MCID: UNV002
MIFTS: 26

Univentricular Heart

Categories: Cardiovascular diseases, Fetal diseases, Rare diseases

Aliases & Classifications for Univentricular Heart

MalaCards integrated aliases for Univentricular Heart:

Name: Univentricular Heart 58 36
Double Inlet Left Ventricle 58 6

Classifications:

Orphanet: 58  
Rare cardiac malformations
Developmental anomalies during embryogenesis


External Ids:

KEGG 36 H01787
ICD10 via Orphanet 33 Q20.4
Orphanet 58 ORPHA1464

Summaries for Univentricular Heart

KEGG : 36 Univentricular heart is a term used to describe a wide variety of structural cardiac abnormalities associated with a functional single ventricular chamber. Truly solitary ventricles are exceedingly rare. It is generally agreed that the atrioventricular connection is central in defining the univentricular heart. More specifically, the well-developed ventricle may be designated left, right, or indeterminate. Clinical manifestations hinge on the presence or absence of pulmonary outflow obstruction. Without pulmonary stenosis, infants with low pulmonary resistance present with signs and symptoms typical of large left-to-right shunting, ie, congestive heart failure and failure-to-thrive. A certain degree of pulmonary stenosis is physiologically desirable to prevent pulmonary overcirculation. However, severe pulmonary stenosis or atresia may result in profound hypoxemia and cyanosis.

MalaCards based summary : Univentricular Heart, also known as double inlet left ventricle, is related to univentricular heart with single atrio-ventricular valve and single ventricular heart. An important gene associated with Univentricular Heart is MIR129-2 (MicroRNA 129-2). The drugs Sodium citrate and Citric acid have been mentioned in the context of this disorder. Affiliated tissues include heart, atrioventricular node and trachea.

Related Diseases for Univentricular Heart

Diseases related to Univentricular Heart via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 87)
# Related Disease Score Top Affiliating Genes
1 univentricular heart with single atrio-ventricular valve 11.2
2 single ventricular heart 10.7
3 heart septal defect 10.5
4 tricuspid atresia 10.5
5 cyanosis, transient neonatal 10.5
6 hypoplastic left heart syndrome 10.5
7 dextro-looped transposition of the great arteries 10.4
8 heart disease 10.4
9 subvalvular aortic stenosis 10.4
10 ventricular septal defect 10.4
11 heterotaxy 10.4
12 mitral atresia 10.3
13 atrioventricular block 10.3
14 double outlet right ventricle 10.3
15 atrioventricular septal defect 10.2
16 dextrocardia 10.2
17 infective endocarditis 10.2
18 scoliosis 10.2
19 atrial heart septal defect 10.2
20 levocardia 10.2
21 progressive familial heart block, type ia 10.1
22 coarctation of aorta 10.1
23 varicose veins 10.1
24 aortic aneurysm, familial thoracic 1 10.1
25 aortic dissection 10.1
26 left bundle branch hemiblock 10.1
27 long qt syndrome 10.1
28 aortic aneurysm 10.1
29 aortic arch interruption 10.1
30 congenitally corrected transposition of the great arteries 10.1
31 double outlet right atrium 10.1
32 pulmonary venous return anomaly 10.1
33 juxtaposition of the atrial appendages 10.1
34 right atrial isomerism 10.1
35 second-degree atrioventricular block 10.1
36 sinoatrial node disease 10.1
37 cardiac arrest 10.1
38 endocarditis 10.1
39 protein-losing enteropathy 10.1
40 arteriovenous malformation 10.1
41 sick sinus syndrome 10.1
42 pulmonary valve stenosis 10.1
43 polycythemia 10.1
44 47,xyy 10.1
45 double discordia 10.1
46 cardiac arrhythmia 9.9
47 fibrosis of extraocular muscles, congenital, 1 9.9
48 tetralogy of fallot 9.9
49 wolff-parkinson-white syndrome 9.9
50 charge syndrome 9.9

Graphical network of the top 20 diseases related to Univentricular Heart:



Diseases related to Univentricular Heart

Symptoms & Phenotypes for Univentricular Heart

Drugs & Therapeutics for Univentricular Heart

Drugs for Univentricular Heart (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 18)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Sodium citrate Approved, Investigational Phase 3 68-04-2
2
Citric acid Approved, Nutraceutical, Vet_approved Phase 3 77-92-9 311
3 Vasodilator Agents Phase 3
4 Phosphodiesterase Inhibitors Phase 3
5 Phosphodiesterase 5 Inhibitors Phase 3
6 Citrate Phase 3
7 Sildenafil Citrate Phase 3 171599-83-0
8 Angiotensin-Converting Enzyme Inhibitors Phase 2
9 HIV Protease Inhibitors Phase 2
10
protease inhibitors Phase 2
11
Nitric Oxide Approved 10102-43-9 145068
12 Respiratory System Agents
13 Anti-Asthmatic Agents
14 Antioxidants
15 Protective Agents
16 Neurotransmitter Agents
17 Endothelium-Dependent Relaxing Factors
18 Bronchodilator Agents

