MCID: PLM068
MIFTS: 39

Pulmonary Vein Stenosis

Categories: Blood diseases, Cardiovascular diseases, Rare diseases, Respiratory diseases

Aliases & Classifications for Pulmonary Vein Stenosis

MalaCards integrated aliases for Pulmonary Vein Stenosis:

Name: Pulmonary Vein Stenosis 20 70
Pulmonary Veins Stenosis 20

Classifications:



External Ids:

UMLS 70 C0340756

Summaries for Pulmonary Vein Stenosis

GARD : 20 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.

MalaCards based summary : Pulmonary Vein Stenosis, also known as pulmonary veins stenosis, is related to spindle cell sarcoma and sarcoma. An important gene associated with Pulmonary Vein Stenosis is ACTC1 (Actin Alpha Cardiac Muscle 1), and among its related pathways/superpathways are p70S6K Signaling and G-protein signaling RAC1 in cellular process. The drugs Methotrexate and Levoleucovorin have been mentioned in the context of this disorder. Affiliated tissues include heart, lung and skin, and related phenotypes are cardiovascular system and cellular

Wikipedia : 73 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 216)
# Related Disease Score Top Affiliating Genes
1 spindle cell sarcoma 29.9 KIT DES ACTC1
2 sarcoma 29.4 PDGFRA KIT KDR ACTC1
3 atrial fibrillation 10.8
4 pulmonary hypertension 10.7
5 heart septal defect 10.5
6 ventricular septal defect 10.5
7 fibrosing mediastinitis 10.4
8 atrial heart septal defect 10.3
9 mediastinitis 10.3
10 down syndrome 10.3
11 lung disease 10.3
12 single ventricular heart 10.3
13 fibromatosis 10.3 DES ACTC1
14 fibroblastic rheumatism 10.3 DES ACTC1
15 benign metastasizing leiomyoma 10.3 DES ACTC1
16 pulmonary hypertension, primary, 1 10.3
17 pulmonary embolism 10.3
18 bronchopulmonary dysplasia 10.3
19 cor triatriatum 10.3
20 cutaneous leiomyosarcoma 10.3 DES ACTC1
21 botryoid rhabdomyosarcoma 10.3 DES ACTC1
22 parachordoma 10.3 DES ACTC1
23 syringocystadenoma papilliferum 10.3 DES ACTC1
24 reticulum cell sarcoma 10.2 DES ACTC1
25 ectomesenchymoma 10.2 DES CD99
26 ossifying fibromyxoid tumor 10.2 DES ACTC1
27 non-langerhans-cell histiocytosis 10.2 DES ACTC1
28 perineurioma 10.2 KIT ACTC1
29 ischemic fasciitis 10.2 DES ACTC1
30 thymic epithelial tumor 10.2 KIT CD99
31 cyanosis, transient neonatal 10.2
32 heart disease 10.2
33 chondromyxoid fibroma 10.2 DES ACTC1
34 epithelioid leiomyosarcoma 10.2 DES ACTC1
35 epithelioid hemangioendothelioma 10.2 KDR ACTC1
36 congenital fibrosarcoma 10.2 DES ACTC1
37 sarcomatoid mesothelioma 10.2 DES ACTC1
38 cutaneous fibrous histiocytoma 10.2 DES ACTC1
39 necrotizing sialometaplasia 10.2 MKI67 ACTC1
40 mobitz type ii atrioventricular block 10.2 KDR ACTC1
41 total anomalous pulmonary venous return 1 10.2
42 varicose veins 10.2
43 smith-lemli-opitz syndrome 10.2
44 opitz gbbb syndrome, type i 10.2
45 patent ductus arteriosus 1 10.2
46 heterotaxy 10.2
47 chromosomal triplication 10.2
48 pulmonary venous return anomaly 10.2
49 epithelioid trophoblastic tumor 10.1 MKI67 ACTC1
50 malignant teratoma 10.1 KIT CD99

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:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 cardiovascular system MP:0005385 9.8 ACTC1 DES F2RL1 KDR KIT PDGFRA
2 cellular MP:0005384 9.76 ACTC1 DES F2RL1 KDR KIT PDGFRA
3 muscle MP:0005369 9.43 ACTC1 DES KDR KIT PDGFRA PECAM1
4 no phenotypic analysis MP:0003012 9.02 F2RL1 KDR KIT MKI67 PDGFRA

