MCID: SNG003
MIFTS: 30

Single Ventricular Heart

Categories: Rare diseases

Aliases & Classifications for Single Ventricular Heart

MalaCards integrated aliases for Single Ventricular Heart:

Name: Single Ventricular Heart 20 37
Single Ventricle 20 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 pulmonary atresia with intact ventricular septum. The drugs Ambrisentan and Dobutamine have been mentioned in the context of this disorder. Affiliated tissues include heart, endothelial and brain.

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 217)
# Related Disease Score Top Affiliating Genes
1 holoprosencephaly 1 11.1
2 pulmonary atresia with intact ventricular septum 11.1
3 critical congenital heart disease 11.0
4 polysyndactyly with cardiac malformation 11.0
5 microphthalmia, syndromic 9 11.0
6 hypoplastic left heart syndrome 10.8
7 heart disease 10.7
8 heart septal defect 10.6
9 univentricular heart 10.6
10 tricuspid atresia 10.6
11 heterotaxy 10.6
12 dextro-looped transposition of the great arteries 10.5
13 ventricular septal defect 10.5
14 atrioventricular septal defect 10.4
15 protein-losing enteropathy 10.4
16 subvalvular aortic stenosis 10.4
17 pulmonary hypertension 10.3
18 mitral atresia 10.3
19 double outlet right ventricle 10.3
20 right atrial isomerism 10.3
21 patent ductus arteriosus 1 10.3
22 cardiac arrest 10.3
23 dextrocardia 10.3
24 bronchitis 10.2
25 plastic bronchitis 10.2
26 coarctation of aorta 10.2
27 arteriovenous malformation 10.2
28 atrial heart septal defect 10.2
29 tricuspid valve insufficiency 10.2
30 complete atrioventricular canal 10.2
31 atrioventricular block 10.2
32 congestive heart failure 10.2
33 down syndrome 10.1
34 visceral heterotaxy 10.1
35 47,xyy 10.1
36 double discordia 10.1
37 eisenmenger syndrome 10.1
38 pheochromocytoma 10.1
39 tetralogy of fallot 10.1
40 enterocolitis 10.1
41 adrenal gland pheochromocytoma 10.1
42 thrombosis 10.1
43 pulmonary valve stenosis 10.1
44 hypoxia 10.1
45 atrial standstill 1 10.0
46 pulmonic stenosis 10.0
47 paraganglioma 10.0
48 sinoatrial node disease 10.0
49 respiratory failure 10.0
50 sick sinus syndrome 10.0

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 66)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Ambrisentan Approved, Investigational Phase 4 177036-94-1 6918493
2
Dobutamine Approved Phase 4 34368-04-2 36811
3
Carvedilol Approved, Investigational Phase 4 72956-09-3 2585
4 Vasodilator Agents Phase 4
5 Sildenafil Citrate Phase 4 171599-83-0
6 Antihypertensive Agents Phase 4
7 Adrenergic Antagonists Phase 4
8 Adrenergic beta-Antagonists Phase 4
9 Antioxidants Phase 4
10 Adrenergic alpha-1 Receptor Antagonists Phase 4
11 Hormones Phase 4
12 Adrenergic alpha-Antagonists Phase 4
13 Adrenergic Agents Phase 4
14 Neurotransmitter Agents Phase 4
15 calcium channel blockers Phase 4
16 Calcium, Dietary Phase 4
17
Calcium Nutraceutical Phase 4 7440-70-2 271
18
Enalapril Approved, Vet_approved Phase 3 75847-73-3 5362032 40466924
19
Enalaprilat Approved Phase 3 76420-72-9 6917719
20
Udenafil Approved, Investigational Phase 3 268203-93-6 6918523
21
Sodium citrate Approved, Investigational Phase 3 68-04-2
22
Colchicine Approved Phase 2, Phase 3 64-86-8 6167 2833
23
Citric acid Approved, Nutraceutical, Vet_approved Phase 3 77-92-9 311
24 Angiotensin-Converting Enzyme Inhibitors Phase 3
25
protease inhibitors Phase 3
26 HIV Protease Inhibitors Phase 3
27 Phosphodiesterase Inhibitors Phase 3
28 Phosphodiesterase 5 Inhibitors Phase 3
29 Citrate Phase 3
30 Antirheumatic Agents Phase 2, Phase 3
31 Antimitotic Agents Phase 2, Phase 3
32 Tubulin Modulators Phase 2, Phase 3
33
Bosentan Approved, Investigational Phase 2 147536-97-8 104865
34
Epinephrine Approved, Vet_approved Phase 2 51-43-4 5816
35
Racepinephrine Approved Phase 2 329-65-7 838
36 Kava Approved, Investigational, Nutraceutical Phase 2 9000-38-8
37 Endothelin Receptor Antagonists Phase 2
38 Epinephryl borate Phase 2
39
Tadalafil Approved, Investigational Phase 1 171596-29-5 110635
40
Heparin Approved, Investigational Phase 1 9005-49-6 772 9812414
41 calcium heparin Phase 1
42
Spironolactone Approved 1952-01-7, 52-01-7 5833
43
tannic acid Approved 1401-55-4
44
Benzocaine Approved, Investigational 1994-09-7, 94-09-7 2337
45
Treprostinil Approved, Investigational 81846-19-7 54786 6918140
46
Digoxin Approved 20830-75-5 30322 2724385
47
Vitamin C Approved, Nutraceutical 50-81-7 5785 54670067
48 Antibiotics, Antitubercular
49 Anti-Bacterial Agents
50
Substance P 33507-63-0 44359816

