MCID: PLM068
MIFTS: 41

Pulmonary Vein Stenosis malady

Rare diseases, Cardiovascular diseases categories

Summaries for Pulmonary Vein Stenosis

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44NIH Rare Diseases, 34MalaCards
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NIH Rare Diseases:44 Pulmonary vein stenosis is a very rare and serious condition in which there is a blockage in the blood vessels that bring oxygen-rich blood from the lungs back to the heart. this condition can be isolated to one vein, but often affects multiple veins. stenosis occurs when there is an abnormal thickening and narrowing of the walls of the veins. pulmonary vein stenosis is a progressive condition and may lead to total obstruction to a blood vessel. most commonly, all of the pulmonary veins of one lung are affected, causing pulmonary hypertension and pulmonary arterial hypertension. surgery and catheterization to widen the narrow veins is usually a short-term solution since the obstruction typically recurs. last updated: 9/1/2011

MalaCards: Pulmonary Vein Stenosis, also known as pulmonary veins stenosis, is related to hypertension and cor triatriatum. An important gene associated with Pulmonary Vein Stenosis is ACTC1 (actin, alpha, cardiac muscle 1), and among its related pathways are Striated Muscle Contraction and Cardiac Progenitor Differentiation. The compounds orcein and s 100 have been mentioned in the context of this disorder. Affiliated tissues include lung and heart, and related mouse phenotypes are skeleton and muscle.

Aliases & Classifications for Pulmonary Vein Stenosis

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44NIH Rare Diseases, 63UMLS
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Classifications:

Malacards categories (disease lists): (See all malacards categories)
Global: Rare diseases
Anatomical: Cardiovascular diseases


Aliases & Descriptions:

pulmonary vein stenosis 44 63
pulmonary veins stenosis 44


Related Diseases for Pulmonary Vein Stenosis

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Sources:
18GeneCards, 19GeneDecks
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Diseases related to Pulmonary Vein Stenosis via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show top 50)    (show all 95)
idRelated DiseaseScoreTop Affiliating Genes
1hypertension10.4
2cor triatriatum10.4
3congenital heart disease10.3
4mediastinitis10.3
5pulmonary embolism10.3
6fibrosing mediastinitis10.3
7transposition of the great arteries10.3
8scimitar syndrome10.1
9tetralogy of fallot10.1
10patent ductus arteriosus10.1
11thoracic aortic aneurysm10.1
12down syndrome10.1
13primary pulmonary hypertension10.1
14aortic aneurysm10.1
15esophagitis10.1
16lung cancer10.1
17bronchogenic cyst10.1
18eisenmenger syndrome10.1
19heterotaxy10.1
20aneurysm10.1
21fasciitis10.1ACTC1
22peripheral primitive neuroectodermal tumor10.1CD99
23infantile myofibromatosis10.1DES
24papilloma10.1ACTC1
25carney triad10.1PDGFRA
26pyogenic granuloma10.1PECAM1, ACTC1
27lymphangioma10.1PECAM1, ACTC1
28adenosarcoma10.1ACTC1, DES
29epithelioid leiomyosarcoma10.1DES, ACTC1
30pseudosarcomatous fibromatosis10.1DES, ACTC1
31spindle cell carcinoma10.1DES, ACTC1
32spindle cell sarcoma10.1DES, ACTC1
33teratoma10.1CD99, ACTC1
34cutaneous fibrous histiocytoma10.1ACTC1, DES
35dermatofibrosarcoma protuberans10.1ACTC1, DES
36fibrous histiocytoma10.1DES, ACTC1
37rhabdoid tumor10.1DES, ACTC1
38hemangiopericytoma10.1DES, ACTC1
39angiomyolipoma10.1DES, ACTC1
40embryonal rhabdomyosarcoma10.1ACTC1, DES
41glomus tumor10.1ACTC1, DES
42malignant mixed mullerian tumor10.1ACTC1, DES
43fibroma10.1ACTC1, DES
44fibromatosis10.1ACTC1, DES
45abrikosov's tumor10.1DES, ACTC1
46giant cell tumor10.1ACTC1, DES
47mucoepidermoid carcinoma10.1ACTC1, MKI67
48pleomorphic adenoma10.0DES, ACTC1
49adenoid cystic carcinoma10.0MKI67, ACTC1
50hemangioma10.0PECAM1, ACTC1

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



Diseases related to pulmonary vein stenosis

Symptoms for Pulmonary Vein Stenosis

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Drugs & Therapeutics for Pulmonary Vein Stenosis

