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MCID: PLM037
MIFTS: 69

Pulmonary Hypertension

Categories: Blood diseases, Cardiovascular diseases, Genetic diseases, Rare diseases, Respiratory diseases
Genes Tissues Related diseases Publications Pathways Symptoms & Phenotypes Drugs
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Aliases & Classifications for Pulmonary Hypertension

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MalaCards integrated aliases for Pulmonary Hypertension:

Name: Pulmonary Hypertension 41 12 75 43 3 15 38 63 72
Primary Pulmonary Hypertension 41 12 75 15
Idiopathic Pulmonary Arterial Hypertension 72
Idiopathic Pulmonary Hypertension 72
Hypertension, Pulmonary 44
Hypertension Pulmonary 55

Classifications:

MalaCards categories:
Global: Genetic diseases Rare diseases
Anatomical: Cardiovascular diseases Respiratory diseases Blood diseases
See all MalaCards categories (disease lists)
ICD10: 33


External Ids:

Disease Ontology 12 DOID:14557 DOID:6432
ICD9CM 35 416.0
MeSH 44 D006976
NCIt 50 C3120 C97119
SNOMED-CT 68 26174007 70995007
ICD10 33 I27.0 I27.20
UMLS 72 C0020542 C0152171 C3203102
Sources

Summaries for Pulmonary Hypertension

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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 DREAM Repression and Dynorphin Expression and Human Embryonic Stem Cell Pluripotency. The drugs Nitric Oxide and Angiotensin II have been mentioned in the context of this disorder. Affiliated tissues include Lung, heart and lung, and related phenotypes are muscle and 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

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

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Related Diseases for Pulmonary Hypertension

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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 1072)
# Related Disease Score Top Affiliating Genes
1 pulmonary hypertension, primary, 1 35.5 PDE5A NPPB KCNK3 EDN1 BMPR2 ACVRL1
2 chronic thromboembolic pulmonary hypertension 35.4 NPPB BMPR2
3 pulmonary venoocclusive disease 34.4 KCNK3 BMPR2 ACVRL1
4 heritable pulmonary arterial hypertension 33.9 SMAD9 KCNK3 CAV1 BMPR2 ACVRL1
5 persistent fetal circulation syndrome 33.3 PDE5A NOS3 EDNRA EDN1
6 connective tissue disease 32.2 EDNRA EDN1 BMPR2 ACVRL1
7 pulmonary edema 32.1 NPPB NOS3 EDN1
8 sleep apnea 32.1 NPPB NOS3 EDN1
9 heart disease 32.1 PDE5A NPPB NOS3 EDNRA EDN1 BMPR2
10 vascular disease 32.0 NOS3 MIR21 EDN1 BMPR2 ACVRL1
11 portal hypertension 32.0 NOS3 EDNRA EDN1 BMPR2
12 congestive heart failure 31.7 NPPB MIR21 EDN1
13 hepatopulmonary syndrome 31.6 NOS3 EDN1 BMPR2 ACVRL1
14 chronic mountain sickness 31.5 NPPB NOS3 EDN1
15 impotence 31.5 PDE5A NOS3 EDN1
16 angina pectoris 31.3 NPPB NOS3 EDN1
17 chronic pulmonary heart disease 31.2 SMAD9 EDN1 BMPR2 ACVRL1
18 arteries, anomalies of 31.1 NPPB NOS3 EDN1 BMPR2 ACVRL1
19 central sleep apnea 31.1 NPPB NOS3
20 coronary artery anomaly 31.1 NPPB NOS3 EDN1
21 nonarteritic anterior ischemic optic neuropathy 30.6 PDE5A NOS3 EDN1
22 hypertension, essential 30.5 NPPB NOS3 EDNRA EDN1
23 coronary heart disease 1 30.4 NPPB NOS3 MIR17
24 myocardial infarction 30.1 PDE5A NPPB NOS3 MIR21 EDN1
25 leukemia, acute lymphoblastic 28.8 MIR451A MIR22 MIR21
26 psoriasis 28.3 MIR22 MIR21 MIR20A
27 hyperuricemia, pulmonary hypertension, renal failure, and alkalosis syndrome 12.8
28 pulmonary hypertension, primary, 2 12.7
29 pulmonary hypertension, primary, 3 12.7
30 pulmonary hypertension, neonatal 12.7
31 pulmonary hypertension, primary, 4 12.6
32 pulmonary hypertension, chronic thromboembolic, without deep vein thrombosis 12.6
33 pulmonary hypertension owing to lung disease and/or hypoxia 12.5
34 vater-like defects with pulmonary hypertension, laryngeal webs, and growth deficiency 12.4
35 pulmonary hypertension, primary, autosomal recessive 12.4
36 pulmonary hypertension with unclear multifactorial mechanism 12.4
37 rare pulmonary hypertension 12.4
38 syndrome with pulmonary hypertension as a major feature 12.2
39 alveolar capillary dysplasia with misalignment of pulmonary veins 12.2
40 lymphedema and cerebral arteriovenous anomaly 12.0
41 eisenmenger syndrome 11.9
42 pulmonary edema of mountaineers 11.9
43 pulmonary fibrosis, idiopathic 11.8
44 alveolar capillary dysplasia 11.7
45 patent ductus arteriosus 1 11.7
46 sickle cell disease 11.7
47 diaphragmatic hernia, congenital 11.7
48 cantu syndrome 11.7
49 hypoxia 11.6
50 pulmonary venoocclusive disease 2, autosomal recessive 11.6

