MCID: PLM037
MIFTS: 79

Pulmonary Hypertension malady

Categories: Cardiovascular diseases, Respiratory diseases, Genetic diseases

Aliases & Classifications for Pulmonary Hypertension

Aliases & Descriptions for Pulmonary Hypertension:

Name: Pulmonary Hypertension 39 38 12 41 3 14 69
Primary Pulmonary Hypertension 39 12 71 29 52 14
Idiopathic Pulmonary Arterial Hypertension 69
Idiopathic Pulmonary Hypertension 69
Hypertension, Pulmonary 42
Hypertension Pulmonary 52

Classifications:



External Ids:

Disease Ontology 12 DOID:14557 DOID:6432
ICD10 33 I27.0 I27.2
ICD9CM 35 416.0
NCIt 47 C97119 C3120
MeSH 42 D006976

Summaries for Pulmonary Hypertension

MedlinePlus : 41 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 venoocclusive disease 1 and myocardial infarction, and has symptoms including angina pectoris, chest pain and coughing. An important gene associated with Pulmonary Hypertension is BMPR2 (Bone Morphogenetic Protein Receptor Type 2), and among its related pathways/superpathways are PEDF Induced Signaling and Vascular smooth muscle contraction. The drugs Adcirca and Adempas have been mentioned in the context of this disorder. Affiliated tissues include Lung, lung and heart, and related phenotypes are cardiovascular system and homeostasis/metabolism

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

Wikipedia : 71 Pulmonary hypertension (PH or PHTN) is an increase of blood pressure in the pulmonary artery, pulmonary... more...

Related Diseases for Pulmonary Hypertension

Diseases in the Pulmonary Hypertension family:

Pulmonary Hypertension, Primary, 4 Pulmonary Hypertension, Primary, 3
Pulmonary Hypertension, Primary, 2

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

(show top 50) (show all 410)
id Related Disease Score Top Affiliating Genes
1 pulmonary venoocclusive disease 1 31.6 ACVRL1 BMPR2 ENG FOXF1 KCNK3
2 myocardial infarction 30.0 ADM ANGPT1 EDN1 NOS3 NPPA NPPB
3 adenocarcinoma 29.6 EDN1 EDNRA EDNRB
4 pulmonary hypertension, familial primary, 1, with or without hht 12.4
5 chronic thromboembolic pulmonary hypertension 12.3
6 pulmonary hypertension, primary, 4 12.2
7 hyperuricemia, pulmonary hypertension, renal failure, and alkalosis 12.2
8 pulmonary hypertension, primary, 3 12.2
9 pulmonary hypertension, primary, 2 12.2
10 pulmonary hypertension, neonatal 12.1
11 pulmonary hypertension, chronic thromboembolic, without deep vein thrombosis 12.0
12 vater-like defects with pulmonary hypertension, laryngeal webs, and growth deficiency 11.9
13 pulmonary venoocclusive disease 11.6
14 alveolar capillary dysplasia with misalignment of pulmonary veins 11.5
15 cirrhosis, cryptogenic 11.4
16 eisenmenger syndrome 11.4
17 alveolar capillary dysplasia 11.3
18 hypoxia 11.2
19 heritable pulmonary arterial hypertension 11.2
20 persistent fetal circulation syndrome 11.1
21 pulmonary vein stenosis 11.1
22 adams-oliver syndrome 11.1
23 unilateral absence of a pulmonary artery 11.1
24 pulmonary edema of mountaineers 11.0
25 ventricular septal defect 2 10.9
26 ventricular septal defect 1 10.9
27 pulmonary venoocclusive disease 2 10.9
28 hypertrichotic osteochondrodysplasia 10.9
29 arthrogryposis, distal, type 5 10.9
30 ventricular septal defect 3 10.9
31 rowley-rosenberg syndrome 10.8
32 lymphedema and cerebral arteriovenous anomaly 10.8
33 diffuse cutaneous systemic sclerosis 10.8
34 adams-oliver syndrome 6 10.8
35 diaphragmatic hernia 3 10.8
36 adams-oliver syndrome 5 10.8
37 multiple mitochondrial dysfunctions syndrome 3 10.8
38 adams-oliver syndrome 1 10.8
39 multisystemic smooth muscle dysfunction syndrome 10.8
40 multiple mitochondrial dysfunctions syndrome 1 10.8
41 combined oxidative phosphorylation deficiency 22 10.8
42 multiple mitochondrial dysfunctions syndrome 2 10.8
43 pulmonary valve insufficiency 10.8
44 lipoyltransferase 1 deficiency 10.8
45 hydrops, lactic acidosis, and sideroblastic anemia 10.8
46 citrulline transport defect 10.5 BMPR2 EDN1 NPPB
47 histidine metabolism disease 10.4 KCNK3 NOS3 NPPA NPPB
48 ovarian serous adenofibroma 10.4 ACVRL1 BMPR2 KCNK3
49 ectodermal dysplasia 9, hair/nail type 10.4 EDN1 NOS3 NPPB
50 hematocele of tunica vaginalis testis 10.4 ACVRL1 BMPR2 EDN1 SLC6A4

