MCID: PLM037
MIFTS: 68

Pulmonary Hypertension

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

Aliases & Classifications for Pulmonary Hypertension

MalaCards integrated aliases for Pulmonary Hypertension:

Name: Pulmonary Hypertension 40 12 74 42 3 15 37 62 71
Primary Pulmonary Hypertension 40 12 74 15
Idiopathic Pulmonary Arterial Hypertension 71
Idiopathic Pulmonary Hypertension 71
Hypertension, Pulmonary 43
Hypertension Pulmonary 54

Classifications:



External Ids:

Disease Ontology 12 DOID:14557 DOID:6432
ICD9CM 34 416.0
MeSH 43 D006976
NCIt 49 C3120 C97119
SNOMED-CT 67 26174007 70995007
ICD10 32 I27.0 I27.20
UMLS 71 C0020542 C0152171 C3203102

Summaries for Pulmonary Hypertension

MedlinePlus : 42 Pulmonary hypertension (PH) is high blood pressure in the arteries to your lungs. It is a serious condition. If you have it, the blood vessels that carry blood from your heart to your lungs become hard and narrow. Your heart has to work harder to pump the blood through. Over time, your heart weakens and cannot do its job and you can develop heart failure. Symptoms of PH include Shortness of breath during routine activity, such as climbing two flights of stairs Tiredness Chest pain A racing heartbeat Pain on the upper right side of the abdomen Decreased appetite As PH worsens, you may find it hard to do any physical activities. There are two main kinds of PH. One runs in families or appears for no known reason. The other kind is related to another condition, usually heart or lung disease. There is no cure for PH. Treatments can control symptoms. They involve treating the heart or lung disease, medicines, oxygen, and sometimes lung transplantation. NIH: National Heart, Lung, and Blood Institute

MalaCards based summary : Pulmonary Hypertension, also known as primary pulmonary hypertension, is related to pulmonary hypertension, primary, 1 and chronic thromboembolic pulmonary hypertension, and has symptoms including 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 MicroRNAs in cancer. The drugs Nitric Oxide and Iloprost have been mentioned in the context of this disorder. Affiliated tissues include Lung, heart and endothelial, 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 : 62 About pulmonary hypertension: Pulmonary hypertension (PULL-mun-ary HI-per-TEN-shun), or PH, is increased pressure in the pulmonary arteries. These arteries carry blood from your heart to your lungs to pick up oxygen. PH causes symptoms such as shortness of breath during routine activity (for example, climbing two flights of stairs), tiredness, chest pain, and a racing heartbeat. As the condition worsens, its symptoms may limit all physical activity.

Wikipedia : 74 Pulmonary hypertension (PH or PHTN) is a condition of increased blood pressure within the arteries of... more...

Related Diseases for Pulmonary Hypertension

Diseases in the Pulmonary Hypertension family:

Pulmonary Hypertension, Primary, 1 Pulmonary Hypertension, Primary, Autosomal Recessive
Pulmonary Hypertension, Primary, 2 Pulmonary Hypertension, Primary, 3
Pulmonary Hypertension, Primary, 4 Rare Pulmonary Hypertension

