MCID: PLM068
MIFTS: 34

Pulmonary Vein Stenosis malady

Cardiovascular category

Summaries for Pulmonary Vein Stenosis

Sources:
43NIH Rare Diseases, 33MalaCards
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NIH Rare Diseases:43 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 vein disease and pulmonary function. An important gene associated with Pulmonary Vein Stenosis is F2RL1 (coagulation factor II (thrombin) receptor-like 1), and among its related pathways are Cardiac Progenitor Differentiation and Striated Muscle Contraction. The compounds cycloheximide and glucose have been mentioned in the context of this disorder. Affiliated tissues include heart and lung, and related mouse phenotypes are cardiovascular system and homeostasis/metabolism.

Aliases & Classifications for Pulmonary Vein Stenosis

Sources:
43NIH Rare Diseases, 61UMLS
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Classifications:

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


Aliases & Descriptions:

pulmonary vein stenosis 43 61
pulmonary veins stenosis 43


Related Diseases for Pulmonary Vein Stenosis

Sources:
17GeneCards, 18GeneDecks
See all sources

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

(show top 50)    (show all 100)
idRelated DiseaseScoreTop Affiliating Genes
1vein disease10.5
2pulmonary function10.4
3fibrosing mediastinitis10.3
4primary pulmonary hypertension10.1
5scimitar syndrome10.1
6conn's syndrome10.1
7thoracic aortic aneurysm10.1
8patent ductus arteriosus10.1
9aortic aneurysm10.1
10bronchogenic cyst10.1
11eisenmenger syndrome10.1
12heterotaxy10.1
13coronary sinus stenosis10.1
14pancreatitis10.0F2RL1
15cerebral primitive neuroectodermal tumor10.0CD99
16peritonitis10.0PECAM1
17peripheral primitive neuroectodermal tumor10.0CD99
18myoepithelioma10.0ACTC1
19meningioma10.0MKI67
20cervical cancer10.0TYMP
21carney triad10.0PDGFRA
22neurofibromatosis10.0PDGFRA
23osteosarcoma10.0PDGFRA
24myocardial infarction10.0PECAM1
25fasciitis10.0ACTC1
26pulmonary fibrosis10.0F2RL1
27uterine sarcoma10.0DES
28teratoma10.0ACTC1, CD99
29infantile myofibromatosis10.0DES
30hemangioma10.0PECAM1, ACTC1
31lipoma10.0DES
32hepatitis10.0TYMP, CD99
33pyogenic granuloma10.0PECAM1, ACTC1
34pituitary adenoma10.0MKI67
35pancreatic cancer10.0F2RL1, TYMP
36lymphangioma10.0PECAM1, ACTC1
37neurofibroma10.0PDGFRA, ACTC1
38granular cell tumor10.0DES
39mucoepidermoid carcinoma10.0ACTC1, MKI67
40endometrial carcinoma10.0TYMP, MKI67
41oligodendroglioma10.0MKI67, PDGFRA
42adenoid cystic carcinoma10.0MKI67, ACTC1
43malignant peripheral nerve sheath tumor10.0ACTC1, PDGFRA
44ovarian cancer10.0PDGFRA, TYMP
45colorectal cancer10.0PDGFRA, TYMP
46fibromatosis10.0ACTC1, DES
47adenosarcoma10.0DES, ACTC1
48gallbladder cancer10.0MKI67, TYMP
49dermatofibrosarcoma protuberans10.0ACTC1, DES
50epithelioid leiomyosarcoma10.0DES, ACTC1

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



Diseases related to pulmonary vein stenosis

Clinical Features for Pulmonary Vein Stenosis

Drugs & Therapeutics for Pulmonary Vein Stenosis

Sources:
5CenterWatch, 42NIH Clinical Center, 6ClinicalTrials
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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

Anatomical Context for Pulmonary Vein Stenosis

Sources:
33MalaCards
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MalaCards organs/tissues related to Pulmonary Vein Stenosis:

33
Heart, Lung

Animal Models for Pulmonary Vein Stenosis or affiliated genes

Sources:
37MGI, 28inGenious Targeting Laboratory
See all sources

MGI Mouse Phenotypes related to Pulmonary Vein Stenosis:

37
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053858.2PECAM1, ACTC1, F2RL1, DES, PDGFRA
2MP:00053767.4PECAM1, PDGFRA, DES, TYMP, F2RL1, ACTC1

Publications for Pulmonary Vein Stenosis

Sources:
51PubMed
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Articles related to Pulmonary Vein Stenosis:

(show top 50)    (show all 183)
idTitleAuthorsYear
1
Severe pulmonary vein stenosis due to invasion of metastatic lung cancer. (23443870)
2013
2
Pulmonary Vein Stenosis with Down Syndrome: A Rare and Frequently Fatal Cause of Pulmonary Hypertension in Infants and Children. (23650870)
2013
3
Direct visualization of pulmonary vein stenosis after previous catheter ablation. (24184786)
2013
4
Chest Radiographic Findings in Pediatric Patients with Intraluminal Pulmonary Vein Stenosis. (23773478)
2013
5
Pulmonary Vein Stenosis Assessed by Three-Dimensional Transesophageal Echocardiography. (24134452)
2013
6
Pulmonary vein stenosis and the pathophysiology of "upstream" pulmonary veins. (24084286)
2013
7
Clinical characteristics and prognostic factors of primary pulmonary vein stenosis or atresia in children. (23102496)
2013
8
Pulmonary vein stenosis after radiofrequency ablation of lone atrial fibrillation in an ironman triathlete. (23010442)
2013
9
Multimodality imaging of iatrogenic pulmonary vein stenosis. (22207341)
2012
10
Bilateral disease and early age at presentation are associated with shorter survival in patients with congenital heart disease and intraluminal pulmonary vein stenosis. (22469299)
2012
11
Pulmonary vein stenosis and hemoptysis. (22550212)
2012
12
Pulmonary vein stenosis requiring lobectomy after radiofrequency catheter ablation for atrial fibrillation. (23207568)
2012
13
Cryo-balloon angioplasty for pulmonary vein stenosis in pediatric patients. (21892648)
2012
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
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
17
Transhepatic portal vein access for balloon dilation of right upper pulmonary vein stenosis following infradiaphragmatic total anomalous pulmonary venous drainage repair. (21413130)
2011
18
Congenital pulmonary vein stenosis in an adult patient treated with transcatheter balloon angioplasty. (21068023)
2011
19
Multimodality imaging to diagnose pulmonary vein stenosis following atrial fibrillation ablation. (21891815)
2011
20
Pulmonary vein stenosis may still occur after ablation. (20421222)
2010
21
Images in cardiovascular disease. Pulmonary vein stenosis after lung transplantation successfully treated with stent implantation. (21173363)
2010
22
A transthoracic echocardiographic follow-up study after catheter ablation of atrial fibrillation: can we detect pulmonary vein stenosis by transthoracic echocardiography? (20967145)
2010
23
Pulmonary vein stenosis: the UK, Ireland and Sweden collaborative study. (19737738)
2009
24
Pulmonary vein stenosis: intervene early and carry a big stent. (18373597)
2008
25
Comparison of stent versus balloon angioplasty for pulmonary vein stenosis complicating pulmonary vein isolation. (18284494)
2008
26
Unilateral pulmonary vein stenosis secondary to idiopathic fibrosing mediastinitis. (18480351)
2008
27
Detection of pulmonary vein stenosis by transesophageal echocardiography: comparison with multidetector computed tomography. (17452156)
2007
28
Clinical-pathologic conference in surgery for congenital and acquired cardiovascular disease: unilateral pulmonary vein stenosis with a contralateral pulmonary varix. (17662796)
2007
29
Acquired isolated left pulmonary vein stenosis: a complication of bronchogenic cyst removal diagnosed by transesophageal echocardiography. (16412190)
2006
30
Temporary pulmonary vein stenosis during intraoperative transesophageal echocardiography in total cavopulmonary connection. (16235015)
2006
31
Pulmonary vein stenosis following repair of total anomalous pulmonary venous connection. (16638548)
2006
32
An unusual cause of pulmonary vein stenosis: a case report and review of the literature. (16970720)
2006
33
Visualization of pulmonary vein stenosis after radio frequency ablation using multi-slice computed tomography: initial clinical experience in 33 patients. (15982498)
2005
34
Clinical presentation, investigation, and management of pulmonary vein stenosis complicating ablation for atrial fibrillation. (15699274)
2005
35
Incidence and predictors of pulmonary vein stenosis following catheter ablation of atrial fibrillation using the anatomic pulmonary vein ablation approach: results from paired magnetic resonance imaging. (16101625)
2005
36
Functional assessment of pulmonary vein stenosis using radionuclide ventilation/perfusion imaging. (15302759)
2004
37
Pulmonary vein stenosis after radiofrequency pulmonary vein isolation for atrial fibrillation. (15122197)
2004
38
Pulmonary vein stenosis by ostial irrigated-tip ablation: incidence, time course, and prediction. (12693497)
2003
39
Stent implantation and subsequent dilatation for pulmonary vein stenosis in pediatric patients: maximizing effectiveness. (12604863)
2003
40
Incidence of pulmonary vein stenosis 2 years after radiofrequency catheter ablation of refractory atrial fibrillation. (12714028)
2003
41
Pulmonary vein varix in association with bilateral pulmonary vein stenosis. (11428487)
2001
42
Case report: pulmonary vein stenosis following RF ablation of paroxysmal atrial fibrillation: successful treatment with balloon dilation. (11141209)
2000
43
Pulmonary vein stenosis with normal connection: associated cardiac abnormalities and variable outcome. (10421134)
1999
44
Pulmonary vein stenosis after catheter ablation of atrial fibrillation. (9788832)
1998
45
Pulsed wave and color Doppler echocardiography and cardiac catheterization findings in bilateral pulmonary vein stenosis. (9571591)
1998
46
Deringing procedure for congenital pulmonary vein stenosis. (7647600)
1995
47
Unilateral pulmonary vein stenosis with atrial septal defect: report of one case. (8085454)
1994
48
Pulsed wave and color Doppler findings in congenital pulmonary vein stenosis. (1614915)
1992
49
Congenital pulmonary vein stenosis: a radiographic study. (3763853)
1986
50
Report of a successful operation for stenosis of common pulmonary vein (cor triatriatum). (13316660)
1956