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



Diseases related to Pulmonary Hypertension

Symptoms & Phenotypes for Pulmonary Hypertension

UMLS symptoms related to Pulmonary Hypertension:


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

MGI Mouse Phenotypes related to Pulmonary Hypertension:

44 (show all 13)
id Description MGI Source Accession Score Top Affiliating Genes
1 cardiovascular system MP:0005385 10.43 ACVRL1 ADM ANGPT1 BMP2 BMPR2 EDN1
2 homeostasis/metabolism MP:0005376 10.28 ANGPT1 BMP2 BMPR2 EDN1 EDNRA EDNRB
3 embryo MP:0005380 10.26 ACVRL1 ADM ANGPT1 BMP2 BMPR1B BMPR2
4 mortality/aging MP:0010768 10.24 BMP2 BMPR1B BMPR2 EDN1 EDNRA EDNRB
5 growth/size/body region MP:0005378 10.21 EDNRB ENG NOS3 SLC6A4 THBD ACVRL1
6 digestive/alimentary MP:0005381 10.15 ACVRL1 BMP2 EDN1 EDNRA EDNRB ENG
7 endocrine/exocrine gland MP:0005379 10.11 ADM BMPR1B EDN1 EDNRA EDNRB KCNK3
8 muscle MP:0005369 10.1 BMPR2 EDN1 EDNRA EDNRB ENG FOXF1
9 nervous system MP:0003631 10.07 ACVRL1 ANGPT1 BMP2 BMPR1B BMPR2 EDN1
10 craniofacial MP:0005382 10.05 ACVRL1 BMP2 BMPR1B EDN1 EDNRA EDNRB
11 normal MP:0002873 9.93 ACVRL1 BMP2 BMPR1B BMPR2 EDN1 EDNRA
12 renal/urinary system MP:0005367 9.65 ACVRL1 ADM ANGPT1 BMPR2 EDN1 EDNRA
13 respiratory system MP:0005388 9.36 KCNK3 NOS3 THBD VIP ACVRL1 ADM

Drugs & Therapeutics for Pulmonary Hypertension

FDA approved drugs:

(show all 7)
id Drug Name Active Ingredient(s) 17 Company Approval Date
1
Adcirca 17 TADALAFIL Eli Lilly Approved May 2009
2
Adempas 17 RIOCIGUAT Bayer Healthcare Pharmaceuticals Approved October 2013
3
Letairis 17 AMBRISENTAN Gilead Approved June 2007
4
Opsumit 17 MACITENTAN Actelion Pharmaceuticals Approved October 2013
5
Remodulin 17 TREPROSTINIL United Therapeutics Approved May 2002
6
Tracleer 17 BOSENTAN Actelion Approved November 2001
7
Tyvaso 17 TREPROSTINIL United Therapeutics Approved July 2009

