MCID: SNG003
MIFTS: 30

Single Ventricular Heart

Categories: Rare diseases
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Aliases & Classifications for Single Ventricular Heart

MalaCards integrated aliases for Single Ventricular Heart:

Name: Single Ventricular Heart 19 36
Single Ventricle 19 5
Single Ventricle Defect 5

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 Ambrisentan and Sildenafil have been mentioned in the context of this disorder. Affiliated tissues include heart, brain and skeletal muscle.

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 250)
# Related Disease Score Top Affiliating Genes
1 holoprosencephaly 1 11.2
2 polysyndactyly with cardiac malformation 11.2
3 critical congenital heart disease 11.1
4 hypoplastic left heart syndrome 10.8
5 heart disease 10.7
6 heart septal defect 10.6
7 visceral heterotaxy 10.6
8 transposition of the great arteries, dextro-looped 10.6
9 tricuspid atresia 10.6
10 hypoplastic left heart syndrome 1 10.6
11 conotruncal heart malformations 10.5
12 univentricular heart 10.5
13 atrioventricular septal defect 10.5
14 protein-losing enteropathy 10.5
15 ventricular septal defect 10.5
16 right atrial isomerism 10.4
17 cardiac arrest 10.4
18 subvalvular aortic stenosis 10.4
19 double outlet right ventricle 10.4
20 mitral atresia 10.3
21 complete atrioventricular canal 10.3
22 coarctation of aorta 10.3
23 patent ductus arteriosus 1 10.3
24 pulmonary hypertension 10.3
25 third-degree atrioventricular block 10.3
26 atrial heart septal defect 10.3
27 interatrial communication 10.3
28 bronchitis 10.3
29 dextrocardia 10.3
30 plastic bronchitis 10.3
31 aortic valve disease 2 10.2
32 congenitally corrected transposition of the great arteries 10.2
33 down syndrome 10.2
34 ebstein anomaly 10.2
35 pulmonary atresia with intact ventricular septum 10.2
36 atrioventricular block 10.2
37 arteriovenous malformation 10.2
38 tricuspid valve insufficiency 10.2
39 total anomalous pulmonary venous return 1 10.2
40 enterocolitis 10.2
41 pheochromocytoma 10.1
42 tetralogy of fallot 10.1
43 charge syndrome 10.1
44 sick sinus syndrome 10.1
45 pulmonary valve stenosis 10.1
46 perinatal necrotizing enterocolitis 10.1
47 double discordia 10.1
48 eisenmenger syndrome 10.1
49 paraganglioma 10.1
50 mitral valve insufficiency 10.1

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 73)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Ambrisentan Approved, Investigational Phase 4 177036-94-1 6918493
2
Sildenafil Approved, Investigational Phase 4 139755-83-2, 171599-83-0 5212 135398744
3
Carvedilol Approved, Investigational Phase 4 72956-09-3 2585
4
Dobutamine Approved Phase 4 34368-04-2 36811
5
Eplerenone Approved Phase 4 107724-20-9 443872
6 Vasodilator Agents Phase 4
7 Adrenergic beta-Antagonists Phase 4
8 Adrenergic Antagonists Phase 4
9 Adrenergic alpha-Antagonists Phase 4
10 Neurotransmitter Agents Phase 4
11 Adrenergic alpha-1 Receptor Antagonists Phase 4
12 Adrenergic Agents Phase 4
13 Calcium, Dietary Phase 4
14 calcium channel blockers Phase 4
15 Antioxidants Phase 4
16 Protective Agents Phase 4
17 Hormones Phase 4
18 Antihypertensive Agents Phase 4
19 Hormone Antagonists Phase 4
20 Diuretics, Potassium Sparing Phase 4
21 diuretics Phase 4
22 Mineralocorticoids Phase 4
23 Mineralocorticoid Receptor Antagonists Phase 4
24
Calcium Nutraceutical Phase 4 7440-70-2 271
25
Udenafil Approved, Investigational Phase 3 268203-93-6 6918523 135413547
26
Enalaprilat Approved Phase 3 76420-72-9 6917719 5462501
27
Enalapril Approved, Vet_approved Phase 3 75847-73-3 40466924 5388962 5362032
28
Sodium citrate Approved, Investigational Phase 3 68-04-2 23431961
29
Colchicine Approved Phase 2, Phase 3 64-86-8 2833 6167
30
Citric acid Approved, Nutraceutical, Vet_approved Phase 3 77-92-9 311
31
Arginine Approved, Investigational, Nutraceutical Phase 2, Phase 3 74-79-3 6322
32 Angiotensin-Converting Enzyme Inhibitors Phase 3
33 HIV Protease Inhibitors Phase 3
34
protease inhibitors Phase 3
35 Phosphodiesterase Inhibitors Phase 3
36 Phosphodiesterase 5 Inhibitors Phase 3
37 Citrate Phase 3
38 Arginine Vasopressin Phase 2, Phase 3
39 Vasopressins Phase 2, Phase 3
40 Vasoconstrictor Agents Phase 2, Phase 3
41 Hemostatics Phase 2, Phase 3
42 Coagulants Phase 2, Phase 3
43 Antirheumatic Agents Phase 2, Phase 3
44 Antimitotic Agents Phase 2, Phase 3
45 Tubulin Modulators Phase 2, Phase 3
46
Bosentan Approved, Investigational Phase 2 147536-97-8 104865
47
Spironolactone Approved Phase 1, Phase 2 1952-01-7, 52-01-7 5833
48 Endothelin Receptor Antagonists Phase 2
49 Pharmaceutical Solutions Phase 1, Phase 2
50
Tadalafil Approved, Investigational Phase 1 171596-29-5 110635

