MCID: PLM040
MIFTS: 39

Pulmonary Valve Disease

Categories: Cardiovascular 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 71

Classifications:



External Ids:

Disease Ontology 12 DOID:5749
ICD9CM 34 424.3
NCIt 49 C78579
SNOMED-CT 67 76267008
ICD10 32 I37 I37.9 I39.3
UMLS 71 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 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 72)
# Related Disease Score Top Affiliating Genes
1 pulmonary valve insufficiency 32.2 PDZK1IP1 NPPB CPB1
2 tricuspid valve insufficiency 29.4 PTPN11 PDZK1IP1 NPPB CPB1
3 heart valve disease 29.0 PDZK1IP1 NPPB NKX2-5 FBN1 CPB1
4 tricuspid valve disease 28.6 TBX5 PDZK1IP1 NPPB NKX2-5 GATA6 FBN1
5 heart septal defect 28.4 TBX5 SOS1 SHOC2 PTPN11 NKX2-5 LEFTY2
6 atrial heart septal defect 27.1 TBX5 SOS1 SHOC2 PTPN11 PDZK1IP1 NPPB
7 pulmonary valve stenosis 27.1 TBX5 SOS2 SOS1 SHOC2 RASA2 PTPN11
8 rheumatic pulmonary valve disease 12.5
9 atrial septal defect 9 11.2
10 malignant childhood germ cell neoplasm 10.5 GATA6 GATA4
11 complete atrioventricular septal defect with ventricular hypoplasia 10.4 GATA6 GATA4
12 partial atrioventricular canal 10.4 GATA6 GATA4
13 villonodular synovitis 10.3 SOS1 PTPN11
14 heart, malformation of 10.3 TBX5 NKX2-5
15 acute cor pulmonale 10.3 PDZK1IP1 NPPB
16 noonan syndrome 3 10.3 SOS1 SHOC2 PTPN11
17 acute pulmonary heart disease 10.3 PDZK1IP1 NPPB
18 germ cell and embryonal cancer 10.2 NKX2-5 GATA6 GATA4
19 hypoplastic right heart syndrome 10.2 TBX5 NKX2-5
20 jacobsen syndrome 10.2 TBX5 NKX2-5 GATA4
21 diaphragmatic hernia, congenital 10.2 TBX5 GATA6 GATA4
22 aortic valve insufficiency 10.1 PDZK1IP1 NPPB FBN1
23 cryptorchidism, unilateral or bilateral 10.1 SOS1 SHOC2 PTPN11 GATA4
24 hypogonadotropic hypogonadism 5 with or without anosmia 10.1 TBX5 NKX2-5 GATA6 GATA4
25 tricuspid atresia 10.1 TBX5 NKX2-5 GATA6 GATA4
26 eisenmenger syndrome 10.1 NKX2-5 GATA4
27 inguinal hernia 10.1 GATA6 GATA4 FBN1
28 wolff-parkinson-white syndrome 10.1 TBX5 NPPB NKX2-5
29 familial atrial fibrillation 10.1 TBX5 NKX2-5 GATA6 GATA4
30 intrinsic cardiomyopathy 10.0 NPPB NKX2-5 GATA4
31 optic nerve neoplasm 10.0 RASA2 LZTR1
32 total anomalous pulmonary venous return 1 10.0 TBX5 NKX2-5 GATA4 CFC1
33 noonan syndrome and noonan-related syndrome 10.0 SOS1 PTPN11 MAP2K2
34 atrioventricular block 10.0 TBX5 NPPB NKX2-5 GATA4
35 mitral valve stenosis 10.0 PDZK1IP1 NPPB CPB1
36 ventricular septal defect 9.9 TBX5 NPPB NKX2-5 GATA6 GATA4
37 pylorospasm 9.9 SOS2 RASA2 LZTR1 A2ML1
38 lentigines 9.9 PTPN11 MAP2K2 MAP2K1
39 right atrial isomerism 9.9 TBX5 NKX2-5 LEFTY2 CFC1
40 aortic valve disease 2 9.8 TBX5 NPPB NKX2-5 GATA4 FBN1
41 mitral valve disease 9.8 PDZK1IP1 NPPB FBN1 CPB1
42 pulmonary hypertension 9.8
43 infective endocarditis 9.8
44 endocarditis 9.8
45 tricuspid valve stenosis 9.8
46 carcinoid syndrome 9.8
47 aortic valve disease 1 9.7 TBX5 SOS1 NKX2-5 GATA6 GATA4 FBN1
48 digeorge syndrome 9.7 TBX5 NKX2-5 LZTR1 GATA4
49 ebstein anomaly 9.7 TBX5 NPPB NKX2-5 LEFTY2 GATA4
50 cardiofaciocutaneous syndrome 4 9.7 MAP2K2 CFC1

