MCID: PLM040
MIFTS: 45

Pulmonary Valve Disease

Categories: Cardiovascular diseases

Aliases & Classifications for Pulmonary Valve Disease

MalaCards integrated aliases for Pulmonary Valve Disease:

Name: Pulmonary Valve Disease 12 14
Pulmonary Valve Disorder 12 69
Abnormality of the Pulmonary Valve 29

Classifications:



External Ids:

Disease Ontology 12 DOID:5749
ICD9CM 35 424.3
NCIt 47 C78579
SNOMED-CT 64 195001000 76267008
ICD10 33 I37 I39.3
UMLS 69 C0034087

Summaries for Pulmonary Valve Disease

MalaCards based summary : Pulmonary Valve Disease, also known as pulmonary valve disorder, is related to rheumatic pulmonary valve disease and atrial septal defect 9. An important gene associated with Pulmonary Valve Disease is GATA6 (GATA Binding Protein 6), and among its related pathways/superpathways are Signaling by FGFR2 and Kit receptor signaling pathway. The drugs Warfarin and Acetazolamide have been mentioned in the context of this disorder. Affiliated tissues include heart, lung and testes, and related phenotypes are cardiovascular system and growth/size/body region

Related Diseases for Pulmonary Valve Disease

Diseases in the Pulmonary Valve Disease family:

Pulmonary Valve Insufficiency

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

(show all 38)
id Related Disease Score Top Affiliating Genes
1 rheumatic pulmonary valve disease 12.1
2 atrial septal defect 9 10.8
3 pulmonary valve insufficiency 10.8
4 internal hordeolum 10.4 PTPN11 SOS1
5 ascending cholangitis 10.3 NKX2-6 SLC6A11
6 growth restriction, severe, with distinctive facies 10.2 PTPN11 SOS1
7 fleck retina of kandori 10.1 CRELD1 NKX2-6
8 pulmonary valve stenosis 10.1
9 takayasu arteritis 10.1 CRELD1 NKX2-6
10 complete atrioventricular canal-left heart obstruction syndrome 10.0 CRELD1 GATA4
11 inclusion body myopathy with paget disease of bone and frontotemporal dementia 10.0 GATA4 GATA6
12 persistent eustachian valve 10.0 CRELD1 GATA4
13 autosomal dominant deafness-onychodystrophy syndrome 10.0 PTPN11 SPRED1
14 vitelliform macular dystrophy 10.0 GATA4 GATA6 NKX2-6
15 artery disease 9.9 CRELD1 GATA6 NKX2-6
16 adams-oliver syndrome 5 9.9 CRELD1 NKX2-6
17 trophoblastic neoplasm 9.8 CRELD1 NKX2-6 SLC6A11
18 interstitial nephritis, karyomegalic 9.8 PTPN11 SPRED1
19 inferior vena cava interruption 9.8 CRELD1 GATA4 GATA6
20 pasli disease 9.8 CRELD1 GATA4 GATA6
21 macroglossia 9.7 CRELD1 GATA4 NKX2-6
22 malignant pineal area germ cell neoplasm 9.6 GATA4 GATA6 NKX2-6 TBX5
23 dentine erosion 9.6 GATA4 GATA6 NKX2-6 TBX5
24 noonan syndrome 7 9.6 PTPN11 SOS1 SPRED1
25 skin epithelioid hemangioma 9.6 PTPN11 SPRED1
26 leopard syndrome 1 9.6 PTPN11 SOS1 SPRED1
27 second-degree atrioventricular block 9.6 GATA4 TBX5
28 carcinoid syndrome 9.6
29 nephronophthisis 15 9.5 PTPN11 SOS1 SPRED1
30 tricho odonto onycho dermal syndrome 9.5 CRELD1 GATA4 GATA6 NKX2-6
31 surfactant metabolism dysfunction, pulmonary, 2 9.4 CRELD1 GATA4 GATA6 NKX2-6
32 optic nerve disease 9.3 CRELD1 GATA4 NKX2-6 TBX5
33 ichthyosis 9.0 CRELD1 GATA4 GATA6 NKX2-6 TBX5
34 fetal alcohol syndrome 9.0 CRELD1 GATA4 GATA6 NKX2-6 TBX5
35 xanthogranulomatous pyelonephritis 8.9 CRELD1 GATA4 NKX2-6 PTPN11 TBX5
36 hypothyroidism, congenital nongoitrous, 5 8.7 CRELD1 GATA4 GATA6 NKX2-6 PTPN11 TBX5
37 progressive multifocal leukoencephalopathy 7.8 CRELD1 GATA4 NKX2-6 PTPN11 SOS1 SPRED1
38 cervical serous adenocarcinoma 6.5 CRELD1 GATA4 GATA6 ITGB8 NKX2-6 PTPN11

