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 Sodium Citrate have been mentioned in the context of this disorder. Affiliated tissues include testes, heart and brain.

Related Diseases for Univentricular Heart

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 108)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Dobutamine Approved Phase 4 34368-04-2 36811
2
Sodium Citrate Approved, Investigational Phase 4,Phase 2,Phase 1 68-04-2
3
Ambrisentan Approved, Investigational Phase 4,Phase 2 177036-94-1 6918493
4
Carvedilol Approved, Investigational Phase 4 72956-09-3 2585
5
Eplerenone Approved Phase 4 107724-20-9 443872 150310
6
Citric Acid Approved, Nutraceutical, Vet_approved Phase 4,Phase 2,Phase 1 77-92-9 311
7
Calcium Approved, Nutraceutical Phase 4,Phase 1 7440-70-2 271
8 Phosphodiesterase Inhibitors Phase 4,Phase 3,Phase 1,Phase 2
9 Adrenergic beta-Agonists Phase 4
10 Adrenergic Agonists Phase 4
11 Sympathomimetics Phase 4
12 Protective Agents Phase 4,Phase 2,Not Applicable
13 Peripheral Nervous System Agents Phase 4,Phase 3
14 Adrenergic Agents Phase 4
15 Phosphodiesterase 5 Inhibitors Phase 4,Phase 3,Phase 1,Phase 2
16 Sildenafil Citrate Phase 4,Phase 2,Phase 1 171599-83-0
17 Neurotransmitter Agents Phase 4
18 Vasodilator Agents Phase 4,Phase 2,Phase 1
19 Citrate Phase 4,Phase 2,Phase 1
20 Cardiotonic Agents Phase 4,Phase 2
21 Autonomic Agents Phase 4
22 Antihypertensive Agents Phase 4,Phase 3,Phase 2,Not Applicable
23 Antioxidants Phase 4,Not Applicable
24 Calcium, Dietary Phase 4,Phase 1
25 Hormones Phase 4,Not Applicable
26 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 4,Not Applicable
27 Natriuretic Agents Phase 4,Phase 2,Phase 3,Not Applicable
28 Mineralocorticoid Receptor Antagonists Phase 4,Not Applicable
29 diuretics Phase 4,Not Applicable
30 Diuretics, Potassium Sparing Phase 4,Not Applicable
31 Mineralocorticoids Phase 4,Not Applicable
32 Hormone Antagonists Phase 4,Not Applicable
33 Adrenergic beta-Antagonists Phase 4
34 Adrenergic alpha-1 Receptor Antagonists Phase 4
35 calcium channel blockers Phase 4
36 Adrenergic alpha-Antagonists Phase 4
37 Adrenergic Antagonists Phase 4
38
Enalapril Approved, Vet_approved Phase 3 75847-73-3 40466924 5362032
39
Enalaprilat Approved Phase 3 76420-72-9 6917719
40
Udenafil Approved, Investigational Phase 3,Phase 1,Phase 2 268203-93-6 6918523
41
Colchicine Approved Phase 2, Phase 3 64-86-8 2833 6167
42
Aspirin Approved, Vet_approved Phase 3 50-78-2 2244
43
Rivaroxaban Approved Phase 3 366789-02-8
44
Macitentan Approved Phase 3 441798-33-0
45
Serine Approved, Nutraceutical Phase 3 56-45-1 5951
46
protease inhibitors Phase 3,Phase 2
47 HIV Protease Inhibitors Phase 3,Phase 2
48 Angiotensin-Converting Enzyme Inhibitors Phase 3,Phase 2
49 Anticoagulants Phase 3,Phase 1
50 Endothelin Receptor Antagonists Phase 3,Phase 2,Not Applicable

Interventional clinical trials:

(show top 50) (show all 112)
# Name Status NCT ID Phase Drugs
1 Study of Effects of Sildenafil on Patients With Fontan Heart Circulation Completed NCT01815502 Phase 4
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 Colchicine in Postoperative Fontan Patients Recruiting NCT03575572 Phase 2, Phase 3 Colchicine
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 Fontan Udenafil Exercise Longitudinal Assessment Trial Active, not recruiting NCT02741115 Phase 3 Udenafil;Placebo
15 An Upcoming Clinical Study to Measure the Safety and Impact of a Drug Called Macitentan in Teenage and Adult Fontan Patients. Enrolling by invitation NCT03775421 Phase 3 macitentan 10 mg
16 Use of Vasopressin Following the Fontan Operation Suspended NCT02975999 Phase 2, Phase 3 Vasopressin;Normal Saline
17 Cardiac Progenitor Cell Infusion to Treat Univentricular Heart Disease (PERSEUS) Completed NCT01829750 Phase 2
18 Bosentan Therapy in Children With Functional Single Ventricle Completed NCT01662037 Phase 2 Bosentan
19 Study of Placebo or Bosentan to Treat Patients With Single Ventricle Physiology. Completed NCT01292551 Phase 2 Bosentan;Placebo
20 Pharmacokinetic/Pharmacodynamic Study of Udenafil in Adolescents Completed NCT02201342 Phase 1, Phase 2 Udenafil
21 Pathophysiological Mechanisms of Hepatopulmonary Influence in the Fontan Circulation Completed NCT00263406 Phase 2 Administration of an ACE-inhibitor or not
22 Sildenafil After the Fontan Operation Completed NCT00507819 Phase 2 Sildenafil;Placebo
23 Transfusion Strategies in Pediatric Cardiothoracic Surgery Completed NCT00350220 Phase 2
24 Mesoblast Stem Cell Therapy for Patients With Single Ventricle and Borderline Left Ventricle Recruiting NCT03079401 Phase 1, Phase 2
25 Intramyocardial Injection of Autologous Umbilical Cord Blood Derived Mononuclear Cells During Surgical Repair of Hypoplastic Left Heart Syndrome (AutoCell-S2) Recruiting NCT03779711 Phase 2
26 Ambrisentan in Single Ventricle Active, not recruiting NCT02080637 Phase 2 Ambrisentan
27 Effects of Perioperative Nesiritide or Milrinone Infusion on Recovery From Fontan Surgery Terminated NCT00543309 Phase 2 nesiritide;milrinone;placebo
28 Transcoronary Infusion of Cardiac Progenitor Cells in Patients With Single Ventricle Physiology Completed NCT01273857 Phase 1
29 Sildenafil in Single Ventricle Patients Completed NCT01169519 Phase 1 Sildenafil by injection;Sildenafil by injection;Sildenafil by injection;Sildenafil by injection
30 Effect of Tadalafil on Exercise Capacity in Pediatric Fontan Patients Completed NCT01291069 Phase 1 Tadalafil Citrate;Sugar pill
31 A Pilot Study Investigating the Clinical Use of Tissue Engineered Vascular Grafts in Congenital Heart Surgery Completed NCT01034007 Phase 1
32 Liothyronine in Children With Single Ventricle Congenital Cardiac Malformations Undergoing the Fontan Procedure Completed NCT00004828 Phase 1 liothyronine I 131
33 Feasibility and Efficacy of Portable Non-invasive Negative Pressure Ventilation in Fontan Patients Completed NCT03251742 Phase 1
34 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
35 Biomarkers for Feeding Intolerance in Infants With Complex Congenital Heart Defects Undergoing Single Ventricle Staged Palliation Unknown status NCT02995577
36 Global and Regional Myocardial Strain and Power Output In Patients With Single Ventricles Using Novel MRI Techniques Unknown status NCT01107990
37 Cardiovascular, Pulmonary and Skeletal Muscle Evaluation in Late Postoperative Period of the Fontan Surgery Unknown status NCT02283255 Not Applicable
38 Pulmonary Functions Test in Patients After Fontan Operation Unknown status NCT01413308
39 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
40 T1 Mapping of Diffuse Myocardial Fibrosis in Congenital Heart Disease Unknown status NCT02350829 Not Applicable
41 Cerebral Blood Flow in Single Ventricles Throughout Staged Surgical Reconstruction Completed NCT02135081
42 Nutritional Failure in Infants With Single Ventricle Congenital Heart Disease Completed NCT01821287
43 Single Ventricle Outcome Completed NCT00308217
44 Impact of Early Intervention on Maternal Stress in Mothers of Fetuses Diagnosed With Single Ventricle Physiology Completed NCT02462434 Not Applicable
45 Endothelin Blockade in Patients With Single Ventricle Physiology Completed NCT00989911 Not Applicable Bosentan
46 Multisite Feeding Study: Home Surveillance and Feeding Strategies in Infants With Complex Single Ventricle Completed NCT00257517
47 Outcomes of Children With Congenital Single Ventricle Heart Completed NCT00266968
48 Influence of Genetic Polymorphisms on Ventricular Structure and Function in Patients With Single Ventricle Anatomy Completed NCT00165984
49 Training in Exercise Activities and Motion for Growth Completed NCT01917084 Not Applicable
50 Diffuse Myocardial Fibrosis in Fontan Patients Completed NCT01929174