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

(show top 50) (show all 1106)
# Related Disease Score Top Affiliating Genes
1 pulmonary hypertension, primary, 1 35.8 KCNK3 EDN1 BMPR2 ACVRL1
2 chronic thromboembolic pulmonary hypertension 35.3 EDN1 BMPR2
3 pulmonary venoocclusive disease 34.3 SMAD9 PDE5A KCNK3 EDN1 CAV1 BMPR2
4 heritable pulmonary arterial hypertension 34.0 SMAD9 KCNK3 CAV1 BMPR2 BMPR1B ACVRL1
5 eisenmenger syndrome 33.8 EDN1 BMPR2
6 diaphragmatic hernia, congenital 33.7 PDE5A NOS3 EDNRA EDN1 BMPR2
7 persistent fetal circulation syndrome 33.5 PDE5A NOS3 EDNRA EDN1
8 pulmonary fibrosis, idiopathic 33.5 SMAD4 MIR21 EDN1 CAV1
9 pulmonary valve insufficiency 33.0 EDN1 BMPR2
10 pulmonary arterial hypertension associated with congenital heart disease 32.7 SMAD9 CAV1 BMPR2 BMPR1B
11 tricuspid valve insufficiency 32.5 PDE5A EDN1 BMPR2
12 vascular disease 32.5 NOS3 MIR21 EDN1 BMPR2 ACVRL1
13 congestive heart failure 32.4 PDE5A NOS3 EDNRA EDN1
14 heart disease 32.3 SMAD9 SMAD4 PDE5A NOS3 MIR21 EDNRA
15 portal hypertension 32.2 NOS3 EDNRA EDN1 BMPR2
16 atrial heart septal defect 32.1 SMAD9 EDN1 BMPR2
17 meconium aspiration syndrome 32.0 PDE5A EDN1 CPS1
18 raynaud phenomenon 32.0 PDE5A EDN1
19 telangiectasis 31.9 SMAD4 BMPR2 ACVRL1
20 arteriovenous malformation 31.8 EDN1 BMPR2 ACVRL1
21 hereditary hemorrhagic telangiectasia 31.7 SMAD4 NOS3 BMPR2 ACVRL1
22 hepatopulmonary syndrome 31.6 NOS3 EDNRA EDN1 BMPR2 ACVRL1
23 impotence 31.5 PDE5A NOS3 EDNRA EDN1
24 limited scleroderma 31.4 PDE5A EDNRA EDN1 CAV1 BMPR2
25 chronic mountain sickness 31.2 NOS3 EDN1
26 connective tissue disease 31.2 MIR22 MIR21 MIR20A MIR17 EDNRA EDN1
27 arteriosclerosis 31.2 NOS3 MIR21 EDNRA EDN1
28 raynaud disease 31.1 PDE5A EDNRA EDN1
29 chronic pulmonary heart disease 31.1 SMAD9 PDE5A NOS3 EDNRA EDN1 BMPR2
30 aortic valve disease 2 31.1 NOS3 MIR21 MIR17
31 tricuspid valve disease 30.7 PDE5A EDN1 BMPR2
32 nonarteritic anterior ischemic optic neuropathy 30.6 PDE5A NOS3 EDN1
33 cardiovascular system disease 30.5 PDE5A NOS3 MIR21 MIR17 EDNRA EDN1
34 migraine with or without aura 1 30.5 PDE5A NOS3 EDNRA EDN1
35 body mass index quantitative trait locus 11 30.4 PDE5A NOS3 MIR22 MIR21 MIR17 EDNRA
36 hypertension, essential 30.4 PDE5A NOS3 MIR21 MIR17 KCNK3 EDNRA
37 arteries, anomalies of 30.3 NOS3 MIR22 MIR21 MIR17 EDN1
38 rectum cancer 29.9 MIR21 MIR20A MIR17
39 intestinal disease 29.4 MIR22 MIR21 MIR20A MIR17
40 central nervous system disease 29.4 MIR22 MIR21 MIR20A MIR17
41 nervous system disease 29.4 MIR22 MIR21 MIR20A MIR17
42 hyperuricemia, pulmonary hypertension, renal failure, and alkalosis syndrome 12.8
43 pulmonary hypertension, primary, 2 12.7
44 pulmonary hypertension, primary, 3 12.7
45 pulmonary hypertension, neonatal 12.7
46 pulmonary hypertension, primary, 4 12.6
47 pulmonary hypertension, chronic thromboembolic, without deep vein thrombosis 12.6
48 pulmonary hypertension owing to lung disease and/or hypoxia 12.5
49 vater-like defects with pulmonary hypertension, laryngeal webs, and growth deficiency 12.4
50 pulmonary hypertension, primary, autosomal recessive 12.4

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, chest pain, hemoptysis, snoring, coughing

MGI Mouse Phenotypes related to Pulmonary Hypertension:

45
# Description MGI Source Accession Score Top Affiliating Genes
1 muscle MP:0005369 9.86 ACVRL1 BMPR2 CAV1 EDN1 EDNRA NOS3
2 normal MP:0002873 9.85 ACVRL1 BMPR1B BMPR2 EDN1 EDNRA KCNK3
3 renal/urinary system MP:0005367 9.5 ACVRL1 BMPR2 CAV1 EDN1 EDNRA NOS3
4 respiratory system MP:0005388 9.17 ACVRL1 BMPR2 CAV1 EDNRA KCNK3 NOS3