Graphical network of the top 20 diseases related to Pulmonary Hypertension:



Diseases related to Pulmonary Hypertension
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Symptoms & Phenotypes for Pulmonary Hypertension

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UMLS symptoms related to Pulmonary Hypertension:


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

MGI Mouse Phenotypes related to Pulmonary Hypertension:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 muscle MP:0005369 9.7 ACVRL1 BMPR2 CAV1 EDN1 EDNRA NOS3
2 normal MP:0002873 9.56 ACVRL1 BMPR1B BMPR2 EDN1 EDNRA KCNK3
3 respiratory system MP:0005388 9.17 ACVRL1 BMPR2 CAV1 EDNRA KCNK3 NOS3
Sources

Drugs & Therapeutics for Pulmonary Hypertension

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PubMed Health treatment related to Pulmonary Hypertension: 63

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

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

(show top 50) (show all 499)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Nitric Oxide Approved Phase 4 10102-43-9 145068
2
Angiotensin II Approved, Investigational Phase 4 11128-99-7, 68521-88-0, 4474-91-3 172198
3
Telmisartan Approved, Investigational Phase 4 144701-48-4 65999
4
Hydrochlorothiazide Approved, Vet_approved Phase 4 58-93-5 3639
5
Zinc Approved, Investigational Phase 4 7440-66-6 32051
6
Ranolazine Approved, Investigational Phase 4 142387-99-3, 95635-55-5 56959
7
Isoflurane Approved, Vet_approved Phase 4 26675-46-7 3763
8
Adenosine Approved, Investigational Phase 4 58-61-7 60961
9
Silver sulfadiazine Approved, Vet_approved Phase 4 22199-08-2 441244
10
Clevidipine Approved, Investigational Phase 4 167221-71-8
11
Dopamine Approved Phase 4 51-61-6, 62-31-7 681
12
Methazolamide Approved Phase 4 554-57-4 4100
13
Dobutamine Approved Phase 4 34368-04-2 36811
14
Magnesium oxide Approved Phase 4 1309-48-4 14792
15
Norepinephrine Approved Phase 4 51-41-2 439260
16
Riociguat Approved Phase 4 625115-55-1
17
Nebivolol Approved, Investigational Phase 4 152520-56-4, 118457-14-0, 99200-09-6 71301
18
Bupivacaine Approved, Investigational Phase 4 2180-92-9, 38396-39-3 2474
19
Ceftaroline fosamil Approved, Investigational Phase 4 229016-73-3
20
Sevoflurane Approved, Vet_approved Phase 4 28523-86-6 5206
21
Macitentan Approved Phase 4 441798-33-0
22
Epoprostenol Approved Phase 4 35121-78-9, 61849-14-7 5282411 5280427
23
Spironolactone Approved Phase 4 1952-01-7, 52-01-7 5833
24
Acetazolamide Approved, Vet_approved Phase 4 59-66-5 1986
25
Lidocaine Approved, Vet_approved Phase 4 137-58-6 3676
26
Tocopherol Approved, Investigational Phase 4 1406-66-2, 54-28-4 14986
27
Sorbitol Approved Phase 4 50-70-4 5780
28 Coconut Approved Phase 4
29
Vitamin E Approved, Nutraceutical, Vet_approved Phase 4 59-02-9 14985
30 Simendan Investigational Phase 4 131741-08-7
31
Lactitol Investigational Phase 4 585-86-4, 585-88-6 493591
32 Tocotrienol Investigational Phase 4 6829-55-6
33 Adrenergic Agonists Phase 4
34 Adrenergic beta-Agonists Phase 4
35 Vardenafil Dihydrochloride Phase 4
36 Angiotensinogen Phase 4
37 Angiotensin II Type 1 Receptor Blockers Phase 4
38 Giapreza Phase 4
39 Angiotensin Receptor Antagonists Phase 4
40 Sodium Chloride Symporter Inhibitors Phase 4
41 insulin Phase 4
42 Insulin, Globin Zinc Phase 4
43 Cardiotonic Agents Phase 4
44 Phosphodiesterase 3 Inhibitors Phase 4
45 Parasympatholytics Phase 4
46 Cholinergic Agents Phase 4
47 Cholinergic Antagonists Phase 4
48 Performance-Enhancing Substances Phase 4
49 Tissue Plasminogen Activator Phase 4
50 Plasminogen Phase 4