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

(show top 50) (show all 651)
id Name Status Phase Clinical Trials Cas Number PubChem Id
1
Bosentan Approved, Investigational Phase 4,Phase 3,Phase 2,Phase 1,Early Phase 1 147536-97-8 104865
2
Iloprost Approved, Investigational Phase 4,Phase 2,Phase 3,Phase 1 78919-13-8 6443959
3
Dalteparin Approved Phase 4,Phase 3 9041-08-1
4
Heparin Approved, Investigational Phase 4,Phase 3,Phase 2 9005-49-6 772 46507594
5
Spironolactone Approved Phase 4,Phase 1,Phase 2 1952-01-7, 52-01-7 5833
6
Iron Approved Phase 4,Phase 2,Phase 1 7439-89-6 23925
7
Epoprostenol Approved Phase 4,Phase 3,Phase 2 61849-14-7, 35121-78-9 5280427 5282411
8
Tadalafil Approved, Investigational Phase 4,Phase 2,Phase 3,Phase 1 171596-29-5 110635
9
Nitric Oxide Approved Phase 4,Phase 3,Phase 2,Phase 1,Early Phase 1 10102-43-9 145068
10
Treprostinil Approved, Investigational Phase 4,Phase 3,Phase 2,Phase 1 81846-19-7 54786 6918140
11
Dexmedetomidine Approved, Vet_approved Phase 4,Phase 2,Phase 3 76631-46-4, 113775-47-6 68602 5311068 56032
12
Epinephrine Approved, Vet_approved Phase 4,Phase 1 51-43-4 5816
13
Midazolam Approved, Illicit Phase 4,Phase 2,Phase 3 59467-70-8 4192
14
Remifentanil Approved Phase 4,Phase 2 132875-61-7 60815
15
Atenolol Approved Phase 4,Phase 3,Phase 2 29122-68-7 2249
16
Benzocaine Approved Phase 4,Phase 3,Phase 2 1994-09-7, 94-09-7 2337
17
Magnesium Sulfate Approved, Vet_approved Phase 4,Phase 3 7487-88-9 24083
18
Ranolazine Approved, Investigational Phase 4,Phase 3,Phase 2,Phase 1 142387-99-3, 95635-55-5 56959
19
Isoflurane Approved, Vet_approved Phase 4 26675-46-7 3763
20
Propofol Approved, Investigational, Vet_approved Phase 4 2078-54-8 4943
21
Clevidipine Approved Phase 4 167221-71-8
22
Acetazolamide Approved, Vet_approved Phase 4,Phase 2,Phase 3 59-66-5 1986
23
Methazolamide Approved Phase 4 554-57-4 4100
24
Magnesium oxide Approved Phase 4,Phase 2 1309-48-4 14792
25
Zinc Approved Phase 4,Phase 3 7440-66-6 32051 23994
26
Cyclosporine Approved, Investigational, Vet_approved Phase 4 79217-60-0, 59865-13-3 5284373 6435893
27
Miconazole Approved, Investigational, Vet_approved Phase 4,Phase 1 22916-47-8 4189
28
Nitroglycerin Approved, Investigational Phase 4,Phase 1 55-63-0 4510
29 Moxonidine Approved Phase 4 75438-57-2 4810
30
Losartan Approved Phase 4,Phase 3,Phase 2 114798-26-4 3961
31
Carvedilol Approved, Investigational Phase 4,Phase 3,Phase 2,Phase 1 72956-09-3 2585
32
Chlorthalidone Approved Phase 4,Phase 3,Phase 2 77-36-1 2732
33
Eplerenone Approved Phase 4 107724-20-9 150310 443872
34
Irbesartan Approved, Investigational Phase 4,Phase 2,Phase 3 138402-11-6 3749
35
Quinapril Approved, Investigational Phase 4 85441-61-8 54892
36
Hydrochlorothiazide Approved, Vet_approved Phase 4,Phase 3 58-93-5 3639
37
Trandolapril Approved Phase 4 87679-37-6 5484727
38
Olmesartan Approved, Investigational Phase 4 144689-24-7, 144689-63-4 158781 130881
39
Telmisartan Approved, Investigational Phase 4 144701-48-4 65999
40
Celecoxib Approved, Investigational Phase 4 169590-42-5 2662
41
Ibuprofen Approved Phase 4,Phase 3 15687-27-1 3672
42
Naproxen Approved, Vet_approved Phase 4 22204-53-1 1302 156391
43
Nebivolol Approved, Investigational Phase 4,Phase 2,Phase 3 152520-56-4, 99200-09-6, 118457-14-0 71301
44
Macitentan Approved Phase 4,Phase 3,Phase 2,Phase 1 441798-33-0
45
Nifedipine Approved Phase 4,Phase 3 21829-25-4 4485
46
Methylprednisolone Approved, Vet_approved Phase 4,Phase 2,Phase 3,Early Phase 1 83-43-2 6741
47
Prednisolone Approved, Vet_approved Phase 4,Phase 2,Phase 3,Early Phase 1 50-24-8 5755
48 Selexipag Approved Phase 4,Phase 3,Phase 2 475086-01-2
49
Pioglitazone Approved, Investigational Phase 4,Phase 3,Phase 2 111025-46-8 4829
50
Progesterone Approved, Vet_approved Phase 4,Phase 3 57-83-0 5994

Interventional clinical trials:

(show top 50) (show all 1665)
id Name Status NCT ID Phase
1 Comparison of Efficacy Different Treatment Regimens in Pulmonary Hypertension Secondary to Lung Disease and or Hypoxia Unknown status NCT01449253 Phase 4
2 Exercise Induced Pulmonary Hypertension in Systemic Sclerosis and Treatment With Ambrisentan Unknown status NCT01051960 Phase 4
3 Inhaled Iloprost for Disproportionate Pulmonary Hypertension in Chronic Obstructive Pulmonary Disease (COPD) Unknown status NCT01116063 Phase 4
4 Bosentan in Pulmonary Hypertension in Interstitial Lung Disease Treatment Study Unknown status NCT00637065 Phase 4
5 Comparison of Thrombgolytic and Anticoagulation Therapy in Submassive Pulmonary Embolism Unknown status NCT02132689 Phase 4
6 Effects of Spironolactone on Collagen Metabolism in Patients With Pulmonary Arterial Hypertension Unknown status NCT01468571 Phase 4
7 Iron Deficiency In Pulmonary Hypertension Unknown status NCT01288651 Phase 4
8 Vardenafil as add-on Therapy for Patients With Pulmonary Hypertension Treated With Inhaled Iloprost Unknown status NCT01649739 Phase 4
9 Long Acting Phosphodiesterase 5 Inhibitors as Add-on Therapy for Patients With Pulmonary Hypertension Treated With Prostanoids. Unknown status NCT00705588 Phase 4
10 Pulmonary Arterial Hypertension Secondary to Idiopathic Pulmonary Fibrosis and Treatment With Bosentan Unknown status NCT00625469 Phase 4
11 Inhaled Nitric Oxide and Inhaled Prostacyclin After Cardiac Surgery for Heart Transplant or LVAD Placement Unknown status NCT01717209 Phase 4
12 Pulmonary Arterial Hypertension Secondary to Idiopathic Pulmonary Fibrosis And Treatment With Sildenafil Unknown status NCT00625079 Phase 4
13 Hemodynamic Response After Six Months of Sildenafil Unknown status NCT00483626 Phase 4
14 Effects of Iloprost Treatment in Adult Patients With Pulmonary Arterial Hypertension Related to Congenital Heart Disease Unknown status NCT01383083 Phase 4
15 Low Dose Prolonged Infusion of Tissue Type Plasminogen Activator Therapy in Massive Pulmonary Embolism Unknown status NCT02029456 Phase 4
16 Evaluation of Sildenafil for the Treatment of Moderate Congestive Heart Failure Unknown status NCT00793338 Phase 4
17 A Clinical Trial of Ambrisentan and Tadalafil in Pulmonary Arterial Hypertension Associated With Systemic Sclerosis Unknown status NCT01042158 Phase 4
18 Hypotonic Treprostinil Subcutaneous Infusion for Control of Treprostinil Related Site Pain Unknown status NCT01615627 Phase 4
19 The Study of Using Esophageal Pressure to Guide the PEEP Setting in Abdominal Hypertension Patients Who Undergoing Mechanical Ventilation Unknown status NCT01825304 Phase 4
20 Acute Effect of Sildenafil on Exercise Tolerance and Functional Capacity in COPD, IPF and Post Pneumonectomy Patients Unknown status NCT01382368 Phase 4
21 Safety Evaluation of Dexmedetomidine for EBUS-TBNA Unknown status NCT01381627 Phase 4
22 Examining Genetic Influence on Response to Beta-Blocker Medications in People With Type 2 Diabetes Unknown status NCT00925119 Phase 4
23 Study of Ranolazine in the Treatment of Pulmonary Hypertension Associated With Diastolic Left Ventricular Dysfunction Completed NCT02133352 Phase 4
24 Natrecor for Pulmonary Hypertension in Lung Transplants Completed NCT00205426 Phase 4