Interventional clinical trials:

(show top 50) (show all 68)
# Name Status NCT ID Phase Drugs
1 The Effects of Ambrisentan on Exercise Capacity in Fontan Patients Completed NCT01971580 Phase 4 Ambrisentan
2 Response to Sildenafil in Patients With Fontan Physiology, A Pressure-volume Loop Analysis Completed NCT01815502 Phase 4
3 Effect of Carvedilol on Exercise Performance in Fontan Patients Completed NCT02946892 Phase 4 Carvedilol;Placebo
4 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
5 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
6 Fontan Udenafil Exercise Longitudinal Assessment Trial (FUEL) Completed NCT02741115 Phase 3 Udenafil;Placebo
7 Trial of ACE Inhibition in Infants With Single Ventricle (A Trial Conducted by the Pediatric Heart Network) Completed NCT00113087 Phase 3 Enalapril;Placebo
8 A Phase III Extension Study of Udenafil in Adolescents With Single Ventricle Physiology After Fontan Palliation Active, not recruiting NCT03013751 Phase 3 Udenafil
9 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
10 Colchicine in Postoperative Fontan Patients (CPFP) Terminated NCT03575572 Phase 2, Phase 3 Colchicine
11 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
12 Bosentan Therapy for High Risk Staged Fontan Procedure in Children With Functional Single Ventricle Completed NCT01662037 Phase 2 Bosentan
13 Safety, Pharmacokinetics (PK) and Hemodynamic Effects of Ambrisentan in Single Ventricle Pediatric Patients Completed NCT02080637 Phase 2 Ambrisentan
14 Phase IIb Study of Intramyocardial Injection of Autologous Umbilical Cord Blood Derived Mononuclear Cells During Stage II Surgical Repair of Right Ventricular Dependent Variants of Hypoplastic Left Heart Syndrome (AutoCell-S2) Recruiting NCT03779711 Phase 2
15 Mesoblast Stem Cell Therapy for Patients With Single Ventricle and Borderline Left Ventricle Recruiting NCT03079401 Phase 1, Phase 2
16 A Pilot Study Investigating the Clinical Use of Tissue Engineered Vascular Grafts in Congenital Heart Surgery Completed NCT01034007 Phase 1
17 Effect of Tadalafil on Exercise Capacity in Pediatric Fontan Patients Completed NCT01291069 Phase 1 Tadalafil Citrate;Sugar pill
18 Phase I Study of Cardiac Progenitor Cell Therapy in Patients With Single Ventricle Physiology Completed NCT01273857 Phase 1
19 Safety, Pharmacokinetics and Hemodynamic Efficacy of Sildenafil in Single Ventricle Patients Completed NCT01169519 Phase 1 Sildenafil by injection;Sildenafil by injection;Sildenafil by injection;Sildenafil by injection
20 Liothyronine in Children With Single Ventricle Congenital Cardiac Malformations Undergoing the Fontan Procedure Completed NCT00004828 Phase 1 liothyronine I 131
21 " 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
22 Biomarkers for Feeding Intolerance in Infants With Complex Congenital Heart Defects Undergoing Single Ventricle Staged Palliation Unknown status NCT02995577
23 Multimodality Biomarkers for Noninvasive Assessment of the Fontan Patient Unknown status NCT03263312
24 Long-term Survival After Single-ventricle Palliation: A Swedish Nationwide Cohort Study Unknown status NCT03356574
25 Assessment of the Outcomes of Children With Congenital Single Ventricle Heart Completed NCT00266968
26 Parental Experience in the Single Ventricle Interstage Utilizing a mHealth Innovation: A Comparative Case Study Completed NCT03231020
27 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
