APVR
MCID: PLM069
MIFTS: 26

Pulmonary Venous Return Anomaly (APVR)

Categories: Cardiovascular diseases, Fetal diseases, Rare diseases

Aliases & Classifications for Pulmonary Venous Return Anomaly

MalaCards integrated aliases for Pulmonary Venous Return Anomaly:

Name: Pulmonary Venous Return Anomaly 52
Total Anomalous Pulmonary Venous Return 52
Anomalous Pulmonary Venous Return 52
Scimitar Syndrome 52
Scimitar Anomaly 52
Tapvr1 52
Tapvr 52
Apvr 52

Classifications:



Summaries for Pulmonary Venous Return Anomaly

NIH Rare Diseases : 52 The following summary is from Orphanet , a European reference portal for information on rare diseases and orphan drugs. Orpha Number: 3090 Definition A rare developmental defect during embryogenesis where some or all of the pulmonary veins drain into the right atrium or the systemic veins, with or without the presence of pulmonary venous obstruction, leading to various manifestations such as fatigue, exertional dyspnea, pulmonary arterial hypertension , cyanosis and progressive congestive heart failure. The two main subtypes are congenital partial pulmonary venous return anomaly (PAPVC; see this term), where one or a few of the pulmonary veins are anomalous, and congenital total pulmonary venous return anomaly (TAPVC, see this term), where all of the pulmonary veins are anomalous. Visit the Orphanet disease page for more resources.

MalaCards based summary : Pulmonary Venous Return Anomaly, also known as total anomalous pulmonary venous return, is related to congenital pulmonary venous return anomaly and congenital partial pulmonary venous return anomaly. An important gene associated with Pulmonary Venous Return Anomaly is ANKRD1 (Ankyrin Repeat Domain 1). The drugs Remifentanil and Rocuronium have been mentioned in the context of this disorder. Affiliated tissues include heart, eye and lung.

Related Diseases for Pulmonary Venous Return Anomaly

Diseases in the Pulmonary Venous Return Anomaly family:

Congenital Pulmonary Venous Return Anomaly

Diseases related to Pulmonary Venous Return Anomaly via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 151)
# Related Disease Score Top Affiliating Genes
1 congenital pulmonary venous return anomaly 12.7
2 congenital partial pulmonary venous return anomaly 12.6
3 total anomalous pulmonary venous return 1 11.8
4 congenital heart defects, multiple types, 6 11.7
5 cardiac-urogenital syndrome 11.7
6 scimitar anomaly, multiple cardiac malformations, and craniofacial and central nervous system abnormalities 11.7
7 lung agenesis, congenital heart defects, and thumb anomalies syndrome 11.6
8 structural heart defects and renal anomalies syndrome 11.6
9 manouvrier syndrome 11.6
10 meacham winn culler syndrome 11.6
11 pulmonary hypertension 10.8
12 cor triatriatum 10.7
13 cyanosis, transient neonatal 10.6
14 mitral valve stenosis 10.6
15 heterotaxy 10.5
16 pulmonary vein stenosis 10.5
17 tetralogy of fallot 10.4
18 patent ductus arteriosus 1 10.4
19 cardiac arrest 10.4
20 dextro-looped transposition of the great arteries 10.4
21 gonadal dysgenesis 10.4
22 turner syndrome 10.4
23 cor triatriatum sinister 10.4
24 single ventricular heart 10.4
25 pulmonary hypertension, primary, 1 10.3
26 patent foramen ovale 10.3
27 congestive heart failure 10.3
28 dextrocardia 10.3
29 pulmonary artery hypoplasia 10.3
30 coarctation of aorta 10.3
31 varicose veins 10.3
32 respiratory failure 10.3
33 pulmonary edema 10.3
34 ventricular septal defect 10.3
35 47,xyy 10.3
36 interatrial communication 10.3
37 progressive familial heart block, type ia 10.2
38 papillomatosis, confluent and reticulated 10.2
39 thrombophilia due to thrombin defect 10.2
40 williams-beuren syndrome 10.2
41 multinucleated neurons, anhydramnios, renal dysplasia, cerebellar hypoplasia, and hydranencephaly 10.2
42 progressive familial heart block, type ib 10.2
43 esophageal varix 10.2
44 right bundle branch block 10.2
45 vascular disease 10.2
46 double outlet right ventricle 10.2
47 situs inversus 10.2
48 hypoplastic left heart syndrome 10.2
49 double discordia 10.2
50 univentricular heart 10.2

Graphical network of the top 20 diseases related to Pulmonary Venous Return Anomaly:



Diseases related to Pulmonary Venous Return Anomaly

Symptoms & Phenotypes for Pulmonary Venous Return Anomaly

Drugs & Therapeutics for Pulmonary Venous Return Anomaly

Drugs for Pulmonary Venous Return Anomaly (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 18)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Remifentanil Approved Phase 4 132875-61-7 60815
2
Rocuronium Approved Phase 4 119302-91-9, 143558-00-3 441290
3
Sevoflurane Approved, Vet_approved Phase 4 28523-86-6 5206
4 Narcotics Phase 4
5 Neuromuscular Blocking Agents Phase 4
6 Anesthetics, General Phase 4
7 Platelet Aggregation Inhibitors Phase 4
8 Anesthetics Phase 4
9 Analgesics Phase 4
10 Analgesics, Opioid Phase 4
11 Anesthetics, Inhalation Phase 4
12
Heparin Approved, Investigational 9005-49-6 46507594 772
13
Aspirin Approved, Vet_approved 50-78-2 2244
14 Fibrinolytic Agents
15 Pharmaceutical Solutions
16 Anticoagulants
17 Coagulants
18 calcium heparin

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Impact of Deep Neuromuscular Block Versus Inhalation and Total Intravenous Anesthesia (TIVA) on Laparoscopic Surgical Workspace Defined as Insufflated Pneumoperitoneum Volume. Completed NCT01930747 Phase 4 rocuronium;Sevoflurane;remifentanyl
2 Comparison of Fibrinogen Concentrate and Cryoprecipitate in Pediatric Cardiac Surgery Patients Not yet recruiting NCT04376762 Phase 4 Fibrinogen;Cryoprecipitate
3 Multicenter, Double-Blind, Placebo-Controlled Study to Evaluate Efficacy/Safety of CY-1503 (Cylexin) in Prevention of Reperfusion Injury in Neonates/Infants Undergoing Hypothermic Cardiopulmonary Bypass Completed NCT00226369 Phase 2, Phase 3 CY-1503
4 Reproduction and Survival After Cardiac Defect Repair Completed NCT00005190
5 Oxygen Saturation and Perfusion Index Screening ın Neonates at High Altitude Completed NCT04290481
6 Sutureless Technique and Conventional Repairs for Total Anomalous Pulmonary Venous Connection: an Observational Study by Chinese Heart Centers Recruiting NCT04234711
7 Anticoagulation Medicine in Surgical Repair for Total Anomalous Pulmonary Venous Connection: a Randomize Multi-centers Study Recruiting NCT04241380 Anticoagulant Solutions;Anticoagulant management
8 A Review of the Diagnosis, Treatment and Outcomes of Children With Critical Congenital Heart Defects Terminated NCT00208689
9 Proposal for Retrospective Review of Descending Aortic Flow Reversal in Total Anomalous Pulmonary Venous Connection Terminated NCT00486070

Search NIH Clinical Center for Pulmonary Venous Return Anomaly

Genetic Tests for Pulmonary Venous Return Anomaly

Anatomical Context for Pulmonary Venous Return Anomaly

MalaCards organs/tissues related to Pulmonary Venous Return Anomaly:

40
Heart, Eye, Lung, Testes, Liver, Brain, Kidney

Publications for Pulmonary Venous Return Anomaly

Articles related to Pulmonary Venous Return Anomaly:

(show top 50) (show all 403)
# Title Authors PMID Year
1
Congenital extracardiac venous system anomaly in two siblings with normal karyotype and increased nuchal translucency thickness: a case report. 61
32551126 2020
2
Adults forms of scimitar syndrome. 61
32445203 2020
3
Pediatric Cardiology Specialist's Opinions Toward the Acceptability of Comfort Care for Congenital Heart Disease. 61
32419096 2020
4
A case of infracardiac total anomalous pulmonary venous return. 61
31487091 2020
5
Outcomes of hypoplastic left heart syndrome: analysis of National Inpatient Sample Database 1998-2004 versus 2005-2014. 61
31741094 2020
6
Myocardial overexpression of ANKRD1 causes sinus venosus defects and progressive diastolic dysfunction. 61
31688894 2019
7
Type A3 truncus arteriosus with infracardiac total anomalous pulmonary venous return and single ventricle physiology: A triad of tribulations. 61
31558378 2019
8
A Rare Case of Cardiogenic Shock: Cor Triatriatum With Anomalous Pulmonary Venous Return. 61
31496401 2019
9
Multimodal Endovascular approach in a Case of Multiple Dural Arteriovenous Fistulae. 61
31536637 2019
10
Characterisation of computed tomography angiography findings in paediatric patients with heterotaxy. 61
31165901 2019
11
Risk factors for death or heart transplantation in single-ventricle physiology (tricuspid atresia, pulmonary atresia, and heterotaxy): A systematic review and meta-analysis. 61
31006521 2019
12
Prenatal diagnosis and prevalence of critical congenital heart defects: an international retrospective cohort study. 61
31270117 2019
13
Post-Extubation Inhaled Nitric Oxide Therapy via High-Flow Nasal Cannula After Fontan Procedure. 61
31065760 2019
14
Infracardiac total anomalous pulmonary venous return in a patient with Williams syndrome: A case report. 61
31261599 2019
15
Comparison of prenatal ultrasound and postmortem findings in fetuses with common pulmonary vein atresia. 61
31116443 2019
16
Vascular Anomalies of the Pediatric Liver. 61
31059404 2019
17
Computed Tomography Angiography and Magnetic Resonance Angiography of Congenital Anomalies of Pulmonary Veins. 61
31082945 2019
18
Computed Tomography Angiography and Magnetic Resonance Angiography of Congenital Anomalies of Pulmonary Veins. 61
31082974 2019
19
A Novel Mouse Model for Cilia-Associated Cardiovascular Anomalies with a High Penetrance of Total Anomalous Pulmonary Venous Return. 61
30289203 2019
20
Contemporary Outcomes After Repair of Isolated and Complex Complete Atrioventricular Septal Defect. 61
30009807 2018
21
Familial total anomalous pulmonary venous return with 15q11.2 (BP1-BP2) microdeletion. 61
30108319 2018
22
A case of mixed drainage total anomalous pulmonary venous return in an adult patient. 61
32082811 2018
23
Neonatal Outcomes in Total Anomalous Pulmonary Venous Return: The Role of Prenatal Diagnosis and Pulmonary Venous Obstruction. 61
29796693 2018
24
Accuracy of echocardiography in diagnosing total anomalous pulmonary venous return. 61
30344556 2018
25
Prevalence of congenital cardiac anomalies in patients with cleft lip and palate - Its implications in surgical management. 61
30191117 2018
26
Pulmonary hypertension in Takayasu arteritis. 61
30146739 2018
27
Outcomes and prognostic factors for postsurgical pulmonary vein stenosis in the current era. 61
29576261 2018
28
Total Anomalous Pulmonary Venous Return with No Connection. 61
30062319 2018
29
A Modified Algorithm for Critical Congenital Heart Disease Screening Using Pulse Oximetry. 61
29691284 2018
30
Successful management of living donor liver transplantation for biliary atresia with single ventricle physiology-from peri-transplant through total cavopulmonary connection: A case report. 61
29457852 2018
31
Congenital second-degree heart block and total anomalous pulmonary venous return associated with microduplication of 1q32.2. 61
29922018 2018
32
An Unusual Neonatal Presentation of Scimitar Syndrome. 61
29930881 2018
33
Beating the odds: a rare case of supracardiac total anomalous pulmonary venous return (TAPVR) in an adult patient. 61
29507009 2018
34
Successful Surgical Repair and Perioperative Management of 6-Month-Old With Total Anomalous Pulmonary Venous Return in a Developing Country: Considerations for the Treatment of Pulmonary Hypertension. 61
29479388 2018
35
Morphological dilemma: Anomalous pulmonary venous confluence or cor triatriatum-does it matter? 61
29296068 2018
36
Mixed Total Anomalous Pulmonary Venous Return With Ascending and Descending Vertical Veins. 61
28901227 2017
37
Holt-Oram Syndrome: A Rare Variant. 61
28761211 2017
38
What Is the Teratogenic Risk of Mycophenolate? 61
28497000 2017
39
Infracardiac type total anomalous pulmonary venous return with obstruction and dilatation of portal vein. 61
28491157 2017
40
Obstructed Infradiaphragmatic Total Anomalous Pulmonary Venous Return in a 13-Day-Old Infant Presenting Acutely to the Emergency Department: A Case Report. 61
28285866 2017
41
Whole-exome sequencing identifies SGCD and ACVRL1 mutations associated with total anomalous pulmonary venous return (TAPVR) in Chinese population. 61
28412737 2017
42
Concordance of fetal echocardiography in the diagnosis of congenital cardiac disease utilizing updated guidelines. 61
28277918 2017
43
Ivemark syndrome: bronchial compression from anomalous pulmonary venous anatomy. 61
28458851 2017
44
Anomalous left coronary artery from the pulmonary artery discovered following total anomalous pulmonary venous return repair: a rare entity. 61
27573031 2017
45
Multidetector Computed Tomographic Angiography Imaging of Congenital Pulmonary Venous Anomalies: A Pictorial Review. 61
27720432 2017
46
Real time three-dimensional transesophageal echocardiography imaging of crescent-shaped superior sinus venosus atrial septal defect. 61
27699850 2017
47
Congenital heart defects in Williams syndrome. 61
29376566 2017
48
Continuous Cardiopulmonary Bypass During the Repair of Total Anomalous Pulmonary Venous Return. 61
27834770 2016
49
Outcomes of Surgery for Total Anomalous Pulmonary Venous Return without Total Circulatory Arrest. 61
27733992 2016
50
[Pediatric Iatrogenic Vertebral Arteriovenous Fistula Successfully Treated with Endovascular Treatment:A Case Report]. 61
27729606 2016

Variations for Pulmonary Venous Return Anomaly

Expression for Pulmonary Venous Return Anomaly

Search GEO for disease gene expression data for Pulmonary Venous Return Anomaly.

Pathways for Pulmonary Venous Return Anomaly

GO Terms for Pulmonary Venous Return Anomaly

Sources for Pulmonary Venous Return Anomaly

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
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
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
53 NINDS
54 Novoseek
56 OMIM
57 OMIM via Orphanet
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
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