MCID: HYP055
MIFTS: 62

Hypoplastic Left Heart Syndrome malady

Categories: Genetic diseases, Rare diseases, Cardiovascular diseases, Fetal diseases

Aliases & Classifications for Hypoplastic Left Heart Syndrome

About this section
Sources:
32LifeMap Discovery®, 10Disease Ontology, 45NIH Rare Diseases, 22GeneTests, 12DISEASES, 51Orphanet, 36MeSH, 65UMLS, 24GTR, 27ICD10, 29ICD9CM, 42NCIt, 59SNOMED-CT, 28ICD10 via Orphanet, 37MESH via Orphanet, 66UMLS via Orphanet
See all MalaCards sources

Aliases & Descriptions for Hypoplastic Left Heart Syndrome:

Name: Hypoplastic Left Heart Syndrome 32 10 45 22 12 51 36 65
Hlhs 45 51
 
Hypoplastic Left Heart 24

Characteristics:

Orphanet epidemiological data:

51
hypoplastic left heart syndrome:
Prevalence: 1-5/10000 (Europe),1-5/10000 (Austria),1-5/10000 (Belgium),1-5/10000 (Croatia),1-5/10000 (Denmark),1-5/10000 (France),1-5/10000 (Germany),1-5/10000 (Hungary),1-5/10000 (Ireland),1-9/100000 (Italy),1-5/10000 (Malta),1-9/100000 (Netherlands),1-5/10000 (Norway),1-9/100000 (Poland),1-9/100000 (Portugal),1-5/10000 (Switzerland),1-5/10000 (United Kingdom),1-5/10000 (Ukraine),1-9/100000 (Spain); Age of onset: Antenatal,Neonatal

Classifications:



External Ids:

Disease Ontology10 DOID:9955
ICD1027 Q23.4
ICD9CM29 746.7
MeSH36 D018636
NCIt42 C98894
SNOMED-CT59 62067003
Orphanet51 2248
ICD10 via Orphanet28 Q23.4
MESH via Orphanet37 D018636
UMLS via Orphanet66 C0152101
UMLS65 C0152101

Summaries for Hypoplastic Left Heart Syndrome

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NIH Rare Diseases:45 Hypoplastic left heart syndrome (hlhs) is a problem with the heart’s structure that is present at birth (congenital). it occurs when parts of the left side of the heart (mitral valve, left ventricle, aortic valve, and aorta) do not develop completely. the underdeveloped left side of the heart is unable to provide enough blood flow to the body, which decreases the oxygen-rich blood supply. babies with hlhs might look normal at birth, but will develop symptoms of hlhs within a few days. these symptoms might include: poor feeding, problems breathing, pounding heart, weak pulse, and ashen or bluish skin color. the cause of hlhs is presently unknown. last updated: 7/21/2011

MalaCards based summary: Hypoplastic Left Heart Syndrome, also known as hlhs, is related to congenital heart disease and tricuspid valve insufficiency, and has symptoms including hypoplastic left heart, abnormality of the aorta and atria septal defect. An important gene associated with Hypoplastic Left Heart Syndrome is GJA1 (Gap Junction Protein Alpha 1), and among its related pathways are Cell adhesion_Endothelial cell contacts by junctional mechanisms and N-cadherin signaling events. The drug alprostadil has been mentioned in the context of this disorder. Affiliated tissues include heart, skin and lung, and related mouse phenotypes are respiratory system and embryo.

Disease Ontology:10 A congenital heart disease characterized by abnormal development of the left-sided structures of the heart.

Wikipedia:68 Hypoplastic left heart syndrome (HLHS) is a rare congenital heart defect in which the left heart is... more...

Related Diseases for Hypoplastic Left Heart Syndrome

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Diseases in the Hypoplastic Left Heart Syndrome family:

Hypoplastic Left Heart Syndrome 2 Hypoplastic Left Heart Syndrome 1

Diseases related to Hypoplastic Left Heart Syndrome via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50)    (show all 106)
idRelated DiseaseScoreTop Affiliating Genes
1congenital heart disease29.9NKX2-5, NKX2-6, TNNI3
2tricuspid valve insufficiency29.7GJA1, NKX2-6, NOTCH1
3hypoplastic left heart syndrome 212.5
4hypoplastic left heart syndrome 112.4
5hemophagocytic lymphohistiocytosis11.7
6left ventricular outflow tract obstruction11.5
7fbln1-related developmental delay-central nervous system anomaly-syndactyly syndrome10.7NOTCH1, SMAD6
8hypotrichosis10.6GJA1, NOTCH1
9mucopolysaccharidosis iva10.6FOXC2, FOXL1
10brachydactyly10.5FOXC2, FOXL1
11mental retardation, autosomal dominant 810.5EHMT1, NOTCH1
12cerebritis10.3
13aneurysm10.2
14pompholyx10.2NPPA, NPPB
15leptomeningeal amyloidosis10.2NPPA, NPPB
16septal myocardial infarction10.2NPPA, NPPB
17right aortic arch10.2
18aortic valve disease 110.2
19heart disease10.2
20cor triatriatum10.2
21transposition of the great arteries10.2
22interatrial communication10.2
23coxa vara, congenital10.1NPPA, NPPB
24syngap1-related non-syndromic intellectual disability10.1NPPA, NPPB
25facial nerve disease10.1NPPA, NPPB
26endomyocardial fibrosis10.1NPPB, TAZ
27diabetic autonomic neuropathy10.1NPPA, NPPB
28idiopathic hypersomnia10.1NPPA, NPPB
29lymphoma10.0
30ventricular septal defect 310.0
31aortic valve disease 210.0
32hypoplastic right heart syndrome10.0
33holt-oram syndrome10.0
34omphalocele10.0
35microcephaly10.0
36endocardial fibroelastosis10.0
37pulmonary valve stenosis10.0
38pagod syndrome10.0
39pentalogy of cantrell10.0
40gerbode defect10.0GATA5, NKX2-5, NKX2-6
41vitelliform macular dystrophy10.0GJA1, NKX2-5, NKX2-6
42griscelli syndrome, type 210.0
43griscelli syndrome10.0
44oral mucosa leukoplakia10.0NPPA, NPPB
45hemolytic disease of the newborn with kell alloimmunization10.0NKX2-5, NKX2-6
463mc syndrome9.9GATA5, NPPA, NPPB
47surfactant metabolism dysfunction, pulmonary, 29.9GATA5, NKX2-5, NKX2-6
48endogenous depression9.9NPPA, NPPB
49adult acute lymphocytic leukemia9.9NPPA, NPPB, TNNI3
50hemophagocytic lymphohistiocytosis, familial, 19.9

