MCID: PLM068
MIFTS: 41

Pulmonary Vein Stenosis malady

Rare diseases, Cardiovascular diseases categories
Download this MalaCard

Summaries for Pulmonary Vein Stenosis

About this section
Sources:
43NIH Rare Diseases, 33MalaCards
See all sources

Fully expand this MalaCard
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 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

About this section
Sources:
43NIH Rare Diseases, 62UMLS
See all sources

Classifications:

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


Aliases & Descriptions:

pulmonary vein stenosis 43 62
pulmonary veins stenosis 43


Related Diseases for Pulmonary Vein Stenosis

About this section
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 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

About this section

Drugs & Therapeutics for Pulmonary Vein Stenosis

About this section
Sources:
42NIH Clinical Center, 6ClinicalTrials
See all sources

Drug clinical trials:

Search ClinicalTrials for Pulmonary Vein Stenosis

Search NIH Clinical Center for Pulmonary Vein Stenosis

Genetic Tests for Pulmonary Vein Stenosis

About this section

Anatomical Context for Pulmonary Vein Stenosis

About this section
Sources:
33MalaCards
See all sources

MalaCards organs/tissues related to Pulmonary Vein Stenosis:

33
Lung, Heart

Animal Models for Pulmonary Vein Stenosis or affiliated genes

About this section
Sources:
37MGI
See all sources

MGI Mouse Phenotypes related to Pulmonary Vein Stenosis:

37
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

About this section
Sources:
52PubMed
See all sources

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

About this section

Expression for genes affiliated with Pulmonary Vein Stenosis

About this section
Sources:
2BioGPS, 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

About this section
Sources:
50PathCards, 38NCBI BioSystems Database, 55Reactome, 5Cell Signaling Technology
See all sources

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

About this section
Sources:
45Novoseek, 24HMDB, 29IUPHAR, 61Tocris Bioscience, 51PharmGKB, 11DrugBank
See all sources

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

(show all 48)
idCompoundScoreTop Affiliating Genes
1orcein4510.1ACTC1, DES
2s 1004510.1ACTC1, DES
3reticulin4510.0DES, ACTC1
4mucicarmine4510.0ACTC1, DES
5b 7234510.0ACTC1, DES
6percoll459.9DES, PECAM1
7procollagen459.6DES, ACTC1
8prostacyclin459.5TYMP, PECAM1, F2RL1
9matrigel459.5PECAM1, TYMP, ACTC1
10methanol45 2410.5ACTC1, MKI67
11fibrinogen459.5F2RL1, DES, PECAM1
12cyclosporin a45 29 6111.5ACTC1, PECAM1, F2RL1
13streptavidin459.5MKI67, DES
14deoxyuridine45 2410.5MKI67, TYMP
15vinorelbine45 51 1111.5MKI67, TYMP
16p00001459.4MKI67, TYMP
17imatinib45 51 1111.4PDGFRA, ACTC1, DES
18octreotide45 61 29 1112.4MKI67, F2RL1
19biotin45 24 1111.2DES, MKI67, ACTC1
20heparin45 29 24 1112.2PECAM1, DES, F2RL1
21creatinine459.2DES, TYMP, ACTC1
22p005459.1MKI67, TYMP, PECAM1
23irinotecan45 51 1111.1MKI67, TYMP
24actinomycin d459.1F2RL1, DES, PECAM1, TYMP
25glycogen45 2410.1DES, MKI67, ACTC1
26cysteine459.0F2RL1, DES, PECAM1, TYMP
27lysine459.0F2RL1, DES, MKI67
28doxorubicin45 51 1110.9MKI67, TYMP, DES
29silver458.9MKI67, DES
30folate458.9TYMP, MKI67, PDGFRA
31hematoxylin458.9DES, PECAM1, MKI67, ACTC1
32estradiol45 24 1110.9DES, TYMP, MKI67
33p003458.8F2RL1, PECAM1, TYMP, MKI67
34cycloheximide458.7F2RL1, PECAM1, TYMP, MKI67
35progesterone45 29 61 24 1112.7DES, TYMP, MKI67, ACTC1
36p002458.7DES, PECAM1, TYMP, MKI67
37formaldehyde45 249.4DES, PECAM1, TYMP, MKI67, ACTC1
38nitric oxide45 24 1110.3F2RL1, DES, PECAM1, TYMP, MKI67
39serine458.3F2RL1, DES, PECAM1, TYMP, MKI67
40retinoic acid45 249.2DES, PECAM1, MKI67, ACTC1, PDGFRA
41oxygen45 249.0F2RL1, DES, PECAM1, TYMP, MKI67, ACTC1
42glucose458.0F2RL1, DES, PECAM1, TYMP, MKI67, ACTC1
43calcium45 51 24 1110.8F2RL1, DES, PECAM1, MKI67, ACTC1, PDGFRA
44estrogen457.7DES, PECAM1, TYMP, MKI67, ACTC1, PDGFRA
45thymidine45 248.7F2RL1, DES, PECAM1, TYMP, MKI67, PDGFRA
46tyrosine457.4F2RL1, DES, PECAM1, TYMP, MKI67, ACTC1
47paraffin456.8CD99, F2RL1, DES, PECAM1, TYMP, MKI67
48vegf456.8F2RL1, DES, PECAM1, TYMP, MKI67, ACTC1

GO Terms for genes affiliated with Pulmonary Vein Stenosis

About this section
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 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

About this section
  • Antibodies
  • Proteins
  • Lysates
  • Antibodies

Sources for Pulmonary Vein Stenosis

About this section
4CDC
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
52PubMed
53QIAGEN
59SNOMED-CT via Orphanet
62UMLS
63UMLS via Orphanet