28 Training in Exercise Activities and Motion for Growth (A Study Conducted by the Pediatric Heart Network) Completed NCT01917084
29 A Pilot Study of Inspiratory Muscle Function and the Effects of Inspiratory Muscle Training in Subjects With Fontan Physiology Completed NCT03339466
30 Cardiac Biomarkers in Patients With Single Ventricle Physiology Completed NCT03678909
31 Impact of Early Intervention on Maternal Stress in Mothers of Fetuses Diagnosed With Single Ventricle Physiology Requiring Neonatal Surgery Completed NCT02462434
32 An Investigation Into The Potential Roles Of Vasoactive Intestinal Peptide And Substance P In The Pathophysiology Of Plastic Bronchitis And Protein Losing Enteropathy In Children With Palliated Single Ventricle Physiology Completed NCT01563757
33 The Impact of Family Factors on Developmental and Psychosocial Outcomes in Children With Hypoplastic Left Heart Syndrome (HLHS) at 6 Years of Age - Pediatric Heart Network Single Ventricle Reconstruction Extension (SVR II) Ancillary Study Completed NCT01582529
34 Identification of Biomarkers for Heart Failure in Children With Single Ventricle Physiology Completed NCT00571233
35 Heart Failure and Fibrosis in Adult Congenital Heart Patients With a Single Ventricle and Prior Fontan Operation. Completed NCT01929174
36 Vitamin C May Improve Endothelial Function and Exercise Capacity in Functional Single Ventricle Patients After Fontan Palliation Completed NCT00974025
37 Endothelin Blockade in Patients With Single Ventricle Physiology Completed NCT00989911 Bosentan
38 Home Surveillance and Feeding Strategies in Infants With Complex Single Ventricle Completed NCT00257517
39 Factors Associated With Normal Exercise Capacity in Patients With Single Ventricle: the Multicenter Cross-sectional Study Completed NCT04026542
40 Influence of Genetic Polymorphisms on Ventricular Structure and Function in Patients With Single Ventricle Anatomy Completed NCT00165984
41 Nutritional Failure in Infants With Single Ventricle Congenital Heart Disease Completed NCT01821287
42 The Examination of Outcome in Children With Single Ventricle Physiology Completed NCT00308217
43 Impact of Spironolactone on Endothelial Function in Patients With Single Ventricle Heart Completed NCT00211081 Spironolactone (drug)
44 To Assess the Initial Safety and Performance of COR-VG-001 Conduit in Pediatric Patients Undergoing Extracardiac Total Cavopulmonary Connection (EC-TCPC) Completed NCT02377674
45 Prospective Randomized Controlled Clinical Trial Comparing a Restrictive Versus Liberal Transfusion Strategy in Neonates and Infants Undergoing Surgery for Congenital Heart Disease Completed NCT01484886
46 RE-ENERGIZE FONTAN: A RandomizEd Exercise INtERvention DesiGned to MaximIZE Fitness in Pediatric FONTAN Patients Recruiting NCT04195451
47 ET1 Concentration, Metabolic Pathway Activation, and Pulmonary Blood Flow in Infants Undergoing Superior Cavo-Pulmonary Anastomosis Recruiting NCT03404258
48 The Effect of Fontan Fenestration Closure on Hepatic Stiffness Recruiting NCT03481985
49 Parents Post-traumatic Stress Before and After Their Infant's Second Palliative Heart Surgery: The PaTHS Descriptive Correlational Longitudinal Study Recruiting NCT04785482
50 Cross-sectional Analysis of Non-invasive Measures of Ventricular Systolic and Diastolic Function and Biomarkers in the Fontan Circulation Recruiting NCT03119090