Interventional clinical trials:

(show top 50) (show all 83)
# Name Status NCT ID Phase Drugs
1 The Effects of Ambrisentan on Exercise Capacity in Fontan Patients Completed NCT01971580 Phase 4 Ambrisentan
2 Effect of Carvedilol on Exercise Performance in Fontan Patients Completed NCT02946892 Phase 4 Carvedilol;Placebo
3 Response to Sildenafil in Patients With Fontan Physiology, A Pressure-volume Loop Analysis Completed NCT01815502 Phase 4
4 Eplerenone to Prevent Myocardial Fibrosis in Congenital Heart Disease Terminated NCT01971593 Phase 4 Eplerenone
5 A Phase III Extension Study of Udenafil in Adolescents With Single Ventricle Physiology After Fontan Palliation Unknown status NCT03013751 Phase 3 Udenafil
6 The Influence of Postoperative Systemic Oxygen Transport on Neurologic and Functional Outcomes in Infants Undergoing Single Ventricle Palliation With Norwood and Hybrid Management Strategies Unknown status NCT01134302 Phase 3
7 Fontan Udenafil Exercise Longitudinal Assessment Trial (FUEL) Completed NCT02741115 Phase 3 Udenafil;Placebo
8 Use of Arginine Vasopressin in Early Postoperative Management After Fontan Palliation Completed NCT03088345 Phase 2, Phase 3 Vasopressin, Arginine;Placebo
9 Trial of Right Ventricular Versus Modified Blalock-Taussig Shunt in Infants With Single Ventricle Defect Undergoing Staged Reconstruction (A Trial Conducted by the Pediatric Heart Network) Completed NCT00115934 Phase 3
10 Trial of ACE Inhibition in Infants With Single Ventricle (A Trial Conducted by the Pediatric Heart Network) Completed NCT00113087 Phase 3 Enalapril;Placebo
11 Efficacy and Safety Study of Autologous Cardiac Stem Cells (JRM-001) Treated After Reconstructive Surgery in Pediatric Patients With Congenital Heart Disease: A Multicenter Randomized Single-blind Parallel-group Study Recruiting NCT02781922 Phase 3
12 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
13 Use of Vasopressin Following the Fontan Operation: Both Pilot and Multicenter Studies Suspended NCT02975999 Phase 2, Phase 3 Vasopressin;Normal Saline
14 Colchicine in Postoperative Fontan Patients (CPFP) Terminated NCT03575572 Phase 2, Phase 3 Colchicine
15 Bosentan Therapy for High Risk Staged Fontan Procedure in Children With Functional Single Ventricle Completed NCT01662037 Phase 2 Bosentan
16 A Phase I/II Dose Escalation Trial of Udenafil in Adolescents With Single Ventricle Physiology After Fontan Palliation Completed NCT02201342 Phase 1, Phase 2 Udenafil
17 Safety, Pharmacokinetics (PK) and Hemodynamic Effects of Ambrisentan in Single Ventricle Pediatric Patients Completed NCT02080637 Phase 2 Ambrisentan
18 Prospective, Open-labeled, Single-arm Clinical Trial to Evaluate the Safety and Efficacy of the Second-generation Tissue Engineered Vascular Graft as Vascular Conduits for Extracardiac Total Cavopulmonary Connection. Recruiting NCT04467671 Phase 2
19 Acute Imposition of Fontan Physiology in The Single Ventricle Patient: Effects on Fibrosis, Function and Drug Intervention Recruiting NCT04901975 Phase 1, Phase 2 Spironolactone
20 Mesoblast Stem Cell Therapy for Patients With Single Ventricle and Borderline Left Ventricle Active, not recruiting NCT03079401 Phase 1, Phase 2
21 A Pilot Study Investigating the Clinical Use of Tissue Engineered Vascular Grafts in Congenital Heart Surgery Completed NCT01034007 Phase 1
22 Phase I Study of Cardiac Progenitor Cell Therapy in Patients With Single Ventricle Physiology Completed NCT01273857 Phase 1
23 Effect of Tadalafil on Exercise Capacity in Pediatric Fontan Patients Completed NCT01291069 Phase 1 Tadalafil Citrate;Sugar pill
24 Liothyronine in Children With Single Ventricle Congenital Cardiac Malformations Undergoing the Fontan Procedure Completed NCT00004828 Phase 1 liothyronine I 131