Interventional clinical trials:

(show top 50) (show all 1425)
# Name Status NCT ID Phase Drugs
1 Intravenous Iron Treatment In Iron Deficient Patients With Idiopathic Pulmonary Arterial Hypertension Unknown status NCT01288651 Phase 4 Ferricarboxymaltose
2 Inhaled Iloprost for Disproportionate Pulmonary Hypertension in Chronic Obstructive Pulmonary Disease Unknown status NCT01116063 Phase 4 Inhaled iloprost
3 Hemodynamic Evaluation of Patients With Pulmonary Arterial Hypertension. Response to Sildenafil Treatment Unknown status NCT00483626 Phase 4 oral sildenafil
4 Effects of Spironolactone on Collagen Metabolism in Pulmonary Arterial Hypertension Unknown status NCT01468571 Phase 4 Spironolactone;Placebo
5 A Double Blind, Placebo Controlled Trial of Oral Riociguat for Sarcoidosis Associated Pulmonary Hypertension Unknown status NCT02625558 Phase 4 Riociguat;Placebo
6 Acute Effect of Recombinant Human Brain Natriuretic Peptide in Patients With Pulmonary Hypertension Associated With Acute Exacerbation of Chronic Pulmonary Disease Unknown status NCT02742909 Phase 4 rhBNP;placebo
7 Comparison of Efficacy Different Treatment Regimens in Pulmonary Hypertension Secondary to Lung Disease and or Hypoxia Unknown status NCT01449253 Phase 4 Sildenafil;Bosentan
8 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 Hypotonic Treprostinil Subcutaneous Infusion for Control of Treprostinil Related Site Pain Unknown status NCT01615627 Phase 4 HypotonicTreprostinil Solution;Eutonic Treprostinil Solution
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 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
12 Auto-PAP for Pulmonary Hypertension Treatment in Decompensated Heart Failure Patients With Obstructive Sleep Apnea: A Two Center Pilot Study Unknown status NCT02963597 Phase 4
13 Vardenafil as add-on Therapy for Patients With Pulmonary Hypertension Treated With Inhaled Iloprost Unknown status NCT01649739 Phase 4 Levitra
14 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
15 Low Dose Prolonged Infusion of Tissue Type Plasminogen Activator Therapy in Massive Pulmonary Embolism: AYKAN Trial Unknown status NCT02029456 Phase 4 25 mg Actilyse ( Boehringer Ingelheim, Germany) infusion in 6 hours
16 Evaluation of Sildenafil for the Treatment of Moderate Congestive Heart Failure Unknown status NCT00793338 Phase 4 Sildenafil
17 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
18 The Effect of Far Infrared (FIR) Therapy on Access Flow of Arteriovenous (AV) Fistula, Echocardiographic Parameters and Endothelial Function in Patients With End Stage Renal Disease Unknown status NCT01138254 Phase 4
19 Comparative Study of the Effects of Telmisartan and Nebivolol on 24-h Ambulatory Blood Pressure and Arterial Stiffness in Patients With Arterial Hypertension Unknown status NCT02057328 Phase 4 TELMISARTAN;NEBIVOLOL
20 Open Label, Non Comparative Study to Investigate the Effect of Bosentan on Pulmonary Artery Remodelling in Pulmonary Arterial Hypertension (PAH). Completed NCT00595049 Phase 4 bosentan
21 Phase IV Study of Chronic Infusional Epoprostenol for Severe Primary Pulmonary Hypertension Completed NCT00004754 Phase 4 epoprostenol
22 Inhaled Iloprost for Sarcoidosis Associated Pulmonary Hypertension Completed NCT00403650 Phase 4 Iloprost
23 A Phase IV, Prospective, Single-Arm, Open-Label Study to Measure Outcomes in Patients With Pulmonary Arterial Hypertension Not on Active Treatment Completed NCT02191137 Phase 4 Riociguat (Adempas, BAY63-2521)
24 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®