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:

45
# Description MGI Source Accession Score Top Affiliating Genes
1 cardiovascular system MP:0005385 10.21 FBN1 GATA4 GATA6 LEFTY2 LZTR1 MAP2K1
2 growth/size/body region MP:0005378 10.03 FBN1 GATA4 GATA6 LEFTY2 LZTR1 MAP2K1
3 embryo MP:0005380 10.02 FBN1 GATA4 GATA6 LEFTY2 MAP2K1 NKX2-5
4 craniofacial MP:0005382 9.95 FBN1 LZTR1 MAP2K1 MAP2K2 NKX2-5 PTPN11
5 digestive/alimentary MP:0005381 9.91 GATA4 LEFTY2 MAP2K1 MAP2K2 NKX2-5 PTPN11
6 muscle MP:0005369 9.76 FBN1 GATA4 GATA6 LZTR1 NKX2-5 PTPN11
7 normal MP:0002873 9.61 GATA4 GATA6 MAP2K1 MAP2K2 NKX2-5 PTPN11
8 respiratory system MP:0005388 9.17 FBN1 GATA4 GATA6 LEFTY2 NKX2-5 PTPN11

Drugs & Therapeutics for Pulmonary Valve Disease

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Review of Outcomes Following Pulmonary Valve Replacement for Congenital Heart Disease in Adults Completed NCT00276237
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
3 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
4 Adult Pulmonary Valve Replacement: A Simple and Reproducible Technique Terminated NCT00384163

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

Publications for Pulmonary Valve Disease

Articles related to Pulmonary Valve Disease:

(show all 48)
# Title Authors PMID Year
1
Doppler gradients, valve area and ventricular function in pregnant women with aortic or pulmonary valve disease: Left versus right. 61
31785953 2020
2
Neo-aortic Root Dilatation, Aortic Stiffness, and Ventricular interactions in Long-Term Follow-Up After the Ross Procedure in Childhood. 61
32367305 2020
3
Pulmonary endarteritis and endocarditis complicated with septic embolism: a case report and review of the literature. 61
32164584 2020
4
Pulmonary valve infective endocarditis with atrial septal defect and pulmonary valve disease-too coincidental to be true? 61
32034668 2020
5
Successful transcatheter pulmonary valve implantation in a dog: first clinical report. 61
31785529 2019
6
Modified delivery of SAPIEN 3 valve in the pulmonary position: Evolution of technique or dire need? 61
30408317 2019
7
Pulmonary valve and right ventricular outflow tract surgery in adults: 23-year experience. 61
29297190 2018
8
Psychosocial and clinical outcomes of percutaneous versus surgical pulmonary valve implantation. 61
30018768 2018
9
Patient and physician view on patient information and decision-making in congenital aortic and pulmonary valve surgery. 61
30487977 2018
10
Epidemiology of valvular heart disease in a Swedish nationwide hospital-based register study. 61
28432156 2017
11
Imaging of the pulmonary valve in the adults. 61
28678047 2017
12
Development of an Online, Evidence-Based Patient Information Portal for Congenital Heart Disease: A Pilot Study. 61
28507990 2017
13
Abnormal Haemodynamic Flow Patterns in Bicuspid Pulmonary Valve Disease. 61
28620320 2017
14
Approach to residual pulmonary valve dysfunction in adults with repaired tetralogy of Fallot. 61
27329296 2016
15
Transcatheter implantation of SAPIEN 3 valve in native right ventricular outflow tract for severe pulmonary regurgitation following tetralogy of fallot repair. 61
26945801 2016
16
Routine intra-operative trans-oesophageal echocardiography yields better outcomes in surgical repair of CHD. 61
25730612 2016
17
Percutaneous pulmonary valve placement. 61
26175629 2015
18
CT and MRI of pulmonary valvular abnormalities. 61
24582177 2014
19
Importance of pulmonary valve disease during implantation of a biventricular assist device. 61
24268185 2014
20
Melody valve implantation in the pulmonary and tricuspid position. 61
23197462 2013
21
Cardiovascular disease in late survivors of tetralogy of fallot: a tertiary care center experience. 61
24082371 2013
22
Heart valve disease: investigation by cardiovascular magnetic resonance. 61
22260363 2012
23
Pulmonary valve interventions. 61
22059793 2011
24
Tricuspid and pulmonary valve disease evaluation and management. 61
21070730 2010
25
Late pulmonary valve replacement in congenital heart disease patients without original congenital pulmonary valve pathology. 61
19915895 2010
26
Surgical advances in the treatment of adults with congenital heart disease. 61
19745740 2009
27
Experience and intermediate-term results using the Contegra heterograft for right ventricular outflow reconstruction in adults. 61
19638357 2009
28
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
29
Personalized pulmonary trunk modeling for intervention planning and valve assessment estimated from CT data. 61
20425966 2009
30
Finite element analysis of stent deployment: understanding stent fracture in percutaneous pulmonary valve implantation. 61
18042059 2007
31
[Reconstruction of the right ventricular outflow tract using a Medtronic Freestyle aortic root bioprosthesis]. 61
17580055 2007
32
Pulmonary valve replacement: extending the indications to the whole spectrum of pulmonary valve disease. 61
19804205 2007
33
Percutaneous pulmonary valve implantation. 61
16638543 2006
34
Percutaneous stent-mounted valve for treatment of aortic or pulmonary valve disease. 61
15343576 2004
35
Carcinoid syndrome: an unusual cause of tricuspid and pulmonary valve disease. 61
12876616 2003
36
Tricuspid and pulmonary valve involvement in carcinoid disease. 61
11198317 2000
37
Rheumatic pulmonary valve disease. 61
11273310 1998
38
Pregnancy in patients with pulmonary autograft valve replacement. 61
9347279 1997
39
Pulmonary autograft procedure for aortic valve disease: long-term results of the pioneer series. 61
9337191 1997
40
[Pulmonary valve disease]. 61
9117674 1996
41
[A transesophageal Doppler echo-color study of pulmonary venous flow before and after the correction of valvular defects]. 61
8076727 1994
42
Echocardiographic evaluation of isolated pulmonary valve disease in adolescents and adults. 61
10146329 1993
43
Valvular heart disease in the primary antiphospholipid syndrome. 61
1733383 1992
44
[Congenital heart defects and abnormalities newly detected with echocardiography in adolescents and adults]. 61
2251488 1990
45
[Heart involvement in carcinoid syndrome]. 61
2950393 1986
46
A case of congenital triple valve disease. 61
6657537 1983
47
Childhood rheumatic heart disease in Nigeria. 61
7245345 1981
48
Rheumatic pulmonary valve disease. 61
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

