MCID: CMP035
MIFTS: 30

Complete Atrioventricular Canal

Categories: Cardiovascular diseases, Fetal diseases, Rare diseases

Aliases & Classifications for Complete Atrioventricular Canal

MalaCards integrated aliases for Complete Atrioventricular Canal:

Name: Complete Atrioventricular Canal 54 60
Common Atrioventricular Canal 54 60 74
Complete Atrioventricular Septal Defect 54 60
Cavc 54 60

Characteristics:

Orphanet epidemiological data:

60
complete atrioventricular canal
Inheritance: Not applicable; Prevalence: 1-5/10000 (Europe); Age of onset: Infancy,Neonatal;

Classifications:



External Ids:

ICD10 via Orphanet 35 Q21.2
UMLS via Orphanet 75 C0221215 C0344787
Orphanet 60 ORPHA1329
SNOMED-CT via HPO 70 253273004 396351009 59494005
UMLS 74 C0221215

Summaries for Complete Atrioventricular Canal

MalaCards based summary : Complete Atrioventricular Canal, also known as common atrioventricular canal, is related to complete atrioventricular canal-tetralogy of fallot syndrome and complete atrioventricular canal-ventricle hypoplasia syndrome. An important gene associated with Complete Atrioventricular Canal is LEFTY2 (Left-Right Determination Factor 2). The drugs Sodium Citrate and Fentanyl have been mentioned in the context of this disorder. Affiliated tissues include heart and small intestine, and related phenotypes are abnormal cardiac septum morphology and abnormality of the cardiac septa

Related Diseases for Complete Atrioventricular Canal

Diseases related to Complete Atrioventricular Canal via text searches within MalaCards or GeneCards Suite gene sharing:

(show all 45)
# Related Disease Score Top Affiliating Genes
1 complete atrioventricular canal-tetralogy of fallot syndrome 12.4
2 complete atrioventricular canal-ventricle hypoplasia syndrome 12.4
3 complete atrioventricular canal-left heart obstruction syndrome 12.4
4 partial atrioventricular canal 11.5
5 atrioventricular septal defect 10.6
6 tetralogy of fallot 10.6
7 double outlet right ventricle 10.4
8 down syndrome 10.3
9 pulmonary hypertension 10.3
10 aortopulmonary window 10.3
11 cor triatriatum 10.2
12 pulmonary hypertension, primary, 1 10.2
13 aortic arch interruption 10.2
14 pulmonary vein stenosis 10.2
15 ventricular septal defect 10.1
16 vascular disease 10.1
17 transposition of the great arteries 10.1
18 atrial heart septal defect 10.1
19 hypertension, essential 10.0
20 ebstein anomaly 10.0
21 infective endocarditis 10.0
22 aortic coarctation 10.0
23 hypoxia 10.0
24 noonan syndrome 1 9.9
25 williams-beuren syndrome 9.9
26 heterotaxy, visceral, 1, x-linked 9.9
27 tricuspid atresia 9.9
28 chromosome 3pter-p25 deletion syndrome 9.9
29 single ventricular heart 9.9
30 endocarditis 9.9
31 mitral valve insufficiency 9.9
32 situs inversus 9.9
33 dextrocardia 9.9
34 heterotaxy 9.9
35 hypertrophic cardiomyopathy 9.9
36 atrial septal defect coronary sinus 9.9
37 chromosomal triplication 9.9
38 congenitally corrected transposition of the great arteries 9.9
39 double discordia 9.9
40 pseudo-turner syndrome 9.9
41 right atrial isomerism 9.9
42 conotruncal heart malformations 9.9
43 ellis-van creveld syndrome 9.9
44 mitral valve stenosis 9.9
45 mitral atresia 9.9

Graphical network of the top 20 diseases related to Complete Atrioventricular Canal:



Diseases related to Complete Atrioventricular Canal

Symptoms & Phenotypes for Complete Atrioventricular Canal

Human phenotypes related to Complete Atrioventricular Canal:

33 60
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 abnormal cardiac septum morphology 33 hallmark (90%) HP:0001671
2 abnormality of the cardiac septa 60 Very frequent (99-80%)

Drugs & Therapeutics for Complete Atrioventricular Canal

Drugs for Complete Atrioventricular Canal (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 34)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Sodium Citrate Approved, Investigational Phase 2 68-04-2
2
Fentanyl Approved, Illicit, Investigational, Vet_approved Phase 2 437-38-7 3345
3
Dexmedetomidine Approved, Vet_approved Phase 2 113775-47-6 5311068 68602
4
Calcium Approved, Nutraceutical Phase 2 7440-70-2 271
5
Citric Acid Approved, Nutraceutical, Vet_approved Phase 2 77-92-9 311
6 Anesthetics, General Phase 2
7 Anticoagulants Phase 2
8 Adrenergic Agents Phase 2
9 Adrenergic alpha-Agonists Phase 2
10 Adrenergic Agonists Phase 2
11 Anesthetics Phase 2
12 Hypnotics and Sedatives Phase 2
13 Anesthetics, Intravenous Phase 2
14 Adjuvants, Anesthesia Phase 2
15 Neurotransmitter Agents Phase 2
16 Adrenergic alpha-2 Receptor Agonists Phase 2
17 Calcium, Dietary Phase 2
18 Analgesics, Opioid Phase 2
19 Narcotics Phase 2
20 Analgesics, Non-Narcotic Phase 2,Not Applicable
21 Central Nervous System Depressants Phase 2
22 Chelating Agents Phase 2
23 Peripheral Nervous System Agents Phase 2,Not Applicable
24 Citrate Phase 2
25 Analgesics Phase 2,Not Applicable
26 Plasma Substitutes Phase 2
27 Dextrans Phase 2
28 Blood Substitutes Phase 2
29 Pharmaceutical Solutions Phase 2
30
Nitric Oxide Approved Phase 1 10102-43-9 145068
31 carnitine Phase 1
32
Mechlorethamine Approved, Investigational 51-75-2 4033
33
Acetaminophen Approved Not Applicable 103-90-2 1983
34 Antipyretics Not Applicable

Interventional clinical trials:

(show all 13)
# Name Status NCT ID Phase Drugs
1 Study of Triostat in Infants During Heart Surgery Completed NCT00027417 Phase 3 Liothyronine sodium/triiodothyronine
2 Intravenous L-Citrulline to Treat Children Undergoing Heart Bypass Surgery : Revised Protocol Completed NCT01120964 Phase 1, Phase 2 Intravenous L-Citrulline;Placebo of Intravenous L-Citrulline
3 Measures to Lower the Stress Response in Pediatric Cardiac Surgery Completed NCT00848393 Phase 2 Fentanyl (High Dose);Fentanyl (Low Dose);Fentanyl (Low Dose) + Dexmedetomidine
4 Hypertonic Saline Dextran in Pediatric Cardiac Surgery Completed NCT00199771 Phase 2 7.5% NaCl in 6% dextran 70 solution
5 Mesoblast Stem Cell Therapy for Patients With Single Ventricle and Borderline Left Ventricle Recruiting NCT03079401 Phase 1, Phase 2
6 Aberrations in Carnitine Homeostasis in Congenital Heart Disease With Increased Pulmonary Blood Flow Active, not recruiting NCT01825369 Phase 1 IV L-carnitine
7 National Congenital Heart Disease Audit Unknown status NCT02303535
8 Molecular Genetic Epidemiology of Endocardial Cushion Defects - SCOR in Pediatric Cardiovascular Disease Completed NCT00005322
9 Molecular Genetic Epidemiology of Three Cardiac Defects -SCOR in Pediatric Cardiovascular Disease Completed NCT00005546
10 Evolution of Two Cohorts of Children (Univentricular and Bi-ventricular Heart) After Strapping of the Pulmonary Artery Completed NCT03047343
11 Acetaminophen for Oxidative Stress After Cardiopulmonary Bypass Completed NCT01228305 Not Applicable
12 Infant Weight Gain With Trisomy 21 and CAVC Terminated NCT00327951
13 Optimal Timing for Repair of Left to Right Shunt Lesions Terminated NCT00229827

