MCID: HYP055
MIFTS: 60

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:
33LifeMap Discovery®, 11Disease Ontology, 46NIH Rare Diseases, 23GeneTests, 13DISEASES, 52Orphanet, 25GTR, 37MeSH, 66UMLS, 28ICD10, 30ICD9CM, 43NCIt, 29ICD10 via Orphanet, 38MESH via Orphanet, 67UMLS via Orphanet, 60SNOMED-CT
See all MalaCards sources

Aliases & Descriptions for Hypoplastic Left Heart Syndrome:

Name: Hypoplastic Left Heart Syndrome 33 11 46 23 13 52 37 66
Hlhs 46 52
 
Hypoplastic Left Heart 25

Characteristics:

Orphanet epidemiological data:

52
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 Ontology11 DOID:9955
ICD1028 Q23.4
ICD9CM30 746.7
MeSH37 D018636
NCIt43 C98894
SNOMED-CT60 62067003
Orphanet52 ORPHA2248
ICD10 via Orphanet29 Q23.4
MESH via Orphanet38 D018636
UMLS via Orphanet67 C0152101

Summaries for Hypoplastic Left Heart Syndrome

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NIH Rare Diseases:46 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 hypoplastic left heart syndrome 2 and hypoplastic left heart syndrome 1, 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 Cardiac Progenitor Differentiation. The drug alprostadil has been mentioned in the context of this disorder. Affiliated tissues include heart, skin and brain, and related mouse phenotypes are respiratory system and digestive/alimentary.

Disease Ontology:11 A congenital heart disease characterized by abnormal development of the left-sided structures of the heart.

Wikipedia:69 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 87)
idRelated DiseaseScoreTop Affiliating Genes
1hypoplastic left heart syndrome 212.5
2hypoplastic left heart syndrome 112.4
3hemophagocytic lymphohistiocytosis11.7
4left ventricular outflow tract obstruction11.5
5acquired kidney disease-associated renal cell carcinoma10.7NOTCH1, SMAD6
66-phosphogluconate dehydrogenase deficiency10.6GATA5, NKX2-5
7pyloric stenosis, infantile hypertrophic, 510.5FOXC2, FOXL1
8handigodu joint disease10.4FOXC2, FOXL1
9hallux varus and preaxial polysyndactyly10.4GJA1, NOTCH1
10gummatous syphilis10.4FOXC2, FOXL1
11mental retardation, autosomal dominant 810.4EHMT1, NOTCH1
12testicular trophoblastic tumor10.4GJA1, NOTCH1
13cerebritis10.3
14aneurysm10.2
15right aortic arch10.2
16macroglossia10.2NKX2-5, NPPB
17aortic valve disease 110.2
18heart disease10.1
19cor triatriatum10.1
20transposition of the great arteries10.1
21interatrial communication10.1
22gonococcal endophthalmia10.1NPPB, TNNI3
23melanotic neuroectodermal tumor10.1GATA5, NKX2-5, NPPB
24congenital heart disease10.1
25adams-oliver syndrome 510.0NOTCH1, NPPB, TNNI3
26lymphoma10.0
27ventricular septal defect 310.0
28aortic valve disease 210.0
29hypoplastic right heart syndrome10.0
30ceroid lipofuscinosis, neuronal, 610.0NPPB, SMAD6, TNNI3
31holt-oram syndrome10.0
32pagod syndrome10.0
33endocardial fibroelastosis10.0
34omphalocele10.0
35microcephaly10.0
36pulmonary valve stenosis10.0
37pentalogy of cantrell10.0
38vitelliform macular dystrophy10.0GATA5, NKX2-5, NPPA
39pseudomarfanism9.9NPPA, NPPB
40griscelli syndrome, type 29.9
41griscelli syndrome9.9
42hemophagocytic lymphohistiocytosis, familial, 19.9
43lymphoproliferative syndrome, x-linked, 19.9
44myocardial infarction9.8
45coronary artery disease9.8
46wolf-hirschhorn syndrome9.8
47kleefstra syndrome9.8
48moebius syndrome9.8
49alveolar capillary dysplasia with misalignment of pulmonary veins9.8
50total anomalous pulmonary venous return9.8