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

(show top 50) (show all 83)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
13.54 SOS2 SOS1 RASA2 PTPN11 MAP2K2 MAP2K1
2
Show member pathways
13.43 SOS2 SOS1 RASA2 MAP2K2 MAP2K1 LEFTY2
3
Show member pathways
13.11 SOS2 SOS1 MAP2K2 MAP2K1 LEFTY2 FBN1
4
Show member pathways
12.92 SOS2 SOS1 PTPN11 MAP2K2 MAP2K1
5
Show member pathways
12.89 SOS2 SOS1 PTPN11 MAP2K2 MAP2K1
6
Show member pathways
12.85 SOS2 SOS1 NKX2-5 MAP2K2 MAP2K1 LEFTY2
7
Show member pathways
12.81 SOS2 SOS1 PTPN11 MAP2K2 MAP2K1
8 12.8 SOS2 SOS1 RASA2 MAP2K2 MAP2K1
9
Show member pathways
12.76 SOS2 SOS1 PTPN11 MAP2K2 MAP2K1
10
Show member pathways
12.68 SOS2 SOS1 MAP2K2 MAP2K1 GATA6 GATA4
11
Show member pathways
12.67 SOS2 SOS1 PTPN11 MAP2K2 MAP2K1
12
Show member pathways
12.67 SOS2 SOS1 SHOC2 RASA2 PTPN11 MAP2K2
13
Show member pathways
12.65 SOS2 SOS1 PTPN11 MAP2K2 MAP2K1
14
Show member pathways
12.63 SOS2 SOS1 PTPN11 MAP2K2 MAP2K1
15
Show member pathways
12.61 SOS2 SOS1 PTPN11 MAP2K2 MAP2K1
16
Show member pathways
12.61 SOS2 SOS1 NPPB MAP2K2 MAP2K1 GATA4
17
Show member pathways
12.6 SOS2 SOS1 MAP2K2 MAP2K1
18
Show member pathways
12.56 NPPB MAP2K2 MAP2K1 GATA4
19
Show member pathways
12.56 SOS2 SOS1 PTPN11 MAP2K2 MAP2K1
20 12.55 SOS2 SOS1 MAP2K2 MAP2K1
21
Show member pathways
12.55 SOS2 SOS1 PTPN11 MAP2K2 MAP2K1
22
Show member pathways
12.52 SOS2 SOS1 MAP2K2 MAP2K1
23
Show member pathways
12.52 SOS2 SOS1 MAP2K2 MAP2K1
24
Show member pathways
12.52 SOS2 SOS1 PTPN11 MAP2K2 MAP2K1
25
Show member pathways
12.5 SOS2 SOS1 PTPN11 MAP2K2 MAP2K1
26
Show member pathways
12.49 SOS2 SOS1 PTPN11 MAP2K2 MAP2K1
27
Show member pathways
12.45 SOS2 SOS1 PTPN11 MAP2K2 MAP2K1
28
Show member pathways
12.45 TBX5 NPPB NKX2-5 LEFTY2 GATA4 CFC1
29
Show member pathways
12.44 SOS2 SOS1 MAP2K2 MAP2K1 LEFTY2
30
Show member pathways
12.44 SOS2 SOS1 PTPN11 MAP2K2 MAP2K1
31
Show member pathways
12.43 SOS2 SOS1 MAP2K2 MAP2K1
32
Show member pathways
12.43 SOS2 SOS1 PTPN11 MAP2K2 MAP2K1
33
Show member pathways
12.42 SOS2 SOS1 PTPN11 MAP2K2 MAP2K1
34
Show member pathways
12.41 SOS2 SOS1 MAP2K2 MAP2K1
35
Show member pathways
12.41 SOS2 SOS1 PTPN11 MAP2K2 MAP2K1
36
Show member pathways
12.37 SOS2 SOS1 MAP2K2 MAP2K1
37
Show member pathways
12.36 SOS2 SOS1 MAP2K2 MAP2K1
38
Show member pathways
12.36 SOS2 SOS1 MAP2K2 MAP2K1
39
Show member pathways
12.36 SOS2 SOS1 MAP2K2 MAP2K1
40
Show member pathways
12.35 SOS2 SOS1 PTPN11 MAP2K2 MAP2K1
41 12.32 SOS2 SOS1 PTPN11 MAP2K2 MAP2K1
42
Show member pathways
12.3 SOS2 SOS1 PTPN11 MAP2K2 MAP2K1
43
Show member pathways
12.25 SOS2 SOS1 MAP2K2 MAP2K1
44
Show member pathways
12.23 SOS1 PTPN11 MAP2K2 MAP2K1
45
Show member pathways
12.22 NPPB MAP2K2 MAP2K1 GATA4
46
Show member pathways
12.19 SOS2 SOS1 MAP2K2 MAP2K1
47 12.19 SOS2 SOS1 MAP2K2 MAP2K1 LEFTY2
48 12.17 SOS2 SOS1 PTPN11 MAP2K2 MAP2K1
49
Show member pathways
12.13 SOS2 SOS1 MAP2K2 MAP2K1
50
Show member pathways
12.12 SOS1 PTPN11 MAP2K2 MAP2K1