25 Safely Change From Bosentan to Ambrisentan in Pulmonary Hypertension Completed NCT01330108 Phase 4 ambrisentan
26 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
27 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®
28 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
29 Randomised Cross-over Pilot Study to Determine the Effects of Isoflurane and Propofol on Pulmonary Vascular Resistance in Children With Pulmonary Hypertension. Completed NCT01212523 Phase 4 propofol and isoflurane
30 Effects of Combination of Bosentan and Sildenafil Versus Sildenafil Monotherapy on Morbidity and Mortality in Symptomatic Patients With Pulmonary Arterial Hypertension - A Multicenter, Double-blind, Randomized, Placebo-controlled, Parallel Group, Prospective, Event Driven Phase IV Study Completed NCT00303459 Phase 4 bosentan;placebo
31 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
32 Echo Study - Characterization of LV Strain Patterns in Patients With Mildly Elevated PCWP and Pulmonary Hypertension Completed NCT01800292 Phase 4 sildenafil
33 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
34 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
35 TRACLEER® (Bosentan) Pulmonary Arterial Hypertension A Multicenter, Open-label, Single-arm Safety Study to Investigate the Effects of Chronic TRACLEER® Treatment on Testicular Function in Male Patients With Pulmonary Arterial Hypertension Completed NCT00082186 Phase 4 bosentan
36 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
37 Vasoreactivity Testing With Intravenous Sildenafil in Patients With Precapillary Pulmonary Hypertension (Treatment Optimisation Study) Completed NCT01889966 Phase 4 Sildenafil
38 Efficacy of Beraprost in Lowering Pulmonary Arterial Pressure in Pulmonary Arterial Hypertension Children Associated With Left to Right Shunt Congenital Heart Defect Completed NCT03431649 Phase 4 Beraprost Sodium;Sildenafil Citrate
39 A Single-arm, Open Label Study Evaluating the Impact on Lifestyle of a New Thermo Stable Formulation of FLOLAN® in Subjects With Pulmonary Arterial Hypertension (PAH). (FLOLAN® is a Registered Trademark of the GlaxoSmithKline Group of Companies.) Completed NCT01462565 Phase 4 current marketed FLOLAN (epoprostenol sodium);new thermo stable formulation of epoprostenol sodium
40 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
41 Therapy of Pulmonary Arterial Hypertension (PAH) With Bosentan in Patients With Eisenmenger Syndrome Completed NCT00266162 Phase 4 Bosentan administration
42 The Effect of Riociguat on Gas Exchange, Exercise Performance, and Pulmonary Artery Pressure During Acute Altitude Exposure Completed NCT02024386 Phase 4 Riociguat
43 Sildenafil for Improving Outcomes After Valvular Correction Completed NCT00862043 Phase 4 Sildenafil Citrate;Placebo
44 Natrecor® (Nesiritide) as a Nitric Oxide Sparing Agent in Patients Undergoing Lung Transplantation Completed NCT00205426 Phase 4 Natrecor
45 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
46 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
47 CombinatiON Up-FRON t Therapy for PAH - A Phase 4, Randomized, Multicenter Study of Inhaled Treprostinil in Treatment naïve Pulmonary Arterial Hypertension Patients Starting on Tadalafil Completed NCT01305252 Phase 4 treprostinil inhalations;tadalafil
48 Exercise Induced Pulmonary Hypertension in Systemic Sclerosis and Treatment With Ambrisentan: A Prospective Single Center, Open Label, Pilot Study Completed NCT01051960 Phase 4 Ambrisentan
49 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
50 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