Drugs & Therapeutics for Pulmonary Vein Stenosis

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

(show all 31)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Methotrexate Approved Phase 2 1959-05-2, 59-05-2 126941
2
Levoleucovorin Approved, Investigational Phase 2 68538-85-2 149436
3
Vinblastine Approved Phase 2 865-21-4 13342 241903
4 Orange Approved Phase 1, Phase 2
5
Bevacizumab Approved, Investigational Phase 1, Phase 2 216974-75-3
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
8
Folic acid Approved, Nutraceutical, Vet_approved Phase 2 59-30-3 6037
9 Tubulin Modulators Phase 2
10 Vitamin B9 Phase 2
11 Folic Acid Antagonists Phase 2
12 Antirheumatic Agents Phase 2
13 Antimitotic Agents Phase 2
14 Immunosuppressive Agents Phase 2
15 Folate Phase 2
16 Vitamin B Complex Phase 2
17 Antimetabolites Phase 2
18 Dermatologic Agents Phase 2
19 Immunologic Factors Phase 2
20 Imatinib Mesylate Phase 1, Phase 2 220127-57-1 123596
21 Angiogenesis Inhibitors Phase 1, Phase 2
22 Antineoplastic Agents, Immunological Phase 1, Phase 2
23 Anti-Bacterial Agents Phase 1, Phase 2
24 Protein Kinase Inhibitors Phase 1, Phase 2
25 Anti-Arrhythmia Agents Phase 1, Phase 2
26 Angiotensin II Type 1 Receptor Blockers Phase 1, Phase 2
27 Angiotensin Receptor Antagonists Phase 1, Phase 2
28 Giapreza Phase 1, Phase 2
29 Angiotensinogen Phase 1, Phase 2
30 Antihypertensive Agents Phase 1, Phase 2
31 Mitogens

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Vinblastine and Methotrexate in Children With Multivessel Pulmonary Vein Stenosis-A Phase II Study Completed NCT00215046 Phase 2 Vinblastine;Methotrexate
2 Adjunct Targeted Biologic Inhibition in Children With Multivessel Intraluminal Pulmonary Vein Stenosis Completed NCT00891527 Phase 1, Phase 2 Bevacizumab (Avastin) and Imatinib Mesylate (Gleevec)
3 Pilot Trial: the Safety and Feasibility of Losartan Therapy for Treatment of Pulmonary Vein Stenosis in Pediatric Patients Suspended NCT02769130 Phase 1, Phase 2 Losartan
4 Pulmonary Vein Ablation for Atrial Fibrillation: Safety Monitoring by Transesphoageal Echo, Intracardiac Echo and Computed Tomography and Assessment of Predictors of Recurrence and of Hypercoagulable State Completed NCT00590668
5 Allogeneic Left Atrial and Pulmonary Vein Transplant for Pulmonary Vein Stenosis Recruiting NCT03456193
6 Predicting Pediatric Intraluminal Pulmonary Vein Stenosis Outcomes Using a Comprehensive Standardized Catheterization Assessment Recruiting NCT04696289
7 Measurement of Angiogenic Related Growth Factors in Urine and Blood From Patients With Multivessel Pulmonary Vein Stenosis Terminated NCT00215059

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:

40
Heart, Lung, Skin, Breast, Bone, Smooth Muscle, Small Intestine

Publications for Pulmonary Vein Stenosis

Articles related to Pulmonary Vein Stenosis:

(show top 50) (show all 664)
# Title Authors PMID Year
1
Intravascular Ultrasound for Pulmonary Vein Stenosis Interventions in Congenital Heart Disease. 61
33723085 2021
2
Single-institution outcomes of surgical repair of infracardiac total anomalous pulmonary venous connection. 61
32739162 2021
3
Pulmonary Vein Stenosis as a Pitfall of Ventilation/Perfusion SPECT/CT for Pulmonary Embolism. 61
33323737 2021
4
Clinical Outcomes Predictors and Surgical Management of Primary Pulmonary Vein Stenosis. 61
33745903 2021
5
Hemodynamic Basis for Dyspnea in Pulmonary Vein Stenosis. 61
33761753 2021
6
Primary pulmonary vein stenosis during infancy: state of the art review. 61
33674714 2021
7
Pulmonary Vein Stenosis: A Rare Disease with a Global Reach. 61
33800765 2021
8
Outcomes in Establishing Individual Vessel Patency for Pediatric Pulmonary Vein Stenosis. 61
33802089 2021
9
Correlation of Intravascular Ultrasound with Histology in Pediatric Pulmonary Vein Stenosis. 61
33806479 2021
10
The many faces and outcomes of pulmonary vein stenosis in early childhood. 61
32506838 2021
11
Evolution and Current Results of a Unified Strategy for Sinus Venosus Surgery. 61
32437676 2021
12
Mid-term outcomes of cor triatriatum repair: comparison of biventricular physiology and univentricular physiology. 61
33168116 2021
13
From Other Journals: A Review of Recent Articles in Pediatric Cardiology. 61
33025027 2021
14
Neonatal and Postneonatal Pulmonary Hypertension. 61
33670256 2021
15
Clinical Syndromic Phenotypes and the Potential Role of Genetics in Pulmonary Vein Stenosis. 61
33578785 2021
16
Successful Repair of Infantile Scimitar Syndrome with Contralateral Pulmonary Vein Stenosis in an Infant. 61
33594868 2021
17
Preliminary findings on the use of intravascular ultrasound in the assessment of pediatric pulmonary vein stenosis. 61
32936535 2021
18
Histopathology of anastomotic stenosis after total anomalous pulmonary vein connection. 61
33532846 2021
19
Pulsed field ablation: a promise that came true. 61
33264171 2021
20
Early Outcomes for In Situ Pericardial Roll Repair for Distant Anomalous Pulmonary Venous Return. 61
32339505 2021
21
Longer Exposure to Left-to-Right Shunts Is a Risk Factor for Pulmonary Vein Stenosis in Patients with Trisomy 21. 61
33401418 2021
22
Heart-Lung Transplant via an Eighth-Time Sternotomy. 61
31018757 2021
23
Experience using drug-coated balloon venoplasty for acquired pulmonary vein stenosis after radiofrequency ablation. 61
33437330 2021
24
Pulmonary vein stenosis after lung transplantation: a case report and literature review. 61
33569483 2021
25
Pulmonary Vein Stenosis After Atrial Fibrillation Ablation: Insights From the ADVICE Trial. 61
33157186 2020
26
Interventional therapy for Takayasu arteritis with pulmonary artery and pulmonary vein stenosis. 61
33226081 2020
27
The Brightest Rainbow Follows the Darkest Storm. 61
33182595 2020
28
Surgical Outcomes for Obstructive Total Anomalous Pulmonary Venous Return of the Non-Infracardiac Type Compared with the Infracardiac Type. 61
33234209 2020
29
Bifurcation pulmonary venoplasty and stenting for recalcitrant pulmonary vein stenosis after surgical pulmonary vein reconstruction. 61
33133309 2020
30
Treatment of Congenital and Acquired Pulmonary Vein Stenosis. 61
33009955 2020
31
Impact of body mass index on procedural complications, procedure duration, and radiation dose in patients with atrial fibrillation undergoing radiofrequency ablation: A systematic review and meta-analysis. 61
32492246 2020
32
Long-Term Outcomes and Improvements in Quality of Life in Patients with Atrial Fibrillation Treated with Catheter Ablation vs. Antiarrhythmic Drugs. 61
33000397 2020
33
Congenitally corrected transposition and mitral atresia complicated by restrictive atrial septum. 61
32748445 2020
34
Primary pulmonary vein stenosis among premature infants with single-vessel disease. 61
32989220 2020
35
Reduction in Pulmonary Vein Stenosis and Collateral Damage With Pulsed Field Ablation Compared With Radiofrequency Ablation in a Canine Model. 61
32877256 2020
36
Radiologic Manifestations of Pulmonary Vein Ablation Complications: A Pictorial Review. 61
32960836 2020
37
Paclitaxel-coated drug-eluting balloon for pulmonary vein stenosis after repair of total anomalous pulmonary venous return with asplenia. 61
32884589 2020
38
Commentary: Targeting neointimal lesions in pulmonary vein stenosis: Fact or fiction? 61
32145910 2020
39
Modified sutureless repair using left atrial appendage flap for acquired left-sided pulmonary vein stenosis. 61
32034909 2020
40
Computed tomography-based surgical strategy for total anomalous pulmonary venous connection. 61
32048709 2020
41
Changes in Prognosis of Heterotaxy Syndrome Over Time. 61
32647064 2020
42
Pulmonary Vein Stenosis and Pulmonary Hypertension Following a Catheter-Based Radiofrequency Ablation for Atrial Fibrillation: A Case Report. 61
32844783 2020
43
Percutaneous Pulmonary Vein Stenting to Treat Severe Pulmonary Vein Stenosis After Surgical Reconstruction. 61
32639846 2020
44
Sutureless technique using the left atrial appendage for left pulmonary vein occlusion after catheter ablation. 61
32705435 2020
45
Severe pulmonary vein stenosis requiring angioplasty after hot balloon ablation for pulmonary vein isolation. 61
32695604 2020
46
Commentary: Pulmonary vein stenosis: Not just a surgical solution. 61
32868046 2020
47
Pulmonary vein stenosis: Treatment and challenges. 61
32763037 2020
48
Double-Orifice Mitral Valve Associated with Bicuspid Aortic Valve and Primary Pulmonary Vein Stenosis. 61
32577596 2020
49
Pulmonary vein stenosis mimicking interstitial lung disease. 61
32571674 2020
50
[Impact factors of in-stent restenosis following successful stent implantation for acquired severe pulmonary vein stenosis post atrial fibrillation ablation]. 61
32450653 2020