GO Terms for Pulmonary Valve Disease

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

(show all 18)
# Name GO ID Score Top Affiliating Genes
1 positive regulation of transcription, DNA-templated GO:0045893 9.93 TBX5 NKX2-5 MAP2K2 MAP2K1 GATA6 GATA4
2 MAPK cascade GO:0000165 9.88 SOS1 RASA2 MAP2K2 MAP2K1
3 heart development GO:0007507 9.63 TBX5 PTPN11 NKX2-5 MAP2K1 FBN1 CFC1
4 endocardial cushion development GO:0003197 9.57 TBX5 GATA4
5 positive regulation of production of miRNAs involved in gene silencing by miRNA GO:1903800 9.56 MAP2K2 MAP2K1
6 regulation of stress-activated MAPK cascade GO:0032872 9.55 MAP2K2 MAP2K1
7 cardiac ventricle morphogenesis GO:0003208 9.54 NKX2-5 GATA4
8 intestinal epithelial cell differentiation GO:0060575 9.52 GATA6 GATA4
9 Bergmann glial cell differentiation GO:0060020 9.51 PTPN11 MAP2K1
10 cardiac muscle tissue development GO:0048738 9.5 NKX2-5 GATA6 GATA4
11 regulation of early endosome to late endosome transport GO:2000641 9.49 MAP2K2 MAP2K1
12 positive regulation of small GTPase mediated signal transduction GO:0051057 9.48 SOS2 SOS1
13 regulation of Golgi inheritance GO:0090170 9.43 MAP2K2 MAP2K1
14 atrial septum morphogenesis GO:0060413 9.43 TBX5 NKX2-5 GATA4
15 cerebellar cortex formation GO:0021697 9.4 PTPN11 MAP2K1
16 bundle of His development GO:0003166 9.37 TBX5 NKX2-5
17 cardiac muscle cell differentiation GO:0055007 9.26 TBX5 NKX2-5 GATA6 GATA4
18 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 MAP kinase kinase activity GO:0004708 8.96 MAP2K2 MAP2K1
2 MAP-kinase scaffold activity GO:0005078 8.62 MAP2K2 MAP2K1

Sources for Pulmonary Valve Disease

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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