Comorbidity relations with Pulmonary Valve Disease via Phenotypic Disease Network (PDN): (show all 15)


Acute Cystitis Chronic Myocardial Ischemia
Deficiency Anemia Familial Atrial Fibrillation
Heart Disease Hypertension, Essential
Intermediate Coronary Syndrome Ischemic Heart Disease
Left Ventricular Outflow Tract Obstruction Mitral Valve Disease
Pulmonary Hypertension, Familial Primary, 1, with or Without Hht Respiratory Failure
Sinoatrial Node Disease Transient Cerebral Ischemia
Tricuspid Valve Disease

Graphical network of the top 20 diseases related to Pulmonary Valve Disease:



Diseases related to Pulmonary Valve Disease

Symptoms & Phenotypes for Pulmonary Valve Disease

MGI Mouse Phenotypes related to Pulmonary Valve Disease:

44
id Description MGI Source Accession Score Top Affiliating Genes
1 cardiovascular system MP:0005385 9.98 GATA4 GATA6 ITGB8 NKX2-6 PTPN11 SOS1
2 growth/size/body region MP:0005378 9.97 GATA4 GATA6 ITGB8 NKX2-6 PTPN11 SOS1
3 embryo MP:0005380 9.95 TBX5 GATA4 GATA6 ITGB8 NKX2-6 PTPN11
4 mortality/aging MP:0010768 9.86 ITGB8 NKX2-6 PTPN11 SLC6A11 SOS1 TBX5
5 limbs/digits/tail MP:0005371 9.72 GATA4 GATA6 PTPN11 SPRED1 TBX5
6 muscle MP:0005369 9.55 GATA4 GATA6 PTPN11 SOS1 TBX5
7 normal MP:0002873 9.43 GATA4 GATA6 ITGB8 NKX2-6 PTPN11 TBX5
8 respiratory system MP:0005388 9.02 GATA4 GATA6 NKX2-6 PTPN11 SPRED1

Drugs & Therapeutics for Pulmonary Valve Disease

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

(show top 50) (show all 217)
id Name Status Phase Clinical Trials Cas Number PubChem Id
1
Warfarin Approved Phase 4,Phase 3 81-81-2 6691 54678486
2
Acetazolamide Approved, Vet_approved Phase 4 59-66-5 1986
3
Remifentanil Approved Phase 4,Phase 2 132875-61-7 60815
4
Bupivacaine Approved, Investigational Phase 4,Phase 2 2180-92-9, 38396-39-3 2474
5
Morphine Approved, Investigational Phase 4,Phase 2 57-27-2 5288826
6
Benzocaine Approved Phase 4,Phase 3,Phase 2,Phase 1 1994-09-7, 94-09-7 2337
7
Tadalafil Approved, Investigational Phase 4 171596-29-5 110635
8
Neostigmine Approved, Vet_approved Phase 4 59-99-4 4456
9
Rocuronium Approved Phase 4 119302-91-9, 143558-00-3 441290
10 tannic acid Approved, Nutraceutical Phase 4,Phase 3,Phase 2,Phase 1
11
Lactitol Investigational Phase 4 585-86-4 3871
12 Anticoagulants Phase 4,Phase 3,Phase 2
13 Anesthetics Phase 4,Phase 3,Phase 2
14 Phosphodiesterase 5 Inhibitors Phase 4,Phase 3,Phase 2
15 Phosphodiesterase Inhibitors Phase 4,Phase 3,Phase 2
16 Sildenafil Citrate Phase 4,Phase 3,Phase 2 171599-83-0
17 Vasodilator Agents Phase 4,Phase 3,Phase 2,Phase 1,Early Phase 1
18 Calcium, Dietary Phase 4
19 Chelating Agents Phase 4
20 Anticonvulsants Phase 4
21 Carbonic Anhydrase Inhibitors Phase 4
22 diuretics Phase 4,Phase 3,Phase 1
23 Natriuretic Agents Phase 4,Phase 3,Phase 1
24 Analgesics Phase 4,Phase 2
25 Analgesics, Opioid Phase 4,Phase 2
26 Anesthetics, General Phase 4,Phase 3
27 Anesthetics, Intravenous Phase 4,Phase 3
28 Central Nervous System Depressants Phase 4,Phase 3,Phase 2
29 Hypnotics and Sedatives Phase 4,Phase 3
30 Narcotics Phase 4,Phase 2
31 Peripheral Nervous System Agents Phase 4,Phase 2,Phase 3,Early Phase 1
32 Natriuretic Peptide, Brain Phase 4,Phase 3
33 Anesthetics, Local Phase 4,Phase 2
34 Autonomic Agents Phase 4,Phase 2,Phase 3,Early Phase 1
35 Cholinergic Agents Phase 4,Phase 2
36 Neurotransmitter Agents Phase 4,Phase 2,Phase 3,Phase 1,Early Phase 1
37 Cholinesterase Inhibitors Phase 4
38 Citrate Nutraceutical Phase 4,Phase 3,Phase 2
39
Citric Acid Nutraceutical, Vet_approved Phase 4,Phase 3,Phase 2 77-92-9 311
40
Amiodarone Approved, Investigational Phase 3 1951-25-3 2157
41
Menthol Approved Phase 3 2216-51-5 16666
42
Milrinone Approved Phase 3 78415-72-2 4197
43
Propofol Approved, Investigational, Vet_approved Phase 3,Phase 2 2078-54-8 4943
44
Metoprolol Approved, Investigational Phase 2, Phase 3 37350-58-6, 51384-51-1 4171
45
Furosemide Approved, Vet_approved Phase 3 54-31-9 3440
46
Dobutamine Approved Phase 3 34368-04-2 36811
47
Apixaban Approved Phase 3 503612-47-3 10182969
48
Rivaroxaban Approved Phase 3,Phase 2 366789-02-8
49 Beractant Approved Phase 3 108778-82-1
50
Nitric Oxide Approved Phase 3,Phase 2,Early Phase 1 10102-43-9 145068