Search NIH Clinical Center for Complete Atrioventricular Canal

Genetic Tests for Complete Atrioventricular Canal

Anatomical Context for Complete Atrioventricular Canal

MalaCards organs/tissues related to Complete Atrioventricular Canal:

42
Heart, Small Intestine

Publications for Complete Atrioventricular Canal

Articles related to Complete Atrioventricular Canal:

(show top 50) (show all 217)
# Title Authors Year
1
Echocardiographic Assessment of Complete Atrioventricular Canal Defects: A Balancing Act. ( 30447289 )
2019
2
Rare Occurrence of Absent Pulmonary Valve Syndrome in the Setting of Double-Outlet Right Ventricle with Unbalanced Complete Atrioventricular Canal Defect. ( 31049480 )
2019
3
Preoperative Clinical and Echocardiographic Factors Associated with Surgical Timing and Outcomes in Primary Repair of Common Atrioventricular Canal Defect. ( 31065759 )
2019
4
Complete Atrioventricular Canal Defect: Towards a More Physiological Repair. ( 28705664 )
2018
5
Retrospective study of complete atrioventricular canal defects: Anesthetic and perioperative challenges. ( 29336386 )
2018
6
Complete atrioventricular canal repair with a decellularized porcine small intestinal submucosa patch. ( 30242970 )
2018
7
Bloodless Heart Surgery for an 11-kg Infant of the Jehovah's Witness Faith Undergoing Second Repair for Complete Atrioventricular Canal. ( 30250346 )
2018
8
Cardiac Function After Tetralogy of Fallot/Complete Atrioventricular Canal Repair. ( 28329461 )
2017
9
Biventricular repair for common atrioventricular canal defect with parachute left atrioventricular valve. ( 25838456 )
2016
10
Early Complete Atrioventricular Canal Repair Yields Outcomes Equivalent to Late Repair. ( 25886812 )
2015
11
Atypical Williams syndrome in an infant with complete atrioventricular canal defect. ( 26271350 )
2015
12
Palliation of Truncus Arteriosus Associated With Complete Atrioventricular Canal--Results of Single Ventricle Palliation. ( 26467884 )
2015
13
A Novel Technique for Repair of Complete Atrioventricular Canal Defect: The Central Patch Technique. ( 24958047 )
2014
14
A Rare Case of Pulmonary Artery Sling and Complete Atrioventricular Canal Defect in an Infant With Trisomy 21. ( 24958055 )
2014
15
Noonan syndrome with complete atrioventricular canal defect with pulmonary stenosis. ( 24055746 )
2013
16
Fetal complete common atrioventricular canal defect: spontaneous closure of the ventricular septal defect--in utero anatomic evolution and postnatal outcomes. ( 23799731 )
2013
17
Changes in left atrioventricular valve geometry after surgical repair of complete atrioventricular canal. ( 22078711 )
2012
18
Descriptive epidemiology of non-syndromic complete atrioventricular canal defects. ( 23061687 )
2012
19
Anesthesia for an infant with Beckwith-Wiedman syndrome who underwent open heart surgery for complete atrioventricular canal defect. ( 25631696 )
2012
20
Improving left ventricular outflow tract obstruction repair in common atrioventricular canal defects. ( 22626757 )
2012
21
Outcome after two-patch repair of complete common atrioventricular canal defects in patients weighing four kilograms or less. ( 23804859 )
2012
22
Complete atrioventricular canal with guarded primum septal defect. ( 21188369 )
2011
23
Prolonged QT in a 13-year-old patient with Down syndrome and complete atrioventricular canal defect. ( 22008496 )
2011
24
No-patch technique for complete atrioventricular canal repair. ( 20609816 )
2010
25
Complete atrioventricular canal in a dog. ( 20615778 )
2010
26
Individualized approach to repair of complete atrioventricular canal: selective use of the traditional single-patch technique versus the Australian technique. ( 23804726 )
2010
27
Management of complete atrioventricular canal defect with aortic arch obstruction: an unresolved debate. ( 23804821 )