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:

 52 (show all 8)
  • patent ductus arteriosus
  • mitral stenosis
  • maternal diabetes
  • hypoplastic aortic arch
  • defect in the atrial septum
  • abnormality of chromosome segregation
  • hypoplastic left heart
  • mitral atresia

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 12)
idNameStatusPhaseClinical TrialsCas NumberPubChem Id
1
BosentanPhase 2102147536-97-8104865
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
C27H29N5O6S
CHEBI:51450
CHEMBL957
CID104865
D07538
DB00559
 
EN002863
HMS2090N14
I06-1873
KS-5062
L001086
LS-31532
MolPort-003-845-300
NCGC00167440-01
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
bosentan
bosentanum
bosentán
p-tert-Butyl-N-(6-(2-hydroxyethoxy)-5-(o-methoxyphenoxy)-2-(2-pyrimidinyl)-4-pyrimidinyl)benzenesulfonamide
p-tert-Butyl-N-[6-(2-hydroxyethoxy)
2Sildenafil citratePhase 2, Phase 1315171599-83-0
3
Citric AcidPhase 2, Phase 1106377-92-9311
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
4Epoetin alfaPhase 1, Phase 2638113427-24-0
5
PhenoxybenzaminePhase 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 [INN:BAN]
Phenoxybenzaminum
Phenoxybenzaminum [INN-Latin]
Prestwick0_000944
Prestwick1_000944
Prestwick2_000944
Prestwick3_000944
SPBio_001829
SPBio_003067
Spectrum2_001815
Spectrum4_000769
Spectrum5_001370
Spectrum_000378
UNII-0TTZ664R7Z
phenoxybenzamine
6CitrateNutraceuticalPhase 2, Phase 11063
7
DexmedetomidinePhase 151576631-46-4, 113775-47-668602, 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)
Dexmedetomidinum
Dexmédétomidine
MPV 1440
MPV-1440
Medetomidina [Spanish]
Medetomidine
Medetomidinum [Latin]
NCGC00025347-01
PRECEDEX
Precedex (TN)
Tocris-2023
ZINC04632106
8
Dopamine367851-61-6, 62-31-7681
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 [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
9
Bupropion31334841-39-9, 34911-55-2444
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]
BPBio1_000042
BRD-A05186015-003-05-7
BRN 2101062
BSPBio_000038
BSPBio_002247
Bupropion (INN)
Bupropion (Old RN)
Bupropion (USAN)
Bupropion Hcl
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