25 Safety, Pharmacokinetics and Hemodynamic Efficacy of Sildenafil in Single Ventricle Patients Completed NCT01169519 Phase 1 Sildenafil by injection
26 " An Open Label Pilot Study Evaluating Safety and Evidence of Therapeutic Effect of IV Admin of 2-0, 3-0 Desulfated Heparin, Treatment of Exacerbation of Protein Losing Enteropathy (PLE) Associated With 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
27 Biomarkers for Feeding Intolerance in Infants With Complex Congenital Heart Defects Undergoing Single Ventricle Staged Palliation Unknown status NCT02995577
28 Imaging and Biomarker Evaluation of Hepatic Stiffness in Children Enrolled in the Fontan Udenafil Exercise Longitudinal Study Unknown status NCT03430583 MZ101
29 Long-term Survival After Single-ventricle Palliation: A Swedish Nationwide Cohort Study Unknown status NCT03356574
30 Cognitive Intervention to Improve Working Memory in Adolescents With Single Ventricle Heart Disease Completed NCT03687008
31 Redesigning the Care Journey of Patients With Single-ventricle Congenital Heart Disease Completed NCT04613934
32 Adaptation of Alveolar-capillary Diffusion at Effort of Subjects Suffering From Complex Congenital Heart Disease Completed NCT02561975
33 A Magnetic Resonance Imaging Study to Determine Cerebral Blood Flow Changes in Single Ventricle Patients During Staged Reconstruction as Compared to Normal Children Completed NCT02135081
34 Improving Functional Capacity in Fontan Patients Through Inspiratory Muscle Training Completed NCT01977222
35 Heart Failure and Fibrosis in Adult Congenital Heart Patients With a Single Ventricle and Prior Fontan Operation. Completed NCT01929174
36 Assessment of Exercise Capacity in Single-ventricle Patients Undergoing Fontan Procedure Completed NCT05063903
37 Cardiovascular, Pulmonary and Skeletal Muscle Evaluation in Patients With Univentricular Physiology in the Late Postoperative Period of the Fontan Surgery: Effects of Exercise Training Completed NCT02283255
38 A Pilot Study of Inspiratory Muscle Function and the Effects of Inspiratory Muscle Training in Subjects With Fontan Physiology Completed NCT03339466
39 Training in Exercise Activities and Motion for Growth (A Study Conducted by the Pediatric Heart Network) Completed NCT01917084
40 Parents Post-traumatic Stress Before and After Their Infant's Second Palliative Heart Surgery: The PaTHS Descriptive Correlational Longitudinal Study Completed NCT04785482
41 The Examination of Outcome in Children With Single Ventricle Physiology Completed NCT00308217
42 Cross-sectional Analysis of Non-invasive Measures of Ventricular Systolic and Diastolic Function and Biomarkers in the Fontan Circulation Completed NCT03119090
43 Impact of Early Intervention on Maternal Stress in Mothers of Fetuses Diagnosed With Single Ventricle Physiology Requiring Neonatal Surgery Completed NCT02462434
44 Pharmacokinetics and Safety Profile of Digoxin in Infants With Single Ventricle Congenital Heart Disease Completed NCT03877965 Digoxin
45 Single Ventricle Reconstruction III: Brain Connectome and Neurodevelopmental Outcomes Completed NCT02692443
46 Cardiac Biomarkers in Patients With Single Ventricle Physiology Completed NCT03678909
47 Vitamin C May Improve Endothelial Function and Exercise Capacity in Functional Single Ventricle Patients After Fontan Palliation Completed NCT00974025
48 Influence of Genetic Polymorphisms on Ventricular Structure and Function in Patients With Single Ventricle Anatomy Completed NCT00165984
49 Factors Associated With Normal Exercise Capacity in Patients With Single Ventricle: the Multicenter Cross-sectional Study Completed NCT04026542
50 Parental Experience in the Single Ventricle Interstage Utilizing a mHealth Innovation: A Comparative Case Study Completed NCT03231020