Interventional clinical trials:

(show top 50) (show all 349)

id Name Status NCT ID Phase Drugs
1 The Study of Warfarin Maintenance Dose in Chinese Patients Unknown status NCT01855737 Phase 4 Warfarin
2 Applying Pharmacogenetics to Warfarin Dosing in Chinese Patients Unknown status NCT01610141 Phase 4
3 EUROPT Clinical Trial to Study the Efficacy of One-Way Valve Implantation (New Treatment Algorithm) in Patients With Heterogeneous Emphysema Unknown status NCT00730301 Phase 4
4 Physiological Response to Heliox21 and Air O2 Unknown status NCT01498432 Phase 4 Heliox21;Air O2
5 Comparative Study of Non-Invasive Mask Ventilation vs Cuirass Ventilation in Patients With Acute Respiratory Failure. Unknown status NCT00331656 Phase 4
6 Effect of Combined Remote Ischemic Preconditioning and Postconditioning on Acute Pulmonary Injury in Patients Undergoing Valvular Heart Surgery Completed NCT01427621 Phase 4
7 Sildenafil for Secondary Pulmonary Hypertension Due to Valvular Disease Completed NCT00862043 Phase 4 Sildenafil Citrate;Placebo
8 Acute Hemodynamic Effects of Sildenafil in Patients With Severe Aortic Stenosis Completed NCT01060020 Phase 4 Sildenafil
9 Acetazolamide for Respiratory Failure in Combination With Metabolic Alkalosis Completed NCT00222534 Phase 4 Acetazolamide;Placebo
10 Acute Effect of Pulmonary Desufflation on Cardiac Performance in COPD Patients Completed NCT01996124 Phase 4 Indacaterol Fumarate;Placebo
11 Effect of Non-invasive Ventilation (NIV) on Cerebral Oxygenation Completed NCT02454582 Phase 4
12 Effects of Remifentanil on Respiratory Function in Mechanically Ventilated Patients Completed NCT00665119 Phase 4 remifentanil;placebo
13 Brain Natriuretic Peptide (BNP) Levels in Patients in Non Invasive Mechanical Ventilation Completed NCT00453726 Phase 4
14 B-type Natriuretic Peptide (BNP)-Guided Diagnostic Strategy in Intensive Care Unit (ICU) Patients With Respiratory Failure Completed NCT00130559 Phase 4
15 Evaluation Of The Total Face Mask For Emergency Application In Acute Respiratory Failure Completed NCT00686257 Phase 4
16 General Plus Spinal Anesthesia and General Anesthesia Alone on Right Ventricular Function Recruiting NCT03013075 Phase 4 Bupivacaine heavy and Morphine;General Anesthetics
17 Aortic Stenosis and PhosphodiEsterase Type 5 iNhibition (ASPEN): A Pilot Study Recruiting NCT01275339 Phase 4 Tadalafil;Placebo
18 Acute Effect of EPAP and Noninvasive Ventilation on Pulmonary Function and Lung Regional Ventilation on Bariatric Surgery Recruiting NCT02577744 Phase 4
19 Sugammadex vs. Placebo to Prevent Residual Neuromuscular Block in the Post--‐Anesthesia Care Unit Recruiting NCT02822001 Phase 4 Sugammadex;Placebo
20 Effect of Neuromuscular Blockade and Reversal on Breathing Not yet recruiting NCT02845375 Phase 4 Sugammadex;Neostigmine;Placebo
21 Aortic Stenosis and PhosphodiEsterase iNhibition With Aortic Valve Replacement (ASPEN-AVR): A Pilot Study Terminated NCT01272388 Phase 4 Tadalafil;Placebo
22 Effectiveness of the Vasodilator Test With Revatio, Made in Patients With Acute Pulmonary Hypertension Unknown status NCT01091012 Phase 3 Sildenafil 20mg oral vs Sildenafil 10mg intravenous
23 Efficiency of Radiofrequency Ablation for Surgical Treatment of Chronic Atrial Fibrillation With Rheumatic Valve Disease Unknown status NCT01013688 Phase 3
24 Multi-center Trial of Percutaneous Pulmonary Valve Implantation With Venus-p Unknown status NCT02590679 Phase 2, Phase 3