Zyban
alpha-(tert-butylamino)-m-chloropropiophenone
amfebutamonum
bupropion
10
Ethanol197164-17-5702
Synonyms:
(C6-C9)Alkyl alcohol
02483_FLUKA
02851_FLUKA
02853_FLUKA
02854_FLUKA
02855_FLUKA
02856_FLUKA
02856_SIAL
02857_FLUKA
02857_SIAL
02858_FLUKA
02858_SIAL
02860_FLUKA
02865_FLUKA
02865_SIAL
02870_FLUKA
02870_SIAL
02875_FLUKA
02877_FLUKA
02878_FLUKA
02882_FLUKA
02882_SIAL
02883_FLUKA
02884_FLUKA
02890_FLUKA
02890_SIAL
02891_FLUKA
02891_SIAL
1-Hydroxyethane
100C.NPA
121182-78-3
187380_ALDRICH
187380_SIAL
24102_RIEDEL
24102_SIAL
24103_RIEDEL
24103_SIAL
24105_RIEDEL
24105_SIAL
24106_RIEDEL
24106_SIAL
24194_RIEDEL
24194_SIAL
245119_ALDRICH
245119_SIAL
270741_ALDRICH
270741_SIAL
277649_ALDRICH
277649_SIAL
2858_SIGMA
29221_FLUKA
32205_RIEDEL
32205_SIAL
32221_RIEDEL
32221_SIAL
32294_RIEDEL
32294_SIAL
34870_SIAL
34963_RIEDEL
39278_FLUKA
40210_ALDRICH
40210_RIEDEL
41322_FLUKA
458600_ALDRICH
458600_SIAL
459828_ALDRICH
459828_SIAL
459836_ALDRICH
459836_SIAL
459844_SIAL
48075_SUPELCO
493511_SIAL
493538_ALDRICH
493538_SIAL
493546_ALDRICH
493546_SIAL
64-17-5
676829_SIAL
68475-56-9
71076-86-3
71329-38-9
8000-16-6
8024-45-1
AC1L19TW
AC1Q31MM
AHD 2000
AI3-01706
ALCOHOL 5% IN D5-W
Absolute Alcohol
Absolute Ethanol
Absolute alcohol
Absolute ethanol
Absolute ethyl alcohol
Aethanol
Aethanol [German]
Aethylalkohol
Alcare Hand Degermer
Alcohol
Alcohol (USP)
Alcohol (ethyl)
Alcohol Anhydrous
Alcohol [USP]
Alcohol anhydrous
Alcohol dehydrated
Alcohol denatured
Alcohol etílico
Alcohol, Absolute
Alcohol, Dehydrated
Alcohol, Diluted
Alcohol, Grain
Alcohol, anhydrous
Alcohol, dehydrated
Alcohol, denatured
Alcohol, diluted
Alcohol, ethyl
Alcohols
Alcohols, C1-3
Alcohols, C30
Alcohols, C6-9
Alcool Ethylique
Alcool Etilico
Alcool ethylique
Alcool etilico
Alcool éthylique
Algrain
Alkohol
Alkohol [German]
Alkoholu Etylowego
Alkoholu etylowego
Aminoethanol
Anhydrol
Anhydrol PM 4085
Anhydrous alcohol
Anhydrous ethanol
Beta-Aminoethanol
Beta-Aminoethyl Alcohol
Beta-Ethanolamine
Beta-Hydroxyethylamine
C00469
C2H5OH
C2H6O
CCRIS 945
CDA 19
CDA 19-200
CHEBI:16236
CHEMBL545
CID702
Caswell No. 426
Caswell No. 430
Colamine
Cologne Spirit
Cologne spirit
Cologne spirits
 