25 VasoREACTivity Testing With Intravenous SILdenafil in Patients With Precapillary Pulmonary Hypertension Completed NCT01889966 Phase 4
26 Study of Sildenafil Effects in Combination With Rehabilitation in Patients With Chronic Obstructive Pulmonary Disease (COPD) and Associated Pulmonary Hypertension Completed NCT01055405 Phase 4
27 Phase IV Study of Chronic Infusional Epoprostenol for Severe Primary Pulmonary Hypertension Completed NCT00004754 Phase 4
28 Post-Marketing Surveillance of Ventavis® in Chinese Patients With Primary Pulmonary Hypertension (PPH) Completed NCT00882947 Phase 4
29 Measuring Outcomes In Patients With Pulmonary Arterial Hypertension Not on Active Treatment (MOTION) Completed NCT02191137 Phase 4
30 Oral Sildenafil in Persistent Pulmonary Hypertension Secondary to Meconium Aspiration Syndrome in Newborns Completed NCT01757782 Phase 4
31 Effects of Propofol and Isoflurane on Pulmonary Vascular Resistance Completed NCT01212523 Phase 4
32 Characterization of LV Strain Patterns in Mildly Elevated PCWP and PAH. Completed NCT01800292 Phase 4
33 Sildenafil for Secondary Pulmonary Hypertension Due to Valvular Disease Completed NCT00862043 Phase 4
34 Clevidipine for Vasoreactivity Evaluation of the Pulmonary Arterial Bed Completed NCT01121458 Phase 4
35 Safely Change From Bosentan to Ambrisentan in Pulmonary Hypertension Completed NCT01330108 Phase 4
36 Sildenafil Effects on Pulmonary Haemodynamics and Gas Exchange in Chronic Obstructive Pulmonary Disease (COPD) Completed NCT00491803 Phase 4
37 Inhaled Iloprost for Sarcoidosis-associated Pulmonary Hypertension Completed NCT00403650 Phase 4
38 Efficacy Study of Riociguat and Its Effects on Exercise Performance and Pulmonary Artery Pressure at High Altitude Completed NCT02024386 Phase 4
39 Pulmonary Artery Remodelling With Bosentan Completed NCT00595049 Phase 4
40 The Effect of Tracleer® on Male Fertility Completed NCT00082186 Phase 4
41 Study of a New Thermo Stable Formulation of Epoprostenol Sodium to Treat Pulmonary Arterial Hypertension (PAH) Completed NCT01462565 Phase 4
42 A 48-week Study of the Effect of Dual Therapy (Inhaled Treprostinil and Tadafafil) Versus Monotherapy (Tadalafil). Completed NCT01305252 Phase 4
43 Safety, Efficacy and Treatment Satisfaction in Patients With PAH Rapidly Switched From Epoprostenol to Remodulin Completed NCT00373360 Phase 4
44 Acute Hemodynamic Effects of Sildenafil in Patients With Severe Aortic Stenosis Completed NCT01060020 Phase 4
45 Evaluate Effect of Optison on Pulmonary Artery Systolic Pressure (PASP) and Pulmonary Vascular Resistance (PVR). Completed NCT00878878 Phase 4
46 AZ, MZ, and the Pulmonary System Response to Hypoxia Completed NCT02760121 Phase 4
47 A Transition Study From Flolan® to Remodulin® in Patients With Pulmonary Arterial Hypertension Completed NCT00058929 Phase 4
48 BREATHE 5-OL: Tracleer (Bosentan) in Patients With Pulmonary Arterial Hypertension Related to Eisenmenger Physiology Completed NCT00367770 Phase 4
49 Effects of the Combination of Bosentan and Sildenafil Versus Sildenafil Monotherapy on Pulmonary Arterial Hypertension (PAH) Completed NCT00303459 Phase 4
50 Study of Add-on Ambrisentan Therapy to Background Phosphodiesterase Type-5 Inhibitor (PDE5i) Therapy in Pulmonary Arterial Hypertension (ATHENA-1) Completed NCT00617305 Phase 4