Drugs & Therapeutics for Pulmonary Hypertension

PubMed Health treatment related to Pulmonary Hypertension: 62

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

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

(show top 50) (show all 428)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Nitric Oxide Approved Phase 4 10102-43-9 145068
2
Iloprost Approved, Investigational Phase 4 78919-13-8 6443959
3
Angiotensin II Approved, Investigational Phase 4 68521-88-0, 4474-91-3, 11128-99-7 172198
4
Telmisartan Approved, Investigational Phase 4 144701-48-4 65999
5
Hydrochlorothiazide Approved, Vet_approved Phase 4 58-93-5 3639
6
Isoflurane Approved, Vet_approved Phase 4 26675-46-7 3763
7
Zinc Approved, Investigational Phase 4 7440-66-6 32051
8
Ranolazine Approved, Investigational Phase 4 95635-55-5, 142387-99-3 56959
9
Adenosine Approved, Investigational Phase 4 58-61-7 60961
10
Silver sulfadiazine Approved, Vet_approved Phase 4 22199-08-2 441244
11
Clevidipine Approved, Investigational Phase 4 167221-71-8
12
Dopamine Approved Phase 4 51-61-6, 62-31-7 681
13
Methazolamide Approved Phase 4 554-57-4 4100
14
Dobutamine Approved Phase 4 34368-04-2 36811
15
Magnesium oxide Approved Phase 4 1309-48-4 14792
16
Norepinephrine Approved Phase 4 51-41-2 439260
17
Nebivolol Approved, Investigational Phase 4 152520-56-4, 118457-14-0, 99200-09-6 71301
18
Bupivacaine Approved, Investigational Phase 4 38396-39-3, 2180-92-9 2474
19
Ceftaroline fosamil Approved, Investigational Phase 4 229016-73-3
20
Riociguat Approved Phase 4 625115-55-1
21
Selexipag Approved Phase 4 475086-01-2
22
Sevoflurane Approved, Vet_approved Phase 4 28523-86-6 5206
23
Macitentan Approved Phase 4 441798-33-0
24
Epoprostenol Approved Phase 4 61849-14-7, 35121-78-9 5280427 5282411
25
Acetazolamide Approved, Vet_approved Phase 4 59-66-5 1986
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 Angiotensin II Type 1 Receptor Blockers Phase 4
36 Angiotensin Receptor Antagonists Phase 4
37 Giapreza Phase 4
38 Angiotensinogen Phase 4
39 Vardenafil Dihydrochloride Phase 4
40 Sodium Chloride Symporter Inhibitors Phase 4
41 Neurotransmitter Agents Phase 4
42 Anti-Asthmatic Agents Phase 4
43 Respiratory System Agents Phase 4
44 Autonomic Agents Phase 4
45 Bronchodilator Agents Phase 4
46 Protective Agents Phase 4
47 Phosphodiesterase 3 Inhibitors Phase 4
48 Cholinergic Agents Phase 4
49 Parasympatholytics Phase 4
50 Cholinergic Antagonists Phase 4

Interventional clinical trials:

(show top 50) (show all 1423)
# 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 Patients With Pulmonary Hypertension or Interstitial Lung Disease Travelling to Altitude - Effect of Nocturnal Oxygen Therapy on Breathing and Sleep Unknown status NCT02150616 Phase 4 Oxygen;Sham oxygen (room air)
5 A Double Blind, Placebo Controlled Trial of Oral Riociguat for Sarcoidosis Associated Pulmonary Hypertension Unknown status NCT02625558 Phase 4 Riociguat;Placebo
6 Comparison of Efficacy Different Treatment Regimens in Pulmonary Hypertension Secondary to Lung Disease and or Hypoxia Unknown status NCT01449253 Phase 4 Sildenafil;Bosentan
7 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)
8 Effects of Spironolactone on Collagen Metabolism in Pulmonary Arterial Hypertension Unknown status NCT01468571 Phase 4 Spironolactone;Placebo
9 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
10 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
11 Randomized Controlled Trial to Compare the Efficacy of Combination Therapy vs Monotherapy for Pulmonary Arterial Hypertension in Systemic Sclerosis Unknown status NCT03053739 Phase 4 Sildenafil 20mg and Bosentan 62.5mg;Sildenafil 20mg and Placebo
12 Long Acting Phosphodiesterase 5 Inhibitors as Add-on Therapy for Patients With Pulmonary Hypertension Treated With Prostanoids. Unknown status NCT00705588 Phase 4 Tadalafil;Vardenafil
13 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
14 Evaluation of Sildenafil for the Treatment of Moderate Congestive Heart Failure Unknown status NCT00793338 Phase 4 Sildenafil
15 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
16 Vardenafil as add-on Therapy for Patients With Pulmonary Hypertension Treated With Inhaled Iloprost Unknown status NCT01649739 Phase 4 Levitra
17 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
18 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
19 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
20 Phase IV Study of Chronic Infusional Epoprostenol for Severe Primary Pulmonary Hypertension Completed NCT00004754 Phase 4 epoprostenol
21 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®
22 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
23 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®
24 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
25 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
26 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
27 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
28 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
29 Echo Study - Characterization of LV Strain Patterns in Patients With Mildly Elevated PCWP and Pulmonary Hypertension Completed NCT01800292 Phase 4 sildenafil
30 Inhaled Iloprost for Sarcoidosis Associated Pulmonary Hypertension Completed NCT00403650 Phase 4 Iloprost
31 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
32 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
33 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
34 Vasoreactivity Testing With Intravenous Sildenafil in Patients With Precapillary Pulmonary Hypertension (Treatment Optimisation Study) Completed NCT01889966 Phase 4 Sildenafil
35 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)
36 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
37 Safely Change From Bosentan to Ambrisentan in Pulmonary Hypertension Completed NCT01330108 Phase 4 ambrisentan
38 Therapy of Pulmonary Arterial Hypertension (PAH) With Bosentan in Patients With Eisenmenger Syndrome Completed NCT00266162 Phase 4 Bosentan administration
39 The Effect of Riociguat on Gas Exchange, Exercise Performance, and Pulmonary Artery Pressure During Acute Altitude Exposure Completed NCT02024386 Phase 4 Riociguat
40 Acute Effect of Recombinant Human Brain Natriuretic Peptide in Patients With Pulmonary Hypertension Associated With Acute Exacerbation of Chronic Pulmonary Disease Completed NCT02742909 Phase 4 rhBNP;placebo
41 Sildenafil for Improving Outcomes After Valvular Correction Completed NCT00862043 Phase 4 Sildenafil Citrate;Placebo
42 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
43 Natrecor® (Nesiritide) as a Nitric Oxide Sparing Agent in Patients Undergoing Lung Transplantation Completed NCT00205426 Phase 4 Natrecor
44 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
45 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
46 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
47 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
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 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
50 Clevidipine for Vasoreactivity Evaluation of the Pulmonary Arterial Bed (CARVE) Completed NCT01121458 Phase 4 Clevidipine

Search NIH Clinical Center for Pulmonary Hypertension

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


Alprostadil
bosentan
Epoprostenol
Epoprostenol Sodium
Hydralazine
Hydralazine Hydrochloride
Nitroglycerin
Treprostinil

Cell-based therapeutics:


LifeMap Discovery
Data from LifeMap, the Embryonic Development and Stem Cells Database
Read about Pulmonary Hypertension cell therapies at LifeMap Discovery.

Cochrane evidence based reviews: hypertension, pulmonary

Genetic Tests for Pulmonary Hypertension

Anatomical Context for Pulmonary Hypertension

MalaCards organs/tissues related to Pulmonary Hypertension:

40
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

Publications for Pulmonary Hypertension

Articles related to Pulmonary Hypertension:

(show top 50) (show all 30226)
# 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. 54 61 46
19390056 2009
2
Susceptibility to chronic thromboembolic pulmonary hypertension may be conferred by miR-759 via its targeted interaction with polymorphic fibrinogen alpha gene. 61 46
20677013 2010
3
Dynamic changes in lung microRNA profiles during the development of pulmonary hypertension due to chronic hypoxia and monocrotaline. 61 46
20110569 2010
4
New advances in the management of pulmonary sarcoidosis. 42
31641045 2019
5
Correlation between right ventricular-pulmonary artery coupling and the prognosis of patients with pulmonary arterial hypertension. 42
31577738 2019
6
Pulmonary vasodilator therapy for patients after Fontan procedure: A protocol for a systematic review and meta-analysis. 42
31626139 2019
7
Transcripts from a novel BMPR2 termination mutation escape nonsense mediated decay by downstream translation re-initiation: implications for treating pulmonary hypertension. 54 61
20095988 2010
8
Emerging therapies for the treatment of pulmonary hypertension. 54 61
20216170 2010
9
Placenta growth factor in sickle cell disease: association with hemolysis and inflammation. 54 61
20040765 2010
10
Pulmonary hemodynamic response to acute combination and monotherapy with sildenafil and brain natriuretic peptide in rats with monocrotaline-induced pulmonary hypertension. 54 61
19996941 2010
11
Sequence variants in BMPR2 and genes involved in the serotonin and nitric oxide pathways in idiopathic pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension: relation to clinical parameters and comparison with left heart disease. 54 61
19844076 2010
12
B-type natriuretic peptide utilization as an adjunct to management in a case of conjoined twins with pulmonary hypertension. 54 61
20085871 2010
13
Phosphodiesterase type 5 inhibitors for high-altitude pulmonary hypertension: a meta-analysis. 54 61
20225909 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. 54 61
19556740 2010
15
Dichloroacetate treatment partially regresses established pulmonary hypertension in mice with SM22alpha-targeted overexpression of the serotonin transporter. 54 61
19679640 2009
16
Brain natriuretic peptide in pulmonary arterial hypertension: biomarker and potential therapeutic agent. 54 61
20054445 2009
17
Interaction between bone morphogenetic proteins and endothelin-1 in human pulmonary artery smooth muscle. 54 61
19786120 2009
18
Nitric oxide synthases in infants and children with pulmonary hypertension and congenital heart disease. 54 61
19912632 2009
19
Expression of vasoactive intestinal peptide and related receptors in overcirculation-induced pulmonary hypertension in piglets. 54 61
19581838 2009
20
A novel insight into the mechanism of pulmonary hypertension involving caveolin-1 deficiency and endothelial nitric oxide synthase activation. 54 61
20382348 2009
21
Soluble guanylate cyclase agonists inhibit expression and procoagulant activity of tissue factor. 54 61
19592462 2009
22
Brain natriuretic peptide in chronic thromboembolic pulmonary hypertension. 54 61
19468826 2009
23
Endothelin-1 across the lung circulation in patients with pulmonary arterial hypertension and influence of epoprostenol infusion. 54 61
19632577 2009
24
Chronic treatment with sildenafil stimulates Leydig cell and testosterone secretion. 54 61
19659904 2009
25
Intratracheal gene transfer of adrenomedullin using polyplex nanomicelles attenuates monocrotaline-induced pulmonary hypertension in rats. 54 61
19337232 2009
26
Effects of roflumilast, a phosphodiesterase-4 inhibitor, on hypoxia- and monocrotaline-induced pulmonary hypertension in rats. 54 61
19386793 2009
27
Persistent eNOS activation secondary to caveolin-1 deficiency induces pulmonary hypertension in mice and humans through PKG nitration. 54 61
19487814 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. 54 61
18793807 2009
29
RhoA and Rho kinase activation in human pulmonary hypertension: role of 5-HT signaling. 54 61
19299501 2009
30
Prognostic value of B-type natriuretic peptide in children with pulmonary hypertension. 54 61
18599134 2009
31
Discovery of riociguat (BAY 63-2521): a potent, oral stimulator of soluble guanylate cyclase for the treatment of pulmonary hypertension. 54 61
19263460 2009
32
Phosphodiesterase 5 inhibition in heart failure: mechanisms and clinical implications. 54 61
19377497 2009
33
Inhibition of cGMP phosphodiesterase 5 suppresses serotonin signalling in pulmonary artery smooth muscles cells. 54 61
19416631 2009
34
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. 54 61
18682983 2009
35
Caveolin-1 Expression and Hemodynamics in COPD Patients. 54 61
19572028 2009
36
Brain natriuretic peptide as a preclinical marker of chronic pulmonary hypertension in patients with pulmonary embolism. 54 61
19247590 2009
37
Novel approaches to the pharmacotherapy of pulmonary arterial hypertension. 54 61
19121410 2009
38
Combined use of PDE5 inhibitors and nitrates in the treatment of pulmonary arterial hypertension in patients with heart failure. 54 61
19181291 2009
39
[Endothelin-1 and brain natriuretic peptide in the development of pulmonary hypertension in interstitial lung diseases]. 54 61
20481052 2009
40
[Estimation of plasmic concentration of the brain natriuretic peptide in interstitial pulmonary diseases with pulmonary hypertension: clinical role]. 54 61
19459423 2009
41
Endothelin-1 inhibition by ambrisentan as a potential treatment adjunct after debulking surgery in epithelial ovarian cancer. 54 61
19544975 2009
42
Role of nitric oxide in shock: the large animal perspective. 54 61
19273179 2009
43
NO-independent, haem-dependent soluble guanylate cyclase stimulators. 54 61
19089334 2009
44
Hypoxic upregulation of preproendothelin-1 gene expression is associated with protein tyrosine kinase-PI3K signaling in cultured lung vascular endothelial cells. 54 61
19436835 2009
45
Superoxide dismutase restores eNOS expression and function in resistance pulmonary arteries from neonatal lambs with persistent pulmonary hypertension. 54 61
18790993 2008
46
Inhalation of vasoactive intestinal peptide in pulmonary hypertension. 54 61
18978135 2008
47
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. 54 61
18567602 2008
48
["Pulmonary hypertension" of the gastrointestinal tract in patients with systemic sclerosis: thoughts on a not (yet) existing disease entity]. 54 61
18814097 2008
49
Inflammation, endothelial injury, and persistent pulmonary hypertension in heterozygous BMPR2-mutant mice. 54 61
18552156 2008
50
Differential proinflammatory and prooxidant effects of bone morphogenetic protein-4 in coronary and pulmonary arterial endothelial cells. 54 61
18539760 2008

