MCID: PLM068
MIFTS: 41

Pulmonary Vein Stenosis malady

Rare diseases, Cardiovascular diseases categories
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Summaries for Pulmonary Vein Stenosis

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NIH Rare Diseases:42 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 based summary: Pulmonary Vein Stenosis, also known as pulmonary veins stenosis, is related to sarcoma and fasciitis. 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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Pulmonary Vein Stenosis, Aliases & Descriptions:

Name: Pulmonary Vein Stenosis 42 62
 
Pulmonary Veins Stenosis 42


Classifications:

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


Related Diseases for Pulmonary Vein Stenosis

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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 96)
idRelated DiseaseScoreTop Affiliating Genes
1sarcoma29.3DES, PECAM1, MKI67, ACTC1, PDGFRA, CD99
2fasciitis10.5ACTC1
3peripheral primitive neuroectodermal tumor10.5CD99
4hypertension10.4
5pulmonary hypertension10.4
6infantile myofibromatosis10.4DES
7cor triatriatum10.4
8papilloma10.4ACTC1
9carney triad10.3PDGFRA
10congenital heart disease10.3
11mediastinitis10.3
12pulmonary embolism10.3
13fibrosing mediastinitis10.3
14transposition of the great arteries10.3
15pyogenic granuloma10.3PECAM1, ACTC1
16lymphangioma10.3PECAM1, ACTC1
17adenosarcoma10.3ACTC1, DES
18epithelioid leiomyosarcoma10.3ACTC1, DES
19pseudosarcomatous fibromatosis10.3DES, ACTC1
20spindle cell carcinoma10.3ACTC1, DES
21spindle cell sarcoma10.3DES, ACTC1
22teratoma10.3CD99, ACTC1
23cutaneous fibrous histiocytoma10.3DES, ACTC1
24dermatofibrosarcoma protuberans10.3DES, ACTC1
25fibrous histiocytoma10.3DES, ACTC1
26rhabdoid tumor10.3ACTC1, DES
27hemangiopericytoma10.3DES, ACTC1
28angiomyolipoma10.3ACTC1, DES
29paraganglioma10.3DES, ACTC1
30fibroma10.2DES, ACTC1
31embryonal rhabdomyosarcoma10.2DES, ACTC1
32malignant mixed mullerian tumor10.2DES, ACTC1
33fibromatosis10.2ACTC1, DES
34abrikosov's tumor10.2DES, ACTC1
35giant cell tumor10.2DES, ACTC1
36mucoepidermoid carcinoma10.2MKI67, ACTC1
37pleomorphic adenoma10.2DES, ACTC1
38salivary gland adenoid cystic carcinoma10.2ACTC1, MKI67
39hemangioma10.2PECAM1, ACTC1
40bronchopulmonary dysplasia10.1
41down syndrome10.1
42primary pulmonary hypertension10.1
43tetralogy of fallot10.1
44patent ductus arteriosus10.1
45scimitar syndrome10.1
46clear cell sarcoma10.1
47esophagitis10.1
48lung cancer10.1
49ventricular septal defect10.1
50bronchogenic cyst10.1

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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Drug clinical trials:

Search ClinicalTrials for Pulmonary Vein Stenosis

Search NIH Clinical Center for Pulmonary Vein Stenosis

Genetic Tests for Pulmonary Vein Stenosis

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Anatomical Context for Pulmonary Vein Stenosis

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MalaCards organs/tissues related to Pulmonary Vein Stenosis:

32
Lung, Heart

Animal Models for Pulmonary Vein Stenosis or affiliated genes

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MGI Mouse Phenotypes related to Pulmonary Vein Stenosis:

36
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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Articles related to Pulmonary Vein Stenosis:

(show top 50)    (show all 188)
idTitleAuthorsYear
1
Congenital pulmonary vein stenosis in an adult patient. (24184251)
2014
2
Massive Hemoptysis Due to Pulmonary Vein Stenosis Following Catheter Ablation for Atrial Fibrillation. (25316885)
2014
3
Hemoptysis from bronchial varices associated with pulmonary vein stenosis: role of surgical repair. (22997173)
2013
4
Severe pulmonary vein stenosis due to invasion of metastatic lung cancer. (23443870)
2013
5
Pulmonary Vein Stenosis with Down Syndrome: A Rare and Frequently Fatal Cause of Pulmonary Hypertension in Infants and Children. (23650870)
2013
6
Proximal pulmonary vein stenosis detection in pediatric patients: value of multiplanar and 3-D VR imaging evaluation. (23475282)
2013
7
Haemoptysis and pulmonary vein stenosis after ablation for atrial fibrillation: pathophysiology and therapeutic options. (23561921)
2013
8
Angioplasty of acquired pulmonary vein stenosis using covered stent. (23592594)
2013
9
Relentless pulmonary vein stenosis: A contemporary approach to a recurring problem. (23436711)
2013
10
Bifurcation angioplasty using drug eluting stents of post-AF ablation severe pulmonary vein stenosis. (22394418)
2012
11
Percutaneous stenting to treat pulmonary vein stenosis after single-lung transplantation. (22949779)
2012
12
Sutureless technique for recurrent pulmonary vein stenosis after pericardial patchplasty in an adult. (22901499)
2012
13
Successful correction of unroofed coronary sinus with pulmonary vein stenosis. (22753625)
2012
14
Isolated congenital left-sided pulmonary vein stenosis in an adult patient misdiagnosed as primary pulmonary hypertension. (21349107)
2011
15
Surgical management of post-repair pulmonary vein stenosis. (21444056)
2011
16
Evaluation of pulmonary vein stenosis after pulmonary vein isolation using a novel circular mapping and ablation catheter (PVAC). (21841188)
2011
17
Cardiopulmonary hemodynamic clues for pulmonary vein stenosis diagnosis. (22530105)
2011
18
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
19
Acquired pulmonary vein stenosis: one problem, two mechanisms. (20138470)
2010
20
Hypoplastic right lung associated with right pulmonary vein stenosis and systemic collateral. (20136863)
2010
21
Multimodality imaging assessment of anatomic and functional pulmonary vein stenosis. (19808631)
2009
22
Importance of totally anomalous pulmonary venous connection and postoperative pulmonary vein stenosis in outcomes of heterotaxy syndrome. (18004616)
2008
23
Pulmonary vein stenosis: intervene early and carry a big stent. (18373597)
2008
24
Primary pulmonary vein stenosis. (17293205)
2007
25
Detection of pulmonary vein stenosis by transesophageal echocardiography: comparison with multidetector computed tomography. (17452156)
2007
26
Transesophageal echocardiography: a follow-up tool after catheter ablation of atrial fibrillation and interventional therapy of pulmonary vein stenosis and occlusion. (17458690)
2007
27
Management of congenital and acquired pulmonary vein stenosis. (16488708)
2006
28
Acquired isolated left pulmonary vein stenosis: a complication of bronchogenic cyst removal diagnosed by transesophageal echocardiography. (16412190)
2006
29
Temporary pulmonary vein stenosis during intraoperative transesophageal echocardiography in total cavopulmonary connection. (16235015)
2006
30
Usefulness of helical computed tomography in diagnosing pulmonary vein stenosis in infants. (16049562)
2005
31
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
32
Decreased pulmonary perfusion in pulmonary vein stenosis after radiofrequency ablation: assessment with dynamic magnetic resonance perfusion imaging. (15302728)
2004
33
A case of congenital pulmonary vein stenosis in an adult. (14872119)
2004
34
Pulmonary vein isolation using transvenous catheter cryoablation for treatment of atrial fibrillation without risk of pulmonary vein stenosis. (12932615)
2003
35
Transcatheter angioplasty for acquired pulmonary vein stenosis after radiofrequency ablation. (12952852)
2003
36
Pulmonary vein stenosis by ostial irrigated-tip ablation: incidence, time course, and prediction. (12693497)
2003
37
Pulmonary vein stenosis after catheter ablation of atrial fibrillation: emergence of a new clinical syndrome. (12693885)
2003
38
Successful stent treatment of pulmonary vein stenosis following atrial fibrillation radiofrequency ablation. (12082196)
2002
39
Congenital pulmonary vein stenosis associated with cor triatriatum. (11235744)
2001
40
Left upper pulmonary vein stenosis 2 months after radiofrequency catheter ablation of atrial fibrillation. (10747360)
2000
41
Pulmonary vein stenosis complicating catheter ablation of focal atrial fibrillation. (10868741)
2000
42
Editorial comment: can we avoid pulmonary vein stenosis following ablation of atrial fibrillation? (11141210)
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
Lung transplantation for congenital pulmonary vein stenosis. (7574992)
1995
46
Pulsed wave and color Doppler findings in congenital pulmonary vein stenosis. (1614915)
1992
47
Radionuclide evaluation of unilateral pulmonary vein stenosis. Correlation with chest roentgenogram and clinical profiles. (3295141)
1986
48
Congenital pulmonary vein stenosis: a radiographic study. (3763853)
1986
49
Unilateral pulmonary vein stenosis. (3718800)
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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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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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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Compounds related to Pulmonary Vein Stenosis according to GeneCards/GeneDecks:

(show all 48)
idCompoundScoreTop Affiliating Genes
1orcein4410.1ACTC1, DES
2s 1004410.1ACTC1, DES
3reticulin4410.0DES, ACTC1
4mucicarmine4410.0ACTC1, DES
5b 7234410.0ACTC1, DES
6percoll449.9DES, PECAM1
7procollagen449.6DES, ACTC1
8prostacyclin449.5TYMP, PECAM1, F2RL1
9matrigel449.5PECAM1, TYMP, ACTC1
10methanol44 2410.5ACTC1, MKI67
11fibrinogen449.5F2RL1, DES, PECAM1
12cyclosporin a44 28 6111.5ACTC1, PECAM1, F2RL1
13streptavidin449.5MKI67, DES
14deoxyuridine44 2410.5MKI67, TYMP
15vinorelbine44 50 1111.5MKI67, TYMP
16p00001449.4MKI67, TYMP
17imatinib44 50 1111.4PDGFRA, ACTC1, DES
18octreotide44 61 28 1112.4MKI67, F2RL1
19biotin44 24 1111.2DES, MKI67, ACTC1
20heparin44 28 24 1112.2PECAM1, DES, F2RL1
21creatinine449.2DES, TYMP, ACTC1
22p005449.1MKI67, TYMP, PECAM1
23irinotecan44 50 1111.1MKI67, TYMP
24actinomycin d449.1F2RL1, DES, PECAM1, TYMP
25glycogen44 2410.1DES, MKI67, ACTC1
26cysteine449.0F2RL1, DES, PECAM1, TYMP
27lysine449.0F2RL1, DES, MKI67
28doxorubicin44 50 1110.9MKI67, TYMP, DES
29silver448.9MKI67, DES
30folate448.9TYMP, MKI67, PDGFRA
31hematoxylin448.9DES, PECAM1, MKI67, ACTC1
32estradiol44 24 1110.9DES, TYMP, MKI67
33p003448.8F2RL1, PECAM1, TYMP, MKI67
34cycloheximide448.7F2RL1, PECAM1, TYMP, MKI67
35progesterone44 28 61 24 1112.7DES, TYMP, MKI67, ACTC1
36p002448.7DES, PECAM1, TYMP, MKI67
37formaldehyde44 249.4DES, PECAM1, TYMP, MKI67, ACTC1
38nitric oxide44 24 1110.3F2RL1, DES, PECAM1, TYMP, MKI67
39serine448.3F2RL1, DES, PECAM1, TYMP, MKI67
40retinoic acid44 249.2DES, PECAM1, MKI67, ACTC1, PDGFRA
41oxygen44 249.0F2RL1, DES, PECAM1, TYMP, MKI67, ACTC1
42glucose448.0F2RL1, DES, PECAM1, TYMP, MKI67, ACTC1
43calcium44 50 24 1110.8F2RL1, DES, PECAM1, MKI67, ACTC1, PDGFRA
44estrogen447.7DES, PECAM1, TYMP, MKI67, ACTC1, PDGFRA
45thymidine44 248.7F2RL1, DES, PECAM1, TYMP, MKI67, PDGFRA
46tyrosine447.4F2RL1, DES, PECAM1, TYMP, MKI67, ACTC1
47paraffin446.8CD99, F2RL1, DES, PECAM1, TYMP, MKI67
48vegf446.8F2RL1, DES, PECAM1, TYMP, MKI67, ACTC1

GO Terms for genes affiliated with Pulmonary Vein Stenosis

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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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Sources for Pulmonary Vein Stenosis

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3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
28IUPHAR
29KEGG
34MeSH
35MESH via Orphanet
36MGI
39NCIt
40NDF-RT
43NINDS
44Novoseek
46OMIM
47OMIM via Orphanet
51PubMed
52QIAGEN
58SNOMED-CT via Orphanet
62UMLS
63UMLS via Orphanet