Search NIH Clinical Center for Pulmonary Hypertension

Inferred drug relations via UMLS 69 / NDF-RT 48 :


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:

id Genetic test Affiliating Genes
1 Primary Pulmonary Hypertension 29

Anatomical Context for Pulmonary Hypertension

MalaCards organs/tissues related to Pulmonary Hypertension:

39
Lung, Heart, Endothelial, Smooth Muscle, Brain, Bone, Liver
LifeMap Discovery
Data from LifeMap, the Embryonic Development and Stem Cells Database

Cells/anatomical compartments in embryo or adult related to Pulmonary Hypertension:
id 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 2452)
id Title Authors Year
1
Pulmonary hypertension in Nigerian adults with sickle cell anemia. ( 28507438 )
2017
2
Ventricular diastolic pressure ratio as a marker of treatment response in pulmonary hypertension. ( 28527879 )
2017
3
Correlation of right atrial enlargement on ECG to right atrial volume by echocardiography in patients with pulmonary hypertension. ( 28511781 )
2017
4
Activated TAFI Promotes the Development of Chronic Thromboembolic Pulmonary Hypertension: A Possible Novel Therapeutic Target. ( 28289017 )
2017
5
Reversal of pre-capillary pulmonary hypertension in a patient with sickle cell anemia who underwent haploidentical peripheral blood stem cell transplantation. ( 28067884 )
2017
6
Transition of Intravenous Treprostinil to Oral Therapy in a Patient with Functional Class IV Chronic Thromboembolic Pulmonary Hypertension. ( 28513869 )
2017
7
Predictors of Exercise-Induced Pulmonary Hypertension in Patients with Asymptomatic Degenerative Mitral Regurgitation: Mechanistic Insights from 2D Speckle-Tracking Echocardiography. ( 28071674 )
2017
8
Pentraxin 3 in neonates with and without diagnosis of pulmonary hypertension. ( 27838407 )
2017
9
Depressive Status in Patients With Chronic Thromboembolic Pulmonary Hypertension. ( 28529263 )
2017
10
Pulmonary hypertension in lymphangioleiomyomatosis: prevalence, severity and the role of carbon monoxide diffusion capacity as a screening method. ( 28427470 )
2017
11
Osteoprotegerin Disruption Attenuates HySu-Induced Pulmonary Hypertension Through Integrin I+vI^3/FAK/AKT Pathway Suppression. ( 28077433 )
2017
12
Pulmonary Hypertension Prevalence and Prognosis in a Cohort of Patients with Hereditary Hemorrhagic Telangiectasia Undergoing Embolization of pAVMs. ( 28375020 )
2017
13
Diagnostic accuracy of lung subtraction iodine mapping CT for the evaluation of pulmonary perfusion in patients with chronic thromboembolic pulmonary hypertension: Correlation with perfusion SPECT/CT. ( 28526539 )
2017
14
Different efficacy of inhaled and oral medications in pulmonary hypertension. ( 28527831 )
2017
15
Identification of Pulmonary Hypertension Caused by Left Heart Disease (World Health Organization Group 2) Based on Cardiac Chamber Volumes Derived from Chest CT. ( 28506612 )
2017
16
Altered Peripheral Blood Myeloid Cell Subpopulations in Children With Down Syndrome and Pulmonary Hypertension. ( 28060127 )
2017
17
Epidemiology and management of chronic thromboembolic pulmonary hypertension: Experience from two expert centers. ( 28528258 )
2017
18
Pulmonary Hypertension in Infants, Children, and Young Adults. ( 28521893 )
2017
19
Pulmonary Hypertension-Back to the Future. ( 28532977 )
2017
20
Pharmacologic stress myocardial perfusion imaging in patients with pulmonary hypertension: What do we know, and what remains to be learned? ( 28074408 )
2017
21
Bronchopulmonary dysplasia and pulmonary hypertension: a meta-analysis. ( 28079864 )
2017
22
To screen or not to screen for chronic thromboembolic pulmonary hypertension after acute pulmonary embolism. ( 28073030 )
2017
23
An Update on the Management of Chronic Thromboembolic Pulmonary Hypertension. ( 27989311 )
2017
24