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

Genetic Tests for Single Ventricular Heart

Anatomical Context for Single Ventricular Heart

MalaCards organs/tissues related to Single Ventricular Heart:

40
Heart, Endothelial, Brain, Liver, Kidney, Placenta, Bone Marrow

Publications for Single Ventricular Heart

Articles related to Single Ventricular Heart:

(show top 50) (show all 3425)
# Title Authors PMID Year
1
Pulmonary artery banding in patients with functional single ventricle associated with pulmonary hypertension. 61
33541150 2021
2
COVID-19 in Adults With Congenital Heart Disease. 61
33795039 2021
3
Standardized Feeding Approach Mitigates Weight Loss in Infants with Congenital Heart Disease. 61
33359473 2021
4
Increasing Pump Speed During Exercise Training Improves Exercise Capacity in Children with Ventricular Assist Devices. 61
32701623 2021
5
Recurrent Pulmonary Artery Interventions Following the Norwood Procedure Are Not Associated With Conduit Type. 61
32512161 2021
6
Outcomes of systemic-to-pulmonary artery shunt for single ventricular heart with extracardiac total anomalous pulmonary venous connection. 61
32886275 2021
7
Perforation of an Aortopulmonary Collateral: A Scary Sight in the Lab! 61
33794479 2021
8
Outcomes of single ventricle palliation in infants with heterotaxy syndrome. 61
33783481 2021
9
Post-operative arrhythmias in patients with hypoplastic left heart syndrome and anatomic variants: incidence, type, and course. 61
33685535 2021
10
CT and MRI for Repaired Complex Adult Congenital Heart Diseases. 61
33289363 2021
11
[Absence of arterial duct in fetus: an autopsy analysis]. 61
33677884 2021
12
Poor Weight Recovery Between Stage 1 Palliation and Hospital Discharge for Infants with Single Ventricle Physiology: An Analysis of the NPC-QIC Phase II Dataset. 61
33774055 2021
13
Dilatable Pulmonary Artery Banding Palliation in Congenital Heart Disease. 61
33684011 2021
14
Identification of patient variables that are associated with ventricular end-diastolic pressure before the bidirectional Glenn operation. 61
33686934 2021
15
Peripheral Pulmonary Artery Stenosis is Not a Contraindication to Single Ventricle Pathway. 61
33811885 2021
16
Poor Late Outcomes After Tricuspid Valve Repair in a Single Ventricle: Experience of 103 Patients. 61
32634383 2021
17
Myocardial deformation in patients with a single left ventricle using 2D cardiovascular magnetic resonance feature tracking: a case-control study. 61
33788063 2021
18
Single systemic right ventricle longitudinal strain: Intravendor reproducibility and intervendor agreement in children. 61
33523543 2021
19
Development of a biventricular conversion program: A new paradigm. 61
33783014 2021
20
Hybrid Subxiphoid Perventricular Approach as an Alternative Access in Neonates and Small Children Undergoing Complex Congenital Heart Interventions. 61
33263794 2021
21
Lymphatic pathway evaluation in congenital heart disease using 3D whole-heart balanced steady state free precession and T2-weighted cardiovascular magnetic resonance. 61
33641664 2021
22
Proteomic profiling identifies key differences between inter-stage infants with single ventricle heart disease and healthy controls. 61
33045409 2021
23
Delivery room oxygen physiology and respiratory interventions for newborns with cyanotic congenital heart disease. 61
33758390 2021
24
The choice of palliative arterial switch operation as an alternative for selected cases in a single center: Experience and mid term results. 61
33694295 2021
25
Prognostic Value of Liver and Spleen Stiffness in Patients with Fontan Associated Liver Disease (FALD): A Case Series with Histopathologic Comparison. 61
33809668 2021
26
