MCID: PLM040
MIFTS: 40

Pulmonary Valve Disease

Categories: Cardiovascular diseases, Respiratory diseases

Aliases & Classifications for Pulmonary Valve Disease

MalaCards integrated aliases for Pulmonary Valve Disease:

Name: Pulmonary Valve Disease 12 15 17
Pulmonary Valve Disorder 12 70

Classifications:



External Ids:

Disease Ontology 12 DOID:5749
ICD9CM 34 424.3
NCIt 50 C78579
SNOMED-CT 67 76267008
ICD10 32 I37 I37.9 I39.3
UMLS 70 C0034087

Summaries for Pulmonary Valve Disease

MalaCards based summary : Pulmonary Valve Disease, also known as pulmonary valve disorder, is related to pulmonary valve insufficiency and tricuspid valve insufficiency. An important gene associated with Pulmonary Valve Disease is GATA6 (GATA Binding Protein 6), and among its related pathways/superpathways are Developmental Biology and Integrin Pathway. Affiliated tissues include heart and skin, 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 top 50) (show all 86)
# Related Disease Score Top Affiliating Genes
1 pulmonary valve insufficiency 31.7 NPPB FBN1
2 tricuspid valve insufficiency 30.0 PTPN11 NPPB FBN1
3 heart valve disease 29.8 NPPB NKX2-5 FBN1
4 tricuspid valve disease 29.4 TBX5 NPPB NKX2-5 GATA6 GATA4 FBN1
5 heart septal defect 28.7 ZIC3 TBX5 SOS1 SHOC2 PTPN11 NKX2-5
6 atrial heart septal defect 27.9 ZIC3 TBX5 SOS1 SHOC2 PTPN11 NPPB
7 tetralogy of fallot 27.6 ZIC3 TBX5 SOS1 PTPN11 NPPB NKX2-5
8 rheumatic pulmonary valve disease 11.2
9 atrial septal defect 9 11.1
10 malignant childhood germ cell neoplasm 10.3 GATA6 GATA4
11 partial atrioventricular canal 10.3 GATA6 GATA4
12 villonodular synovitis 10.3 SOS1 PTPN11
13 congenital heart defects, multiple types, 4 10.2 GATA6 GATA4
14 hypoplastic right heart syndrome 10.2 TBX5 NKX2-5
15 germ cell and embryonal cancer 10.2 NKX2-5 GATA6 GATA4
16 ebstein anomaly 10.2 TBX5 NKX2-5 GATA4
17 keratosis pilaris atrophicans faciei 10.1 SOS1 PTPN11 MAP2K1
18 testicular spermatocytic seminoma 10.1 PTPN11 HRAS
19 isolated congenitally uncorrected transposition of the great arteries 10.1 ZIC3 CFC1
20 mitral valve insufficiency 10.1 TBX5 NPPB FBN1
21 alagille syndrome 1 10.1 TBX5 NKX2-5 CFC1
22 cryptorchidism, unilateral or bilateral 10.1 SOS1 SHOC2 PTPN11 GATA4
23 interatrial communication 10.1 TBX5 NKX2-5 GATA6 GATA4
24 atrial septal defect 2 10.1 TBX5 PTPN11 NKX2-5 GATA4
25 noonan syndrome-like disorder with loose anagen hair 1 10.1 SHOC2 PTPN11 HRAS
26 intrinsic cardiomyopathy 10.1 NPPB NKX2-5 GATA4
27 lumbosacral lipoma 10.1 TBX5 LEFTY2
28 familial atrial fibrillation 10.0 TBX5 NKX2-5 GATA6 GATA4
29 optic nerve neoplasm 10.0 RASA2 LZTR1
30 diaphragmatic hernia, congenital 10.0 TBX5 GATA6 GATA4 FBN1
31 embryonal rhabdomyosarcoma 10.0 SOS1 PTPN11 HRAS
32 inguinal hernia 10.0 GATA6 GATA4 FBN1
33 atrioventricular block 10.0 TBX5 NPPB NKX2-5 GATA4
34 acneiform dermatitis 10.0 MAP2K1 HRAS
35 heart conduction disease 10.0 TBX5 NPPB NKX2-5 GATA4
36 noonan syndrome 3 10.0 SOS1 SHOC2 PTPN11 HRAS
37 noonan syndrome and noonan-related syndrome 9.9 SOS1 PTPN11 MAP2K2
38 pylorospasm 9.9 SOS2 RASA2 LZTR1 A2ML1
39 digeorge syndrome 9.9 TBX5 NKX2-5 LZTR1 GATA4
40 neurofibromatosis 9.9 SOS1 RASA2 PTPN11 HRAS
41 jacobsen syndrome 9.9 ZIC3 TBX5 NKX2-5 GATA4
42 cardiofaciocutaneous syndrome 3 9.9 MAP2K1 CFC1
43 contractures, pterygia, and spondylocarpotarsal fusion syndrome 1a 9.9 TBX5 PTPN11 HRAS FBN1
44 aortic valve disease 2 9.9 TBX5 NPPB NKX2-5 GATA4 FBN1
45 pulmonary atresia with intact ventricular septum 9.9
46 pulmonary hypertension 9.9
47 infective endocarditis 9.9
48 endocarditis 9.9
49 cardiac tamponade 9.9
50 tricuspid valve stenosis 9.9