25 Efficacy and Safety of Venus P-valve to Treat RVOT Stenosis With Pulmonary Regurgitation Unknown status NCT02071654 Phase 3
26 Hemofiltration for Respiratory Failure After Bone Marrow Transplantation Unknown status NCT00120575 Phase 2, Phase 3
27 The Effect and Mechanism of Bronchoscopic Lung Volume Reduction by Endobronchial Valve in Korean Emphysema Patients Completed NCT01869205 Phase 3
28 Pulmonary Valve Replacement in Large Right Ventricular Outflow Tract Completed NCT00259207 Phase 3
29 Effect of Brief Nebulization of Milrinone on Pulmonary Arterial Pressure Before Cardiopulmonary Bypass on Mitral Valve Surgery Patients Completed NCT01621971 Phase 3 inhaled milrinone;intravenous milrinone
30 Broncho-alveolar Lavage Under Noninvasive Ventilation With Propofol TCI in Patient With AHRF Completed NCT00741949 Phase 3 Propofol;Placebo
31 Endobronchial Valve for Emphysema Palliation Trial (VENT) Completed NCT00129584 Phase 3
32 Sedation Management in Pediatric Patients With Acute Respiratory Failure (The RESTORE Study) Completed NCT00814099 Phase 3
33 Use of Endobronchial Valves in Non-Upper Lobe Heterogeneous Emphysema Completed NCT00825578 Phase 3
34 Nasal Intermittent Positive Pressure Ventilation in Premature Infants (NIPPV) Completed NCT00433212 Phase 3
35 Molecular Mechanisms of Volume Overload-Aim 1(SCCOR in Cardiac Dysfunction and Disease) Completed NCT01052428 Phase 2, Phase 3 metoprolol succinate (Toprol XL);Placebo
36 Canadian Oxygen Trial (COT) Completed NCT00637169 Phase 3
37 B-type Natriuretic Peptide for the Management of Weaning Completed NCT00473148 Phase 3
38 Efficacy of Early Tracheostomy to Reduce Incidence of Ventilator Acquired Pneumonia (VAP) Completed NCT00262431 Phase 3
39 Fluid Restriction Keeps Children Dry Recruiting NCT02989051 Phase 2, Phase 3
40 Anti-Thrombotic Strategy After Trans-Aortic Valve Implantation for Aortic Stenosis Recruiting NCT02664649 Phase 3 Apixaban;Standard of care
41 A Study of Rivaroxaban (JNJ-39039039) on the Venous Thromboembolic Risk in Post-Hospital Discharge Patients Recruiting NCT02111564 Phase 3 Rivaroxaban, 10 mg;Rivaroxaban, 7.5 mg;Placebo
42 Study to Evaluate the Treatment Effect of PT003 on Cardiovascular Hemodynamics in Subjects With Moderate to Severe COPD Recruiting NCT02685293 Phase 3 GFF MDI (PT003);Placebo MDI
43 Pulmonary Rehabilitation Before Lung Cancer Resection Recruiting NCT02887521 Phase 3
44 Effect of Sildenafil for Sustained PAH After MV Surgery (SUPERIOR Trial) Active, not recruiting NCT02435303 Phase 2, Phase 3 Sildenafil;Placebo
45 Efficacy and Tolerance of Early Launching of Nocturnal Non Invasive Active, not recruiting NCT01225614 Phase 3
46 LVR in Severe Emphysema Using Bronchoscopic Autologous Blood Instillation in Combination With Intra-bronchial Valves Not yet recruiting NCT03010449 Phase 2, Phase 3
47 Safety and Efficacy Study of the PTMA Device to Reduce Mitral Valve Regurgitation in Patients With Heart Failure Suspended NCT00815386 Phase 2, Phase 3
48 Endobronchial Valve for Emphysema PalliatioN Trial (VENT) Cost-effectiveness Sub-Study Terminated NCT00137956 Phase 3
49 Early Surfactant to Reduce Use of Mechanical Breathing in Low Birth Weight Infants Terminated NCT00005774 Phase 3 Early surfactant;Standard practice
50 Inhaled Nitric Oxide Study for Respiratory Failure in Newborns Terminated NCT00005776 Phase 3 Inhaled nitric oxide;Placebo

