiology and risk factors for chronic thromboembolic pulmonary hypertension. ( 29338883 )
2018
29
Use of direct oral anticoagulants for chronic thromboembolic pulmonary hypertension. ( 29791520 )
2018
30
Bosentan Therapy for Pulmonary Arterial Hypertension and Chronic Thromboembolic Pulmonary Hypertension: A Systemic Review and Meta-Analysis. ( 29393580 )
2018
31
Pulmonary Hypertension in Aortic and Mitral Valve Disease. ( 29876357 )
2018
32
Severe Compression of the Left Main Coronary Artery in a Patient with Chronic Thromboembolic Pulmonary Hypertension. ( 29321385 )
2018
33
Screening for pulmonary hypertension in interstitial lung disease: Many reasons to ECHO! ( 29415370 )
2018
34
Poor Subpleural Perfusion Predicts Failure After Balloon Pulmonary Angioplasty for Nonoperable Chronic Thromboembolic Pulmonary Hypertension. ( 29730328 )
2018
35
Macitentan in pulmonary hypertension due to left ventricular dysfunction. ( 29437943 )
2018
36
MRI-derived Regional Biventricular Function in Patients with Chronic Thromboembolic Pulmonary Hypertension Before and After Pulmonary Endarterectomy. ( 29730148 )
2018
37
Perioperative events in children with pulmonary hypertension undergoing non-cardiac procedures. ( 28971729 )
2018
38
Biomarkers in Pulmonary Hypertension. ( 29966636 )
2018
39
Pulmonary Hypertension in Glycogen Storage Disease Type II. ( 29786057 )
2018
40
Impact of histologic chorioamnionitis on pulmonary hypertension and respiratory outcomes in preterm infants. ( 29480140 )
2018
41
Haemodynamics to predict outcome in pulmonary hypertension due to left heart disease: a meta-analysis. ( 29439019 )
2018
42
Usefulness of scoring right ventricular function for assessment of prognostic factors in patients with chronic thromboembolic pulmonary hypertension. ( 29704099 )
2018
43
Twist1 in Hypoxia-induced Pulmonary Hypertension through Transforming Growth Factor-I^-Smad Signaling. ( 28915063 )
2018
44
Will we be singing a different tune on combined post- and pre-capillary pulmonary hypertension? ( 29437950 )
2018
45
Surgery for partial atrioventricular septal defect with pulmonary hypertension in an adult dog. ( 29877312 )
2018
46
Severe Pulmonary Hypertension Due to Adult-Onset Still's Disease. ( 29468168 )
2018
47
Lethal persistent pulmonary hypertension of the newborn in Bohring-Opitz syndrome. ( 29681105 )
2018
48
Persistent Pulmonary Hypertension and Histologic Chorioamnionitis in Preterm Infants: Controlled Study. ( 29453682 )
2018
49
NTproBNP as a surrogate biomarker for early screening of pulmonary hypertension in preterm infants with bronchopulmonary dysplasia. ( 29977013 )
2018
50
Coping, social support and information in patients with pulmonary arterial hypertension or chronic thromboembolic pulmonary hypertension: A 2-year retrospective cohort study. ( 29326818 )
2018

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:

37
# 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 9.56 BMPR2 EDN1 KCNK3 MIR20A MIR21 MIR22
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 ExPASy
19 FMA
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 LOVD
42 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
54 NINDS
55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 SNOMED-CT via Orphanet
71 TGDB
72 Tocris
73 UMLS
74 UMLS via Orphanet
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