Comorbidity relations with Pulmonary Valve Disease via Phenotypic Disease Network (PDN): (show all 13)


Acute Cystitis Aortic Valve Disease 1
Deficiency Anemia Familial Atrial Fibrillation
Heart Disease Hypertension, Essential
Intermediate Coronary Syndrome Mitral Valve Disease
Pulmonary Hypertension, Primary, 1 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:

46 (show all 11)
# Description MGI Source Accession Score Top Affiliating Genes
1 cardiovascular system MP:0005385 10.34 FBN1 GATA4 GATA6 HRAS LEFTY2 LZTR1
2 growth/size/body region MP:0005378 10.25 FBN1 GATA4 GATA6 HRAS LEFTY2 LZTR1
3 embryo MP:0005380 10.17 FBN1 GATA4 GATA6 LEFTY2 MAP2K1 NKX2-5
4 mortality/aging MP:0010768 10.17 FBN1 GATA4 GATA6 HRAS LEFTY2 LZTR1
5 craniofacial MP:0005382 10.13 FBN1 HRAS LZTR1 MAP2K1 MAP2K2 NKX2-5
6 digestive/alimentary MP:0005381 10.11 GATA4 HRAS LEFTY2 MAP2K1 MAP2K2 NKX2-5
7 integument MP:0010771 10.02 FBN1 GATA4 HRAS MAP2K1 MAP2K2 NKX2-5
8 muscle MP:0005369 9.91 FBN1 GATA4 GATA6 HRAS LZTR1 NKX2-5
9 normal MP:0002873 9.9 GATA4 GATA6 HRAS MAP2K1 MAP2K2 NKX2-5
10 respiratory system MP:0005388 9.61 FBN1 GATA4 GATA6 HRAS LEFTY2 NKX2-5
11 skeleton MP:0005390 9.32 FBN1 GATA4 HRAS LZTR1 MAP2K1 MAP2K2

Drugs & Therapeutics for Pulmonary Valve Disease

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 A Feasibility Study to Evaluate the Safety and Short-term Effectiveness of Implantation of Transcatheter Pulmonary Valve (TPV) for the Treatment of Congenital Heart Disease With Pulmonary Valve Disease Active, not recruiting NCT02555319
2 A Prospective, Multicenter, Single Arm Study to Evaluate the Safety and Effectiveness of Implantation of 'Transcatheter Pulmonary Valve (TPV)' for the Treatment of Congenital Heart Disease With Pulmonary Valve Disease Active, not recruiting NCT03110861

Search NIH Clinical Center for Pulmonary Valve Disease

Genetic Tests for Pulmonary Valve Disease

Anatomical Context for Pulmonary Valve Disease

MalaCards organs/tissues related to Pulmonary Valve Disease:

40
Heart, Skin

Publications for Pulmonary Valve Disease

Articles related to Pulmonary Valve Disease:

(show top 50) (show all 53)
# Title Authors PMID Year
1
Patient information portal for congenital aortic and pulmonary valve disease: a stepped-wedge cluster randomised trial. 61
33757975 2021
2
Balloon Valvuloplasty via the Pulmonary Artery Trunk for Treating Neonates With Severe Pulmonary Valve Disease. 61
33635246 2021
3
Echocardiographic assessment of the tricuspid and pulmonary valves: a practical guideline from the British Society of Echocardiography. 61
33339003 2020
4
Preclinical study of a self-expanding pulmonary valve for the treatment of pulmonary valve disease. 61
33365146 2020
5
Pulmonary valve infective endocarditis with atrial septal defect and pulmonary valve disease-too coincidental to be true? 61
32034668 2020
6
Neo-aortic Root Dilatation, Aortic Stiffness, and Ventricular interactions in Long-Term Follow-Up After the Ross Procedure in Childhood. 61
32367305 2020
7
Combined Transcatheter Tricuspid and Pulmonary Valve Replacement. 61
32645786 2020
8
Doppler gradients, valve area and ventricular function in pregnant women with aortic or pulmonary valve disease: Left versus right. 61
31785953 2020
9
Pulmonary endarteritis and endocarditis complicated with septic embolism: a case report and review of the literature. 61
32164584 2020
10
Successful transcatheter pulmonary valve implantation in a dog: first clinical report. 61
31785529 2019
11
Modified delivery of SAPIEN 3 valve in the pulmonary position: Evolution of technique or dire need? 61
30408317 2019
12
Patient and physician view on patient information and decision-making in congenital aortic and pulmonary valve surgery. 61
30487977 2018
13
Psychosocial and clinical outcomes of percutaneous versus surgical pulmonary valve implantation. 61
30018768 2018
14
Pulmonary valve and right ventricular outflow tract surgery in adults: 23-year experience. 61
29297190 2018
15
Epidemiology of valvular heart disease in a Swedish nationwide hospital-based register study. 61
28432156 2017
16
Imaging of the pulmonary valve in the adults. 61
28678047 2017
17
Development of an Online, Evidence-Based Patient Information Portal for Congenital Heart Disease: A Pilot Study. 61
28507990 2017
18
Abnormal Haemodynamic Flow Patterns in Bicuspid Pulmonary Valve Disease. 61
28620320 2017
19
Approach to residual pulmonary valve dysfunction in adults with repaired tetralogy of Fallot. 61
27329296 2016
20
Transcatheter implantation of SAPIEN 3 valve in native right ventricular outflow tract for severe pulmonary regurgitation following tetralogy of fallot repair. 61
26945801 2016
21
Routine intra-operative trans-oesophageal echocardiography yields better outcomes in surgical repair of CHD. 61
25730612 2016
22
Percutaneous pulmonary valve placement. 61
26175629 2015
23
CT and MRI of pulmonary valvular abnormalities. 61
24582177 2014
24
Importance of pulmonary valve disease during implantation of a biventricular assist device. 61
24268185 2014
25
Melody valve implantation in the pulmonary and tricuspid position. 61
23197462 2013
26
Cardiovascular disease in late survivors of tetralogy of fallot: a tertiary care center experience. 61
24082371 2013
27
Heart valve disease: investigation by cardiovascular magnetic resonance. 61
22260363 2012
28
Pulmonary valve interventions. 61
22059793 2011
29
Tricuspid and pulmonary valve disease evaluation and management. 61
21070730 2010
30
Late pulmonary valve replacement in congenital heart disease patients without original congenital pulmonary valve pathology. 61
19915895 2010
31
Experience and intermediate-term results using the Contegra heterograft for right ventricular outflow reconstruction in adults. 61
19638357 2009
32
Surgical advances in the treatment of adults with congenital heart disease. 61
19745740 2009
33
Experimental off-pump transventricular pulmonary valve replacement using a self-expandable valved stent: A new approach for pulmonary incompetence after repaired tetralogy of Fallot? 61
19379981 2009
34
Personalized pulmonary trunk modeling for intervention planning and valve assessment estimated from CT data. 61
20425966 2009
35
Finite element analysis of stent deployment: understanding stent fracture in percutaneous pulmonary valve implantation. 61
18042059 2007
36
[Reconstruction of the right ventricular outflow tract using a Medtronic Freestyle aortic root bioprosthesis]. 61
17580055 2007
37
Pulmonary valve replacement: extending the indications to the whole spectrum of pulmonary valve disease. 61
19804205 2007
38
Percutaneous pulmonary valve implantation. 61
16638543 2006
39
Percutaneous stent-mounted valve for treatment of aortic or pulmonary valve disease. 61
15343576 2004
40
Carcinoid syndrome: an unusual cause of tricuspid and pulmonary valve disease. 61
12876616 2003
41
Tricuspid and pulmonary valve involvement in carcinoid disease. 61
11198317 2000
42
Rheumatic pulmonary valve disease. 61
11273310 1998
43
Pregnancy in patients with pulmonary autograft valve replacement. 61
9347279 1997
44
Pulmonary autograft procedure for aortic valve disease: long-term results of the pioneer series. 61
9337191 1997
45
[Pulmonary valve disease]. 61
9117674 1996
46
[A transesophageal Doppler echo-color study of pulmonary venous flow before and after the correction of valvular defects]. 61
8076727 1994
47
Echocardiographic evaluation of isolated pulmonary valve disease in adolescents and adults. 61
10146329 1993
48
Valvular heart disease in the primary antiphospholipid syndrome. 61
1733383 1992
49
[Congenital heart defects and abnormalities newly detected with echocardiography in adolescents and adults]. 61
2251488 1990
50
[Heart involvement in carcinoid syndrome]. 61
2950393 1986