Graphical network of the top 20 diseases related to Hypoplastic Left Heart Syndrome:



Diseases related to hypoplastic left heart syndrome

Symptoms for Hypoplastic Left Heart Syndrome

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Symptoms:

 51 (show all 8)
  • hypoplastic left heart/ventricle
  • stillbirth/neonatal death
  • hypoplastic aorta/coarctation/stenosis/anomaly/aortic arch interruption
  • mitral valve prolapse/incompetence/insufficiency/regurgitation/ring anomaly
  • atrial septal defect/interauricular communication
  • patent ductus arteriosus
  • total/partial trisomy/duplication
  • maternal diabetes

HPO human phenotypes related to Hypoplastic Left Heart Syndrome:

(show all 7)
id Description Frequency HPO Source Accession
1 hypoplastic left heart hallmark (90%) HP:0004383
2 abnormality of the aorta typical (50%) HP:0001679
3 atria septal defect occasional (7.5%) HP:0001631
4 abnormality of the mitral valve occasional (7.5%) HP:0001633
5 patent ductus arteriosus occasional (7.5%) HP:0001643
6 abnormality of chromosome segregation occasional (7.5%) HP:0002916
7 maternal diabetes occasional (7.5%) HP:0009800

Drugs & Therapeutics for Hypoplastic Left Heart Syndrome

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Drugs for Hypoplastic Left Heart Syndrome (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 44)
idNameStatusPhaseClinical TrialsCas NumberPubChem Id
1
Bosentanapproved, investigationalPhase 2100147536-97-8104865
Synonyms:
-5-(o-methoxyphenoxy)-2-(2-pyrimidinyl)-4-pyrimidinyl]benzenesulfonamide
147536-97-8
174227-18-0
4-(1,1-Dimethylethyl)-N-(6-(2-hydroxyethoxy)-5-(2-methoxyphenoxy)-(2,2'-bipyrimidin)-4-yl) benzenesulfornamide
4-(1,1-Dimethylethyl)-N-(6-(2-hydroxyethoxy)-5-(2-methoxyphenoxy)-(2,2'-bipyrimidin)-4-yl)benzenesulfornamide
4-t-butyl-N-(6-(2-hydroxyethoxy)-5-(2-methoxyphenoxy)-2,2'-bipyrimidin-4-yl)benzenesulfonamide
4-tert-butyl-N-[6-(2-hydroxyethoxy)-5-(2-methoxyphenoxy)-2,2'-bipyrimidin-4-yl]benzenesulfonamide
4-tert-butyl-N-[6-(2-hydroxyethoxy)-5-(2-methoxyphenoxy)-2-pyrimidin-2-ylpyrimidin-4-yl]benzenesulfonamide
AC-148
AC1L2XN2
Actelion
Bosentan
Bosentan (INN)
Bosentan [USAN:INN:BAN]
Bosentan hydrate
Bosentanum
C27H29N5O6S
CHEBI:51450
CHEMBL957
CID104865
D07538
 
DB00559
EN002863
HMS2090N14
I06-1873
KS-5062
L001086
LS-31532
MolPort-003-845-300
NCGC00167440-01
P-Tert-butyl-N-(6-(2-hydroxyethoxy)-5-(O-methoxyphenoxy)-2-(2-pyrimidinyl)-4-pyrimidinyl)benzenesulfonamide
PDSP1_001731
PDSP2_001714
Ro 47-0203
Ro 47-0203/039
Ro-47-0203
Ro-47-0203/029
Ro-47-0203/039
TL8001039
Tracleer
UNII-XUL93R30K2
bosentanum
p-tert-Butyl-N-(6-(2-hydroxyethoxy)-5-(o-methoxyphenoxy)-2-(2-pyrimidinyl)-4-pyrimidinyl)benzenesulfonamide
p-tert-Butyl-N-[6-(2-hydroxyethoxy)
2
Epoetin alfaapprovedPhase 1, Phase 2626113427-24-0
Synonyms:
Epogen
 
Erythropoietin precursor
Procrit
3
PhenoxybenzamineapprovedPhase 2459-96-14768
Synonyms:
102737-84-8
2-(N-Benzyl-2-chloroethylamino)-1-phenoxypropane
4-12-00-02204 (Beilstein Handbook Reference)
59-96-1
63-92-3 (hydrochloride)
688A
A 688
AB00053702
AC-13214
AC1L1IWT
BPBio1_001000
BRN 2129697
BSPBio_000908
BSPBio_001278
BSPBio_002356
Bensylyt
Bensylyt NEN
Bensylyte
Benzyl(2-chloroethyl)-(1-methyl-2-phenoxyethyl)amine
Benzylyt
Bio2_000479
Bio2_000959
C07435
CCRIS 505
CHEBI:141434
CHEMBL753
CID4768
D08358
DB00925
Dibenylene
Dibenylin
Dibenyline
Dibenziran
Dibenzylene
Dibenzylin
Dibenzyline
Dibenzyran
DivK1c_000800
EINECS 200-446-8
Fenossibenzamina
Fenossibenzamina [DCIT]
Fenossibenzamina [Dcit]
Fenoxibenzamina
Fenoxibenzamina [INN-Spanish]
HMS1362P19
HMS1792P19
HMS1990P19
HMS2089J09
HSDB 4005
IDI1_000800
IDI1_002234
 
