MCID: UNL013
MIFTS: 20

Unilateral Absence of a Pulmonary Artery

Categories: Cardiovascular diseases, Rare diseases, Respiratory diseases

Aliases & Classifications for Unilateral Absence of a Pulmonary Artery

MalaCards integrated aliases for Unilateral Absence of a Pulmonary Artery:

Name: Unilateral Absence of a Pulmonary Artery 20
Isolated Unilateral Absence of a Pulmonary Artery 20
Pulmonary Artery, Isolated Unilateral Absence of 20
Congenital Absence of the Pulmonary Artery 20
Isolated Uapa 20

Classifications:



Summaries for Unilateral Absence of a Pulmonary Artery

GARD : 20 Unilateral absence of the pulmonary artery (UAPA) is a heart defect that is present from birth. The pulmonary artery takes blood from the heart to the lungs. In the absence of a pulmonary artery, other blood vessels compensate by supplying blood to the lungs. Pressure can build inside these vessels and lead to heart and lung complications. UAPA can be left sided or right sided. It most often occurs alone, but can occur with other heart anomalies and birth defects. Signs and symptoms include repeat lung infections, lower exercise tolerance, shortness of breath, chest pain, and fluid in the lungs. Risk for these symptoms increase with age. Complications of UAPA include, hemoptysis (coughing up blood), respiratory hemorrhage, and high blood pressure in the arteries to the lung ( pulmonary hypertension ). Serious complications may be triggered by stress on the body, such as pregnancy and altitude sickness.

MalaCards based summary : Unilateral Absence of a Pulmonary Artery, also known as isolated unilateral absence of a pulmonary artery, is related to pulmonary hypertension and vascular disease. An important gene associated with Unilateral Absence of a Pulmonary Artery is THBD (Thrombomodulin), and among its related pathways/superpathways are Formation of Fibrin Clot (Clotting Cascade) and Complement and coagulation cascades. Affiliated tissues include heart, lung and thyroid.

Related Diseases for Unilateral Absence of a Pulmonary Artery

Diseases related to Unilateral Absence of a Pulmonary Artery via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 52)
# Related Disease Score Top Affiliating Genes
1 pulmonary hypertension 30.3 VWF THBD
2 vascular disease 29.9 VWF THBD
3 tetralogy of fallot 10.4
4 patent ductus arteriosus 1 10.2
5 pain agnosia 10.2
6 cyanosis, transient neonatal 10.1
7 pulmonary valve insufficiency 10.1
8 dextrocardia 10.1
9 absence of the pulmonary artery 10.1
10 right aortic arch 10.1
11 thrombotic microangiopathy 9.9 VWF THBD
12 eisenmenger syndrome 9.9 VWF THBD
13 thyroid angiosarcoma 9.9 VWF THBD
14 thrombophilia due to activated protein c resistance 9.9 VWF THBD
15 thrombophilia 9.9 VWF THBD
16 diabetic angiopathy 9.9 VWF THBD
17 spotted fever 9.9 VWF THBD
18 malignant hypertension 9.9 VWF THBD
19 pulmonary artery disease 9.9 VWF THBD
20 hepatic vascular disease 9.9 VWF THBD
21 factor xi deficiency 9.9 VWF THBD
22 collagen disease 9.9 VWF THBD
23 thrombotic thrombocytopenic purpura 9.9 VWF THBD
24 purpura 9.8 VWF THBD
25 thrombophilia due to thrombin defect 9.8 VWF THBD
26 varicose veins 9.8 VWF THBD
27 homocysteinemia 9.8 VWF THBD
28 disseminated intravascular coagulation 9.8 VWF THBD
29 antiphospholipid syndrome 9.8 VWF THBD
30 pulmonary embolism 9.8 VWF THBD
31 hemolytic-uremic syndrome 9.8 VWF THBD
32 hellp syndrome 9.8 VWF THBD
33 hemolytic uremic syndrome, atypical 1 9.8 VWF THBD
34 peripheral vascular disease 9.8 VWF THBD
35 blood platelet disease 9.8 VWF THBD
36 vasculitis 9.8 VWF THBD
37 blood coagulation disease 9.8 VWF THBD
38 atherosclerosis susceptibility 9.8 VWF THBD
39 polycythemia vera 9.8 VWF THBD
40 essential thrombocythemia 9.8 VWF THBD
41 behcet syndrome 9.8 VWF THBD
42 acute myocardial infarction 9.8 VWF THBD
43 cerebrovascular disease 9.8 VWF THBD
44 thrombosis 9.8 VWF THBD
45 hemorrhagic disease 9.8 VWF THBD
46 stroke, ischemic 9.7 VWF THBD
47 cardiovascular system disease 9.7 VWF THBD
48 hemolytic anemia 9.7 VWF THBD
49 pre-eclampsia 9.7 VWF THBD
50 aplastic anemia 9.6 VWF THBD