Search NIH Clinical Center for Pulmonary Hypertension

Inferred drug relations via UMLS 72 / NDF-RT 51 :


Alprostadil
bosentan
Diltiazem
Epoprostenol
Epoprostenol Sodium
Hydralazine
Hydralazine Hydrochloride
Nifedipine
Nitroglycerin
Treprostinil

Cell-based therapeutics:


LifeMap Discovery
Data from LifeMap, the Embryonic Development and Stem Cells Database
Read about Pulmonary Hypertension cell therapies at LifeMap Discovery.
Stem-cell-based therapeutic approaches for Pulmonary Hypertension:
Endothelial progenitor cells for pulmonary hypertension
Endothelial progenitors for treatment of idiopathic pulmonary arterial hypertension
Embryonic/Adult Cultured Cells Related to Pulmonary Hypertension:
eNOS-transfected endothelial progenitor cells PMIDs: 21653527 20117135
eNOS-transfected endothelial progenitor cells PMIDs: 21653527 20117135
Peripheral blood-derived endothelial progenitor cells PMIDs: 17418297

Cochrane evidence based reviews: hypertension, pulmonary

Sources

Genetic Tests for Pulmonary Hypertension

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Sources

Anatomical Context for Pulmonary Hypertension

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MalaCards organs/tissues related to Pulmonary Hypertension:

41
Heart, Lung, Endothelial, Smooth Muscle, Testes, 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
Sources

Publications for Pulmonary Hypertension

About this section

Articles related to Pulmonary Hypertension:

(show top 50) (show all 30183)
# Title Authors PMID Year
1
Interleukin-6 modulates the expression of the bone morphogenic protein receptor type II through a novel STAT3-microRNA cluster 17/92 pathway. 9 38 88
19390056 2009
2
Retrospective observational analysis of hospital discharge database to characterize primary pulmonary hypertension and its outcomes in Spain from 2004 to 2015. 38 17
31045844 2019
3
Pulmonary Hypertension. 38 17
30955510 2019
4
Effect of beraprost on pulmonary hypertension due to left ventricular systolic dysfunction. 38 17
31008926 2019
5
Susceptibility to chronic thromboembolic pulmonary hypertension may be conferred by miR-759 via its targeted interaction with polymorphic fibrinogen alpha gene. 38 88
20677013 2010
6
Dynamic changes in lung microRNA profiles during the development of pulmonary hypertension due to chronic hypoxia and monocrotaline. 38 88
20110569 2010
7
Emerging therapies for the treatment of pulmonary hypertension. 9 38
20216170 2010
8
Transcripts from a novel BMPR2 termination mutation escape nonsense mediated decay by downstream translation re-initiation: implications for treating pulmonary hypertension. 9 38
20095988 2010
9
Placenta growth factor in sickle cell disease: association with hemolysis and inflammation. 9 38
20040765 2010
10
Phosphodiesterase type 5 inhibitors for high-altitude pulmonary hypertension: a meta-analysis. 9 38
20225909 2010
11
Pulmonary hemodynamic response to acute combination and monotherapy with sildenafil and brain natriuretic peptide in rats with monocrotaline-induced pulmonary hypertension. 9 38
19996941 2010
12
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. 9 38
19844076 2010
13
B-type natriuretic peptide utilization as an adjunct to management in a case of conjoined twins with pulmonary hypertension. 9 38
20085871 2010
14
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. 9 38
19556740 2010
15
Dichloroacetate treatment partially regresses established pulmonary hypertension in mice with SM22alpha-targeted overexpression of the serotonin transporter. 9 38
19679640 2009
16
Brain natriuretic peptide in pulmonary arterial hypertension: biomarker and potential therapeutic agent. 9 38
20054445 2009
17
Interaction between bone morphogenetic proteins and endothelin-1 in human pulmonary artery smooth muscle. 9 38
19786120 2009
18
Nitric oxide synthases in infants and children with pulmonary hypertension and congenital heart disease. 9 38
19912632 2009
19
Soluble guanylate cyclase agonists inhibit expression and procoagulant activity of tissue factor. 9 38
19592462 2009
20
A novel insight into the mechanism of pulmonary hypertension involving caveolin-1 deficiency and endothelial nitric oxide synthase activation. 9 38
20382348 2009
21
Brain natriuretic peptide in chronic thromboembolic pulmonary hypertension. 9 38
19468826 2009
22
Expression of vasoactive intestinal peptide and related receptors in overcirculation-induced pulmonary hypertension in piglets. 9 38
19581838 2009
23
Chronic treatment with sildenafil stimulates Leydig cell and testosterone secretion. 9 38
19659904 2009
24
Endothelin-1 across the lung circulation in patients with pulmonary arterial hypertension and influence of epoprostenol infusion. 9 38
19632577 2009
25
Persistent eNOS activation secondary to caveolin-1 deficiency induces pulmonary hypertension in mice and humans through PKG nitration. 9 38
19487814 2009
26
Intratracheal gene transfer of adrenomedullin using polyplex nanomicelles attenuates monocrotaline-induced pulmonary hypertension in rats. 9 38
19337232 2009
27
Effects of roflumilast, a phosphodiesterase-4 inhibitor, on hypoxia- and monocrotaline-induced pulmonary hypertension in rats. 9 38
19386793 2009
28
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. 9 38
18793807 2009
29
RhoA and Rho kinase activation in human pulmonary hypertension: role of 5-HT signaling. 9 38
19299501 2009
30
Prognostic value of B-type natriuretic peptide in children with pulmonary hypertension. 9 38
18599134 2009
31
Phosphodiesterase 5 inhibition in heart failure: mechanisms and clinical implications. 9 38
19377497 2009
32
Inhibition of cGMP phosphodiesterase 5 suppresses serotonin signalling in pulmonary artery smooth muscles cells. 9 38
19416631 2009
33
Discovery of riociguat (BAY 63-2521): a potent, oral stimulator of soluble guanylate cyclase for the treatment of pulmonary hypertension. 9 38
19263460 2009
34
Caveolin-1 Expression and Hemodynamics in COPD Patients. 9 38
19572028 2009
35
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. 9 38
18682983 2009
36
Brain natriuretic peptide as a preclinical marker of chronic pulmonary hypertension in patients with pulmonary embolism. 9 38
19247590 2009
37
Novel approaches to the pharmacotherapy of pulmonary arterial hypertension. 9 38
19121410 2009
38
Combined use of PDE5 inhibitors and nitrates in the treatment of pulmonary arterial hypertension in patients with heart failure. 9 38
19181291 2009
39
Endothelin-1 inhibition by ambrisentan as a potential treatment adjunct after debulking surgery in epithelial ovarian cancer. 9 38
19544975 2009
40
[Endothelin-1 and brain natriuretic peptide in the development of pulmonary hypertension in interstitial lung diseases]. 9 38
20481052 2009
41
Role of nitric oxide in shock: the large animal perspective. 9 38
19273179 2009
42
Hypoxic upregulation of preproendothelin-1 gene expression is associated with protein tyrosine kinase-PI3K signaling in cultured lung vascular endothelial cells. 9 38
19436835 2009
43
NO-independent, haem-dependent soluble guanylate cyclase stimulators. 9 38
19089334 2009
44
[Estimation of plasmic concentration of the brain natriuretic peptide in interstitial pulmonary diseases with pulmonary hypertension: clinical role]. 9 38
19459423 2009
45
Superoxide dismutase restores eNOS expression and function in resistance pulmonary arteries from neonatal lambs with persistent pulmonary hypertension. 9 38
18790993 2008
46
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. 9 38
18567602 2008
47
Inhalation of vasoactive intestinal peptide in pulmonary hypertension. 9 38
18978135 2008
48
["Pulmonary hypertension" of the gastrointestinal tract in patients with systemic sclerosis: thoughts on a not (yet) existing disease entity]. 9 38
18814097 2008
49
Differential proinflammatory and prooxidant effects of bone morphogenetic protein-4 in coronary and pulmonary arterial endothelial cells. 9 38
18539760 2008
50
Inflammation, endothelial injury, and persistent pulmonary hypertension in heterozygous BMPR2-mutant mice. 9 38
18552156 2008
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Variations for Pulmonary Hypertension