The Impact of Sevoflurane on Coupling of the Left Ventricular-to-Systemic Vasculature in Rats With Chronic Pulmonary Hypertension. ( 28533073 )
2017
25
Pulmonary hypertension and mitral valve disease: Still a beast. ( 28411749 )
2017
26
Aquaporin 1 controls the functional phenotype of pulmonary smooth muscle cells in hypoxia-induced pulmonary hypertension. ( 28409279 )
2017
27
Imaging in Chronic Thromboembolic Pulmonary Hypertension. ( 28060193 )
2017
28
Combination therapy with riociquat and inhaled treprostinil in inoperable and progressive chronic thromboembolic pulmonary hypertension. ( 27942445 )
2017
29
A chronic thromboembolic pulmonary hypertension catch-22 situation: inferior vena caval filter plays a pivotal role in an unlikely situation. ( 28523175 )
2017
30
Energy efficiency and pulmonary artery flow after balloon pulmonary angioplasty for inoperable, chronic thromboembolic pulmonary hypertension: Analysis by phase-contrast MRI. ( 28065382 )
2017
31
Pulmonary hypertension and vasculopathy in incontinentia pigmenti: a case report. ( 28533687 )
2017
32
Role of microRNA-130a in the pathogeneses of obstructive sleep apnea hypopnea syndrome-associated pulmonary hypertension by targeting the GAX gene. ( 28514291 )
2017
33
Safety and tolerability of subcutaneous treprostinil in newborns with congenital diaphragmatic hernia and life-threatening pulmonary hypertension. ( 28389079 )
2017
34
Impact of High-Priority Allocation on Lung and Heart-Lung Transplantation for Pulmonary Hypertension. ( 28527964 )
2017
35
Comparison of echocardiographic parameters to assess right ventricular function in pulmonary hypertension. ( 28527022 )
2017
36
Right ventricular-vascular coupling in heart failure with preserved ejection fraction and pre- vs. post-capillary pulmonary hypertension. ( 28531295 )
2017
37
Fatal postoperative systemic pulmonary hypertension in benfluorex-induced valvular heart disease surgery: A case report. ( 28079786 )
2017
38
Mouse Genome-wide Association Study of Pre-clinical Group II Pulmonary Hypertension Identifies Egfr. ( 28085498 )
2017
39
Thin Air Resulting in High Pressure: Mountain Sickness and Hypoxia-Induced Pulmonary Hypertension. ( 28522921 )
2017
40
Machine Learning of Three-dimensional Right Ventricular Motion Enables Outcome Prediction in Pulmonary Hypertension: A Cardiac MR Imaging Study. ( 28092203 )
2017
41
A Review of Clinical Trial Endpoints of Patients with Pulmonary Arterial Hypertension and Chronic Thromboembolic Pulmonary Hypertension and How They Relate to Patient Outcomes in the United States. ( 28025931 )
2017
42
Does Crimean-Congo Hemorrhagic Fever Cause a Vasculitic Reaction with Pulmonary Artery Enlargement and Acute Pulmonary Hypertension? ( 27344326 )
2016
43
Percutaneous treatment of chronic thromboembolic pulmonary hypertension (CTEPH). ( 27174110 )
2016
44
Clinical usefulness of end-tidal CO2 profiles during incremental exercise in patients with chronic thromboembolic pulmonary hypertension. ( 27817818 )
2016
45
Tadalafil therapy for sarcoidosis-associated pulmonary hypertension. ( 28090299 )
2016
46
Resting arterial hypoxaemia in subjects with chronic heart failure, pulmonary hypertension and patent foramen ovale. ( 26990684 )
2016
47
Transcription factors, transcriptional coregulators, and epigenetic modulation in the control of pulmonary vascular cell phenotype: therapeutic implications for pulmonary hypertension (2015 Grover Conference series). ( 28090287 )
2016
48
YIA 03-10 ENDOTHELIAL KRUPPEL-LIKE FACTOR 15: A NOVEL TARGET FOR THE TREATMENT OF PULMONARY HYPERTENSION. ( 27642952 )
2016
49
Key Role of ROS in the Process of 15-Lipoxygenase/15-Hydroxyeicosatetraenoiccid-Induced Pulmonary Vascular Remodeling in Hypoxia Pulmonary Hypertension. ( 26871724 )
2016
50
Treatment of pulmonary hypertension in three patients with A9-thalassemia intermedia using pulmonary arterial hypertension-specific medications. ( 26854673 )
2016