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 GeneCards Suite gene sharing:

(show all 14)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
12.47 SMAD9 SMAD4 BMPR2 BMPR1B ACVRL1
2 12.32 MIR451A MIR21 MIR20A MIR17 BMPR2
3
Show member pathways
12.27 SMAD9 SMAD4 BMPR2 BMPR1B
4 11.9 SMAD9 SMAD4 BMPR2 BMPR1B
5 11.71 SMAD4 NOS3 EDN1
6 11.7 NOS3 EDN1 CAV1 BMPR2 BMPR1B
7 11.67 SMAD9 SMAD4 ACVRL1
8 11.64 SMAD9 SMAD4 BMPR2 BMPR1B
9
Show member pathways
11.48 SMAD9 SMAD4 BMPR2 BMPR1B
10 11.11 NOS3 EDNRA EDN1
11 10.61 PDE5A NOS3
12 10.6 SMAD9 SMAD4 BMPR2 BMPR1B
13 10.47 SMAD9 SMAD4 BMPR2
14
Show member pathways
10.45 SMAD9 SMAD4 CAV1 BMPR2 ACVRL1

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 caveola GO:0005901 9.13 NOS3 CAV1 BMPR2
2 SMAD protein complex GO:0071141 8.62 SMAD9 SMAD4

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

(show all 49)
# Name GO ID Score Top Affiliating Genes
1 negative regulation of cell proliferation GO:0008285 10.07 SMAD4 NOS3 MIR21 CAV1 ACVRL1
2 gene silencing by miRNA GO:0035195 10 MIR22 MIR21 MIR20A MIR17
3 positive regulation of angiogenesis GO:0045766 9.91 NOS3 MIR21 MIR20A ACVRL1
4 in utero embryonic development GO:0001701 9.91 SMAD4 NOS3 EDNRA EDN1 ACVRL1
5 miRNA mediated inhibition of translation GO:0035278 9.88 MIR21 MIR20A MIR17
6 cellular response to growth factor stimulus GO:0071363 9.85 BMPR2 BMPR1B ACVRL1
7 negative regulation of gene expression GO:0010629 9.85 MIR451A MIR21 MIR20A MIR17 EDN1 ACVRL1
8 response to hypoxia GO:0001666 9.83 SMAD4 EDNRA EDN1 CAV1 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 9.82 MIR21 BMPR2 BMPR1B
11 positive regulation of endothelial cell migration GO:0010595 9.81 MIR21 EDN1 BMPR2
12 outflow tract morphogenesis GO:0003151 9.8 MIR20A MIR17 BMPR2
13 positive regulation of pathway-restricted SMAD protein phosphorylation GO:0010862 9.79 SMAD4 BMPR2 ACVRL1
14 ventricular septum morphogenesis GO:0060412 9.76 SMAD4 NOS3 BMPR2
15 regulation of blood pressure GO:0008217 9.76 NOS3 EDNRA EDN1 ACVRL1
16 positive regulation of BMP signaling pathway GO:0030513 9.75 SMAD4 BMPR2 ACVRL1
17 blood vessel remodeling GO:0001974 9.73 NOS3 BMPR2 ACVRL1
18 positive regulation of vascular associated smooth muscle cell migration GO:1904754 9.72 MIR451A MIR21 MIR20A
19 positive regulation of cartilage development GO:0061036 9.71 BMPR2 BMPR1B
20 positive regulation of vascular smooth muscle cell proliferation GO:1904707 9.71 MIR21 MIR20A MIR17 EDN1
21 negative regulation of DNA biosynthetic process GO:2000279 9.7 BMPR2 ACVRL1
22 negative regulation of systemic arterial blood pressure GO:0003085 9.7 MIR17 BMPR2
23 positive regulation of cardiac muscle hypertrophy GO:0010613 9.7 PDE5A MIR21 EDN1
24 lymphangiogenesis GO:0001946 9.69 BMPR2 ACVRL1
25 positive regulation of cardiac muscle hypertrophy in response to stress GO:1903244 9.69 MIR20A MIR17