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 stem cells PMIDs: 17502484 14530411 18754813
Cardiac progenitor cells PMIDs: 17502484 14530411 18754813

Genetic Tests for Single Ventricular Heart

Anatomical Context for Single Ventricular Heart

Organs/tissues related to Single Ventricular Heart:

MalaCards : Heart, Brain, Skeletal Muscle, Endothelial, Liver, Bone Marrow, Lung

Publications for Single Ventricular Heart

Articles related to Single Ventricular Heart:

(show top 50) (show all 3881)
# Title Authors PMID Year
1
Motor development in infants with complex congenital heart disease: A longitudinal study. 62
35665492 2023
2
Hemodynamic Response to Fluid Boluses in Patients with Single-Ventricle Parallel Circulation. 62
35511283 2022
3
Overgrowth of the Amygdala in Children with Single Ventricle Congenital Heart Disease. 62
36170242 2022
4
A Novel Strategy for the Mechanical Subpulmonary Support in Failing Fontan Patients. 62
36368697 2022
5
A Review of Biventricular Repair for the Congenital Cardiac Anesthesiologist. 62
36470215 2022
6
Computational analysis of serum-derived extracellular vesicle miRNAs in juvenile sheep model of single stage Fontan procedure. 62
36330420 2022
7
Physical Frailty is Modifiable in Young Cardiac Rehabilitation Patients. 62
35511282 2022
8
Cardiovascular surgery in very low birth weight (≤ 1500g) neonates. 62
36469322 2022
9
Update on combined heart and liver transplantation: evolving patient selection, improving outcomes, and outstanding questions. 62
36454232 2022
10
Status of adult outpatients with congenital heart disease in Japan: The Japanese Network of Cardiovascular Departments for Adult Congenital Heart Disease Registry. 62
35995687 2022
11
Sonography of fetal holoprosencephaly: a guide to recognize the lesser varieties. 62
35272544 2022
12
Risk Factors for Perioperative Brain Lesions in Infants With Congenital Heart Disease: A European Collaboration. 62
36300371 2022
13
Prevalence and Associated Factors of Long-term Growth Failure in Infants with Congenital Heart Disease Who Underwent Cardiac Surgery Before the Age of One. 62
35661240 2022
14
Impact of Maternal-Fetal Environment on Outcomes Following the Hybrid Procedure in the Single Ventricle Population. 62
36462027 2022
15
Exploring Noncardiac Surgical Needs From Infancy to Adulthood in Patients With Congenital Heart Disease. 62
36216687 2022
16
Outcomes of Extracorporeal Membrane Oxygenation in Patients After Repair of Congenital Heart Defects. 62
35532807 2022
17
Fatal nocardiosis infection in a pediatric patient with an immunodeficiency after heart re-transplantation. 62
35726843 2022
18
Factors Associated With Gastrostomy Tube Complications in Infants With Congenital Heart Disease. 62
36030602 2022
19
Preterm Birth Is Associated With Adverse Cardiac Remodeling and Worse Outcomes in Patients with a Functional Single Right Ventricle. 62
36470462 2022
20
Perioperative Outcomes in Patients With Failing Single-Ventricle Physiology Undergoing Ventricular Assist Device Placement: A Single Institutional Experience. 62
36050214 2022
21
Survival After Intervention for Single-Ventricle Heart Disease Over 15 Years at a Single Institution. 62
35430225 2022
22
The Surprise Question as a Trigger for Primary Palliative Care Interventions for Children with Advanced Heart Disease. 62
35503117 2022
23
Postoperative opioid administration and post-traumatic stress symptoms in preschool children after cardiac surgery. 62
36333168 2022
24
Evolution of Ventricular Assist Device Support Strategy in Children With Univentricular Physiology. 62
34710386 2022
25
Prevalence and Treatment Outcomes of Arrhythmias in Patients with Single Ventricle Physiology over the Age of 40 Years. 62
36362800 2022
26
Forty-year survival after Glenn procedure without Fontan procedure in patients with single ventricle. 62
36322816 2022
27