Variations for Pulmonary Hypertension

ClinVar genetic disease variations for Pulmonary Hypertension:

6 (show top 50) (show all 381)
id Gene Variation Type Significance SNP ID Assembly Location
1 BMPR2 BMPR2, 1-BP DEL, 2579T deletion Pathogenic
2 BMPR2 NM_001204.6(BMPR2): c.2695C> T (p.Arg899Ter) single nucleotide variant Pathogenic rs137852741 GRCh37 Chromosome 2, 203421083: 203421083
3 BMPR2 NM_001204.6(BMPR2): c.218C> G (p.Ser73Ter) single nucleotide variant Pathogenic rs137852742 GRCh37 Chromosome 2, 203329673: 203329673
4 BMPR2 BMPR2, 1-BP DEL, 355A deletion Pathogenic
5 BMPR2 NM_001204.6(BMPR2): c.354T> G (p.Cys118Trp) single nucleotide variant Pathogenic rs137852743 GRCh37 Chromosome 2, 203332348: 203332348
6 BMPR2 NM_001204.6(BMPR2): c.1040G> A (p.Cys347Tyr) single nucleotide variant Pathogenic rs137852744 GRCh37 Chromosome 2, 203395589: 203395589
7 BMPR2 NM_001204.6(BMPR2): c.1454A> G (p.Asp485Gly) single nucleotide variant Pathogenic rs137852745 GRCh37 Chromosome 2, 203417479: 203417479
8 BMPR2 NM_001204.6(BMPR2): c.1471C> T (p.Arg491Trp) single nucleotide variant Pathogenic rs137852746 GRCh37 Chromosome 2, 203417496: 203417496
9 BMPR2 BMPR2, 5-BP DEL, NT1099 deletion Pathogenic
10 BMPR2 NM_001204.6(BMPR2): c.507C> A (p.Cys169Ter) single nucleotide variant Pathogenic rs137852747 GRCh37 Chromosome 2, 203378530: 203378530
11 BMPR2 NM_001204.6(BMPR2): c.2617C> T (p.Arg873Ter) single nucleotide variant Pathogenic rs137852748 GRCh37 Chromosome 2, 203421005: 203421005
12 BMPR2 NM_001204.6(BMPR2): c.1472G> A (p.Arg491Gln) single nucleotide variant Pathogenic rs137852749 GRCh37 Chromosome 2, 203417497: 203417497
13 BMPR2 BMPR2, 2-BP DEL, 1-BP INS, NT690 indel Pathogenic
14 BMPR2 NM_001204.6(BMPR2): c.367T> C (p.Cys123Arg) single nucleotide variant Pathogenic rs137852750 GRCh37 Chromosome 2, 203332361: 203332361
15 BMPR2 NM_001204.6(BMPR2): c.367T> A (p.Cys123Ser) single nucleotide variant Pathogenic rs137852750 GRCh37 Chromosome 2, 203332361: 203332361
16 BMPR2 NM_001204.6(BMPR2): c.994C> T (p.Arg332Ter) single nucleotide variant Pathogenic rs137852751 GRCh37 Chromosome 2, 203395543: 203395543
17 BMPR2 NM_001204.6(BMPR2): c.2696G> C (p.Arg899Pro) single nucleotide variant Pathogenic rs137852752 GRCh37 Chromosome 2, 203421084: 203421084
18 BMPR2 NM_001204.6(BMPR2): c.631C> T (p.Arg211Ter) single nucleotide variant Pathogenic rs137852753 GRCh37 Chromosome 2, 203383554: 203383554
19 BMPR2 NM_001204.6(BMPR2): c.44delC (p.Pro15Hisfs) deletion Pathogenic rs483352902 GRCh37 Chromosome 2, 203242241: 203242241
20 BMPR2 NM_001204.6(BMPR2): c.77-?_247+?del (p.A26_Q82del) deletion Pathogenic GRCh37 Chromosome 2, 203329532: 203329702
21 BMPR2 BMPR2, EX10DEL deletion Pathogenic
22 BMPR2 BMPR2, EX1-13DEL deletion Pathogenic
23 BMPR2 NM_001204.6(BMPR2): c.1297C> T (p.Gln433Ter) single nucleotide variant Pathogenic rs137852756 GRCh37 Chromosome 2, 203407054: 203407054
24 KCNK3 NM_002246.2(KCNK3): c.608G> A (p.Gly203Asp) single nucleotide variant Pathogenic rs398123039 GRCh37 Chromosome 2, 26950859: 26950859
25 BMPR2 NM_001204.6(BMPR2): c.295T> C (p.Cys99Arg) single nucleotide variant Pathogenic rs863223425 GRCh37 Chromosome 2, 203332289: 203332289
26 BMPR2 NM_001204.6(BMPR2): c.377A> G (p.Asn126Ser) single nucleotide variant Pathogenic rs863223426 GRCh37 Chromosome 2, 203332371: 203332371
27 BMPR2 NM_001204.6(BMPR2): c.797G> C (p.Arg266Thr) single nucleotide variant Pathogenic rs374694591 GRCh37 Chromosome 2, 203383720: 203383720
28 BMPR2 NM_001204.6(BMPR2): c.853-2A> G single nucleotide variant Pathogenic rs863223424 GRCh37 Chromosome 2, 203384808: 203384808
29 BMPR2 NM_001204.6(BMPR2): c.1128+1G> A single nucleotide variant Pathogenic rs863223420 GRCh37 Chromosome 2, 203395678: 203395678
30 BMPR2 NM_001204.6(BMPR2): c.1426_1450del25 (p.Leu476Glyfs) deletion Pathogenic rs863223422 GRCh37 Chromosome 2, 203417451: 203417475
31 ACVRL1 NM_000020.2(ACVRL1): c.430C> T (p.Arg144Ter) single nucleotide variant Pathogenic rs758683062 GRCh37 Chromosome 12, 52307459: 52307459
32 BMPR2 NM_001204.6(BMPR2): c.439C> T (p.Arg147Ter) single nucleotide variant Pathogenic rs869025366 GRCh37 Chromosome 2, 203378462: 203378462
33 BMPR2 NM_001204.6(BMPR2): c.1276G> C (p.Gly426Arg) single nucleotide variant Pathogenic/Likely pathogenic rs869025367 GRCh37 Chromosome 2, 203397455: 203397455
34 FOXF1 NC_000016.10: g.(?_86510584)_(86513143_?)del deletion Pathogenic GRCh38 Chromosome 16, 86510584: 86513143