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Phosphodiesterase-type 5 Inhibitors in Adult and Adolescent Patients With Univentricular Heart Disease: a Multi-center, Randomized, Double Blind Phase III Study Not yet recruiting NCT03997097 Phase 3 Sildenafil;Placebos
2 Pathophysiological Mechanisms of Hepatopulmonary Influence in the Fontan Circulation Completed NCT00263406 Phase 2 Administration of an ACE-inhibitor or not
3 Phase 2 Study of Intracoronary Infusion of Cardiac Progenitor Cells in Patients With Univentricular Heart Disease Completed NCT01829750 Phase 2
4 Effect of Aesculus Hippocastanum L. (Sapindaceae, "Horse Chestnut") on Fontan Circulation - a Randomized Clinical Trial Withdrawn NCT04035317 Phase 2 Aesculus Hippocastanum / Horse Chestnut;Placebo
5 Survival of Double Inlet Left Ventricle Patients Without Fontan Circulation: Intracardiac Anatomy and Hemodynamics, Functional Status and Quality of Life Unknown status NCT03667144
6 Lymphatic Function and Interstitial Fluid Filtration in Patients With a Fontan Circulation From Brazil Completed NCT04394507
7 Factors Associated With Normal Exercise Capacity in Patients With Single Ventricle: the Multicenter Cross-sectional Study Completed NCT04026542
8 Can Ventilatory Response at Rest Predict Ventilatory Efficacy and Exercise Tolerance in Patients With a Univentricular Congenital Heart Disease? Recruiting NCT03818373
9 Pulmonary Capillary Recruitment With Pulmonary Vasodilator Therapy in Patients With Univentricular Heart Physiology and Failing Total Cavo-pulmonary Connection. Recruiting NCT04292483 Nitric Oxide

Search NIH Clinical Center for Univentricular Heart

Genetic Tests for Univentricular Heart

Anatomical Context for Univentricular Heart

MalaCards organs/tissues related to Univentricular Heart:

40
Heart, Atrioventricular Node, Trachea, Liver

Publications for Univentricular Heart

Articles related to Univentricular Heart:

(show top 50) (show all 930)
# Title Authors PMID Year
1
Perioperative management of Fontan operation for the child with panhypopituitarism: a case report. 61
33449198 2021
2
Upper mini sternotomy approach for pulmonary artery banding: A single centre experience. 61
33797797 2021
3
Monthly variation in univentricular heart and transposition of the great arteries - 10-year national population-based cohort study. 61
33567397 2021
4
Risk analysis for patients with a functionally univentricular heart after systemic-to-pulmonary shunt placement. 61
33712829 2021
5
Myocardial deformation in patients with a single left ventricle using 2D cardiovascular magnetic resonance feature tracking: a case-control study. 61
33788063 2021
6
[Therapy and Outcome of Neonates with Congenital Diaphragmatic Hernia and Congenital Heart Defects]. 61
33694149 2021
7
Risk Factors for Thrombus Formation at Stage 2 Palliation and Its Effect on Long-Term Outcome in Patients With Univentricular Heart. 61
33691189 2021
8
Risk factors for morbidity and mortality after a bidirectional Glenn shunt in Northern Thailand. 61
32783183 2021
9
Factors associated with unplanned reinterventions and their relation to early mortality after pediatric cardiac surgery. 61
33419533 2021
10
[THE FINDINGS AND CHAIN OF EVENTS THAT LED PREGNANT WOMEN TO TERMINATION OF PREGNANCY DUE TO FETAL HEART DEFECTS: 17 YEARS EXPERIENCE FROM A SINGLE MEDICAL CENTER]. 61
33760408 2021
11
Outcomes after the Fontan operation in the Middle East: A large Saudi Arabian single centre experience. 61
33148464 2021
12
The mid-term outcome of Fontan conversion compared with primary total cavopulmonary connection. 61
33648806 2021
13
Infected thrombus in a Fontan circulation. 61
33274458 2021
14
Mid-term outcomes of cor triatriatum repair: comparison of biventricular physiology and univentricular physiology. 61
33168116 2021
15
Annuloplasty for aortic regurgitation in univentricular heart on ventricular assist support. 61
33652004 2021
16
Utility of late pulmonary artery banding in single-ventricle physiology: A mid-term follow-up. 61
33679058 2021
17
Heart transplantation at a single tertiary adult congenital heart disease centre: Too little, too late? 61
32798622 2021
18
Glenn surgery: a safe procedure in the path of univentricular correction. 61
33476319 2021
19
Uncommon Association of Aortopulmonary Window in a Patient with Complex Univentricular Heart and CHARGE Syndrome. 61
33654544 2021
20
Pregnancy and early post-natal outcomes of fetuses with functionally univentricular heart in a low-and-middle-income country. 61
32959750 2020
21
Covid-19 in an infant with systemic to pulmonary artery shunt dependent functionally univentricular physiology. 61
33327982 2020
22
Education of children with cyanotic congenital heart disease after neonatal cardiac surgery. 61
33075323 2020
23
Computed Tomography in the Evaluation of Fontan Circulation. 61
33605094 2020
24
Levosimendan as a bridge to heart transplant in a 16-year-old patient with univentricular heart. 61
32698938 2020
25
Assessment of functional capacity among students with univentricular heart. 61
32924398 2020
26
[Management of Functionally Univentricular Heart with Heterotaxy]. 61
33130769 2020
27
Highly atypical double barrel outlet to aorta in double inlet left ventricle, double outlet right ventricle. 61
30902580 2020
28
A case of COVID-19 in a patient with a univentricular heart post total cavopulmonary connection (Fontan) surgery. 61
32613935 2020
29
Extracorporeal cardiopulmonary resuscitation with low pump flow for blocked modified Blalock-Taussig shunt followed by spontaneous recanalization. 61
32762308 2020
30
The Adult Patient with a Fontan. 61
32622492 2020
31
Intra-atrial block requiring dual-site atrial pacing through a femoral approach in a univentricular heart. 61
32695584 2020
32
An Unusual Combination of Double Inlet Left Ventricle With Discordant Ventriculoarterial Connections and Bilateral Arterial Ducts. 61
29334828 2020
33
Fifteen-minute consultation: Assessing the child with a Blalock-Taussig shunt who is unwell in a district general hospital. 61
31278077 2020
34
Reduction of Inflammation by High-Dose Methylprednisolone Does not Attenuate Oxidative Stress in Children Undergoing Bidirectional Glenn Procedure With or Without Aortic Arch or Pulmonary Arterial Repair. 61
32037273 2020
35
Frontal lobe growth is impaired in fetuses with congenital heart disease. 61
32573836 2020
36
A transition clinic intervention to improve follow-up rates in adolescents and young adults with congenital heart disease. 61
32279695 2020
37
Systemic ventricular implantation of a leadless pacemaker in a patient with a univentricular heart and atrioventricular node calcification. 61
32461891 2020
38
Impact of the superior cavopulmonary anastomosis on cerebral oxygenation. 61
32172701 2020
39
Significant Improvements in Mortality After the Fontan Operation in Children With Down Syndrome. 61
31525348 2020
40
Elevated ventricular filling pressures and long-term survival in adults post-Fontan. 61
31145542 2020
41
Catheter ablation of atrial fibrillation in a functionally univentricular heart: a risk-adjusted interventional approach. 61
32020272 2020
42
Efficacy of bubble contrast echocardiography in detecting pulmonary arteriovenous fistulas in children with univentricular heart after total cavopulmonary connection. 61
31916529 2020
43
Catheter ablation for patients with end-stage complex congenital heart disease or cardiomyopathy considered for transplantation: Trials and tribulations. 61
31604655 2020
44
Uncorrected Univentricular Heart in an Adult. 61
32005791 2020
45
Energetics of blood flow in Fontan circulation under VAD support. 61
31489642 2020
46
MicroRNA-29b/c-3p Indicate Advanced Liver Fibrosis/Cirrhosis in Univentricular Heart Patients With and Without Fontan Palliation. 61
33490119 2020
47
Failure of Cellularization of Ventriculotomy Patch Leading to Right Ventricular Pseudoaneurysm. 61
31755355 2020
48
Cyanotic Congenital Cardiac Disease and Scoliosis: Pre-Operative Assessment, Surgical Treatment, and Outcomes. 61
31266026 2020
49
New Horizon of Intervention in Congenital Heart Disease: AFR in a Complex Cyanotic Patient. 61
33425395 2020
50
A 4D flow MRI evaluation of the impact of shear-dependent fluid viscosity on in vitro Fontan circulation flow. 61
31585044 2019

Variations for Univentricular Heart

Expression for Univentricular Heart

Search GEO for disease gene expression data for Univentricular Heart.

Pathways for Univentricular Heart

GO Terms for Univentricular Heart

Sources for Univentricular Heart

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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