Search NIH Clinical Center for Pulmonary Valve Disease

Genetic Tests for Pulmonary Valve Disease

Genetic tests related to Pulmonary Valve Disease:

id Genetic test Affiliating Genes
1 Abnormality of the Pulmonary Valve 29

Anatomical Context for Pulmonary Valve Disease

MalaCards organs/tissues related to Pulmonary Valve Disease:

39
Heart, Lung, Testes, Bone, Liver, Brain, Endothelial

Publications for Pulmonary Valve Disease

Articles related to Pulmonary Valve Disease:

id Title Authors Year
1
Abnormal Haemodynamic Flow Patterns in Bicuspid Pulmonary Valve Disease. ( 28620320 )
2017
2
Importance of pulmonary valve disease during implantation of a biventricular assist device. ( 24268185 )
2013
3
Tricuspid and pulmonary valve disease evaluation and management. ( 21070730 )
2010
4
Pulmonary valve replacement: extending the indications to the whole spectrum of pulmonary valve disease. ( 19804205 )
2007
5
Percutaneous stent-mounted valve for treatment of aortic or pulmonary valve disease. ( 15343576 )
2004
6
Carcinoid syndrome: an unusual cause of tricuspid and pulmonary valve disease. ( 12876616 )
2003
7
Rheumatic pulmonary valve disease. ( 11273310 )
1998
8
Echocardiographic evaluation of isolated pulmonary valve disease in adolescents and adults. ( 10146329 )
1993
9
Rheumatic pulmonary valve disease. ( 5380838 )
1969

Variations for Pulmonary Valve Disease

Expression for Pulmonary Valve Disease

Search GEO for disease gene expression data for Pulmonary Valve Disease.

Pathways for Pulmonary Valve Disease

GO Terms for Pulmonary Valve Disease

Biological processes related to Pulmonary Valve Disease according to GeneCards Suite gene sharing:

(show all 11)
id Name GO ID Score Top Affiliating Genes
1 heart development GO:0007507 9.67 NKX2-6 PTPN11 TBX5
2 positive regulation of cardiac muscle cell proliferation GO:0060045 9.48 GATA6 TBX5
3 positive regulation of BMP signaling pathway GO:0030513 9.46 GATA4 GATA6
4 ventricular septum development GO:0003281 9.43 GATA4 TBX5
5 neurotrophin TRK receptor signaling pathway GO:0048011 9.4 PTPN11 SOS1
6 atrial septum morphogenesis GO:0060413 9.37 GATA4 TBX5
7 cardiac muscle cell differentiation GO:0055007 9.33 GATA4 GATA6 TBX5
8 pericardium development GO:0060039 9.32 NKX2-6 TBX5
9 intestinal epithelial cell differentiation GO:0060575 9.26 GATA4 GATA6
10 endocardial cushion development GO:0003197 9.13 CRELD1 GATA4 TBX5
11 positive regulation of cardioblast differentiation GO:0051891 8.8 GATA4 GATA6 TBX5

Molecular functions related to Pulmonary Valve Disease according to GeneCards Suite gene sharing:

id Name GO ID Score Top Affiliating Genes
1 sequence-specific DNA binding GO:0043565 9.26 GATA4 GATA6 NKX2-6 TBX5
2 RNA polymerase II transcription factor binding GO:0001085 9.16 GATA4 GATA6
3 transcriptional activator activity, RNA polymerase II transcription regulatory region sequence-specific binding GO:0001228 8.8 GATA4 GATA6 TBX5

Sources for Pulmonary Valve Disease

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
16 ExPASy
18 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 MedGen
42 MeSH
43 MESH via Orphanet
44 MGI
46 NCI
47 NCIt
48 NDF-RT
51 NINDS
52 Novoseek
54 OMIM
55 OMIM via Orphanet
59 PubMed
60 QIAGEN
65 SNOMED-CT via HPO
66 SNOMED-CT via Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
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