MCID: PLM068
MIFTS: 43

Pulmonary Vein Stenosis

Categories: Rare diseases, Cardiovascular diseases

Aliases & Classifications for Pulmonary Vein Stenosis

MalaCards integrated aliases for Pulmonary Vein Stenosis:

Name: Pulmonary Vein Stenosis 49 69
Pulmonary Veins Stenosis 49

Classifications:



External Ids:

UMLS 69 C0340756

Summaries for Pulmonary Vein Stenosis

NIH Rare Diseases : 49 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 atrial fibrillation and reticular perineurioma. An important gene associated with Pulmonary Vein Stenosis is PECAM1 (Platelet And Endothelial Cell Adhesion Molecule 1), and among its related pathways/superpathways are p70S6K Signaling and Actin Nucleation by ARP-WASP Complex. The drugs Anti-Arrhythmia Agents and Isoproterenol have been mentioned in the context of this disorder. Affiliated tissues include lung and heart, and related phenotypes are cellular and cardiovascular system

Wikipedia : 72 Pulmonary vein stenosis is a rare cardiovascular disorder. It is recognized as being the stenosis of one... more...

Related Diseases for Pulmonary Vein Stenosis

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

(show top 50) (show all 135)
# Related Disease Score Top Affiliating Genes
1 atrial fibrillation 10.6
2 reticular perineurioma 10.4 KIT PDGFRA
3 sm-ahnmd 10.4 KIT PDGFRA
4 gastric leiomyosarcoma 10.4 KIT PDGFRA
5 desmoid tumor 10.4 KIT PDGFRA
6 carney triad 10.4 KIT PDGFRA
7 hypereosinophilic syndrome, idiopathic 10.4 KIT PDGFRA
8 sarcomatoid renal cell carcinoma 10.4 KIT PDGFRA
9 conventional fibrosarcoma 10.4 KIT PDGFRA
10 endometrial small cell carcinoma 10.4 KIT PDGFRA
11 benign breast phyllodes tumor 10.4 KIT MKI67
12 lung adenoid cystic carcinoma 10.4 KIT PDGFRA
13 hemangioendothelioma 10.4 KDR PECAM1
14 kaposiform hemangioendothelioma 10.4 KDR PECAM1
15 undifferentiated pleomorphic sarcoma 10.4 KIT PDGFRA
16 capillary hemangioma 10.3 KDR PECAM1
17 radiation proctitis 10.3 KDR PECAM1
18 heart sarcoma 10.3 KIT PDGFRA
19 chronic eosinophilic leukemia 10.3 KIT PDGFRA
20 epithelioid hemangioendothelioma 10.3 KDR PECAM1
21 mesenchymal cell neoplasm 10.3 KIT PDGFRA
22 neurofibromatosis, type i 10.3 KIT PDGFRA
23 cervical adenosquamous carcinoma 10.3 KDR PDGFRA
24 myxofibrosarcoma 10.3 DES MKI67
25 fibroblastic rheumatism 10.3 ACTC1 DES
26 angiolipoma 10.3 ACTC1 DES
27 cutaneous leiomyosarcoma 10.3 ACTC1 DES
28 spindle cell lipoma 10.3 ACTC1 DES
29 parachordoma 10.3 ACTC1 DES
30 ossifying fibromyxoid tumor 10.3 ACTC1 DES
31 epithelioid leiomyosarcoma 10.3 ACTC1 DES
32 senile angioma 10.3 KDR KIT
33 syringocystadenoma papilliferum 10.3 ACTC1 DES
34 benign metastasizing leiomyoma 10.3 ACTC1 DES
35 thymic epithelial tumor 10.3 CD99 KIT
36 proctitis 10.3 KDR PECAM1
37 gallbladder sarcoma 10.3 DES KIT
38 congenital fibrosarcoma 10.3 ACTC1 DES
39 pleomorphic liposarcoma 10.2 ACTC1 DES
40 ischemic fasciitis 10.2 ACTC1 DES
41 cutaneous fibrous histiocytoma 10.2 ACTC1 DES
42 reticulum cell sarcoma 10.2 ACTC1 DES
43 dedifferentiated liposarcoma 10.2 ACTC1 DES
44 spindle cell sarcoma 10.2 ACTC1 DES
45 lymphangioma 10.2 ACTC1 KDR
46 8p11 myeloproliferative syndrome 10.2 KDR KIT
47 pseudosarcomatous fibromatosis 10.2 ACTC1 DES
48 glomus tumor 10.2 ACTC1 DES
49 non-langerhans-cell histiocytosis 10.2 ACTC1 DES
50 infantile myofibromatosis 10.2 ACTC1 DES

