MCID: SNG003
MIFTS: 31

Single Ventricular Heart

Categories: Rare diseases

Aliases & Classifications for Single Ventricular Heart

MalaCards integrated aliases for Single Ventricular Heart:

Name: Single Ventricular Heart 39 54
Single Ventricle 54 6
Single Ventricle Defect 6

Classifications:



Summaries for Single Ventricular Heart

MalaCards based summary : Single Ventricular Heart, also known as single ventricle, is related to holoprosencephaly 1 and polysyndactyly with cardiac malformation. The drugs Sodium Citrate and Dobutamine have been mentioned in the context of this disorder. Affiliated tissues include heart, brain and testes.

Related Diseases for Single Ventricular Heart

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

(show top 50) (show all 88)
# Related Disease Score Top Affiliating Genes
1 holoprosencephaly 1 11.2
2 polysyndactyly with cardiac malformation 11.0
3 microphthalmia, syndromic 9 11.0
4 heart disease 10.5
5 hypoplastic left heart syndrome 1 10.2
6 hypoplastic left heart syndrome 10.2
7 heterotaxy 10.2
8 transposition of the great arteries 10.1
9 heterotaxy, visceral, 1, x-linked 10.1
10 tricuspid atresia 10.1
11 rere-related disorders 10.1
12 hypoxia 10.1
13 pulmonary hypertension 9.9
14 down syndrome 9.9
15 atrioventricular septal defect 9.9
16 dextrocardia 9.9
17 univentricular heart 9.9
18 pheochromocytoma 9.8
19 tetralogy of fallot 9.8
20 pulmonary atresia with intact ventricular septum 9.8
21 paraganglioma 9.8
22 adrenal gland pheochromocytoma 9.8
23 cardiac arrest 9.8
24 ventricular septal defect 9.8
25 lymphopenia 9.8
26 pulmonary embolism 9.8
27 aortic coarctation 9.8
28 double discordia 9.8
29 eisenmenger syndrome 9.8
30 thrombosis 9.7
31 coronary thrombosis 9.7
32 coarctation of aorta 9.6
33 tracheoesophageal fistula with or without esophageal atresia 9.6
34 anus, imperforate 9.6
35 right atrial isomerism 9.6
36 atrioventricular dissociation 9.6
37 conotruncal heart malformations 9.6
38 cystic fibrosis 9.6
39 ebstein anomaly 9.6
40 pulmonic stenosis 9.6
41 graves disease 1 9.6
42 thoracoabdominal syndrome 9.6
43 sickle cell anemia 9.6
44 body mass index quantitative trait locus 1 9.6
45 anxiety 9.6
46 aspirin resistance 9.6
47 myocardial infarction 9.6
48 transposition of the great arteries, dextro-looped 1 9.6
49 muscle hypertrophy 9.6
50 acute myocardial infarction 9.6

Graphical network of the top 20 diseases related to Single Ventricular Heart:



Diseases related to Single Ventricular Heart

Symptoms & Phenotypes for Single Ventricular Heart

Drugs & Therapeutics for Single Ventricular Heart

Drugs for Single Ventricular 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
Sodium Citrate Approved, Investigational Phase 4,Phase 2,Phase 1 68-04-2
2
Dobutamine Approved Phase 4 34368-04-2 36811
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 Vasodilator Agents Phase 4,Phase 2,Phase 1
9 Adrenergic beta-Agonists Phase 4
10 Peripheral Nervous System Agents Phase 4,Phase 3
11 Sildenafil Citrate Phase 4,Phase 2,Phase 1 171599-83-0
12 Adrenergic Agonists Phase 4
13 Autonomic Agents Phase 4
14 Neurotransmitter Agents Phase 4
15 Sympathomimetics Phase 4
16 Cardiotonic Agents Phase 4,Phase 2
17 Citrate Phase 4,Phase 2,Phase 1
18 Phosphodiesterase 5 Inhibitors Phase 4,Phase 3,Phase 1,Phase 2
19 Adrenergic Agents Phase 4
20 Phosphodiesterase Inhibitors Phase 4,Phase 3,Phase 1,Phase 2
21 Protective Agents Phase 4,Phase 2,Not Applicable
22 Antihypertensive Agents Phase 4,Phase 3,Phase 2,Not Applicable
23 Hormones Phase 4,Not Applicable
24 Antioxidants Phase 4,Not Applicable
25 Calcium, Dietary Phase 4,Phase 1
26 Hormone Antagonists Phase 4,Not Applicable
27 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 4,Not Applicable
28 diuretics Phase 4,Not Applicable
29 Diuretics, Potassium Sparing Phase 4,Not Applicable
30 Mineralocorticoids Phase 4,Not Applicable
31 Natriuretic Agents Phase 4,Phase 2,Phase 3,Not Applicable
32 Mineralocorticoid Receptor Antagonists Phase 4,Not Applicable
33 calcium channel blockers Phase 4
34 Adrenergic alpha-1 Receptor Antagonists Phase 4
35 Adrenergic beta-Antagonists Phase 4
36 Adrenergic Antagonists Phase 4
37 Adrenergic alpha-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 Angiotensin-Converting Enzyme Inhibitors Phase 3,Phase 2
48 HIV Protease 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 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 Sildenafil After the Fontan Operation Completed NCT00507819 Phase 2 Sildenafil;Placebo
21 Transfusion Strategies in Pediatric Cardiothoracic Surgery Completed NCT00350220 Phase 2
22 Cardiac Progenitor Cell Infusion to Treat Univentricular Heart Disease (PERSEUS) Completed NCT01829750 Phase 2
23 Pathophysiological Mechanisms of Hepatopulmonary Influence in the Fontan Circulation Completed NCT00263406 Phase 2 Administration of an ACE-inhibitor or not
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 Single Ventricular Heart

