MCID: UNV002
MIFTS: 29

Univentricular Heart

Categories: Cardiovascular diseases, Fetal diseases, Rare diseases

Aliases & Classifications for Univentricular Heart

Summaries for Univentricular Heart

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

Related Diseases for Univentricular Heart

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

(show all 23)
# Related Disease Score Top Affiliating Genes
1 univentricular heart with single atrio-ventricular valve 12.1
2 heart disease 10.2
3 aging 10.2
4 tricuspid atresia 10.1
5 ventricular septal defect 10.0
6 cerebritis 10.0
7 thrombosis 9.8
8 endocarditis 9.8
9 protein-losing enteropathy 9.8
10 sick sinus syndrome 9.8
11 tetralogy of fallot 9.8
12 thrombocytosis 9.8
13 wolff-parkinson-white syndrome 9.8
14 right atrial isomerism 9.8
15 heterotaxy 9.8
16 eisenmenger syndrome 9.8
17 transposition of the great arteries 9.8
18 pulmonary hypertension 9.8
19 interatrial communication 9.8
20 cerebral hemorrhage 9.8
21 visceral heterotaxy 9.8
22 atrioventricular block 9.8
23 infective endocarditis 9.8

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 top 50) (show all 91)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Dobutamine Approved Phase 4 34368-04-2 36811
2
Spironolactone Approved Phase 4,Not Applicable 1952-01-7, 52-01-7 5833
3
Carvedilol Approved, Investigational Phase 4 72956-09-3 2585
4
Eplerenone Approved Phase 4 107724-20-9 150310 443872
5
Citric Acid Approved, Nutraceutical, Vet_approved Phase 4,Phase 2,Phase 1 77-92-9 311
6 Sildenafil Citrate Phase 4,Phase 2,Phase 1 171599-83-0
7 Adrenergic Agents Phase 4
8 Adrenergic Agonists Phase 4
9 Adrenergic beta-Agonists Phase 4
10 Neurotransmitter Agents Phase 4
11 Vasodilator Agents Phase 4,Phase 2,Phase 1
12 Peripheral Nervous System Agents Phase 4,Phase 3
13 Phosphodiesterase 5 Inhibitors Phase 4,Phase 3,Phase 1,Phase 2
14 Phosphodiesterase Inhibitors Phase 4,Phase 3,Phase 1,Phase 2
15 Protective Agents Phase 4,Phase 2,Not Applicable
16 Autonomic Agents Phase 4
17 diuretics Phase 4,Not Applicable
18 Mineralocorticoid Receptor Antagonists Phase 4,Not Applicable
19 Mineralocorticoids Phase 4,Not Applicable
20 Natriuretic Agents Phase 4,Phase 2,Phase 3,Not Applicable
21 Hormone Antagonists Phase 4,Not Applicable
22 Hormones Phase 4,Not Applicable
23 Diuretics, Potassium Sparing Phase 4,Not Applicable
24 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 4,Not Applicable
25 Antihypertensive Agents Phase 4,Phase 3,Phase 2,Not Applicable
26 Adrenergic alpha-1 Receptor Antagonists Phase 4
27 Adrenergic alpha-Antagonists Phase 4
28 Adrenergic Antagonists Phase 4
29 Adrenergic beta-Antagonists Phase 4
30 Citrate Nutraceutical Phase 4,Phase 2,Phase 1
31
Enalaprilat Approved Phase 3 76420-72-9 6917719
32
Enalapril Approved, Vet_approved Phase 3 75847-73-3 5362032 40466924
33
Udenafil Approved, Investigational Phase 3,Phase 1,Phase 2 268203-93-6 6918523
34
Aspirin Approved, Vet_approved Phase 3 50-78-2 2244
35
Rivaroxaban Approved Phase 3 366789-02-8
36
Serine Approved, Nutraceutical Phase 3 56-45-1 5951
37
protease inhibitors Phase 3,Phase 2
38 HIV Protease Inhibitors Phase 3,Phase 2
39 Angiotensin-Converting Enzyme Inhibitors Phase 3,Phase 2
40 Anticoagulants Phase 3,Phase 1
41 Analgesics Phase 3
42 Fibrinolytic Agents Phase 3
43 Serine Proteinase Inhibitors Phase 3
44 Coagulants Phase 2, Phase 3,Phase 3
45 Cyclooxygenase Inhibitors Phase 3
46 Hemostatics Phase 2, Phase 3,Phase 3
47 Analgesics, Non-Narcotic Phase 3
48 Vasoconstrictor Agents Phase 2, Phase 3,Phase 3
49 Vasopressins Phase 2, Phase 3,Phase 3
50 Platelet Aggregation Inhibitors Phase 3,Phase 2