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



Diseases related to Pulmonary Vein Stenosis

Symptoms & Phenotypes for Pulmonary Vein Stenosis

MGI Mouse Phenotypes related to Pulmonary Vein Stenosis:

43
# Description MGI Source Accession Score Top Affiliating Genes
1 cellular MP:0005384 9.92 PDGFRA ACTC1 PECAM1 DES F2RL1 TYMP
2 cardiovascular system MP:0005385 9.91 PDGFRA ACTC1 PECAM1 DES F2RL1 KDR
3 homeostasis/metabolism MP:0005376 9.86 KIT PDGFRA ACTC1 PECAM1 DES TYMP
4 muscle MP:0005369 9.63 PDGFRA ACTC1 PECAM1 DES KDR KIT
5 no phenotypic analysis MP:0003012 9.35 KIT MKI67 PDGFRA F2RL1 KDR
6 skeleton MP:0005390 9.1 KIT PDGFRA PECAM1 TYMP F2RL1 KDR

Drugs & Therapeutics for Pulmonary Vein Stenosis

Drugs for Pulmonary Vein Stenosis (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 45)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1 Anti-Arrhythmia Agents Phase 3,Phase 2,Phase 1
2
Isoproterenol Approved, Investigational Phase 2 7683-59-2 3779
3
Vinblastine Approved Phase 2 865-21-4 241903 13342
4
Methotrexate Approved Phase 2 1959-05-2, 59-05-2 126941
5
Adenosine Approved, Investigational Phase 2 58-61-7 60961
6
Losartan Approved Phase 1, Phase 2 114798-26-4 3961
7
Angiotensin II Approved, Investigational Phase 1, Phase 2 68521-88-0, 4474-91-3, 11128-99-7 172198 65143
8
Bevacizumab Approved, Investigational Phase 1, Phase 2 216974-75-3
9
leucovorin Approved, Nutraceutical Phase 2 58-05-9 143 6006
10
Folic Acid Approved, Nutraceutical, Vet_approved Phase 2 59-30-3 6037
11 Orange Approved, Nutraceutical Phase 1, Phase 2
12 Analgesics Phase 2
13 Respiratory System Agents Phase 2
14 Adrenergic Agents Phase 2
15 Adrenergic Agonists Phase 2
16 Folic Acid Antagonists Phase 2
17 Adrenergic beta-Agonists Phase 2
18 Neurotransmitter Agents Phase 2
19 Vasodilator Agents Phase 2
20 Nucleic Acid Synthesis Inhibitors Phase 2
21 Dermatologic Agents Phase 2
22 Vitamin B Complex Phase 2
23 Peripheral Nervous System Agents Phase 2
24 Anti-Asthmatic Agents Phase 2
25 Immunosuppressive Agents Phase 2
26 Antimetabolites Phase 2
27 Antimetabolites, Antineoplastic Phase 2
28 Protective Agents Phase 2
29 Antimitotic Agents Phase 2
30 Antineoplastic Agents, Phytogenic Phase 2
31 Autonomic Agents Phase 2
32 Antirheumatic Agents Phase 2
33 Bronchodilator Agents Phase 2
34 Angiotensin II Type 1 Receptor Blockers Phase 1, Phase 2
35 Angiotensin Receptor Antagonists Phase 1, Phase 2
36 Angiotensinogen Phase 1, Phase 2
37 Antihypertensive Agents Phase 1, Phase 2
38 Angiogenesis Inhibitors Phase 1, Phase 2
39 Angiogenesis Modulating Agents Phase 1, Phase 2
40 Anti-Bacterial Agents Phase 1, Phase 2
41 Imatinib Mesylate Phase 1, Phase 2 123596
42 Protein Kinase Inhibitors Phase 1, Phase 2
43 Folate Nutraceutical Phase 2
44 Vitamin B9 Nutraceutical Phase 2
45 Mitogens

Interventional clinical trials:

(show all 19)