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

Pathways related to Pulmonary Valve Disease according to GeneCards Suite gene sharing:

(show top 50) (show all 137)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
13.61 ZIC3 SOS2 SOS1 RASA2 PTPN11 MAP2K2
2
Show member pathways
13.52 SOS2 SOS1 RASA2 MAP2K2 MAP2K1 LEFTY2
3
Show member pathways
13.18 SOS2 SOS1 MAP2K2 MAP2K1 LEFTY2 HRAS
4
Show member pathways
13.15 SOS2 SOS1 MAP2K2 MAP2K1 LEFTY2 HRAS
5
Show member pathways
12.97 SOS2 SOS1 MAP2K2 MAP2K1 HRAS
6
Show member pathways
12.97 SOS2 SOS1 PTPN11 MAP2K2 MAP2K1 HRAS
7
Show member pathways
12.95 SOS2 SOS1 NKX2-5 MAP2K2 MAP2K1 LEFTY2
8
Show member pathways
12.94 SOS2 SOS1 MAP2K2 MAP2K1 HRAS
9
Show member pathways
12.94 SOS2 SOS1 RASA2 MAP2K2 MAP2K1 HRAS
10 12.92 SOS2 SOS1 MAP2K2 MAP2K1 HRAS
11
Show member pathways
12.92 SOS2 SOS1 PTPN11 MAP2K2 MAP2K1 HRAS
12
Show member pathways
12.9 SOS2 SOS1 MAP2K2 MAP2K1 HRAS
13
Show member pathways
12.9 SOS2 SOS1 PTPN11 MAP2K2 MAP2K1 HRAS
14 12.89 SOS2 SOS1 RASA2 MAP2K2 MAP2K1 HRAS
15
Show member pathways
12.88 SOS2 SOS1 MAP2K2 MAP2K1 HRAS
16
Show member pathways
12.86 SOS2 SOS1 MAP2K2 MAP2K1 HRAS
17
Show member pathways
12.84 SOS2 SOS1 PTPN11 MAP2K2 MAP2K1 HRAS
18
Show member pathways
12.82 SOS2 SOS1 MAP2K2 MAP2K1 HRAS
19
Show member pathways
12.79 SOS2 SOS1 MAP2K2 MAP2K1 HRAS
20
Show member pathways
12.78 SOS2 SOS1 MAP2K2 MAP2K1 HRAS
21
Show member pathways
12.78 SOS2 SOS1 SHOC2 RASA2 PTPN11 MAP2K2
22
Show member pathways
12.77 SOS2 SOS1 MAP2K2 MAP2K1 HRAS GATA6
23
Show member pathways
12.76 SOS2 SOS1 MAP2K2 MAP2K1 HRAS
24 12.75 SOS2 SOS1 MAP2K2 MAP2K1 HRAS
25
Show member pathways
12.74 SOS2 SOS1 MAP2K2 MAP2K1 HRAS
26
Show member pathways
12.74 SOS2 SOS1 PTPN11 MAP2K2 MAP2K1 HRAS
27
Show member pathways
12.73 SOS2 SOS1 PTPN11 MAP2K2 MAP2K1 HRAS
28
Show member pathways
12.71 SOS2 SOS1 MAP2K2 MAP2K1 HRAS
29
Show member pathways
12.71 SOS2 SOS1 PTPN11 MAP2K2 MAP2K1 HRAS
30
Show member pathways
12.7 SOS2 SOS1 MAP2K2 MAP2K1 HRAS
31
Show member pathways
12.7 SOS2 SOS1 MAP2K2 MAP2K1 HRAS
32
Show member pathways
12.66 SOS2 SOS1 PTPN11 MAP2K2 MAP2K1 HRAS
33 12.65 SOS2 SOS1 MAP2K2 MAP2K1 HRAS
34
Show member pathways
12.65 SOS2 SOS1 PTPN11 MAP2K2 MAP2K1 HRAS
35
Show member pathways
12.63 SOS2 SOS1 MAP2K2 MAP2K1 HRAS
36
Show member pathways
12.62 SOS2 SOS1 MAP2K2 MAP2K1 HRAS
37
Show member pathways
12.61 SOS2 SOS1 PTPN11 MAP2K2 MAP2K1 HRAS
38
Show member pathways
12.6 TBX5 NPPB NKX2-5 LEFTY2 GATA4 CFC1
39
Show member pathways
12.59 ZIC3 LEFTY2 GATA6 GATA4
40
Show member pathways
12.57 SOS2 SOS1 PTPN11 MAP2K2 MAP2K1 HRAS
41
Show member pathways
12.56 NPPB MAP2K2 MAP2K1 HRAS
42
Show member pathways
12.53 SOS2 SOS1 MAP2K2 MAP2K1 HRAS
43
Show member pathways
12.53 SOS2 SOS1 PTPN11 MAP2K2 MAP2K1 HRAS
44
Show member pathways
12.52 SOS2 SOS1 MAP2K2 MAP2K1 LEFTY2 HRAS
45
Show member pathways
12.52 SOS2 SOS1 PTPN11 MAP2K2 MAP2K1 HRAS
46
Show member pathways
12.52 SOS2 SOS1 PTPN11 MAP2K2 MAP2K1 HRAS
47
Show member pathways
12.51 SOS2 SOS1 PTPN11 MAP2K2 MAP2K1 HRAS
48
Show member pathways
12.51 SOS2 SOS1 PTPN11 MAP2K2 MAP2K1 HRAS
49
Show member pathways
12.5 SOS2 SOS1 PTPN11 HRAS
50
Show member pathways
12.48 SOS2 SOS1 MAP2K2 MAP2K1 HRAS