Interventional clinical trials:

(show top 50) (show all 102)
# Name Status NCT ID Phase Drugs
1 Study of Effects of Sildenafil on Patients With Fontan Heart Circulation Unknown status NCT01815502 Phase 4 Dobutamine
2 The Effects of Ambrisentan on Exercise Capacity in Fontan Patients Completed NCT01971580 Phase 4 Ambrisentan
3 Effect of Carvedilol on Exercise Performance in Fontan Patients Recruiting NCT02946892 Phase 4 Carvedilol;Placebo
4 The Effects of Eplerenone on Markers of Myocardial Fibrosis in Adult Congenital Heart Disease Terminated NCT01971593 Phase 4 Eplerenone
5 Hybrid Versus Norwood Management Strategies in Infants Undergoing Single Ventricle Palliation Unknown status NCT01134302 Phase 3
6 Trial of Angiotensin Converting Enzyme Inhibition in Infants With Single Ventricle--Pediatric Heart Network Completed NCT00113087 Phase 3 Enalapril;Placebo
7 Comparison of Two Types of Shunts in Infants With Single Ventricle Defect Undergoing Staged Reconstruction--Pediatric Heart Network Completed NCT00115934 Phase 3
8 Biventricular Pacing In Patients With Left Ventricular Dysfunction After Cardiovascular Surgery Completed NCT00321295 Phase 3
9 A Extension Study of Udenafil in Adolescents Recruiting NCT03013751 Phase 3 Udenafil
10 Cardiac Stem/Progenitor Cell Infusion in Univentricular Physiology (APOLLON Trial) Recruiting NCT02781922 Phase 3
11 Fontan Udenafil Exercise Longitudinal Assessment Trial Recruiting NCT02741115 Phase 3 Udenafil;Placebo
12 Early Use of Vasopressin in Post-Fontan Management Recruiting NCT03088345 Phase 2, Phase 3 Vasopressin, Arginine;Placebos
13 Pharmacokinetic, Pharmacodynamic, Safety, and Efficacy Study of Rivaroxaban for Thromboprophylaxis in Pediatric Participants 2 to 8 Years of Age After the Fontan Procedure Recruiting NCT02846532 Phase 3 Rivaroxaban;Acetylsalicylic Acid
14 Colchicine in Postoperative Fontan Patients Not yet recruiting NCT03575572 Phase 2, Phase 3 Colchicine
15 Use of Vasopressin Following the Fontan Operation Suspended NCT02975999 Phase 2, Phase 3 Vasopressin;Normal Saline
16 Cardiac Progenitor Cell Infusion to Treat Univentricular Heart Disease (PERSEUS) Completed NCT01829750 Phase 2
17 Bosentan Therapy in Children With Functional Single Ventricle Completed NCT01662037 Phase 2 Bosentan
18 Study of Placebo or Bosentan to Treat Patients With Single Ventricle Physiology. Completed NCT01292551 Phase 2 Bosentan;Placebo
19 Pharmacokinetic/Pharmacodynamic Study of Udenafil in Adolescents Completed NCT02201342 Phase 1, Phase 2 Udenafil
20 Pathophysiological Mechanisms of Hepatopulmonary Influence in the Fontan Circulation Completed NCT00263406 Phase 2 Administration of an ACE-inhibitor or not
21 Sildenafil After the Fontan Operation Completed NCT00507819 Phase 2 Sildenafil;Placebo
22 Transfusion Strategies in Pediatric Cardiothoracic Surgery Completed NCT00350220 Phase 2
23 Mesoblast Stem Cell Therapy for Patients With Single Ventricle and Borderline Left Ventricle Recruiting NCT03079401 Phase 1, Phase 2
24 Ambrisentan in Single Ventricle Active, not recruiting NCT02080637 Phase 2 Ambrisentan
25 Effects of Perioperative Nesiritide or Milrinone Infusion on Recovery From Fontan Surgery Terminated NCT00543309 Phase 2 nesiritide;milrinone;placebo