Cell-based therapeutics:


LifeMap Discovery
Data from LifeMap, the Embryonic Development and Stem Cells Database
Read about Single Ventricular Heart cell therapies at LifeMap Discovery.
Stem-cell-based therapeutic approaches for Single Ventricular Heart:
Cardiac progenitor cells for single-ventricle anomalies
Embryonic/Adult Cultured Cells Related to Single Ventricular Heart:
Cardiac progenitor cells PMIDs: 18754813 17502484 14530411
Cardiac stem cells PMIDs: 18754813 17502484 14530411

Genetic Tests for Single Ventricular Heart

Anatomical Context for Single Ventricular Heart

MalaCards organs/tissues related to Single Ventricular Heart:

42
Heart, Brain, Testes, Liver, Endothelial, Kidney, Skeletal Muscle

Publications for Single Ventricular Heart

Articles related to Single Ventricular Heart:

(show top 50) (show all 897)
# Title Authors Year
1
Impact of Perioperative Brain Injury and Development on Feeding Modality in Infants With Single Ventricle Heart Disease. ( 31070077 )
2019
2
Oxygen saturations and neurodevelopmental outcomes in single ventricle heart disease. ( 30919581 )
2019
3
Bridge to Transplant with Ventricular Assist Device Support in Pediatric Patients with Single Ventricle Heart Disease. ( 30864969 )
2019
4
Risk factors for death or heart transplantation in single-ventricle physiology (tricuspid atresia, pulmonary atresia, and heterotaxy): A systematic review and meta-analysis. ( 31006521 )
2019
5
Postoperative heart failure after stage 1 palliative surgery for single ventricle cardiac disease. ( 30937501 )
2019
6
Avoiding Furosemide Ototoxicity Associated With Single-Ventricle Repair in Young Infants. ( 30489485 )
2019
7
The effect of right ventricular function on survival and morbidity following stage 2 palliation: An analysis of the single ventricle reconstruction trial public data set. ( 30506893 )
2019
8
Health-related quality of life in pre-school age children with single-ventricle CHD. ( 30599815 )
2019
9
Feeding methods for infants with single ventricle physiology are associated with length of stay during stage 2 surgery hospitalization. ( 30635975 )
2019
10
Percutaneous transhepatic venous access for atrial tachyarrhythmia ablation in patients with single ventricle and interrupted inferior vena cava. ( 30693202 )
2019
11
Corrigendum to "Indications for and results of axillary arterio-venous fistula in patients with a functionally single ventricle after cavopulmonary anastomosis" [Eur J Cardiothorac Surg 2018;00:1-7]†. ( 30698689 )
2019
12
Outcomes of Common Atrioventricular Valve Repair in Patients With Single-Ventricle Physiology - Indication, Timing and Repair Techniques. ( 30745490 )
2019
13
Outcomes of Single Ventricle Patients Undergoing the Kawashima Procedure: Can We Do Better? ( 30799723 )
2019
14
Oval stenting in left pulmonary artery stenosis: a novel double balloon technique to prevent airway compression in single ventricle. ( 30834894 )
2019
15
Palliation of Functionally Single Ventricle Patients in Sub-Saharan Africa-Is It Justifiable? ( 30841827 )
2019
16
Single Ventricle Palliation in a Developing Sub-Saharan African Country: What Should be Improved? ( 30841832 )
2019
17
A bridge to heart transplant: Systemic atrioventricular valve replacement with a Melody valve in an infant with a single-ventricle physiology. ( 30853228 )
2019
18
Commentary: Individualized surgery for patients with single-ventricle physiology. ( 30902464 )
2019
19
Commentary: Single Ventricle Reconstruction 2.0: A closer look at our understanding of care for infants undergoing the Norwood procedure. ( 30928220 )
2019
20
Early prediction of critical events for infants with single-ventricle physiology in critical care using routinely collected data. ( 30948317 )
2019
21
Factors Associated with Delayed Transition to Oral Feeding in Infants with Single Ventricle Physiology. ( 30952511 )
2019
22
Commentary: Single-ventricle reconstruction: Chronicle of a death foretold? ( 30992208 )
2019
23
Challenges of Caring for Functionally Single Ventricle Patients in Africa. ( 31084309 )
2019
24
Single-ventricle reconstruction 2.0: A closer look at our understanding of care for infants undergoing the Norwood procedure. ( 31101343 )
2019
25
Commentary: Single ventricle patients and shunts-ACute event is not pretty. ( 31109708 )
2019
26
Contrast Echocardiogram for the Evaluation of Streaming in a Single-Ventricle Patient. ( 30306199 )
2019
27
Global strain and dyssynchrony of the single ventricle predict adverse cardiac events after the Fontan procedure: Analysis using feature-tracking cine magnetic resonance imaging. ( 30190094 )