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Expression for Pulmonary Hypertension

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Search GEO for disease gene expression data for Pulmonary Hypertension.
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Pathways for Pulmonary Hypertension

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

(show all 11)
# Super pathways Score Top Affiliating Genes
1
DREAM Repression and Dynorphin Expression 64
Show member pathways
 12.39 SMAD9 BMPR2 BMPR1B ACVRL1
2
Human Embryonic Stem Cell Pluripotency 64
Show member pathways
 12.22 SMAD9 NPPB BMPR2 BMPR1B
3
MicroRNAs in cancer 37
12.21 MIR451A MIR21 MIR20A MIR17 BMPR2
4
Fluid shear stress and atherosclerosis 37
11.7 NOS3 EDN1 CAV1 BMPR2 BMPR1B
5
TGF-beta signaling pathway (KEGG) 37
11.58 SMAD9 BMPR2 BMPR1B
6
Signaling by BMP 66
Show member pathways
 11.4 SMAD9 BMPR2 BMPR1B
7
Endothelin Pathways 1
10.96 NOS3 EDNRA EDN1
8
ALK1 signaling events 1
Show member pathways
 10.69 SMAD9 CAV1 BMPR2 ACVRL1
9
NO-dependent CFTR activation (normal and CF) 22
10.57 PDE5A NOS3
10
ALK2 signaling events 1
10.45 SMAD9 BMPR2
11
Cardiomyocyte Differentiation through BMP Receptors 64
10.27 SMAD9 NPPB BMPR2 BMPR1B
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GO Terms for Pulmonary Hypertension

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

# Name GO ID Score Top Affiliating Genes
1 extracellular space GO:0005615 9.23 NPPB MIR451A MIR22 MIR21 MIR20A MIR17
2 caveola GO:0005901 9.13 NOS3 CAV1 BMPR2