Genetic Variations for Pulmonary Vein Stenosis

Expression for genes affiliated with Pulmonary Vein Stenosis

Sources:
1BioGPS, 15Gene Expression Omnibus DataSets
See all sources
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

Sources:
38NCBI BioSystems Database, 54Reactome, 4Cell Signaling Technology
See all sources

Pathways related to Pulmonary Vein Stenosis according to GeneCards/GeneDecks:

idSuper pathways (with members indented)ScoreTop Affiliating Genes
19.6PDGFRA, ACTC1
2
Hide members
9.5ACTC1, DES
39.1DES, F2RL1, ACTC1
48.7PECAM1, F2RL1, TYMP, PDGFRA

Compounds for genes affiliated with Pulmonary Vein Stenosis

Sources:
45Novoseek, 24HMDB, 11DrugBank, 50PharmGKB, 60Tocris Bioscience, 29IUPHAR
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Compounds related to Pulmonary Vein Stenosis according to GeneCards/GeneDecks:

(show all 39)
idCompoundScoreTop Affiliating Genes
1cycloheximide4510.4TYMP
2glucose4510.0TYMP, ACTC1
3orcein459.9ACTC1, DES
4s 100459.8ACTC1, DES
5reticulin459.8ACTC1, DES
6mucicarmine459.8ACTC1, DES
7deoxyuridine45 2410.8TYMP, MKI67
8vinorelbine45 1110.8MKI67, TYMP
9p00001459.7TYMP, MKI67
10percoll459.7DES, PECAM1
11b 723459.7DES, ACTC1
12methanol45 2410.6ACTC1, MKI67
13prostacyclin459.6PECAM1, F2RL1, TYMP
14p003459.5PECAM1, MKI67, TYMP
15streptavidin459.5MKI67, DES
16matrigel459.5ACTC1, PECAM1, TYMP
17p005459.5MKI67, TYMP, PECAM1
18biotin45 11 2411.3ACTC1, MKI67, DES
19imatinib45 50 1111.3ACTC1, DES, PDGFRA
20folate459.3PDGFRA, TYMP, MKI67
21fibrinogen459.2DES, F2RL1, PECAM1
22actinomycin d459.0F2RL1, DES, TYMP, PECAM1
23hematoxylin459.0PECAM1, DES, MKI67, ACTC1
24estradiol45 11 2410.9TYMP, MKI67, DES
25p002458.9DES, PECAM1, TYMP, MKI67
26progesterone45 60 29 11 2412.8MKI67, ACTC1, TYMP, DES
27doxorubicin45 50 1110.8TYMP, DES, MKI67
28glycogen45 249.7DES, ACTC1, MKI67
29cysteine458.6TYMP, DES, PECAM1, F2RL1
30formaldehyde45 249.5DES, PECAM1, ACTC1, TYMP, MKI67
31nitric oxide45 11 2410.5DES, F2RL1, MKI67, PECAM1, TYMP
32retinoic acid45 249.4PECAM1, MKI67, ACTC1, DES, PDGFRA
33serine458.4MKI67, DES, F2RL1, PECAM1, TYMP
34oxygen45 249.1PECAM1, ACTC1, DES, MKI67, TYMP, F2RL1
35calcium45 50 11 2411.0DES, PECAM1, ACTC1, F2RL1, PDGFRA, MKI67
36thymidine45 249.0PDGFRA, DES, MKI67, TYMP, F2RL1, PECAM1
37tyrosine457.6TYMP, PECAM1, MKI67, DES, PDGFRA, F2RL1
38paraffin456.8PDGFRA, DES, MKI67, ACTC1, TYMP, CD99
39vegf456.8PECAM1, PDGFRA, DES, MKI67, TYMP, CD99

GO Terms for genes affiliated with Pulmonary Vein Stenosis

Sources:
16Gene Ontology
See all sources

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

idNameGO IDScoreTop Affiliating Genes
1positive regulation of phosphatidylinositol 3-kinase cascadeGO:0140689.6F2RL1, PDGFRA
2cardiac myofibril assemblyGO:0550039.5ACTC1, PDGFRA
3muscle filament slidingGO:0300499.2ACTC1, DES

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

idNameGO IDScoreTop Affiliating Genes
1platelet-derived growth factor receptor bindingGO:0051619.5TYMP, PDGFRA

Products for genes affiliated with Pulmonary Vein Stenosis

  • Antibodies
  • Proteins
  • Lysates
  • Antibodies

Sources for Pulmonary Vein Stenosis

3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
29IUPHAR
30KEGG
35MeSH
36MESH via Orphanet
37MGI
40NCIt
41NDF-RT
44NINDS
45Novoseek
47OMIM
48OMIM via Orphanet
51PubMed
52QIAGEN
58SNOMED-CT via Orphanet
61UMLS
62UMLS via Orphanet