MCID: PRP101
MIFTS: 20

Peripheral Pulmonary Stenosis

Categories: Cardiovascular diseases, Fetal diseases, Rare diseases

Aliases & Classifications for Peripheral Pulmonary Stenosis

MalaCards integrated aliases for Peripheral Pulmonary Stenosis:

Name: Peripheral Pulmonary Stenosis 59
Peripheral Pulmonary Artery Stenosis 73
Branch Pulmonary Artery Stenosis 59
Pulmonary Branch Stenosis 59

Classifications:



External Ids:

Orphanet 59 ORPHA99084
ICD10 via Orphanet 34 Q25.6
UMLS 73 C0345030

Summaries for Peripheral Pulmonary Stenosis

MalaCards based summary : Peripheral Pulmonary Stenosis, also known as peripheral pulmonary artery stenosis, is related to pulmonary branches stenosis and keutel syndrome. An important gene associated with Peripheral Pulmonary Stenosis is MGP (Matrix Gla Protein). Affiliated tissues include lung and liver.

Related Diseases for Peripheral Pulmonary Stenosis

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

(show all 14)
# Related Disease Score Top Affiliating Genes
1 pulmonary branches stenosis 12.3
2 keutel syndrome 11.7
3 alagille syndrome 1 11.6
4 alagille syndrome 2 11.3
5 tetralogy of fallot 10.2
6 supravalvular aortic stenosis 10.1
7 pulmonary valve insufficiency 10.1
8 williams-beuren syndrome 10.1
9 ventricular septal defect 9.9
10 aortitis 9.9
11 transposition of the great arteries 9.9
12 pulmonary supravalvular stenosis 9.8
13 takayasu arteritis 9.8
14 cholestasis 9.8

Graphical network of the top 20 diseases related to Peripheral Pulmonary Stenosis:



Diseases related to Peripheral Pulmonary Stenosis

Symptoms & Phenotypes for Peripheral Pulmonary Stenosis

Drugs & Therapeutics for Peripheral Pulmonary Stenosis

Search Clinical Trials , NIH Clinical Center for Peripheral Pulmonary Stenosis

Genetic Tests for Peripheral Pulmonary Stenosis

Anatomical Context for Peripheral Pulmonary Stenosis

MalaCards organs/tissues related to Peripheral Pulmonary Stenosis:

41
Lung, Liver

Publications for Peripheral Pulmonary Stenosis

Articles related to Peripheral Pulmonary Stenosis:

(show top 50) (show all 66)
# Title Authors Year
1
Nonsyndromic Peripheral Pulmonary Artery Stenosis Is Associated With Homozygosity of RNF213 p.Arg4810Lys Regardless of Co-occurrence of Moyamoya Disease. ( 28962888 )
2018
2
Computational simulation of postoperative pulmonary flow distribution in Alagille patients with peripheral pulmonary artery stenosis. ( 29194961 )
2018
3
Surgical Techniques for Repair of Peripheral Pulmonary Artery Stenosis. ( 28823329 )
2017
4
Intravascular imaging-guided percutaneous transluminal pulmonary angioplasty for peripheral pulmonary stenosis and pulmonary Takayasu arteritis. ( 26776920 )
2016
5
Adaptive outflow boundary conditions improve post-operative predictions after repair of peripheral pulmonary artery stenosis. ( 26843118 )
2016
6
Surgical Technique for Repair of Peripheral Pulmonary Artery Stenosis and Other Complex Peripheral Reconstructions. ( 27449462 )
2016
7
Surgical Techniques for Repair of Peripheral Pulmonary Artery Stenosis. ( 28043454 )
2016
8
Case images: D-transposition of the great arteries with bilateral peripheral pulmonary artery stenosis and arcus hypoplasia: A rare association. ( 26142800 )
2015
9
Peripheral pulmonary artery stenosis in three cats. ( 25650057 )
2015
10
Peripheral pulmonary artery stenosis as a cause of pulmonary hypertension in adults. ( 25992283 )
2015
11
Classical Supravalvar Aortic Stenosis and Peripheral Pulmonary Stenosis. ( 26236365 )
2014
12
Peripheral pulmonary artery stenosis: an unusual case and discussion of genetic associations. ( 24965779 )
2014
13
Surgical reconstruction of peripheral pulmonary artery stenosis in Williams and Alagille syndromes. ( 23228407 )
2013
14
Outcomes after primary transcatheter therapy in infants and young children with severe bilateral peripheral pulmonary artery stenosis. ( 23941859 )
2013
15
Peripheral pulmonary artery stenosis masquerading as pulmonary hypertension: a diagnostic and therapeutic challenge. ( 22848086 )
2012
16
Effects of stent implantation for peripheral pulmonary artery stenosis on pulmonary vascular hemodynamics and right ventricular function in a patient with repaired tetralogy of Fallot. ( 21416114 )
2011
17
Agitated saline contrast use in a case with peripheral pulmonary artery stenosis. ( 21983770 )
2011
18
How we approach peripheral pulmonary stenosis in Williams-Beuren syndrome. ( 19349025 )
2009
19
Demonstration of peripheral pulmonary stenosis and supravalvular aortic stenosis by different cardiac imaging modalities in a patient with Williams syndrome--usefulness of noninvasive imaging studies. ( 17945365 )
2008
20
Comparison of efficacy of medium-sized cutting balloons versus standard balloons for dilation of peripheral pulmonary stenosis. ( 16563916 )
2006
21
Severe peripheral pulmonary artery stenosis is not a contraindication to liver transplantation in Alagille syndrome. ( 16499598 )
2006
22
Peripheral pulmonary artery stenosis in a four-month-old West Highland White Terrier. ( 16863051 )
2006
23
Visualization of peripheral pulmonary artery stenosis using high-resolution multidetector computed tomography. ( 16235778 )
2005
24
Bladed balloon angioplasty for peripheral pulmonary artery stenosis. ( 15103608 )
2004
25
Transcatheter implantation of intravascular stents for postoperative residual stenosis of peripheral pulmonary artery stenosis. ( 15378111 )
2004
26
Interventional strategies in the management of peripheral pulmonary artery stenosis. ( 12768922 )
2003
27
Supravalvular aortic stenosis and peripheral pulmonary stenosis coexisting with a straight thoracic spine. ( 12030351 )
2002
28
Lung perfusion scintigraphy in the diagnosis of peripheral pulmonary stenosis in patients after repair of Fallot tetralogy. ( 14600940 )
2002
29
Interventional catheterization management of perioperative peripheral pulmonary stenosis: balloon angioplasty or endovascular stenting. ( 12112928 )
2002
30
Factors determining peripheral pulmonary artery stenosis remodeling in children after percutaneous transluminal balloon angioplasty. ( 11954947 )
2002
31
Balloon dilation angioplasty of peripheral pulmonary stenosis associated with Williams syndrome. ( 11331257 )
2001
32
Case 2. Congenital multiple peripheral pulmonary artery stenosis (Pulmonary branch stenosis or supravalvular pulmonary stenosis). ( 10954480 )
2000
33
Peripheral pulmonary stenosis murmurs. ( 10693479 )
1999
34
Images in cardiology: Cutting balloon for treatment of severe peripheral pulmonary stenosis in a child. ( 10377323 )
1999
35
Long-term effect of a new oral prostacyclin analogue, beraprost sodium, in isolated peripheral pulmonary artery stenosis in the adult. ( 10447792 )
1999
36
Balloon pulmonary valvuloplasty and stent implantation. For peripheral pulmonary artery stenosis in Alagille syndrome. ( 9566070 )
1998
37
Endovascular treatment of life-threatening peripheral pulmonary artery stenosis. ( 9840042 )
1998
38
Congenital total lipodystrophy and peripheral pulmonary artery stenosis. ( 9196367 )
1997
39
Congenital total lipodystrophy and peripheral pulmonary artery stenosis. ( 9487983 )
1997
40
Spontaneous regression of peripheral pulmonary artery stenosis in Williams syndrome. ( 8803725 )
1996
41
Idiopathic peripheral pulmonary artery stenosis: an unusual cause of ventilation-perfusion mismatch. ( 7658219 )
1995
42
Concurrence of supravalvular aortic stenosis and peripheral pulmonary stenosis in three generations of a family: a form of arterial dysplasia. ( 8456853 )
1993
43
Quantitative perfusion imaging assessing acquired discrete peripheral pulmonary artery stenosis. ( 8229249 )
1993
44
Peripheral pulmonary artery stenosis in pregnancy. A report of two cases. ( 1593566 )
1992
45
Closure of the ductus arteriosus: determinant factor in the appearance of transient peripheral pulmonary stenosis of the neonate. ( 1960617 )
1991
46
Hemodynamic confirmation of peripheral pulmonary stenosis caused by aortic dissection. ( 2729120 )
1989
47
The natural course of supravalvar aortic stenosis and peripheral pulmonary artery stenosis in Williams's syndrome. ( 2803879 )
1989
48
Combined factor VII and protein C deficiency found in a patient with peripheral pulmonary artery stenosis accompanied by progressive pulmonary hypertension and hemoptysis. ( 3187953 )
1988
49
Transient unilateral pulmonary edema after successful balloon dilation of peripheral pulmonary artery stenosis. ( 3400614 )
1988
50
Percutaneous angioplasty for peripheral pulmonary stenosis in an adult. ( 2949577 )
1987

Variations for Peripheral Pulmonary Stenosis

ClinVar genetic disease variations for Peripheral Pulmonary Stenosis:

6
# Gene Variation Type Significance SNP ID Assembly Location
1 DLX3 NM_005220.2(DLX3): c.574dupG (p.Glu192Glyfs) duplication Likely pathogenic rs1057518764 GRCh38 Chromosome 17, 49991807: 49991807
2 DLX3 NM_005220.2(DLX3): c.574dupG (p.Glu192Glyfs) duplication Likely pathogenic rs1057518764 GRCh37 Chromosome 17, 48069171: 48069171

Expression for Peripheral Pulmonary Stenosis

Search GEO for disease gene expression data for Peripheral Pulmonary Stenosis.

Pathways for Peripheral Pulmonary Stenosis

GO Terms for Peripheral Pulmonary Stenosis

Sources for Peripheral Pulmonary Stenosis

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 ExPASy
19 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 LOVD
42 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
54 NINDS
55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 SNOMED-CT via Orphanet
71 TGDB
72 Tocris
73 UMLS
74 UMLS via Orphanet
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