# Name Status NCT ID Phase Drugs
1 Cardioversion vs. Catheter Ablation for Persistent Atrial Fibrillation Unknown status NCT00196209 Phase 4
2 PV-Isolation With the Cryoballoon Versus RF:a Randomized Controlled Prospective Non-inferiority Trial (FreezeAF) Completed NCT00774566 Phase 4
3 Randomized Trial of Two Ablation Catheters in Paroxysmal Atrial Fibrillation Completed NCT00678340 Phase 4
4 Additional Linear Ablation Beyond Pulmonary Vein Isolation for Paroxysmal Atrial Fibrillation Recruiting NCT02637453 Phase 4
5 Mesh Ablator Versus Cryoballoon Pulmonary Vein Ablation of Symptomatic Paroxysmal Atrial Fibrillation Terminated NCT01061931 Phase 4
6 SOLAR & ATRILAZE Atrial Fibrillation Trial Unknown status NCT00734058 Phase 3
7 Study of HD Mesh Ablation System for Treatment of Paroxysmal Atrial Fibrillation Terminated NCT00741611 Phase 3 Anti-arrhythmic drugs
8 Vinblastine and Methotrexate in Children With Pulmonary Vein Stenosis Completed NCT00215046 Phase 2 Vinblastine;Methotrexate
9 Adenosine to Assess Complete Conduction Block During Catheter Ablation of Paroxysmal Atrial Fibrillation Completed NCT03032965 Phase 2 Adenosine;Isoproterenol
10 Treatment of Atrial Fibrillation by Minimal Invasive Surgery Completed NCT00448656 Phase 2
11 Pilot Trial: the Safety and Feasibility of Losartan for Pulmonary Vein Stenosis Recruiting NCT02769130 Phase 1, Phase 2 Losartan
12 Pilot Study Using Avastin and Gleevec to Treat the Progression of Intraluminal Pulmonary Vein Stenosis Active, not recruiting NCT00891527 Phase 1, Phase 2 Bevacizumab (Avastin) and Imatinib Mesylate (Gleevec)
13 RF Ablation of Atrial Fibrillation Completed NCT00265629 Phase 1
14 Safety Monitoring of Patients Having Pulmonary Vein Ablation Completed NCT00590668
15 Effectiveness Study of Circumferential vs. Segmental Ablation in Paroxysmal Atrial Fibrillation Recruiting NCT02106663
16 Pulmonary Vein Isolation in Athletes Recruiting NCT03226847
17 Cryoballoon Atrial Fibrillation Ablation Registry Recruiting NCT03040037
18 Measurement of Angiogenic Related Growth Factors in Urine and Blood From Patients With Pulmonary Vein Stenosis Terminated NCT00215059
19 Evaluation of the Phased Radio Frequency Ablation System Terminated NCT01693120

Search NIH Clinical Center for Pulmonary Vein Stenosis

Genetic Tests for Pulmonary Vein Stenosis

Anatomical Context for Pulmonary Vein Stenosis

MalaCards organs/tissues related to Pulmonary Vein Stenosis:

38
Lung, Heart

Publications for Pulmonary Vein Stenosis

Articles related to Pulmonary Vein Stenosis:

(show top 50) (show all 263)
# Title Authors Year
1
Head-to-head comparison of acute and chronic pulmonary vein stenosis for cryoballoon versus radiofrequency ablation. ( 29380388 )
2018
2
Fibrosing mediastinitis with pulmonary hypertension as a complication of pulmonary vein stenosis: A case report and review of the literature. ( 29369193 )
2018
3
Balloon angioplasty versus stenting for pulmonary vein stenosis after pulmonary vein isolation for atrial fibrillation: A meta-analysis. ( 29273240 )
2018
4
Spontaneous massive hemothorax presenting as a late complication of stent implantation in a patient with pulmonary vein stenosis following radiofrequency ablation for atrial fibrillation. ( 29325041 )
2018
5
Complex cases of acquired pulmonary vein stenosis after radiofrequency ablation: is surgical repair an option? ( 29444219 )
2018
6
Impact of Radiofrequency Ablation of Atrial Fibrillation on Pulmonary Vein Cross Sectional Area: Implications for the Diagnosis of Pulmonary Vein Stenosis. ( 29250220 )
2017
7
Primary Pulmonary Vein Stenosis: Outcomes, Risk Factors, and Severity Score in a Multicentric Study. ( 28633223 )
2017
8
Pulmonary vein stenosis or occlusion after catheter ablation of atrial fibrillation: long-term comparison of drug-eluting versus large bare metal stents. ( 29069364 )
2017
9
Paucicellular Fibrointimal Proliferation Characterizes Pediatric Pulmonary Vein Stenosis: Clinicopathologic Analysis of 213 Samples From 97 Patients. ( 28622179 )
2017
10
Successful Percutaneous Stenting of Severe Radiofrequency-Induced Pulmonary Vein Stenosis in 3 Veins Post-Failed Surgical Correction. ( 28109876 )
2017
11
Pulmonary Vein Varix with Pulmonary Vein Stenosis. ( 28007075 )
2017
12
Congenital Pulmonary Vein Stenosis and Pulmonary Artery Branch Stenosis: A Rare Combination. ( 28465986 )
2017
13
Pulmonary vein stenosis of ex-premature infants with pulmonary hypertension and bronchopulmonary dysplasia, epidemiology, and survival from a multicenter cohort. ( 28152279 )
2017
14
Real-time three dimensional CT and MRI to guide interventions for congenital heart disease and acquired pulmonary vein stenosis. ( 28455631 )
2017
15
Pulmonary Vein Widening Plasty for Pulmonary Vein Stenosis and Occlusion After Catheter Ablation. ( 29054242 )
2017
16
Pulmonary vein stenosis with collateralization via esophageal varices: Long-term follow-up after successful treatment with drug-eluting stent. ( 28924998 )
2017
17
Pulmonary vein stenosis in patients with Smith-Lemli-Opitz syndrome. ( 28719049 )
2017
18
Response by Fender et al to Letter Regarding Article, "Severe Pulmonary Vein Stenosis Resulting From Ablation for Atrial Fibrillation: Presentation, Management, and Clinical Outcomes". ( 28461424 )
2017
19
Surgical Repair for the Treatment of Pulmonary Vein Stenosis After Radiofrequency Ablation. ( 28838520 )
2017
20
Pulmonary vein stenosis after second-generation cryoballoon ablation for atrial fibrillation. ( 28491764 )
2017
21
Persistent Oxygen Requirement beyond Prematurity: A Case of Acquired Pulmonary Vein Stenosis. ( 28392954 )
2017
22
Recurrent haemoptysis as a symptom of severe pulmonary vein stenosis-a rare complication of catheter ablation in atrial fibrillation. ( 28096996 )
2017
23
Correction to: Severe Pulmonary Vein Stenosis Resulting From Ablation for Atrial Fibrillation: Presentation, Management, and Clinical Outcomes. ( 28461425 )
2017
24
Pulmonary Vein Stenosis Following Single-Lung Transplantation Successfully Treated with Intravascular Ultrasound-Guided Angioplasty and Stent Placement. ( 29203761 )
2017
25
Letter by Jazayeri et al Regarding Article, "Severe Pulmonary Vein Stenosis Resulting From Ablation for Atrial Fibrillation: Presentation, Management, and Clinical Outcomes". ( 28461423 )
2017
26
A History Lesson: Pulmonary Vein Stenosis. ( 28522385 )
2017
27
Computed Tomographic and Fluoroscopic Image Fusion for Pulmonary Vein Stenosis Stenting. ( 28843331 )
2017
28
Asymptomatic Pulmonary Vein Stenosis: Hemodynamic Adaptation and Successful Ablation. ( 28105376 )
2016
29
Recurrent Pulmonary Vein Stenosis After Pulmonary Vein Isolation. ( 28041807 )
2016
30
Pulmonary Vein Stenosis After Second-Generation Cryoballoon Ablation. ( 28032927 )
2016
31
Drug-Coated Balloon Venoplasty for In-Stent Restenosis in a Patient With Recurrent Pulmonary Vein Stenosis Post Ablation for Atrial Fibrillation: Initial Experience With a New Treatment Technique. ( 27145055 )
2016
32
Surgical approaches to pulmonary vein stenosis in pediatric heart transplant recipients: Opportunity for success in a difficult situation. ( 27453521 )
2016
33
Pulmonary vein stenosis after atrial fibrillation ablation. ( 27174109 )
2016
34
Emergency Covered Stent Implantation For Rupture Of A Pulmonary Vein After Balloon Angioplasty For Postinterventional Pulmonary Vein Stenosis. ( 28496923 )
2016
35
Pulmonary vein stenosis: Etiology, diagnosis and management. ( 26839659 )
2016
36
Pulmonary Vein Remodeling Following Atrial Fibrillation Ablation: Implications For The Radiographic Diagnosis Of Pulmonary Vein Stenosis. ( 27909538 )
2016
37
Pulmonary vein stenosis complicating radiofrequency catheter ablation for atrial fibrillation: A literature review. ( 27492534 )
2016
38
Pulmonary Vein Stenosis in Neonates with Severe Bronchopulmonary Dysplasia. ( 26862723 )
2016
39
Pulmonary veins stenosis after catheter ablation of atrial fibrillation as the cause of haemoptysis: Three cases and a literature review. ( 27216646 )
2016
40
Pulmonary vein stenosis: Plea for a multi-institutional registry. ( 26602902 )
2016
41
Severe Pulmonary Vein Stenosis Resulting From Ablation for Atrial Fibrillation: Presentation, Management, and Clinical Outcomes. ( 27793993 )
2016
42
TCT-809 Stenting for the treatment of pulmonary vein stenosis after atrial fibrillation radiofrequency catheter ablation: initial experience with peripheral stents plus drug-coated balloons and coronary drug-eluting stents. ( 27970199 )
2016
43
Pulmonary vein collateral formation as a long-term result of post-interventional pulmonary vein stenosis. ( 26795444 )
2016
44
Hemoptysis and lung disease as a manifestation of pulmonary vein stenosis after cryoballoon catheter ablation for atrial fibrillation. ( 26811238 )
2016
45
Pulmonary vein stenosis-novel strategies for a challenging and resistant condition? ( 26896354 )
2016
46
Congenital pulmonary lymphangiectasia in patient with pulmonary vein stenosis/atresia. ( 27546704 )
2016
47
Pulmonary Vein Stenosis After Minimally Invasive Stand-alone Surgical Ablation of Atrial Fibrillation. ( 25976163 )
2015
48
Catheter-based intervention for pulmonary vein stenosis due to fibrosing mediastinitis: The Mayo Clinic experience. ( 26925456 )
2015
49
Pulmonary vein stenosis after radiofrequency ablation: in vivo optical coherence tomography insights. ( 25525060 )
2015
50
Lung pathology in pediatric pulmonary vein stenosis. ( 26458037 )
2015