26 Transcoronary Infusion of Cardiac Progenitor Cells in Patients With Single Ventricle Physiology Completed NCT01273857 Phase 1
27 Sildenafil in Single Ventricle Patients Completed NCT01169519 Phase 1 Sildenafil by injection;Sildenafil by injection;Sildenafil by injection;Sildenafil by injection
28 Effect of Tadalafil on Exercise Capacity in Pediatric Fontan Patients Completed NCT01291069 Phase 1 Tadalafil Citrate;Sugar pill
29 A Pilot Study Investigating the Clinical Use of Tissue Engineered Vascular Grafts in Congenital Heart Surgery Completed NCT01034007 Phase 1
30 Liothyronine in Children With Single Ventricle Congenital Cardiac Malformations Undergoing the Fontan Procedure Completed NCT00004828 Phase 1 liothyronine I 131
31 Feasibility and Efficacy of Portable Non-invasive Negative Pressure Ventilation in Fontan Patients Completed NCT03251742 Phase 1
32 Pilot/Ph I Safety and Efficacy of ODSH in Protein Losing Enteropathy Secondary to Single Ventricle Palliative Surgery Terminated NCT01161641 Phase 1 ODSH at 0.125 mg/kg/h;ODSH at 0.375 mg/kg/h;ODSH at 0.250 mg/kg/h
33 Global and Regional Myocardial Strain and Power Output In Patients With Single Ventricles Using Novel MRI Techniques Unknown status NCT01107990
34 Cardiovascular, Pulmonary and Skeletal Muscle Evaluation in Late Postoperative Period of the Fontan Surgery Unknown status NCT02283255 Not Applicable
35 Pulmonary Functions Test in Patients After Fontan Operation Unknown status NCT01413308
36 A Prospective Study of Patients With Hypoplastic Left Heart Syndrome (HLHS) Following Stage II Surgical Palliation Unknown status NCT01708863
37 Cardio Pulmonary Exercise Testing in Patients Post the Fontan Palliation in Comparison With Patients With Repaired Congenital Heart Disease and Healthy Volunteers. Unknown status NCT02659462
38 Prospective Analysis of Cerebral Perfusion Using Head Ultrasound and Multi-source-detector Near Infrared Spectroscopy (NIRS) Imaging Unknown status NCT01082536
39 T1 Mapping of Diffuse Myocardial Fibrosis in Congenital Heart Disease Unknown status NCT02350829 Not Applicable
40 Cerebral Blood Flow in Single Ventricles Throughout Staged Surgical Reconstruction Completed NCT02135081
41 Nutritional Failure in Infants With Single Ventricle Congenital Heart Disease Completed NCT01821287
42 Single Ventricle Outcome Completed NCT00308217
43 Impact of Early Intervention on Maternal Stress in Mothers of Fetuses Diagnosed With Single Ventricle Physiology Completed NCT02462434 Not Applicable
44 Endothelin Blockade in Patients With Single Ventricle Physiology Completed NCT00989911 Not Applicable Bosentan
45 Multisite Feeding Study: Home Surveillance and Feeding Strategies in Infants With Complex Single Ventricle Completed NCT00257517
46 Outcomes of Children With Congenital Single Ventricle Heart Completed NCT00266968
47 Influence of Genetic Polymorphisms on Ventricular Structure and Function in Patients With Single Ventricle Anatomy Completed NCT00165984
48 Training in Exercise Activities and Motion for Growth Completed NCT01917084 Not Applicable
49 Biomarker Study for Heart Failure in Children With Single Ventricle Physiology Completed NCT00571233
50 Spironolactone in Patients With Single Ventricle Heart Completed NCT00211081 Not Applicable Spironolactone (drug)