Graphical network of the top 20 diseases related to Unilateral Absence of a Pulmonary Artery:



Diseases related to Unilateral Absence of a Pulmonary Artery

Symptoms & Phenotypes for Unilateral Absence of a Pulmonary Artery

Drugs & Therapeutics for Unilateral Absence of a Pulmonary Artery

Search Clinical Trials , NIH Clinical Center for Unilateral Absence of a Pulmonary Artery

Genetic Tests for Unilateral Absence of a Pulmonary Artery

Anatomical Context for Unilateral Absence of a Pulmonary Artery

MalaCards organs/tissues related to Unilateral Absence of a Pulmonary Artery:

40
Heart, Lung, Thyroid

Publications for Unilateral Absence of a Pulmonary Artery

Articles related to Unilateral Absence of a Pulmonary Artery:

(show top 50) (show all 56)
# Title Authors PMID Year
1
Unilateral absence of pulmonary artery associated with lung cancer: analysis of operation strategy based on digital preoperative planning and surgical simulation with three-dimensional visualization. 61
33559715 2021
2
Long-term outcomes of percutaneous closure of patent ductus arteriosus associated with unilateral absence of a pulmonary artery. 61
33738816 2021
3
Coronary Artery Fistula Unmasking the Absence of Left Pulmonary Artery in an Adult. 61
32206543 2020
4
Unilateral Pulmonary Fibrosis Due to Absence of Right Pulmonary Artery. 61
31528513 2019
5
Autologous Tissue Technique to Repair Unilateral Isolated Absence of a Pulmonary Artery. 61
31018755 2019
6
Isolated Unilateral Absence of Pulmonary Artery Associated with Contralateral Lung Cancer. 61
30109208 2018
7
[Surgery for a Metastatic Tumor in the Right Lung in Case of Congenital Left Pulmonary Artery Defect]. 61
30042243 2018
8
Anesthesia for cesarean section in a patient with isolated unilateral absence of a pulmonary artery. 61
28017175 2017
9
[Anesthesia for cesarean section in a patient with isolated unilateral absence of a pulmonary artery]. 61
25896642 2017
10
Unilateral absence of pulmonary artery associated with contralateral lung cancer. 61
27747032 2016
11
Selective Embolization of Systemic Collaterals for the Treatment of Recurrent Hemoptysis Secondary to the Unilateral Absence of a Pulmonary Artery in a Child. 61
25145922 2015
12
Bosentan in pulmonary hypertension secondary to unilateral absence of a pulmonary artery. 61
25965593 2015
13
Repair of congenital heart defects associated with single pulmonary artery. 61
24855279 2015
14
[Unilateral agenesis of pulmonal artery in adults: a rare disease frequently undiagnosed]. 61
25668609 2015
15
Right pulmonary artery agenesis with patent ductus arteriosus and Eisenmenger syndrome: a rare case diagnosed during the postpartum period. 61
24856223 2014
16
A rare cause of recurrent respiratory tract infection: isolated absence of the right pulmonary artery. 61
24911855 2014
17
[Unilateral pulmonary artery agenesis with ipsilateral pulmonary hypoplasia as incidental finding in an asthmatic patient]. 61
24913001 2014
18
Congenital absence of left pulmonary artery in association with isolated pulmonary valvular stenosis:. 61
24669680 2013
19
A hidden cause of recurrent hemoptysis: unilateral absence of a pulmonary artery. 61
23786954 2013
20
Unilateral absence of right proximal pulmonary artery in the setting of severe pulmonary hypertension: considerations for surgical repair and perioperative management. 61
22566264 2012
21
Unilateral absence of a pulmonary artery: a rare disorder with variable presentation. 61
22481838 2012
22
Absence of the right pulmonary artery associated with a partial anomalous pulmonary venous connection. 61
21938540 2012
23
Age-related clinical characteristics of isolated congenital unilateral absence of a pulmonary artery. 61