KBio1_000800
KBio2_000618
KBio2_000858
KBio2_003186
KBio2_003426
KBio2_005754
KBio2_005994
KBio3_001095
KBio3_001096
KBioGR_000618
KBioGR_001158
KBioSS_000618
KBioSS_000858
L001197
LS-43286
Lopac0_000235
MolPort-001-785-595
N-(2-Chloroethyl)-N-(1-methyl-2-phenoxyethyl)benzenemethanamine
N-(2-Chloroethyl)-N-(1-methyl-2-phenoxyethyl)benzylamine
N-(2-chloroethyl)-N-(phenylmethyl)-1-(phenyloxy)propan-2-amine
N-Phenoxyisopropyl-N-benzyl-beta-chloroethylamine
N-benzyl-N-(2-chloroethyl)-1-phenoxypropan-2-amine
NCGC00015121-11
NCGC00089748-03
NCGC00089748-04
NCGC00089748-05
NCGC00089748-07
NINDS_000800
NSC 37448
POB
POB HCl
Phenoxybenzamine
Phenoxybenzamine (INN)
Phenoxybenzamine Hcl
Phenoxybenzamine Hydrochloride
Phenoxybenzamine [INN:BAN]
Phenoxybenzaminum
Phenoxybenzaminum [INN-Latin]
Prestwick0_000944
Prestwick1_000944
Prestwick2_000944
Prestwick3_000944
SID104171113
SID124879342
SID124879343
SPBio_001829
SPBio_003067
Spectrum2_001815
Spectrum4_000769
Spectrum5_001370
Spectrum_000378
UNII-0TTZ664R7Z
phenoxybenzamine
4Endothelin Receptor AntagonistsPhase 2189
5Adrenergic AgentsPhase 2, Phase 14204
6Neurotransmitter AgentsPhase 2, Phase 114795
7Vasodilator AgentsPhase 2, Phase 12926
8Phosphodiesterase 5 InhibitorsPhase 2, Phase 1513
9Phosphodiesterase InhibitorsPhase 2, Phase 11062
10Sildenafil CitratePhase 2, Phase 1304171599-83-0
11Antihypertensive AgentsPhase 23618
12AnestheticsPhase 27385
13HematinicsPhase 1, Phase 21121
14Adrenergic alpha-AntagonistsPhase 2586
15Adrenergic AntagonistsPhase 21253
16
Citric AcidnutraceuticalPhase 2, Phase 1100177-92-9311
Synonyms:
2-Hydroxy-1,2,3-propanetricarboxylate
2-Hydroxy-1,2,3-propanetricarboxylic acid
2-Hydroxytricarballylic acid
3-Carboxy-3-hydroxypentane-1,5-dioate
3-Carboxy-3-hydroxypentane-1,5-dioic acid
Aciletten
Anhydrous citrate
Anhydrous citric acid
Chemfill
Citraclean
 
Citrate
Citretten
Citric acid
Citro
Citronensäure
E 330
Hydrocerol A
Kyselina citronova
Suby G
Uro-trainer
beta-Hydroxytricarballylate
beta-Hydroxytricarballylic acid
17CitrateNutraceuticalPhase 2, Phase 11001
18
DexmedetomidineapprovedPhase 1470113775-47-668602, 5311068, 56032
Synonyms:
(+)-4-((S)-alpha,2,3-Trimethylbenzyl)imidazole
(+)-4-((S)-alpha,2,3-trimethylbenzyl)imidazole
(+)-medetomidine
(S)-medetomidine
113775-47-6
4-[(1S)-1-(2,3-Dimethylphenyl)ethyl]-1H-imidazole
4-[(1S)-1-(2,3-dimethylphenyl)ethyl]-1H-imidazole
5-[(1S)-1-(2,3-dimethylphenyl)ethyl]-1H-imidazole
AC1NSJXT
C07450
CHEBI:4466
CHEMBL778
CID5311068
D00514
DEXMEDETOMIDINE
Dexdor (T)
 
Dexmedetomidin
Dexmedetomidina
Dexmedetomidine
Dexmedetomidine (USAN/INN)
Dexmedetomidine Hydrochloride
Dexmedetomidinum
Dexmédétomidine
MPV 1440
MPV-1440
Medetomidina [Spanish]
Medetomidine
Medetomidinum [Latin]
NCGC00025347-01
PRECEDEX
Precedex
Precedex (TN)
Tocris-2023
ZINC04632106
19Central Nervous System DepressantsPhase 110016
20Hypnotics and SedativesPhase 12032
21Peripheral Nervous System AgentsPhase 118510
22Analgesics, Non-NarcoticPhase 15184
23AnalgesicsPhase 19358
24Adrenergic alpha-2 Receptor AgonistsPhase 1790
25Adrenergic AgonistsPhase 12331
26Adrenergic alpha-AgonistsPhase 11300
27
Bupropionapproved30834841-39-9444
Synonyms:
( -)-2-(tert-Butylamino)-3'-chloropropiophenone
( -)-2-(tert-Butylamino)-3'-chlorpropiophenon
(+-)-1-(3-chlorophenyl)-2-((1,1-dimethylethyl)amino)-1-propanone
(+-)-Bupropion
.alpha.-(tert-Butylamino)-m-chloropropiophenone
1-(3-chlorophenyl)-2-[(1,1-dimethylethyl)amino]propan-1-one
2-(Tert-Butylamino)-3'-chloropropiophenone
2-(tert-butylamino)-1-(3-chlorophenyl)propan-1-one
34841-36-6 (hydrochloride)
34841-39-9
34911-55-2
AB00053756
AC-197
AC1L198Y
AMFEBUTAMONE HCl
Amfebutamon
Amfebutamona
Amfebutamona [INN-Spanish]
Amfebutamone
Amfebutamonum
Amfebutamonum [INN-Latin]
Aplenzin
BPBio1_000042
BRD-A05186015-003-05-7
BRN 2101062
BSPBio_000038
BSPBio_002247
Budeprion
Buproban
Bupropion
Bupropion (INN)
Bupropion (Old RN)
Bupropion (USAN)
Bupropion Hcl
Bupropion Hydrochloride
Bupropion SR
Bupropion [INN:BAN]
Bupropion hydrocloride
C06860
CHEBI:3219
CHEMBL894
CID444
CPD000472526
D07591
DB01156
 