Search NIH Clinical Center for Univentricular Heart

Genetic Tests for Univentricular Heart

Anatomical Context for Univentricular Heart

MalaCards organs/tissues related to Univentricular Heart:

42
Testes, Heart, Brain, Kidney, Endothelial, Skeletal Muscle, Atrioventricular Node

Publications for Univentricular Heart

Articles related to Univentricular Heart:

(show top 50) (show all 282)
# Title Authors Year
1
Highly atypical double barrel outlet to aorta in double inlet left ventricle, double outlet right ventricle. ( 30902580 )
2019
2
Oligosymptomatic cyanotic heart disease in an adult with a univentricular heart. ( 30912113 )
2019
3
A restrictive ventilatory pattern is common in patients with univentricular heart after Fontan palliation and associated with a reduced exercise capacity and quality of life. ( 30378270 )
2019
4
Optimum age for performing Fontan operation in patients with univentricular heart. ( 30945806 )
2019
5
Subclavicular arteriovenous fistula for univentricular heart palliation. ( 30070780 )
2018
6
State of the art of the Fontan strategy for treatment of univentricular heart disease. ( 30002816 )
2018
7
First-stage palliation strategy for univentricular heart disease may impact risk for acute kidney injury. ( 28889816 )
2018
8
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 )
2018
9
Revisitation of Double Inlet Left Ventricle or Tricuspid Atresia with Transposed Great Arteries. ( 30586578 )
2018
10
Anatomy of the Functionally Univentricular Heart. ( 30322358 )
2018
11
Incidence and Outcomes of Patients with Functionally Univentricular Heart Born in Latvia, 2007 to 2015. ( 30344275 )
2018
12
Living with functionally univentricular heart: beating the odds? ( 29214833 )
2018
13
Blood-threshold CMR volume analysis of functional univentricular heart. ( 29357037 )
2018
14
Diagnostic accuracy of low-dose dual-source cardiac computed tomography as compared to surgery in univentricular heart patients. ( 29769130 )
2018
15
Long-Term Effects of Percutaneous Fenestration Following the Fontan Procedure in Adult Patients with Congenital Univentricular Heart. ( 29802801 )
2018
16
201Tl Myocardial Perfusion Imaging in a Case of Double-Inlet Left Ventricle: Correlate Anatomic Information of SPECT With MRI. ( 29293140 )
2018
17
An Unusual Combination of Double Inlet Left Ventricle With Discordant Ventriculoarterial Connections and Bilateral Arterial Ducts. ( 29334828 )
2018
18
Extracardiac Conduit Fontan Operation with Reduction Aortoplasty for Left Pulmonary Artery Compression after a Norwood Procedure in a Patient with Double-Inlet Left Ventricle. ( 29430429 )
2018
19
Improvements in exercise capacity following cardiac transplantation in a patient born with double inlet left ventricle. ( 29437740 )
2018
20
Arterial Switch, Ventricular Septation, and Fontan Takedown for Double Inlet Left Ventricle. ( 29673637 )
2018
21
Plasma microvesicle analysis identifies microRNA 129-5p as a biomarker of heart failure in univentricular heart disease. ( 28859128 )
2017
22
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
23
Stenting of the ductus arteriosus in infants with functionally univentricular heart disease and ductal-dependent pulmonary blood flow: A single-center experience. ( 27862912 )
2017
24
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
25