GO Terms for Pulmonary Valve Disease

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

(show all 22)
# Name GO ID Score Top Affiliating Genes
1 positive regulation of transcription, DNA-templated GO:0045893 9.87 ZIC3 TBX5 NKX2-5 MAP2K2 MAP2K1 GATA6
2 positive regulation of ERK1 and ERK2 cascade GO:0070374 9.84 PTPN11 MAP2K1 HRAS GATA4
3 MAPK cascade GO:0000165 9.77 SOS1 RASA2 MAP2K2 MAP2K1 HRAS
4 Ras protein signal transduction GO:0007265 9.75 SOS1 SHOC2 HRAS
5 heart looping GO:0001947 9.69 ZIC3 NKX2-5 GATA4
6 heart development GO:0007507 9.63 TBX5 PTPN11 NKX2-5 MAP2K1 FBN1 CFC1
7 regulation of T cell proliferation GO:0042129 9.61 SOS2 SOS1
8 positive regulation of production of miRNAs involved in gene silencing by miRNA GO:1903800 9.6 MAP2K2 MAP2K1
9 endocardial cushion development GO:0003197 9.59 TBX5 GATA4
10 regulation of stress-activated MAPK cascade GO:0032872 9.58 MAP2K2 MAP2K1
11 intestinal epithelial cell differentiation GO:0060575 9.57 GATA6 GATA4
12 Bergmann glial cell differentiation GO:0060020 9.56 PTPN11 MAP2K1
13 lymphocyte homeostasis GO:0002260 9.55 SOS2 SOS1
14 regulation of early endosome to late endosome transport GO:2000641 9.52 MAP2K2 MAP2K1
15 positive regulation of small GTPase mediated signal transduction GO:0051057 9.49 SOS2 SOS1
16 regulation of T cell differentiation in thymus GO:0033081 9.48 SOS2 SOS1
17 regulation of pro-B cell differentiation GO:2000973 9.46 SOS2 SOS1
18 regulation of Golgi inheritance GO:0090170 9.43 MAP2K2 MAP2K1
19 atrial septum morphogenesis GO:0060413 9.43 TBX5 NKX2-5 GATA4
20 cerebellar cortex formation GO:0021697 9.4 PTPN11 MAP2K1
21 cardiac muscle cell differentiation GO:0055007 9.26 TBX5 NKX2-5 GATA6 GATA4
22 positive regulation of cardioblast differentiation GO:0051891 8.92 TBX5 NKX2-5 GATA6 GATA4

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

# Name GO ID Score Top Affiliating Genes
1 bacterial-type RNA polymerase transcriptional activator activity, sequence-specific DNA binding GO:0001216 9.16 NKX2-5 GATA4
2 sequence-specific DNA binding GO:0043565 9.02 ZIC3 TBX5 NKX2-5 GATA6 GATA4
3 MAP-kinase scaffold activity GO:0005078 8.96 MAP2K2 MAP2K1

Sources for Pulmonary Valve Disease

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
20 GARD
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
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
53 NINDS
54 Novoseek
56 OMIM via Orphanet
57 OMIM® (Updated 20-May-2021)
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 Tocris
70 UMLS
71 UMLS via Orphanet
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