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Sources:
6CenterWatch, 43NIH Clinical Center, 7ClinicalTrials
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Approved drugs:

Search CenterWatch for Pulmonary Vein Stenosis

Drug clinical trials:

Search ClinicalTrials for Pulmonary Vein Stenosis

Search NIH Clinical Center for Pulmonary Vein Stenosis

Search CenterWatch for Pulmonary Vein Stenosis

Genetic Tests for Pulmonary Vein Stenosis

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Anatomical Context for Pulmonary Vein Stenosis

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34MalaCards
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MalaCards organs/tissues related to Pulmonary Vein Stenosis:

34
Lung, Heart

Animal Models for Pulmonary Vein Stenosis or affiliated genes

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38MGI
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MGI Mouse Phenotypes related to Pulmonary Vein Stenosis:

38
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053908.5PDGFRA, TYMP, PECAM1, F2RL1
2MP:00053698.5DES, PECAM1, ACTC1, PDGFRA
3MP:00053858.3F2RL1, DES, PECAM1, ACTC1, PDGFRA
4MP:00053847.9F2RL1, PECAM1, TYMP, ACTC1, PDGFRA
5MP:00053767.8PDGFRA, ACTC1, TYMP, PECAM1, DES, F2RL1

Publications for Pulmonary Vein Stenosis

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53PubMed
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Articles related to Pulmonary Vein Stenosis:

(show top 50)    (show all 175)
idTitleAuthorsYear
1
Congenital pulmonary vein stenosis in an adult patient. (24184251)
2014
2
Hemoptysis from bronchial varices associated with pulmonary vein stenosis: role of surgical repair. (22997173)
2013
3
Severe pulmonary vein stenosis due to invasion of metastatic lung cancer. (23443870)
2013
4
Pulmonary Vein Stenosis with Down Syndrome: A Rare and Frequently Fatal Cause of Pulmonary Hypertension in Infants and Children. (23650870)
2013
5
Proximal pulmonary vein stenosis detection in pediatric patients: value of multiplanar and 3-D VR imaging evaluation. (23475282)
2013
6
Haemoptysis and pulmonary vein stenosis after ablation for atrial fibrillation: pathophysiology and therapeutic options. (23561921)
2013
7
Angioplasty of acquired pulmonary vein stenosis using covered stent. (23592594)
2013
8
Relentless pulmonary vein stenosis: A contemporary approach to a recurring problem. (23436711)
2013
9
Bifurcation angioplasty using drug eluting stents of post-AF ablation severe pulmonary vein stenosis. (22394418)
2012
10
Percutaneous stenting to treat pulmonary vein stenosis after single-lung transplantation. (22949779)
2012
11
Sutureless technique for recurrent pulmonary vein stenosis after pericardial patchplasty in an adult. (22901499)
2012
12
Successful correction of unroofed coronary sinus with pulmonary vein stenosis. (22753625)
2012
13
Isolated congenital left-sided pulmonary vein stenosis in an adult patient misdiagnosed as primary pulmonary hypertension. (21349107)
2011
14
Surgical management of post-repair pulmonary vein stenosis. (21444056)
2011
15
Evaluation of pulmonary vein stenosis after pulmonary vein isolation using a novel circular mapping and ablation catheter (PVAC). (21841188)
2011
16
Cardiopulmonary hemodynamic clues for pulmonary vein stenosis diagnosis. (22530105)
2011
17
Unmasking of patent ductus arteriosus on cardiopulmonary bypass: role of intraoperative trans-esophageal echocardiography in a patient with severe pulmonary hypertension due to pulmonary vein stenosis and cor triatriatum. (21636939)
2011
18
Acquired pulmonary vein stenosis: one problem, two mechanisms. (20138470)
2010
19
Hypoplastic right lung associated with right pulmonary vein stenosis and systemic collateral. (20136863)
2010
20
Multimodality imaging assessment of anatomic and functional pulmonary vein stenosis. (19808631)
2009
21
Importance of totally anomalous pulmonary venous connection and postoperative pulmonary vein stenosis in outcomes of heterotaxy syndrome. (18004616)
2008
22
Pulmonary vein stenosis: intervene early and carry a big stent. (18373597)
2008
23
Primary pulmonary vein stenosis. (17293205)
2007
24
Detection of pulmonary vein stenosis by transesophageal echocardiography: comparison with multidetector computed tomography. (17452156)
2007
25
Transesophageal echocardiography: a follow-up tool after catheter ablation of atrial fibrillation and interventional therapy of pulmonary vein stenosis and occlusion. (17458690)
2007
26
Management of congenital and acquired pulmonary vein stenosis. (16488708)
2006
27
Acquired isolated left pulmonary vein stenosis: a complication of bronchogenic cyst removal diagnosed by transesophageal echocardiography. (16412190)
2006
28
Temporary pulmonary vein stenosis during intraoperative transesophageal echocardiography in total cavopulmonary connection. (16235015)
2006
29
Usefulness of helical computed tomography in diagnosing pulmonary vein stenosis in infants. (16049562)
2005
30
Pulmonary haemodynamics at rest and during exercise in patients with significant pulmonary vein stenosis after radiofrequency catheter ablation for drug resistant atrial fibrillation. (15855192)
2005
31
Decreased pulmonary perfusion in pulmonary vein stenosis after radiofrequency ablation: assessment with dynamic magnetic resonance perfusion imaging. (15302728)
2004
32
A case of congenital pulmonary vein stenosis in an adult. (14872119)
2004
33
Pulmonary vein isolation using transvenous catheter cryoablation for treatment of atrial fibrillation without risk of pulmonary vein stenosis. (12932615)
2003
34
Transcatheter angioplasty for acquired pulmonary vein stenosis after radiofrequency ablation. (12952852)
2003
35
Pulmonary vein stenosis by ostial irrigated-tip ablation: incidence, time course, and prediction. (12693497)
2003
36
Pulmonary vein stenosis after catheter ablation of atrial fibrillation: emergence of a new clinical syndrome. (12693885)
2003
37
Successful stent treatment of pulmonary vein stenosis following atrial fibrillation radiofrequency ablation. (12082196)
2002
38
Congenital pulmonary vein stenosis associated with cor triatriatum. (11235744)
2001
39
Left upper pulmonary vein stenosis 2 months after radiofrequency catheter ablation of atrial fibrillation. (10747360)
2000
40
Pulmonary vein stenosis complicating catheter ablation of focal atrial fibrillation. (10868741)
2000
41
Editorial comment: can we avoid pulmonary vein stenosis following ablation of atrial fibrillation? (11141210)
2000
42
Pulmonary vein stenosis with normal connection: associated cardiac abnormalities and variable outcome. (10421134)
1999
43
Pulmonary vein stenosis after catheter ablation of atrial fibrillation. (9788832)
1998
44
Lung transplantation for congenital pulmonary vein stenosis. (7574992)
1995
45
Pulsed wave and color Doppler findings in congenital pulmonary vein stenosis. (1614915)
1992
46
Radionuclide evaluation of unilateral pulmonary vein stenosis. Correlation with chest roentgenogram and clinical profiles. (3295141)
1986
47
Congenital pulmonary vein stenosis: a radiographic study. (3763853)
1986
48
Unilateral pulmonary vein stenosis. (3718800)
1986
49
Left pulmonary vein stenosis associated with transposition of the great arteries. (3947174)
1986
50
Congenital pulmonary vein stenosis. (6465018)
1984

Variations for Pulmonary Vein Stenosis

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Expression for genes affiliated with Pulmonary Vein Stenosis

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Sources:
2BioGPS, 16Gene Expression Omnibus DataSets
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Expression patterns in normal tissues for genes affiliated with Pulmonary Vein Stenosis

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

Pathways for genes affiliated with Pulmonary Vein Stenosis

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51PathCards, 39NCBI BioSystems Database, 56Reactome, 5Cell Signaling Technology
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Pathways related to Pulmonary Vein Stenosis according to GeneCards/GeneDecks:

idSuper pathways (with members indented)ScoreTop Affiliating Genes
1
Show member pathways
9.7ACTC1, DES
29.5PDGFRA, ACTC1
39.3F2RL1, DES, ACTC1
48.5PDGFRA, TYMP, PECAM1, F2RL1

Compounds for genes affiliated with Pulmonary Vein Stenosis

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46Novoseek, 25HMDB, 30IUPHAR, 62Tocris Bioscience, 52PharmGKB, 12DrugBank
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Compounds related to Pulmonary Vein Stenosis according to GeneCards/GeneDecks:

(show all 48)
idCompoundScoreTop Affiliating Genes
1orcein4610.1ACTC1, DES
2s 1004610.1ACTC1, DES
3reticulin4610.0DES, ACTC1
4mucicarmine4610.0ACTC1, DES
5b 7234610.0ACTC1, DES
6percoll469.9DES, PECAM1
7procollagen469.6DES, ACTC1
8prostacyclin469.5TYMP, PECAM1, F2RL1
9matrigel469.5PECAM1, TYMP, ACTC1
10methanol46 2510.5ACTC1, MKI67
11fibrinogen469.5F2RL1, DES, PECAM1
12cyclosporin a46 30 6211.5ACTC1, PECAM1, F2RL1
13streptavidin469.5MKI67, DES
14deoxyuridine46 2510.5MKI67, TYMP
15vinorelbine46 52 1211.5MKI67, TYMP
16p00001469.4MKI67, TYMP
17imatinib46 52 1211.4PDGFRA, ACTC1, DES
18octreotide46 62 30 1212.4MKI67, F2RL1
19biotin46 25 1211.2DES, MKI67, ACTC1
20heparin46 30 25 1212.2PECAM1, DES, F2RL1
21creatinine469.2DES, TYMP, ACTC1
22p005469.1MKI67, TYMP, PECAM1
23irinotecan46 52 1211.1MKI67, TYMP
24actinomycin d469.1F2RL1, DES, PECAM1, TYMP
25glycogen46 2510.1DES, MKI67, ACTC1
26cysteine469.0F2RL1, DES, PECAM1, TYMP
27lysine469.0F2RL1, DES, MKI67
28doxorubicin46 52 1210.9MKI67, TYMP, DES
29silver468.9MKI67, DES
30folate468.9TYMP, MKI67, PDGFRA
31hematoxylin468.9DES, PECAM1, MKI67, ACTC1
32estradiol46 25 1210.9DES, TYMP, MKI67
33p003468.8F2RL1, PECAM1, TYMP, MKI67
34cycloheximide468.7F2RL1, PECAM1, TYMP, MKI67
35progesterone46 30 62 25 1212.7DES, TYMP, MKI67, ACTC1
36p002468.7DES, PECAM1, TYMP, MKI67
37formaldehyde46 259.4DES, PECAM1, TYMP, MKI67, ACTC1
38nitric oxide46 25 1210.3F2RL1, DES, PECAM1, TYMP, MKI67
39serine468.3F2RL1, DES, PECAM1, TYMP, MKI67
40retinoic acid46 259.2DES, PECAM1, MKI67, ACTC1, PDGFRA
41oxygen46 259.0F2RL1, DES, PECAM1, TYMP, MKI67, ACTC1
42glucose468.0F2RL1, DES, PECAM1, TYMP, MKI67, ACTC1
43calcium46 52 25 1210.8F2RL1, DES, PECAM1, MKI67, ACTC1, PDGFRA
44estrogen467.7DES, PECAM1, TYMP, MKI67, ACTC1, PDGFRA
45thymidine46 258.7F2RL1, DES, PECAM1, TYMP, MKI67, PDGFRA
46tyrosine467.4F2RL1, DES, PECAM1, TYMP, MKI67, ACTC1
47paraffin466.8CD99, F2RL1, DES, PECAM1, TYMP, MKI67
48vegf466.8F2RL1, DES, PECAM1, TYMP, MKI67, ACTC1

GO Terms for genes affiliated with Pulmonary Vein Stenosis

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17Gene Ontology
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Biological processes related to Pulmonary Vein Stenosis according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1positive regulation of phosphatidylinositol 3-kinase signalingGO:0140689.5PDGFRA, F2RL1
2cardiac myofibril assemblyGO:0550039.4PDGFRA, ACTC1
3muscle filament slidingGO:0300499.4ACTC1, DES

Molecular functions related to Pulmonary Vein Stenosis according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1platelet-derived growth factor receptor bindingGO:0051619.3PDGFRA, TYMP

Products for genes affiliated with Pulmonary Vein Stenosis

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  • Antibodies
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Sources for Pulmonary Vein Stenosis

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4CDC
14ExPASy
15FMA
23GTR
24HGMD
25HMDB
26ICD10
27ICD10 via Orphanet
28ICD9CM
30IUPHAR
31KEGG
36MeSH
37MESH via Orphanet
38MGI
41NCIt
42NDF-RT
45NINDS
46Novoseek
48OMIM
49OMIM via Orphanet
53PubMed
54QIAGEN
60SNOMED-CT via Orphanet
63UMLS
64UMLS via Orphanet