26 response to transforming growth factor beta GO:0071559 9.69 SMAD4 EDN1
27 artery smooth muscle contraction GO:0014824 9.68 EDNRA EDN1
28 positive regulation of metalloendopeptidase activity GO:1904685 9.68 MIR21 MIR17
29 response to dexamethasone GO:0071548 9.68 EDN1 CPS1
30 negative regulation of smooth muscle cell apoptotic process GO:0034392 9.68 MIR21 EDN1
31 positive regulation of transcription from RNA polymerase II promoter involved in cellular response to chemical stimulus GO:1901522 9.67 SMAD9 SMAD4
32 transmembrane receptor protein serine/threonine kinase signaling pathway GO:0007178 9.67 BMPR2 BMPR1B ACVRL1
33 retina vasculature development in camera-type eye GO:0061298 9.66 BMPR2 ACVRL1
34 endothelial tube morphogenesis GO:0061154 9.66 MIR21 ACVRL1
35 artery development GO:0060840 9.65 BMPR2 ACVRL1
36 regulation of glucose transmembrane transport GO:0010827 9.65 EDNRA EDN1
37 vasoconstriction GO:0042310 9.65 EDNRA EDN1 CAV1
38 transforming growth factor beta receptor signaling pathway GO:0007179 9.65 SMAD9 SMAD4 MIR21 BMPR2 ACVRL1
39 negative regulation of vascular associated smooth muscle cell apoptotic process GO:1905460 9.64 MIR21 MIR17
40 lymphatic endothelial cell differentiation GO:0060836 9.62 BMPR2 ACVRL1
41 regulation of systemic arterial blood pressure by endothelin GO:0003100 9.62 NOS3 EDN1
42 negative regulation of endothelial cell proliferation GO:0001937 9.62 MIR22 MIR21 CAV1 ACVRL1
43 venous blood vessel development GO:0060841 9.6 BMPR2 ACVRL1
44 endochondral bone morphogenesis GO:0060350 9.59 BMPR2 BMPR1B
45 positive regulation of pulmonary blood vessel remodeling GO:1905111 9.57 MIR20A MIR17
46 regulation of the force of heart contraction by chemical signal GO:0003057 9.54 NOS3 CAV1
47 cellular response to BMP stimulus GO:0071773 9.35 SMAD9 SMAD4 BMPR2 BMPR1B ACVRL1
48 negative regulation of chondrocyte proliferation GO:1902731 9.33 MIR21 BMPR2 BMPR1B
49 BMP signaling pathway GO:0030509 9.1 SMAD9 SMAD4 MIR21 BMPR2 BMPR1B ACVRL1

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

# Name GO ID Score Top Affiliating Genes
1 mRNA binding involved in posttranscriptional gene silencing GO:1903231 9.62 MIR22 MIR21 MIR20A MIR17
2 transforming growth factor beta-activated receptor activity GO:0005024 9.26 BMPR2 ACVRL1
3 BMP receptor activity GO:0098821 9.16 BMPR2 ACVRL1
4 transforming growth factor beta receptor activity, type I GO:0005025 8.96 BMPR1B ACVRL1
5 transmembrane receptor protein serine/threonine kinase activity GO:0004675 8.8 BMPR2 BMPR1B ACVRL1

Sources for Pulmonary Hypertension

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
53 NINDS
54 Novoseek
56 OMIM
57 OMIM via Orphanet
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
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