20814784 2010
24
Unilateral absence of the left pulmonary artery accompanied by right lung cancer. 61
20609736 2010
25
A 36-year-old woman with a history of dextrocardia and dyspnea. Diagnosis: unilateral absence of a pulmonary artery (UAPA) with right hypoplastic lung. 61
19648732 2010
26
A case of congenital agenesis of the right pulmonary artery presenting with hemoptysis and mimicking pulmonary hemosiderosis. 61
18478262 2009
27
Case of the Month #134. Unilateral absence of a pulmonary artery. 61
18697722 2008
28
Unilateral absence of a pulmonary artery in absent pulmonary valve syndrome: a case report and review of literature. 61
17095983 2006
29
Pregnancy with congenital unilateral absence of a pulmonary artery. 61
16752272 2006
30
Isolated unilateral absence of a pulmonary artery treated by pneumonectomy in an adult: report of a case. 61
16715422 2006
31
Isolated unilateral absence of a pulmonary artery: a case report and review of the literature. 61
12377882 2002
32
Isolated unilateral absence of a pulmonary artery: influence of systemic circulation on alveolar capillary vessels. 61
11564222 2001
33
Unilateral absence of a pulmonary artery. 61
9880979 1998
34
Unilateral pulmonary artery agenesis presenting with hemoptysis: treatment by embolization of systemic collaterals. 61
8581907 1995
35
Absent pulmonary valve syndrome with agenesis of the left pulmonary artery. 61
7974816 1994
36
Unilateral absence of a pulmonary artery. Data from cardiopulmonary exercise testing. 61
8417905 1993
37
MRI of absent left pulmonary artery. 61
1629426 1992
38
Unilateral absence of a pulmonary artery: congenital disease or embolic occlusion? 61
1804679 1991
39
Congenital unilateral absence of a pulmonary artery: a report of two adult cases. 61
2310105 1990
40
[A case of congenital unilateral absence of a pulmonary artery]. 61
3441078 1987
41
Combined unilateral pulmonary artery agenesis and contralateral peripheral pulmonary artery stenosis. 61
3594949 1987
42
Isolated unilateral absence of left pulmonary artery with peribronchial arteriovenous malformation showing recurrent hemoptysis. 61
3601465 1987
43
[Congenital unilateral absence of a pulmonary artery]. 61
3442923 1987
44
Unilateral absence of a pulmonary artery. 61
3843667 1985
45
Fifteen-year experience with surgical repair of truncus arteriosus. 61
3974276 1985
46
High-altitude pulmonary edema with absent right pulmonary artery. 61
3969332 1985
47
Therapeutic considerations in congenital absence of the right pulmonary artery. Use of internal mammary artery as a preparatory shunt. 61
6736120 1984
48
Unilateral absence of a pulmonary artery. 61
6641308 1983
49
Pulmonary vein wedge angiography: indications, results, and surgical correlates in 25 patients. 61
6829468 1983
50
Absent pulmonary artery. A hint to its embryogenesis. 61
6761655 1982

Variations for Unilateral Absence of a Pulmonary Artery

Expression for Unilateral Absence of a Pulmonary Artery

Search GEO for disease gene expression data for Unilateral Absence of a Pulmonary Artery.

Pathways for Unilateral Absence of a Pulmonary Artery

Pathways related to Unilateral Absence of a Pulmonary Artery according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1
Show member pathways
11.16 VWF THBD
2 10.78 VWF THBD

GO Terms for Unilateral Absence of a Pulmonary Artery

Biological processes related to Unilateral Absence of a Pulmonary Artery according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 blood coagulation GO:0007596 8.96 VWF THBD
2 hemostasis GO:0007599 8.62 VWF THBD

Sources for Unilateral Absence of a Pulmonary Artery

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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