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

Pathways related to Pulmonary Vein Stenosis according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1
Show member pathways
12.6 TYMP PDGFRA KIT KDR F2RL1
2
Show member pathways
12.03 TYMP PDGFRA KIT KDR
3
Show member pathways
11.88 TYMP PDGFRA KIT KDR
4 11.75 PDGFRA KIT KDR
5 11.11 PDGFRA KDR
6 10.98 PDGFRA KIT KDR ACTC1
7 10.97 TYMP PECAM1 PDGFRA KDR F2RL1
8
Show member pathways
10.86 PDGFRA KIT KDR
9 10.84 PDGFRA KDR
10 10.56 TYMP PDGFRA KIT KDR DES

GO Terms for Pulmonary Vein Stenosis

Cellular components related to Pulmonary Vein Stenosis according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 receptor complex GO:0043235 9.33 PDGFRA KIT KDR
2 cell-cell junction GO:0005911 9.13 PECAM1 KIT DES
3 integral component of plasma membrane GO:0005887 9.1 PECAM1 PDGFRA KIT KDR F2RL1 CD99

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

(show all 17)
# Name GO ID Score Top Affiliating Genes
1 extracellular matrix organization GO:0030198 9.74 PECAM1 PDGFRA KDR
2 positive regulation of ERK1 and ERK2 cascade GO:0070374 9.72 PDGFRA KDR F2RL1
3 protein autophosphorylation GO:0046777 9.7 PDGFRA KIT KDR
4 peptidyl-tyrosine phosphorylation GO:0018108 9.65 PDGFRA KIT KDR
5 transmembrane receptor protein tyrosine kinase signaling pathway GO:0007169 9.63 PDGFRA KIT KDR
6 muscle filament sliding GO:0030049 9.56 DES ACTC1
7 positive regulation of phosphatidylinositol 3-kinase activity GO:0043552 9.54 PDGFRA KIT
8 establishment of endothelial barrier GO:0061028 9.51 PECAM1 F2RL1
9 embryonic hemopoiesis GO:0035162 9.49 KIT KDR
10 positive regulation of pseudopodium assembly GO:0031274 9.43 KIT F2RL1
11 positive regulation of kinase activity GO:0033674 9.43 PDGFRA KIT KDR
12 positive regulation of phospholipase C activity GO:0010863 9.4 PDGFRA KIT
13 positive regulation of positive chemotaxis GO:0050927 9.37 KDR F2RL1
14 hematopoietic progenitor cell differentiation GO:0002244 9.33 PDGFRA KIT KDR
15 cardiac myofibril assembly GO:0055003 9.32 PDGFRA ACTC1
16 positive regulation of phosphatidylinositol 3-kinase signaling GO:0014068 9.26 PDGFRA KIT KDR F2RL1
17 positive regulation of cell migration GO:0030335 9.02 PECAM1 PDGFRA KIT KDR F2RL1

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

# Name GO ID Score Top Affiliating Genes
1 protein homodimerization activity GO:0042803 9.62 TYMP PECAM1 PDGFRA KIT
2 protein tyrosine kinase activity GO:0004713 9.43 PDGFRA KIT KDR
3 vascular endothelial growth factor-activated receptor activity GO:0005021 9.16 PDGFRA KDR
4 vascular endothelial growth factor binding GO:0038085 8.96 PDGFRA KDR
5 transmembrane receptor protein tyrosine kinase activity GO:0004714 8.8 PDGFRA KIT KDR

Sources for Pulmonary Vein Stenosis

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
20 GARD
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
53 NINDS
54 Novoseek
56 OMIM via Orphanet
57 OMIM® (Updated 20-May-2021)
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 Tocris
70 UMLS
71 UMLS via Orphanet
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