Impact of Abernathy malformation on pulmonary circulatory hemodynamics in a univentricular heart. ( 28163439 )
2017
26
Risk factors for in-hospital shunt thrombosis and mortality in patients weighing less than 3 kg with functionally univentricular heart undergoing a modified Blalock-Taussig shunt. ( 28520941 )
2017
27
Anomalous origin of the left coronary artery from the right pulmonary artery in a univentricular heart. ( 28651676 )
2017
28
Intra-Atrial Y-Graft Fontan for Univentricular Heart With Discontinuous Pulmonary Arteries. ( 28696884 )
2017
29
Stenting of narrow pulmonary artery banding in a patient with univentricular heart. ( 29057003 )
2017
30
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 )
2017
31
Current Results of Single Ventricle Palliation of Patients With Double Inlet Left Ventricle. ( 28709664 )
2017
32
Transvenous biventricular pacing in double-inlet left ventricle following ventricular septation and atrioventricular valve replacement. ( 28398485 )
2017
33
Functionally Univentricular Heart With Right Atrial Isomerism and Mixed Total Anomalous Pulmonary Venous Drainage. ( 26701620 )
2016
34
Evidence for improved outcome for children born with a univentricular heart defect? ( 25904767 )
2016
35
A 74-year-old unoperated univentricular heart: the oldest reported survival. ( 26508431 )
2016
36
Elevated Low-Shear Blood Viscosity is Associated with Decreased Pulmonary Blood Flow in Children with Univentricular Heart Defects. ( 26888364 )
2016
37
Mechanics of the Functionally Univentricular Heart-How Little Do We Understand and Why Does It Matter? ( 26919793 )
2016
38
Changes in Motor Development During a 4-Year Follow-up on Children With Univentricular Heart Defects. ( 27661239 )
2016
39
Commentary on "Changes in Motor Development During a 4-Year Follow-up on Children With Univentricular Heart Defects". ( 27661240 )
2016
40
Surgical approach for systemic-pulmonary shunt in neonates with functionally univentricular heart: comparison between sternotomy and thoracotomy. ( 27300351 )
2016
41
Prenatal diagnosis of functionally univentricular heart, associations and perinatal outcomes. ( 27061183 )
2016
42
Double inlet left ventricle with unrestricted pulmonary blood flow and survival into adulthood. ( 26795443 )
2016
43
Double inlet left ventricle-ventriculoarterial discordance without surgical treatment. ( 27322861 )
2016
44
Thrombocytosis in the Setting of Isomerism and a Functionally Univentricular Heart. ( 26719826 )
2015
45
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 )
2015
46
Truncus arteriosus, tricuspid atresia and partial anomalous pulmonary venous drainage: a unique form of univentricular heart. ( 25972595 )
2015
47
Incidental finding of an univentricular heart on echocardiogram in an adult patient. ( 25468956 )
2015
48
Single-stage septation of a univentricular heart: 26 years later. ( 25623904 )
2015
49
Can univentricular heart be associated with "rigid body rotation"? A case from the threedimensional speckle-tracking echocardiographic MAGYAR-path study. ( 25854450 )
2015
50
Decision-Making for Surgery in the Management of Patients with Univentricular Heart. ( 26284226 )
2015

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 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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