Force Profiles of Single Ventricle Atrioventricular Leaflets in Response to Annular Dilation and Leaflet Tethering. 62
36455710 2022
28
Palliated hypoplastic left heart syndrome patients experience superior waitlist and comparable post-heart transplant survival to non-single ventricle congenital heart disease patients. 62
36455711 2022
29
A computational study of aortic reconstruction in single ventricle patients. 62
36335184 2022
30
Impact of fetal haemodynamics on surgical and neurodevelopmental outcomes in patients with Ebstein anomaly and tricuspid valve dysplasia. 62
34986918 2022
31
Outcomes of infants with pulmonary atresia with intact ventricular septum listed for heart transplantation: A multi-institutional study. 62
35768886 2022
32
Pulmonary atresia with intact ventricular septum: Intended strategies. 62
35414413 2022
33
[A case of recurrent cerebral infarction in an adult patient with false Taussig-Bing anomaly]. 62
36450491 2022
34
Opportunities and short-comings of the axolotl salamander heart as a model system of human single ventricle and excessive trabeculation. 62
36443330 2022
35
Micra pacemaker in adult congenital heart disease patients: A case series. 62
36041216 2022
36
The evolving treatment of congenital heart disease in patient with Down syndrome: Current state of knowledge. 62
35989531 2022
37
Cor triatriatum sinister: Long-term surgical outcomes in children and a proposal for a new classification. 62
36321701 2022
38
Preparatory stage of interventional extracardiac Fontan. 62
36355352 2022
39
Development and Initial Validation of a Frailty Score for Pediatric Patients with Congenital and Acquired Heart Disease. 62
36378279 2022
40
Impact of Abnormal Potassium on Arrhythmia Risk During Pediatric Digoxin Therapy. 62
36403164 2022
41
Determinants and Prognostic Implications of Hepatorenal Dysfunction in Adults With Congenital Heart Disease. 62
35934261 2022
42
Failing Fontan cardiovascular support: Review. 62
36321714 2022
43
An analysis of 183 Heart Transplants for Pediatric or Congenital Heart Disease - Impact of High PRA. 62
36370883 2022
44
Heart Failure in Complex Congenital Heart Disease of the Adult. 62
36197543 2022
45
Impact of ferumoxytol vs gadolinium on 4D flow cardiovascular magnetic resonance measurements in small children with congenital heart disease. 62
36352454 2022
46
Preoperative Management of Neonates With Congenital Heart Disease. 62
36317975 2022
47
Influence of Antegrade Pulmonary Blood Flow on Outcomes of Superior Cavopulmonary Connection. 62
35341786 2022
48
Ventricular Assist Device Outcomes in Infants and Children With Stage 1 Single Ventricle Palliation. 62
36326704 2022
49
The Key to Success in Ventricular Assist Device Support for Single Ventricle. 62
34922914 2022
50
Impact of Udenafil on Echocardiographic Indices of Single Ventricle Size and Function in FUEL Study Participants. 62
36378780 2022

Variations for Single Ventricular Heart

ClinVar genetic disease variations for Single Ventricular Heart:

5
# Gene Name Type Significance ClinVarId dbSNP ID Position
1 FOXP1 NM_001349338.3(FOXP1):c.1702C>T (p.Pro568Ser) SNV Association
55847 rs147674680 GRCh37: 3:71019907-71019907
GRCh38: 3:70970756-70970756
2 NKX2-5 NM_004387.4(NKX2-5):c.809G>A (p.Cys270Tyr) SNV Likely Benign
156161 rs587782931 GRCh37: 5:172659738-172659738
GRCh38: 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

2 CDC
6 CNVD
8 Cosmic
9 dbSNP
10 DGIdb
16 EFO
17 ExPASy
18 FMA
19 GARD
27 GO
28 GTR
29 HMDB
30 HPO
31 ICD10
32 ICD10 via Orphanet
33 ICD11
34 ICD9CM
35 IUPHAR
36 LifeMap
38 LOVD
40 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
52 NINDS
53 Novoseek
55 ODiseA
56 OMIM via Orphanet
57 OMIM® (Updated 08-Dec-2022)
61 PubChem
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 Tocris
71 UMLS
72 UMLS via Orphanet
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