Search NIH Clinical Center for Univentricular Heart

Genetic Tests for Univentricular Heart

Anatomical Context for Univentricular Heart

MalaCards organs/tissues related to Univentricular Heart:

41
Heart, Testes, Brain, Liver, Endothelial, Skeletal Muscle, Atrioventricular Node

Publications for Univentricular Heart

Articles related to Univentricular Heart:

(show top 50) (show all 214)
# Title Authors Year
1
Long-Term Effects of Percutaneous Fenestration Following the Fontan Procedure in Adult Patients with Congenital Univentricular Heart. ( 29802801 )
2018
2
Blood-threshold CMR volume analysis of functional univentricular heart. ( 29357037 )
2018
3
Diagnostic accuracy of low-dose dual-source cardiac computed tomography as compared to surgery in univentricular heart patients. ( 29769130 )
2018
4
Risk factors for in-hospital shunt thrombosis and mortality in patients weighing less than 3a88kg with functionally univentricular heart undergoing a modified Blalock-Taussig shunt. ( 28520941 )
2017
5
Plasma microvesicle analysis identifies microRNA 129-5p as a biomarker of heart failure in univentricular heart disease. ( 28859128 )
2017
6
Intracoronary Cardiac Progenitor Cells in Single Ventricle Physiology: The PERSEUS (Cardiac Progenitor Cell Infusion to Treat Univentricular Heart Disease) Randomized Phase 2 Trial. ( 28052915 )
2017
7
Stenting of narrow pulmonary artery banding in a patient with univentricular heart. ( 29057003 )
2017
8
Anomalous origin of the left coronary artery from the right pulmonary artery in a univentricular heart. ( 28651676 )
2017
9
Improved long-term outcomes in double-inlet left ventricle and tricuspid atresia with transposed great arteries: systemic outflow tract obstruction present at birth defines long-term outcome. ( 28329058 )
2017
10
First-stage palliation strategy for univentricular heart disease may impact risk for acute kidney injury. ( 28889816 )
2017
11
Living with functionally univentricular heart: beating the odds? ( 29214833 )
2017
12
Impact of Abernathy malformation on pulmonary circulatory hemodynamics in a univentricular heart. ( 28163439 )
2017
13
A Comparative Study of Histopathological Changes in the Ascending Aorta and the Risk Factors Related to Histopathological Conditions and Aortic Dilatation in Patients with Tetralogy of Fallot and a Functionally Univentricular Heart. ( 29111162 )
2017
14
Intra-Atrial Y-Graft Fontan for Univentricular Heart With Discontinuous Pulmonary Arteries. ( 28696884 )
2017
15
Stenting of the ductus arteriosus in infants with functionally univentricular heart disease and ductal-dependent pulmonary blood flow: A single-center experience. ( 27862912 )
2016
16
Surgical approach for systemic-pulmonary shunt in neonates with functionally univentricular heart: comparison between sternotomy and thoracotomy. ( 27300351 )
2016
17
Commentary on "Changes in Motor Development During a 4-Year Follow-up on Children With Univentricular Heart Defects". ( 27661240 )
2016
18
Prenatal diagnosis of functionally univentricular heart, associations and perinatal outcomes. ( 27061183 )
2016
19
Comparative Evaluation of Flow Quantification across the Atrioventricular Valve in Patients with Functional Univentricular Heart after Fontan's Surgery and Healthy Controls: Measurement by 4D Flow Magnetic Resonance Imaging and Streamline Visualization. ( 27425568 )
2016
20
Changes in Motor Development During a 4-Year Follow-up on Children With Univentricular Heart Defects. ( 27661239 )
2016
21
Functionally Univentricular Heart With Right Atrial Isomerism and Mixed Total Anomalous Pulmonary Venous Drainage. ( 26701620 )
2016
22
Elevated Low-Shear Blood Viscosity is Associated with Decreased Pulmonary Blood Flow in Children with Univentricular Heart Defects. ( 26888364 )
2016
23