DivK1c_007050
Elont
HMS2051G10
HMS2089G14
Jsp006301
KBio1_001994
KBio2_002143
KBio2_004711
KBio2_007279
KBio3_001467
KBioGR_001168
KBioSS_002143
L000725
LS-122817
Lopac0_000166
MLS001424015
MolPort-003-845-432
NCGC00015122-06
NCGC00089751-02
NCI60_002714
NSC315851
Prestwick0_000249
Prestwick1_000249
Prestwick2_000249
Prestwick3_000249
SAM001246699
SMR000472526
SPBio_001817
SPBio_002257
SpecPlus_000954
Spectrum2_001659
Spectrum3_000644
Spectrum4_000614
Spectrum5_001406
Spectrum_001663
TL8002604
UNII-01ZG3TPX31
Wellbatrin
Wellbutrin
Wellbutrin SR
Wellbutrin XL
Wellbutrin Xl
Zyban
alpha-(tert-butylamino)-m-chloropropiophenone
amfebutamonum
bupropion
28
Dopamineapproved308462-31-7, 51-61-6681
Synonyms:
(3H)-Dopamine
.Beta.-(3,4-Dihydroxyphenyl)ethylamine hydrochloride
.alpha.-(3,4-Dihydroxyphenyl)-.beta.-aminoethane
1,2-Benzenediol, 4-(2-aminoethyl)- (9CI)
1,2-Benzenediol, 4-(2-aminoethyl)-, hydrochloride
1,2-Benzenediol, 4-(2-aminoethyl)-, labeled with tritium
153C5321-5FEE-4B0B-8925-F388F0EEEBD1
2-(3,4-Dihydroxyphenyl)ethylamine
2-(3,4-dihydroxyphenyl)ethylamine
2-benzenediol
3,4-Dihydroxyphenethylamine
3,4-Dihydroxyphenethylamine hydrochloride
3,4-Dihydroxyphenylethylamine
3,4-dihydroxyphenethylamine
3-Hydroxtyramine
3-Hydroxytyramine
3-Hydroxytyramine Hydrobromide
3-Hydroxytyramine hydrochloride
4-(2-Aminoethyl)-1,
4-(2-Aminoethyl)-1,2-benzenediol
4-(2-Aminoethyl)-1,2-bezenediol
4-(2-Aminoethyl)-Pyrocatechol
4-(2-Aminoethyl)benzene-1,2-diol
4-(2-Aminoethyl)catechol
4-(2-Aminoethyl)pyrocatechol
4-(2-Aminoethyl)pyrocatechol hydrochloride
4-(2-aminoethyl)-pyrocatechol
50444-17-2
51-61-6
62-31-7 (HYDROCHLORIDE)
AC1L19S5
AC1Q54AX
AC1Q54AY
AKOS003790978
ASL 279
BIDD:ER0506
BPBio1_001123
BSPBio_001932
Biomol-NT_000001
C03758
CHEBI:18243
CHEMBL59
CID681
D07870
DB00988
Deoxyepinephrine
DivK1c_000780
Dopamin
Dopamina
Dopamina [INN-Spanish]
Dopamine
Dopamine (INN)
Dopamine (USAN)(*hydrochloride*)
Dopamine Hcl
Dopamine Hydrochloride
Dopamine [INN:BAN]
Dopaminum
Dopaminum [INN-Latin]
Dopastat
Dophamine
Dynatra
EINECS 200-110-0
HSDB 3068
Hydroxytyramin
Hydroxytyramine
IDI1_000780
IP 498
Intropin
Intropin [*hydrochloride*]
 
KBio1_000780
KBio2_001492
KBio2_002388
KBio2_002484
KBio2_004060
KBio2_004956
KBio2_005052
KBio2_006628
KBio2_007524
KBio2_007620
KBio3_001152
KBio3_002867
KBio3_002962
KBioGR_001129
KBioGR_002388
KBioGR_002484
KBioSS_001492
KBioSS_002393
KBioSS_002491
KW-3-060
L-DOPAMINE
L000232
LDP
LS-159
Lopac-H-8502
Lopac0_000586
Medopa (TN)
MolPort-001-641-000
NCGC00015519-01
NCGC00015519-08
NCGC00096050-01
NCGC00096050-02
NCGC00096050-03
NCGC00096050-04
NCGC00096050-05
NINDS_000780
NSC 173182
NSC169105
NSC173182
Oprea1_088821
Oxytyramine
Pyrocatechol, 4-(2-aminoethyl)- (8CI)
Pyrocatechol, 4-(2-aminoethyl)-, hydrochloride
Revimine
Revivan
SPBio_001205
SPECTRUM1505155
ST048774
STK301601
Spectrum2_001023
Spectrum3_000406
Spectrum4_000525
Spectrum5_000945
Spectrum_001012
UNII-VTD58H1Z2X
UPCMLD0ENAT5885989:001
a-(3,4-Dihydroxyphenyl)-b-aminoethane
alpha-(3,4-Dihydroxyphenyl)-beta-aminoethane
cMAP_000036
cMAP_000065
dopamine
hydroxytyramine
intropin
m-Hydroxytyramine hydrochloride
nchembio.105-comp9
nchembio.107-comp4
nchembio.284-comp1
nchembio.78-comp16
nchembio.89-comp3
nchembio705-8
nchembio801-comp8
29
Caffeineapproved, Nutraceutical33958-08-22519
Synonyms:
07E4FB58-FD79-4175-8E3D-05BF96954522
1,3,7-Trimethyl-2,6-dioxopurine
1,3,7-Trimethyl-3,7-dihydro-1H-purine-2,6-dione
1,3,7-Trimethylpurine-2,6-dione
1,3,7-Trimethylxanthine
1,3,7-trimethylpurine-2,6-dione
1,3,7-trimethylxanthine
1-3-7-TRIMETHYLXANTHINE
1-Methyl-Theobromine
1-methyltheobromine
137X
1gfz
1l5q
1l7x
27602_FLUKA
2a3b
3,7-Dihydro-1,3,7-trimethyl-1H-purin-2,6-dion
3,7-Dihydro-1,3,7-trimethyl-1H-purine-2,6-dione
3,7-dihydro-1,3,7-trimethyl-1H-purine
5-26-13-00558 (Beilstein)
58-08-2
7-Methyl Theophylline
7-Methyltheophylline
7-methyltheophylline
71701-02-5
75035_FLUKA
95789-13-2
A.S.A. and Codeine Compound
AC-12774
AC1L1DV2
AC1Q3Z23
ACon1_000085
AI3-20154
AKOS000121334
Alert
Alert-Pep
Alert-pep
Allertness Aid
Anacin Maximum Strength
Anhydrous caffeine
Anhydrous caffeine (JP15)
Anhydrous caffeine (TN)
Anoquan
Awake
BIDD:ER0554
BIDD:GT0632
BIDD:PXR0172
BIM-0050216.0001
BRD-K02404261-001-02-7
BRD-K02404261-001-03-5
BRN 0017705
BSPBio_001921
Berlin-Chemie Brand of Caffeine
Bio-0579
Bio1_000473
Bio1_000962
Bio1_001451
Bristol-Myers Squibb Brand of Caffeine
C 0750
C07481
C0750_SIAL
C1778_SIAL
C2042
C6035_FLUKA
C6035_SIGMA
C7731_SIAL
C8960_SIAL
CCRIS 1314
CFF
CHEBI:27732
CHEMBL113
CID2519
CU-01000012617-3
Cafamil
Cafcit
Cafecon
Cafeina
Cafergot
Cafeína
Caffedrine
Caffedrine Caplets
Caffein
Caffeina
Caffeina [Italian]
Caffeine
Caffeine (USP)
Caffeine (natural)
Caffeine Citrate
Caffeine Pure
Caffeine [BAN:JAN]
Caffeine citrate
Caffeine solution
Caffeine, Anhydrous
Caffeine, Monohydrate
Caffeine, Synthetic
Caffeine, anhydrous
Caffeine, synthetic
Caffine
Cafipel
Caféine
Circle K Nosnooze
Coffein
Coffein [German]
Coffeine
Coffeinum
Coffeinum N
Coffeinum Purrum
Compound 65
Conrx Alert
D002110
D00528
DB00201
DHC Plus
DHCplus
Darvon Compound
Darvon Compound-65
Dasin
Dexitac
Dexitac Stay Alert Stimulant
Dhc Plus
Diurex
Diurex Ultra
Diurex WaterCaps
DivK1c_000730
Drowz Away Maximum strength
Durvitan
EINECS 200-362-1
EU-0100228
Eldiatric C
Enerjets
Equaline Stay Awake
Ercatab
Esgic
Esgic-Plus
FEMA No. 2224
Femcet
Fioricet
Fiorinal
GlaxoSmithKline Brand of Caffeine
Good Neighbor Pharmacy Stay Awake
Good Sense Stay Awake
Guaranine
HMS1920I09
HMS2091O11
HMS502E12
HSDB 36
Health Mart Stay Awake
Healthy Accents Stay Awake
Hycomine
Hycomine Compound
I14-4386
IDI1_000730
 