2010
28
Prenatal diagnosis of complete atrioventricular canal associated with tetralogy of Fallot. ( 17663460 )
2008
29
Traditional single patch versus the "Australian" technique for repair of complete atrioventricular canal defects. ( 18958557 )
2008
30
Complete atrioventricular canal repair under 1 year: Rastelli one-patch procedure yields excellent long-term results. ( 19049757 )
2008
31
Echocardiographic evaluation and surgical implications of common atrioventricular canal defects with absent or diminutive ostium primum defect. ( 18489945 )
2008
32
Primary repair of d-transposition with complete atrioventricular canal defect. ( 17244905 )
2007
33
Overview: history, anatomy, timing, and results of complete atrioventricular canal. ( 17433985 )
2007
34
What is the best technique for repair of complete atrioventricular canal? ( 17983953 )
2007
35
Complete atrioventricular canal: comparison of modified single-patch technique with two-patch technique. ( 18036931 )
2007
36
Common atrioventricular canal in a newborn foal--case report and review of the literature. ( 17385556 )
2007
37
Complete atrioventricular canal. ( 16722604 )
2006
38
Analysis of surgical outcome in complex double-outlet right ventricle with heterotaxy syndrome or complete atrioventricular canal defect. ( 16798205 )
2006
39
Biventricular repair of right-dominant complete atrioventricular canal defect. ( 17072673 )
2006
40
No patch technique for complete atrioventricular canal repair. ( 17670589 )
2006
41
No patch technique for complete atrioventricular canal repair. ( 17670590 )
2006
42
Biventricular repair of complete atrioventricular canal, double-outlet right ventricle and common atrium using a modified double switch technique. A valid alternative to univentricular procedure. ( 17670392 )
2005
43
Complete atrioventricular canal defect in a foal: Clinical and pathological features. ( 19083319 )
2005
44
Complete atrioventricular canal and tetralogy of Fallot with pulmonary atresia. ( 15464457 )
2004
45
[Infections as a preoperative problem in patients with tetralogy of Fallot (TOF) associated with complete atrioventricular canal septal defect (AVC)]. ( 15724657 )
2004
46
One-stage repair of complete atrioventricular canal, double-outlet right ventricle, left superior vena cava, cor triatriatum and pulmonary stenosis. ( 12537251 )
2003
47
Complete endocardial cushion defect (complete atrioventricular canal) manifested in adult life by Streptococcus mitis endocarditis of the common atrioventricular valve. ( 12727016 )
2003
48
Single-stage anatomical repair of complete atrioventricular canal, double-outlet right ventricle, and cor triatriatum using ventricular septal defect translocation. ( 11996287 )
2002
49
Play safe: band the late presenting complete atrioventricular canal. ( 12079967 )
2002
50
Macroreentrant atrial flutter around a common atrioventricular canal in an infant with complicated congenital heart anomaly. ( 12418755 )
2002

Variations for Complete Atrioventricular Canal

Expression for Complete Atrioventricular Canal

Search GEO for disease gene expression data for Complete Atrioventricular Canal.

Pathways for Complete Atrioventricular Canal

GO Terms for Complete Atrioventricular Canal

Sources for Complete Atrioventricular Canal

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
20 FMA
29 GO
30 GTR
31 HGMD
32 HMDB
33 HPO
34 ICD10
35 ICD10 via Orphanet
36 ICD9CM
37 IUPHAR
38 KEGG
39 LifeMap
41 LOVD
43 MedGen
45 MeSH
46 MESH via Orphanet
47 MGI
50 NCI
51 NCIt
52 NDF-RT
55 NINDS
56 Novoseek
58 OMIM
59 OMIM via Orphanet
63 PubMed
65 QIAGEN
70 SNOMED-CT via HPO
71 SNOMED-CT via Orphanet
72 TGDB
73 Tocris
74 UMLS
75 UMLS via Orphanet
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