Can Univentricular Heart Be Associated with "Rigid Body Rotation"? A Case from the ThreeDimensional Speckle-Tracking Echocardiographic MAGYAR-Path Study. ( 25854450 )
2015
24
Thrombocytosis in the Setting of Isomerism and a Functionally Univentricular Heart. ( 26719826 )
2015
25
Decision-Making for Surgery in the Management of Patients with Univentricular Heart. ( 26284226 )
2015
26
Mechanics of the Functionally Univentricular Heart-How Little Do We Understand and Why Does It Matter? ( 26919793 )
2015
27
Single-stage septation of a univentricular heart: 26 years later. ( 25623904 )
2015
28
Incidental finding of an univentricular heart on echocardiogram in an adult patient. ( 25468956 )
2015
29
Editorial: Univentricular Heart. ( 26442235 )
2015
30
A 74-year-old unoperated univentricular heart: the oldest reported survival. ( 26508431 )
2015
31
Truncus arteriosus, tricuspid atresia and partial anomalous pulmonary venous drainage: a unique form of univentricular heart. ( 25972595 )
2015
32
Evidence for improved outcome for children born with a univentricular heart defect? ( 25904767 )
2015
33
Heart rate variability in patients undergoing univentricular heart repair. ( 24771727 )
2014
34
The new concept of univentricular heart. ( 25072035 )
2014
35
Anaesthesia in a patient with univentricular heart for emergency craniotomy: A case report. ( 25197131 )
2014
36
Radiofrequency catheter ablation of atrial tachyarrhythmias after an atrial switch operation in a patient with univentricular heart combined with transposition of the great arteries. ( 25275370 )
2014
37
Late presentation of persistent left superior caval vein in a univentricular heart with successful transcutaneous occlusion using cera lifetech atrial septal occluder. ( 25431683 )
2014
38
Intermediate-term outcomes after combined heart-liver transplantation in children with a univentricular heart. ( 23415318 )
2013
39
Functionally univentricular heart and the fontan operation: lessons learned about patterns of practice and outcomes from the congenital heart surgery databases of the European association for cardio-thoracic surgery and the society of thoracic surgeons. ( 24327626 )
2013
40
Univentricular heart with bridging bronchus and sling left pulmonary artery. ( 22639008 )
2013
41
Commentary on "Motor development of infants with univentricular heart at the ages of 16 and 52 weeks". ( 23995668 )
2013
42
Ventricular assist device in univentricular heart physiology. ( 23322095 )
2013
43
Late bidirectional glenn anastomosis in a 54-year-old patient with unoperated functionally univentricular heart. ( 24327645 )
2013
44
Intra-isthmus reentry associated with uncorrected double inlet left ventricle and transposition of the great arteries. ( 22222181 )
2013
45
Major stroke in a 19-year-old patient with a univentricular heart. ( 23345987 )
2013
46
Spinal surgery in the univentricular heart - is it viable? ( 23390991 )
2013
47
Pulmonary artery banding for univentricular heart beyond the neonatal period. ( 24887876 )
2013
48
Motor development of infants with univentricular heart at the ages of 16 and 52 weeks. ( 23995669 )
2013
49
Indication for transplantation in a patient with univentricular heart. ( 24331312 )
2013
50
The functionally univentricular heart: which is better--right or left ventricle? ( 22440218 )
2012

Variations for Univentricular Heart

ClinVar genetic disease variations for Univentricular Heart:

6
# Gene Variation Type Significance SNP ID Assembly Location
1 46;XY;t(2;14)(p22;q24.3)dn Translocation Likely pathogenic

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 ExPASy
19 FMA
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 LOVD
42 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
54 NINDS
55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 SNOMED-CT via Orphanet
71 TGDB
72 Tocris
73 UMLS
74 UMLS via Orphanet
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