Increases in oxygen saturation following discharge from Fontan palliation - an indicator of resolution of pulmonary arteriovenous malformations? 61
33691814 2021
27
Impact of Treatment Strategy on Outcomes in Isolated Pulmonary Artery of Ductal Origin. 61
33394118 2021
28
Surgical Timing and Outcomes of Unilateral Versus Bilateral Superior Cavopulmonary Anastomosis: An Analysis of Pediatric Heart Network Public Databases. 61
33416920 2021
29
Which criteria should be used to select patients for the Fontan operation? 61
33415742 2021
30
Incidence, Predictors, and Impact of Post-operative Acute Kidney Injury following Fontan Conversion Surgery in Young Adult Fontan Survivors. 61
33691191 2021
31
Critical Ebstein's anomaly with circular shunt: from successful fetal therapy with non-steroidal anti-inflammatory drugs to biventricular repair using Da Silva cone technique. 61
33682656 2021
32
The Tricuspid Valve in Single-Ventricle Patients: Forgotten, Unforgivable, and Indispensable. 61
32687825 2021
33
Hemodynamic Response to Device Titration in the Shunted Single Ventricle Circulation: A Patient Cohort Modeling Study. 61
33788799 2021
34
The direct route, alternative paths, and informing the future directions of single ventricle palliation. 61
33781735 2021
35
Performance of epimyocardial leads in patients with a single ventricle circulation. 61
33687754 2021
36
Acquired and progressive coronary arterial fistulae in patients with single-ventricle physiology and treated with pulmonary vasodilators. 61
33745463 2021
37
Cardiac magnetic resonance imaging-derived cardiac index is associated with adverse outcomes in patients with single-ventricle Fontan circulation: a preliminary study. 61
33687862 2021
38
Percutaneous transcatheter occlusion of left atrioventricular valve in a patient with single ventricle and severe atrioventricular valve regurgitation. 61
33022104 2021
39
A 40-Year-Old Man With Tricuspid Atresia, Status Post-Fontan, With Severe COVID-19 Pneumonia and Pneumothorax. 61
33558861 2021
40
Successful 2-Stage Pacemaker Implantation for Atrioventricular Block in a Low-Birth-Weight Infant with Congenital Heart Disease: Case Report. 61
33635254 2021
41
Unique foetal diagnosis of aorto-pulmonary collaterals in right atrial isomerism. 61
33239120 2021
42
Adult Congenital Heart Disease Survivors at Age 50 Years: Medical and Psychosocial Status. 61
32713766 2021
43
Mental Health Disorders in Children With Congenital Heart Disease. 61
33397689 2021
44
Genetic Association Between Hypoplastic Left Heart Syndrome and Cardiomyopathies. 61
33325730 2021
45
Hyperlactataemia as a predictor of adverse outcomes post-cardiac surgery in neonates with congenital heart disease. 61
33557993 2021
46
Trajectories in Neurodevelopmental, Health-Related Quality of Life, and Functional Status Outcomes by Socioeconomic Status and Maternal Education in Children with Single Ventricle Heart Disease. 61
33031800 2021
47
Reduced hippocampal volumes and memory deficits in adolescents with single ventricle heart disease. 61
33410605 2021
48
Defining a new normal: A qualitative exploration of the parent experience during the single ventricle congenital heart disease interstage period. 61
33591609 2021
49
Optimism despite profound uncertainty: school and social relationships in adolescents with single ventricle heart disease. 61
33176897 2021
50
Effect of Atrioventricular Valve Repair on Multistage Palliation Results of Single-Ventricle Defects. 61
32454017 2021

Variations for Single Ventricular Heart

ClinVar genetic disease variations for Single Ventricular Heart:

6
# Gene Name Type Significance ClinVarId dbSNP ID Position
1 FOXP1 NM_032682.6(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

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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