Invagesic
Invagesic Forte
KBio1_000730
KBio2_001781
KBio2_004349
KBio2_006917
KBio3_001141
KBioGR_002325
KBioSS_001781
Keep Alert
Kofein
Kofein [Czech]
Koffein
Koffein [German]
L000155
LS-237
Lanorinal
Leader Alertness Aid
Lil Drug Store Nosnooze
Lopac-C-0750
Lopac0_000228
MEGxp0_001350
MLS001055341
MLS001056714
MLS001066409
Mateina
Mateína
Maximum Strength Caffeine
Maximum Strength Snapback Stimulant Powders
Maximum Strength Stay Awake
Medigesic Plus
Merck dura Brand of Caffeine
Methyltheobromide
Methyltheobromine
Methylxanthine theophylline
Midol Maximum Strength
Migergot
Miudol
MolMap_000054
MolPort-000-730-850
Monohydrate Caffeine
Monomethyl Derivative of Theophylline
Monomethyl derivative of Theophylline
NCGC00015208-01
NCGC00015208-02
NCGC00015208-04
NCGC00015208-14
NCGC00090699-01
NCGC00090699-02
NCGC00090699-03
NCGC00090699-04
NCGC00090699-05
NCGC00090699-06
NCGC00090699-07
NCGC00090699-08
NCGC00090699-09
NCGC00168808-01
NCGC00168808-02
NCI-C02733
NCIOpen2_008255
NINDS_000730
NSC 5036
NSC5036
Nano Ppc
Natural Caffeinum
Nix Nap
No Doz
No-Doz
Nodaca
Nodoz Maximum Strength Caplets
Norgesic
Norgesic Forte
Organex
Orphengesic
Orphengesic Forte
P-A-C Analgesic Tablets
PDSP1_001016
PDSP1_001235
PDSP2_001000
PDSP2_001219
Passauer Brand of Caffeine
Pep-Back
Pep-back Peak Performance
Pep-back Ultra
Percoffedrinol N
Percutafeine
Phensal
Pierre Fabre Brand of Caffeine
Probes1_000150
Probes2_000128
Propoxyphene
Propoxyphene Compound 65
Propoxyphene Compound-65
Quick Pep
Quick-Pep
Refresh'n
Republic Drug Brand of Caffeine
SDCCGMLS-0064595.P001
SDCCGMLS-0064595.P002
SK 65 Compound
SK-65 Compound
SMR000326667
SPBio_001222
SPECTRUM1500155
STK177283
Seid Brand of Caffeine
Smart Sense Stay Awake
Sohmed Alertness Aid
Spectrum2_001261
Spectrum3_000321
Spectrum4_001782
Spectrum5_000423
Spectrum_001301
Stay Alert Aid
Stay Awake
Stay Awake Maximum Strength
Stay Awake Maximum strength
Stim
Sunmark Stay Awake
Synalgos-Dc
TNP00310
Teina
Thein
Theine
Theobromine ME
Theobromine Me
Theophylline ME
Theophylline Me
Theophylline, 7-methyl
Thompson Brand 1 of Caffeine
Thompson Brand 2 of Caffeine
Tirend
Topcare Stay Awake maximum strength
Tri-Aqua
Triad
UNII-3G6A5W338E
Ultra Pep-Back
Vivarin
W222402_ALDRICH
WLN: T56 BN DN FNVNVJ B1 F1 H1
Wake-Up
Wigraine
Xanthine, 1,3,7-trimethyl
ZINC00001084
bmse000206
cafeine
caffeine
caffenium
component of Cafergot
nchembio.243-comp7
nchembio.63-comp5
nchembio774-comp2
teina
30Dopamine Uptake Inhibitors925
31Neurotransmitter Uptake Inhibitors2857
32Vitamins3857
33Psychotropic Drugs5501
34Dopamine Agents3084
35
36Cytochrome P-450 CYP2D6 Inhibitors602
37Cytochrome P-450 Enzyme Inhibitors3036
38Antidepressive Agents, Second-Generation1087
39Antidepressive Agents2367
40Antidepressive Agents, Tricyclic250
41Immunoglobulins4477
42Antibodies4477
43CalamusNutraceutical344
44Kavanutraceutical1329000-38-85281052