2019
28
Prenatal Diagnosis of Single Ventricle Physiology Impacts on Cardiac Morbidity and Mortality. ( 30121866 )
2019
29
Successful management of living donor liver transplantation for biliary atresia with single ventricle physiology-from peri-transplant through total cavopulmonary connection: A case report. ( 29457852 )
2018
30
Single-Ventricle Palliation in a 4-Year-Old With Ehlers-Danlos Syndrome. ( 29233360 )
2018
31
Long-Term Outcomes in Single-Ventricle Congenital Heart Disease. ( 30571273 )
2018
32
Ventricular Pacing in Single-Ventricle Complex Congenital Heart Disease: How Hard it Is to Achieve the Ideal. ( 30336875 )
2018
33
Longitudinal Assessment of Outcome From Prenatal Diagnosis Through Fontan Operation for Over 500 Fetuses With Single Ventricle-Type Congenital Heart Disease: The Philadelphia Fetus-to-Fontan Cohort Study. ( 30371305 )
2018
34
Safety of outpatient cardiac catheterisation in infants with single-ventricle or shunt-dependent biventricular congenital heart disease. ( 30309401 )
2018
35
Fetuses with single ventricle congenital heart disease manifest impairment of regional brain growth. ( 30328635 )
2018
36
Regenerative medicine therapy for single ventricle congenital heart disease. ( 29770299 )
2018
37
Interstage Outcomes in Infants With Single Ventricle Heart Disease Comparing Home Monitoring Technology to Three-Ring Binder Documentation: A Randomized Crossover Study. ( 29692236 )
2018
38
Cell-Based Therapy With Cardiosphere-Derived Cardiocytes: A New Hope for Pediatric Patients With Single Ventricle Congenital Heart Disease? ( 29599273 )
2018
39
Impact of Cardiac Progenitor Cells on Heart Failure and Survival in Single Ventricle Congenital Heart Disease. ( 29367212 )
2018
40
Brain abnormalities in cognition, anxiety, and depression regulatory regions in adolescents with single ventricle heart disease. ( 29315714 )
2018
41
A randomised trial of early palliative care for maternal stress in infants prenatally diagnosed with single-ventricle heart disease. ( 29316996 )
2018
42
Regional brain gray matter changes in adolescents with single ventricle heart disease. ( 29222023 )
2018
43
Reduction of brain volumes after neonatal cardiopulmonary bypass surgery in single-ventricle congenital heart disease before Fontan completion. ( 29278641 )
2018
44
Cardiovascular CT for evaluation of single-ventricle heart disease: risks and accuracy compared with interventional findings. ( 28889811 )
2018
45
Long-term outcomes of single-ventricle palliation for pulmonary atresia with intact ventricular septum: Fontan survivors remain at risk of late myocardial ischaemia and death. ( 29444216 )
2018
46
Primary Draining Vein Stenting for Obstructive Total Anomalous Pulmonary Venous Connection in Neonates with Right Atrial Isomerism and Functional Single Ventricle Improves Outcome. ( 29777280 )
2018
47
Long-term survival of a patient with single atrium and single ventricular heart: a case report ( 30064184 )
2018
48
Effect of fetal hemodynamics on growth in fetuses with single ventricle or transposition of the great arteries. ( 29057564 )
2018
49
Life-threatening airway bleeding after palliation of single ventricle congenital heart disease. ( 28775102 )
2018
50
Hybrid stage 1 palliation as a bridge to cardiac transplantation in patients with high-risk single ventricle physiology. ( 30338630 )
2018

Variations for Single Ventricular Heart

ClinVar genetic disease variations for Single Ventricular Heart:

6
# Gene Variation Type Significance SNP ID Assembly Location
1 FOXP1 NM_032682.5(FOXP1): c.1702C> T (p.Pro568Ser) single nucleotide variant Benign/Likely benign, association rs147674680 GRCh37 Chromosome 3, 71019907: 71019907
2 FOXP1 NM_032682.5(FOXP1): c.1702C> T (p.Pro568Ser) single nucleotide variant Benign/Likely benign, association rs147674680 GRCh38 Chromosome 3, 70970756: 70970756
3 NKX2-5 NM_004387.3(NKX2-5): c.809G> A (p.Cys270Tyr) single nucleotide variant Conflicting interpretations of pathogenicity rs587782931 GRCh37 Chromosome 5, 172659738: 172659738
4 NKX2-5 NM_004387.3(NKX2-5): c.809G> A (p.Cys270Tyr) single nucleotide variant Conflicting interpretations of pathogenicity rs587782931 GRCh38 Chromosome 5, 173232735: 173232735

Expression for Single Ventricular Heart

Search GEO for disease gene expression data for Single Ventricular Heart.

Pathways for Single Ventricular Heart

GO Terms for Single Ventricular Heart

Sources for Single Ventricular 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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