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

(show top 50) (show all 61)
# Name GO ID Score Top Affiliating Genes
1 in utero embryonic development GO:0001701 9.95 NOS3 EDNRA EDN1 ACVRL1
2 negative regulation of gene expression GO:0010629 9.93 MIR21 MIR20A MIR17 EDN1 ACVRL1
3 cellular response to hypoxia GO:0071456 9.9 MIR17 KCNK3 EDN1
4 negative regulation of cell growth GO:0030308 9.9 NPPB BMPR2 ACVRL1
5 response to hypoxia GO:0001666 9.9 EDNRA EDN1 CAV1 ACVRL1
6 positive regulation of angiogenesis GO:0045766 9.88 NOS3 MIR21 MIR20A ACVRL1
7 miRNA mediated inhibition of translation GO:0035278 9.86 MIR21 MIR20A MIR17
8 pattern specification process GO:0007389 9.83 BMPR2 BMPR1B ACVRL1
9 cellular response to transforming growth factor beta stimulus GO:0071560 9.82 EDN1 CAV1 ACVRL1
10 positive regulation of osteoblast differentiation GO:0045669 9.8 MIR21 BMPR2 BMPR1B
11 outflow tract morphogenesis GO:0003151 9.78 MIR20A MIR17 BMPR2
12 positive regulation of endothelial cell migration GO:0010595 9.77 MIR21 EDN1 BMPR2
13 negative regulation of endothelial cell migration GO:0010596 9.73 MIR21 ACVRL1
14 membrane depolarization GO:0051899 9.73 EDN1 CAV1
15 positive regulation of chondrocyte differentiation GO:0032332 9.73 BMPR1B ACVRL1
16 vasodilation GO:0042311 9.72 NOS3 CPS1
17 regulation of nitric-oxide synthase activity GO:0050999 9.72 NOS3 CAV1
18 cellular response to peptide hormone stimulus GO:0071375 9.72 EDN1 CAV1
19 blood vessel remodeling GO:0001974 9.72 NOS3 BMPR2 ACVRL1
20 positive regulation of endothelial cell differentiation GO:0045603 9.71 MIR21 ACVRL1
21 positive regulation of cytokine production involved in inflammatory response GO:1900017 9.71 MIR21 MIR17
22 endocardial cushion morphogenesis GO:0003203 9.71 NOS3 ACVRL1
23 chondrocyte development GO:0002063 9.71 BMPR2 BMPR1B
24 activin receptor signaling pathway GO:0032924 9.71 BMPR2 ACVRL1
25 positive regulation of vascular smooth muscle cell proliferation GO:1904707 9.71 MIR21 MIR20A MIR17 EDN1
26 positive regulation of cartilage development GO:0061036 9.7 BMPR2 BMPR1B
27 proteoglycan biosynthetic process GO:0030166 9.7 BMPR2 BMPR1B
28 positive regulation of urine volume GO:0035810 9.7 NPPB EDN1
29 positive regulation of renal sodium excretion GO:0035815 9.69 NPPB EDN1
30 negative regulation of DNA biosynthetic process GO:2000279 9.69 BMPR2 ACVRL1
31 response to dexamethasone GO:0071548 9.69 EDN1 CPS1
32 positive regulation of vascular associated smooth muscle cell migration GO:1904754 9.69 MIR451A MIR21 MIR20A
33 lymphangiogenesis GO:0001946 9.68 BMPR2 ACVRL1
34 positive regulation of cardiac muscle hypertrophy in response to stress GO:1903244 9.68 MIR20A MIR17
35 negative regulation of systemic arterial blood pressure GO:0003085 9.68 MIR17 BMPR2
36 artery smooth muscle contraction GO:0014824 9.67 EDNRA EDN1
37 positive regulation of metalloendopeptidase activity GO:1904685 9.67 MIR21 MIR17
38 negative regulation of smooth muscle cell apoptotic process GO:0034392 9.67 MIR21 EDN1
39 positive regulation of cardiac muscle hypertrophy GO:0010613 9.67 PDE5A MIR21 EDN1
40 negative regulation of endothelial cell proliferation GO:0001937 9.67 MIR22 MIR21 CAV1 ACVRL1
41 body fluid secretion GO:0007589 9.66 NPPB EDN1
42 retina vasculature development in camera-type eye GO:0061298 9.65 BMPR2 ACVRL1
43 artery development GO:0060840 9.65 BMPR2 ACVRL1
44 transmembrane receptor protein serine/threonine kinase signaling pathway GO:0007178 9.65 BMPR2 BMPR1B ACVRL1
45 transforming growth factor beta receptor signaling pathway GO:0007179 9.65 SMAD9 MIR21 BMPR2 BMPR1B ACVRL1
46 regulation of glucose transmembrane transport GO:0010827 9.64 EDNRA EDN1
47 negative regulation of vascular associated smooth muscle cell apoptotic process GO:1905460 9.63 MIR21 MIR17
48 endothelial tube morphogenesis GO:0061154 9.63 MIR21 ACVRL1
49 regulation of blood vessel size GO:0050880 9.63 NPPB NOS3 EDN1
50 regulation of systemic arterial blood pressure by endothelin GO:0003100 9.62 NOS3 EDN1

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 9.71 MIR22 MIR21 MIR20A MIR17
2 SMAD binding GO:0046332 9.43 BMPR2 BMPR1B ACVRL1
3 transforming growth factor beta receptor activity, type I GO:0005025 9.37 BMPR1B ACVRL1
4 growth factor binding GO:0019838 9.33 BMPR2 BMPR1B ACVRL1
5 BMP receptor activity GO:0098821 9.32 BMPR2 ACVRL1
6 transforming growth factor beta-activated receptor activity GO:0005024 9.13 BMPR2 BMPR1B ACVRL1
7 transmembrane receptor protein serine/threonine kinase activity GO:0004675 8.8 BMPR2 BMPR1B ACVRL1
Sources

Sources for Pulmonary Hypertension

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3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 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 TGDB
71 Tocris
72 UMLS
73 UMLS via Orphanet
The MalaCards human disease database index: 1-9 A B C D E F G H I J K L M N O P Q R S T U V W X Y Z
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