Interventional clinical trials:

(show all 48)
idNameStatusNCT IDPhase
1Prophylactic Peritoneal Dialysis Decreases Time to Achieve a Negative Fluid Balance After the Norwood ProcedureCompletedNCT01215240Phase 3
2Study of Triostat in Infants During Heart SurgeryCompletedNCT00027417Phase 3
3Positive Pressure Ventilation and Sternal Closure in HLHSRecruitingNCT02455713Phase 2, Phase 3
4Cardiac Stem/Progenitor Cell Infusion in Univentricular Physiology (APOLLON Trial)Not yet recruitingNCT02781922Phase 3
5Sildenafil After the Fontan OperationCompletedNCT00507819Phase 2
6Study of Placebo or Bosentan to Treat Patients With Single Ventricle Physiology.CompletedNCT01292551Phase 2
7Fetal Intervention for Aortic Stenosis and Evolving Hypoplastic Left Heart SyndromeRecruitingNCT01736956Phase 1, Phase 2
8Ambrisentan in Single VentricleRecruitingNCT02080637Phase 2
9Erythropoetin Neuroprotection for Neonatal Cardiac SurgeryActive, not recruitingNCT00513240Phase 1, Phase 2
10Cardiac Progenitor Cell Infusion to Treat Univentricular Heart Disease (PERSEUS)Active, not recruitingNCT01829750Phase 2
11Parenteral Phenoxybenzamine During Congenital Heart Disease SurgeryNot yet recruitingNCT00770705Phase 2
12Transcoronary Infusion of Cardiac Progenitor Cells in Patients With Single Ventricle PhysiologyCompletedNCT01273857Phase 1
13Understanding Dexmedetomidine in Neonates After Open Heart SurgeryCompletedNCT00576381Phase 1
14Understanding Dexmedetomidine In Infants Post-Operative From Cardiac SurgeryCompletedNCT00573066Phase 1
15Sildenafil in Single Ventricle PatientsCompletedNCT01169519Phase 1
16Safety Study of Autologous Umbilical Cord Blood Cells for Treatment of Hypoplastic Left Heart SyndromeRecruitingNCT01883076Phase 1
17Allogeneic hMSC Injection in Patients With Hypoplastic Left Heart SyndromeRecruitingNCT02398604Phase 1
18Safety and Feasibility Study of Umbilical Cord Blood Cells for Infants With Hypoplastic Left Heart SyndromeSuspendedNCT01445041Phase 1
19Comparison of Feeding Strategies for Hypoplastic Left Heart Syndrome InfantsCompletedNCT02657629
20Neurodevelopmental Outcomes in Hypoplastic Left Heart SyndromeCompletedNCT00399555
21Biomarker Study for Heart Failure in Children With Single Ventricle PhysiologyCompletedNCT00571233
22Single Ventricle OutcomeCompletedNCT00308217
23Cerebral Perfusion During Neonatal Cardiac SurgeryCompletedNCT01032876
24Bupropion & Cardio Birth Defect (Slone)CompletedNCT01597661
25NIRS in Neonatal Cardiac SurgeryCompletedNCT00166101
26Quality of Life Among Children With Congenital Heart DiseaseCompletedNCT01202916
27Prenatal Effects of Congenital Heart Disease (CHD) on Neurodevelopmental OutcomeCompletedNCT00713635
28A Prospective Study of Patients With Hypoplastic Left Heart Syndrome (HLHS) Following Stage II Surgical PalliationRecruitingNCT01708863
29Umbilical Cord Blood Collection and Processing for Cardiac Regeneration in Hypoplastic Left Heart Syndrome PatientsRecruitingNCT01856049
30SVRII Family Factors StudyRecruitingNCT01582529
31Genetic Determinants of Congenital Heart Disease OutcomesRecruitingNCT01656941
32Examining Developmental Changes in Heart Contractions of Children With Congenital Heart DefectsRecruitingNCT00860327
33Global and Regional Myocardial Strain and Power Output In Patients With Single Ventricles Using Novel MRI TechniquesRecruitingNCT01107990
34Oxygen Consumption-based Assessments of Hemodynamics in Neonates Following Congenital Heart Surgery (Oxy-CAHN Study)RecruitingNCT02184169
35Fluid Balance in Children Undergoing Fontan SurgeryRecruitingNCT02306057
36Association Between a Mother's Exposure to "Strep Throat" and Her Baby's Developing Heart DiseaseRecruitingNCT01603732
37Family Adaptation Study Following the Diagnosis of Hypoplastic Left Heart Syndrome in a NewbornActive, not recruitingNCT00734643
38Near-infrared Spectroscopy (NIRS) Neurodevelopmental OutcomesActive, not recruitingNCT00464100
39Evaluation of Panel Reactive Antibody in Children Following Stage I Palliation for Hypoplastic Left Heart SyndromeActive, not recruitingNCT01135485
40Impact of Early Intervention on Maternal Stress in Mothers of Fetuses Diagnosed With Single Ventricle PhysiologyActive, not recruitingNCT02462434
41Cerebral Blood Flow in Single Ventricles Throughout Staged Surgical ReconstructionActive, not recruitingNCT02135081
42Long-term Outcomes of Children With Hypoplastic Left Heart Syndrome and the Impact of Norwood Shunt TypeEnrolling by invitationNCT02455531
43SVR III: Brain Connectome and Neurodevelopmental OutcomesEnrolling by invitationNCT02692443
44The Use of Optical Oximetry in Determining Gastrointestinal Complications After the Hybrid ProcedureEnrolling by invitationNCT02060825
45Diffuse Myocardial Fibrosis in Fontan PatientsEnrolling by invitationNCT01929174
46Changes in Cerebral Oxygenation During Laparoscopy in Patients With Single Ventricle AnatomyNot yet recruitingNCT02614664
47Aortic Arch ReconstructionTerminatedNCT00277940
48Sleep Disordered Breathing in Children With Single Ventricle PhysiologyWithdrawnNCT00156455