Variations for Pulmonary Vein Stenosis

Expression for Pulmonary Vein Stenosis

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

Pathways for Pulmonary Vein Stenosis

GO Terms for Pulmonary Vein Stenosis

Biological processes related to Pulmonary Vein Stenosis according to GeneCards Suite gene sharing:

(show all 13)
# Name GO ID Score Top Affiliating Genes
1 positive regulation of ERK1 and ERK2 cascade GO:0070374 9.67 F2RL1 KDR PDGFRA
2 protein autophosphorylation GO:0046777 9.65 KDR KIT PDGFRA
3 peptidyl-tyrosine phosphorylation GO:0018108 9.63 KDR KIT PDGFRA
4 transmembrane receptor protein tyrosine kinase signaling pathway GO:0007169 9.58 KDR KIT PDGFRA
5 muscle filament sliding GO:0030049 9.52 ACTC1 DES
6 positive regulation of phosphatidylinositol 3-kinase activity GO:0043552 9.51 KIT PDGFRA
7 embryonic hemopoiesis GO:0035162 9.43 KDR KIT
8 positive regulation of pseudopodium assembly GO:0031274 9.37 F2RL1 KIT
9 positive regulation of positive chemotaxis GO:0050927 9.32 F2RL1 KDR
10 cardiac myofibril assembly GO:0055003 9.26 ACTC1 PDGFRA
11 positive regulation of cell migration GO:0030335 9.26 F2RL1 KDR KIT PDGFRA
12 positive regulation of phospholipase C activity GO:0010863 9.16 KIT PDGFRA
13 positive regulation of phosphatidylinositol 3-kinase signaling GO:0014068 8.92 F2RL1 KDR KIT PDGFRA

Molecular functions related to Pulmonary Vein Stenosis according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 protein tyrosine kinase activity GO:0004713 9.43 KDR KIT PDGFRA
2 signal transducer, downstream of receptor, with protein tyrosine kinase activity GO:0004716 9.16 KDR KIT
3 vascular endothelial growth factor-activated receptor activity GO:0005021 8.96 KDR PDGFRA
4 transmembrane receptor protein tyrosine kinase activity GO:0004714 8.8 KDR KIT PDGFRA

Sources for Pulmonary Vein Stenosis

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
16 ExPASy
18 FMA
27 GO
28 GTR
29 HGMD
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 MedGen
41 MeSH
42 MESH via Orphanet
43 MGI
45 NCI
46 NCIt
47 NDF-RT
50 NINDS
51 Novoseek
53 OMIM
54 OMIM via Orphanet
58 PubMed
60 QIAGEN
65 SNOMED-CT via HPO
66 SNOMED-CT via Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
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