Search NIH Clinical Center for Hypoplastic Left Heart Syndrome

Inferred drug relations via UMLS65/NDF-RT43:

Cell-based therapeutics:


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


Cochrane evidence based reviews: hypoplastic left heart syndrome

Genetic Tests for Hypoplastic Left Heart Syndrome

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Genetic tests related to Hypoplastic Left Heart Syndrome:

id Genetic test Affiliating Genes
1 Hypoplastic Left Heart Syndrome22 GJA1

Anatomical Context for Hypoplastic Left Heart Syndrome

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MalaCards organs/tissues related to Hypoplastic Left Heart Syndrome:

33
Heart, Skin, Lung, Brain, Breast, Thyroid, Prostate

Animal Models for Hypoplastic Left Heart Syndrome or affiliated genes

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MGI Mouse Phenotypes related to Hypoplastic Left Heart Syndrome:

38
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053888.6FOXC2, GJA1, NKX2-5, NKX2-6, NOTCH1, TNNI3
2MP:00053808.4CDH2, EHMT1, FOXC2, GJA1, NKX2-5, NKX2-6
3MP:00053697.0CDH2, FOXC2, GATA5, GJA1, NKX2-5, NOTCH1
4MP:00053786.8CDH2, EHMT1, FOXC2, FOXL1, GJA1, NKX2-5
5MP:00053766.5CDH2, FOXC2, GATA5, GJA1, NKX2-5, NKX2-6
6MP:00053856.4CDH2, EHMT1, FOXC2, GATA5, GJA1, NKX2-5
7MP:00107685.9CDH2, EHMT1, FOXC2, FOXL1, GATA5, GJA1

Publications for Hypoplastic Left Heart Syndrome

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Articles related to Hypoplastic Left Heart Syndrome:

(show top 50)    (show all 595)
idTitleAuthorsYear
1
Intraoperative Transesophageal Echocardiography in the Norwood-Sano Palliation of Hypoplastic Left Heart Syndrome. (27101490)
2016
2
Assessing ST Segment Changes and Ischemia During Exercise Stress Testing in Patients with Hypoplastic Left Heart Syndrome and Fontan Palliation. (26725480)
2016
3
Hybrid stenting of restrictive atrial septum in an infant with hypoplastic left heart syndrome after hybrid stage 1 palliation. (25625457)
2015
4
Regenerative therapy for hypoplastic left heart syndrome: First report of intraoperative intramyocardial injection of autologous umbilical-cord blood-derived cells. (25466856)
2015
5
Self-expanding stent implantation in arterial duct during hybrid palliation of hypoplastic left heart syndrome: midterm experience with a specially designed stent. (25631369)
2015
6
Hybrid Procedure for Neonates With Hypoplastic Left Heart Syndrome at High-Risk for Norwood: Midterm Outcomes. (26433522)
2015
7
Distention of the Immature Left Ventricle Triggers Development of Endocardial Fibroelastosis: An Animal Model of Endocardial Fibroelastosis Introducing Morphopathological Features of Evolving Fetal Hypoplastic Left Heart Syndrome. (26064914)
2015
8
Technical and anatomical factors affecting the size of the branch pulmonary arteries following first-stage Norwood palliation for hypoplastic left heart syndrome. (25681509)
2015
9
Stage 1 hybrid palliation for hypoplastic left heart syndrome-assessment of contemporary patterns of use: An analysis of The Society of Thoracic Surgeons Congenital Heart Surgery Database. (25266878)
2014
10
Hypoplastic left heart syndrome with dextrocardia and situs solitus. (24668985)
2014
11
Tracheal compression following hybrid procedure in an infant with hypoplastic left heart syndrome. (25240547)
2014
12
Cardiac troponin I, cardiac troponin-specific autoantibodies and natriuretic peptides in children with hypoplastic left heart syndrome. (24101702)
2014
13
Psychosocial responses of parents to their infant's diagnosis of hypoplastic left heart syndrome. (25215886)
2014
14
Impaired cerebral perfusion after bilateral pulmonary arterial banding in patients with hypoplastic left heart syndrome. (23993895)
2013
15
Effects of pulmonary artery banding and retrograde aortic arch obstruction on the hybrid palliation of hypoplastic left heart syndrome. (23434295)
2013
16
Abnormal Abdominal Aorta Hemodynamics Are Associated With Necrotizing Enterocolitis in Infants With Hypoplastic Left Heart Syndrome. (24154504)
2013
17
Use of computational fluid dynamics to estimate hemodynamic effects of respiration on hypoplastic left heart syndrome surgery: total cavopulmonary connection treatments. (24385870)
2013
18
QRS duration changes in patients with hypoplastic left heart syndrome undergoing hybrid palliation: prehybrid to post-Fontan. (23305551)
2013
19
Mechanisms of tricuspid valve regurgitation in hypoplastic left heart syndrome: a case-matched echocardiographic-surgical comparison study. (22723622)
2013
20
Microcephaly is associated with early adverse neurologic outcomes in hypoplastic left heart syndrome. (23575878)
2013
21
Right ventricular mechanics in the fetus with hypoplastic left heart syndrome. (23473605)
2013
22
Fifteen-year single-center experience with the Norwood operation for complex lesions with single-ventricle physiology compared with hypoplastic left heart syndrome. (22244563)
2012
23
Does bilateral pulmonary banding in comparison to Norwood procedure improve outcome in neonates with hypoplastic left heart syndrome beyond second-stage palliation? A review of the current literature. (22215495)
2012
24
Heart for bonding: a new protocol of care for hypoplastic left heart syndrome. (22908051)
2012
25
Changing attitudes to the management of hypoplastic left heart syndrome: a European perspective. (22152542)
2011
26
Hypoplastic left heart syndrome with intact atrial septum sequelae of left atrial hypertension in utero. (21565633)
2011
27
Systemic RV in hypoplastic left heart syndrome after surgical palliation. (21679907)
2011
28
Letter by Giardini et al regarding article, "Maladaptive aortic properties in children after palliation of hypoplastic left heart syndrome assessed by cardiovascular magnetic resonance imaging". (21576677)
2011
29
Pathology of the aortic arch in hypoplastic left heart syndrome: surgical implications. (21104077)
2011
30
Unexpected infant death due to hypoplastic left heart syndrome: a case report. (21940189)
2011
31
Reducing the incidence of necrotizing enterocolitis in neonates with hypoplastic left heart syndrome with the introduction of an enteral feed protocol. (19838139)
2010
32
Hypoplastic left heart syndrome. (20237013)
2010
33
Impact of antenatal diagnosis of hypoplastic left heart syndrome on the clinical presentation and surgical outcomes: the Australian experience. (19210602)
2009
34
Exercise capacity of a contemporary cohort of children with hypoplastic left heart syndrome after staged palliation. (19643621)
2009
35
Is "treat your child normally" helpful advice for parents of survivors of treatment of hypoplastic left heart syndrome? (19272201)
2009
36
Right ventricular performance in the fetus with hypoplastic left heart syndrome. (19324154)
2009
37
Stage I palliation for hypoplastic left heart syndrome: Norwood versus Sano modification. (17284981)
2007
38
Hypoplastic left heart syndrome is heritable. (17936159)
2007
39
The nomenclature, definition and classification of hypoplastic left heart syndrome. (16839428)
2006
40
Rescue cardiac transplantation for failing staged palliation in patients with hypoplastic left heart syndrome. (17116269)
2006
41
Anesthetic management of tracheoesophageal fistula repair in a newborn with hypoplastic left heart syndrome. (16677272)
2006
42
The management of hypoplastic left heart syndrome with a right aortic arch. (16984705)
2006
43
Novel approach to the newborn with hypoplastic left heart syndrome and intact atrial septum. (16097012)
2005
44
Hypoplastic left heart syndrome and valvar pulmonary stenosis: presentation and management. (15565270)
2005
45
Cardiac magnetic resonance imaging after stage I Norwood operation for hypoplastic left heart syndrome. (16301358)
2005
46
Balloon dilation of severe aortic stenosis in the fetus: potential for prevention of hypoplastic left heart syndrome: candidate selection, technique, and results of successful intervention. (15466631)
2004
47
Restrictive interatrial communication in hypoplastic left heart syndrome after modified Fontan repair. (14667654)
2003
48
Cardiac homeobox gene NKX2-5 mutations and congenital heart disease: associations with atrial septal defect and hypoplastic left heart syndrome. (12798584)
2003
49
Hypoplastic left heart syndrome with right aortic arch in a newborn. (8518070)
1993
50

Variations for Hypoplastic Left Heart Syndrome

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Expression for genes affiliated with Hypoplastic Left Heart Syndrome

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Search GEO for disease gene expression data for Hypoplastic Left Heart Syndrome.

Pathways for genes affiliated with Hypoplastic Left Heart Syndrome

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GO Terms for genes affiliated with Hypoplastic Left Heart Syndrome

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Cellular components related to Hypoplastic Left Heart Syndrome according to GeneCards Suite gene sharing:

idNameGO IDScoreTop Affiliating Genes
1intercalated discGO:00147049.9CDH2, GJA1

Biological processes related to Hypoplastic Left Heart Syndrome according to GeneCards Suite gene sharing:

(show all 25)
idNameGO IDScoreTop Affiliating Genes
1atrioventricular node developmentGO:000316210.5NKX2-5, NOTCH1
2negative regulation of myotube differentiationGO:001083210.5NKX2-5, NOTCH1
3cardiac muscle cell proliferationGO:006003810.5NKX2-5, NOTCH1
4cardiac ventricle morphogenesisGO:000320810.5NKX2-5, NOTCH1
5cardiac muscle tissue morphogenesisGO:005500810.5NKX2-5, NOTCH1
6glial cell differentiationGO:001000110.4CDH2, NOTCH1
7neuronal stem cell population maintenanceGO:009715010.4CDH2, NOTCH1
8ventricular trabecula myocardium morphogenesisGO:000322210.4NKX2-5, NOTCH1
9receptor guanylyl cyclase signaling pathwayGO:000716810.2NPPA, NPPB
10cGMP biosynthetic processGO:000618210.2NPPA, NPPB
11cardiac muscle tissue developmentGO:004873810.2NKX2-5, TAZ
12heart loopingGO:000194710.2GJA1, NKX2-5, NOTCH1
13negative regulation of BMP signaling pathwayGO:003051410.1NOTCH1, SMAD6
14atrial cardiac muscle cell developmentGO:005501410.1NKX2-5, NKX2-6
15negative regulation of endothelial cell proliferationGO:000193710.0GJA1, XDH
16ureteric bud developmentGO:000165710.0FOXC2, SMAD6
17pharyngeal system developmentGO:00600379.8NKX2-5, NKX2-6
18cardiac muscle contractionGO:00600489.8TAZ, TNNI3
19positive regulation of transcription, DNA-templatedGO:00458939.6FOXC2, GATA5, NKX2-5, NOTCH1
20regulation of cardiac conductionGO:19037799.5NKX2-5, NPPA, TNNI3
21embryonic heart tube developmentGO:00350509.4FOXC2, GJA1, NKX2-5, NKX2-6
22negative regulation of cell growthGO:00303089.3GJA1, NPPA, NPPB
23negative regulation of transcription from RNA polymerase II promoterGO:00001229.1EHMT1, FOXC2, NKX2-5, NOTCH1
24cell differentiationGO:00301549.0FOXC2, FOXL1, NKX2-5, NKX2-6, NOTCH1
25heart developmentGO:00075078.2FOXC2, GATA5, GJA1, NKX2-5, NKX2-6, NOTCH1

Sources for Hypoplastic Left Heart Syndrome

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2CDC
14ExPASy
15FDA
16FMA
24GTR
25HGMD
26HMDB
27ICD10
28ICD10 via Orphanet
29ICD9CM
30IUPHAR
31KEGG
34MedGen
36MeSH
37MESH via Orphanet
38MGI
41NCI
42NCIt
43NDF-RT
46NINDS
47Novoseek
49OMIM
50OMIM via Orphanet
54PubMed
55QIAGEN
60SNOMED-CT via Orphanet
64Tumor Gene Family of Databases
65UMLS
66UMLS via Orphanet