D000431
D00068
DB00898
Dehydrated Ethanol
Dehydrated alcohol
Dehydrated ethanol
Denatured Alcohol
Denatured Alcohol Cd-10
Denatured Alcohol Cd-5
Denatured Alcohol Cd-5a
Denatured Alcohol Sd-1
Denatured Alcohol Sd-13a
Denatured Alcohol Sd-17
Denatured Alcohol Sd-23a
Denatured Alcohol Sd-28
Denatured Alcohol Sd-30
Denatured Alcohol Sd-39b
Denatured Alcohol Sd-39c
Denatured Alcohol Sd-3a
Denatured Alcohol Sd-40m
Denatured Ethanol
Denatured alcohol
Denatured ethanol
Desinfektol EL
Diluted Alcohol
Distilled spirits
E2385_SIGMA
E7023_ALDRICH
E7023_SIAL
E7148_ALDRICH
E7148_SIAL
E7517_SIGMA
EINECS 200-578-6
EINECS 270-649-4
EOH
EOX
ETA
Envision Conditioner Pdd 9020
Esumiru WK 88
EtOH
Etanolo
Etanolo [Italian]
Ethanol (9CI)
Ethanol 200 Proof
Ethanol 200 proof
Ethanol Absolute
Ethanol Absolute Bp
Ethanol Anhydrous
Ethanol Extra Pure
Ethanol Vapor
Ethanol [JAN]
Ethanol solution
Ethanol, Silent Spirit
Ethanol, undenatured
Ethanolum anhydricum
Ethicap
Ethyl Alcohol
Ethyl Alcohol & Water, 10%
Ethyl Alcohol & Water, 20%
Ethyl Alcohol & Water, 30%
Ethyl Alcohol & Water, 40%
Ethyl Alcohol & Water, 5%
Ethyl Alcohol & Water, 50%
Ethyl Alcohol & Water, 60%
Ethyl Alcohol & Water, 70%
Ethyl Alcohol & Water, 80%
Ethyl Alcohol & Water, 95%
Ethyl Alcohol & Water, 96%
Ethyl Alcohol Anhydrous
Ethyl Alcohol, Anhydrous
Ethyl Alcohol, Denatured
Ethyl Hydrate
Ethyl Hydroxide
Ethyl alc
Ethyl alcohol
Ethyl alcohol anhydrous
Ethyl alcohol in alcoholic beverages
Ethyl alcohol usp
Ethyl hydrate
Ethyl hydroxide
Ethylalcohol
Ethylalcohol [Dutch]
Ethylol
Ethylolamine
Ethyloxy Group
Etylowy alkohol
FEMA No. 2419
FEMA Number 2419
Fermentation alcohol
Glycinol
Grain alcohol
HSDB 531
HSDB 82
HYDROXYETHYL GROUP
Hinetoless
Hydroxyethane
I14-12648
IMS 99
Infinity Pure
Jaysol
Jaysol S
LS-1539
LTBB002977
Lux
Methylated Spirit Mineralised
Methylated spirit
Methylcarbinol
MolPort-001-785-844
Molasses alcohol
NCGC00091458-01
NCI-C03134
NSC 85228
NSC85228
Oxydimethylene Group
Potato alcohol
Punctilious ethyl alcohol
Pyro
QMHAIh@
Reagent Alcohol
Ru-Tuss Expectorant
SDA 3A
SDA 40-2
SDM No. 37
SY Fresh M
Sekundasprit
Silent spirit
Spirit
Spirits of wine
Spiritus vini
Spirt
Synasol
Tecsol
Tecsol C
Thanol
Thiofaco M-50
UNII-3K9958V90M
USAF EK-1597
Undenatured Ethanol
WLN: Q2
absolute alcohol
alcohol
alcohol etilico
bmse000297
etanol
ethanol
ethyl alcohol
grain alcohol
nchem.651-comp3c
nchembio.552-comp10
nchembio.94-comp20
spiritus vini
Äthanol
Äthylalkohol
éthanol
11
CaffeineNutraceutical35858-08-22519
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-Pep
Alert-pep
Anacin Maximum Strength
Anhydrous caffeine
Anhydrous caffeine (JP15)
Anhydrous caffeine (TN)
Anoquan
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 Pure
Caffeine [BAN:JAN]
Caffeine solution
Caffeine, Anhydrous
Caffeine, Monohydrate
Caffeine, Synthetic
Caffeine, anhydrous
Caffeine, synthetic
Caffine
Cafipel
Caféine
Coffein
Coffein [German]
Coffeine
Coffeinum
Coffeinum N
Coffeinum Purrum
Compound 65
D002110
D00528
DB00201
DHC Plus
DHCplus
Darvon Compound
Darvon Compound-65
Dasin
Dexitac
Dexitac Stay Alert Stimulant
Dhc Plus
Diurex
DivK1c_000730
Durvitan
EINECS 200-362-1
EU-0100228
Eldiatric C
Enerjets
Ercatab
Esgic
Esgic-Plus
FEMA No. 2224
Femcet
Fioricet
Fiorinal
GlaxoSmithKline Brand of Caffeine
Guaranine
HMS1920I09
HMS2091O11
HMS502E12
HSDB 36
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
Lopac-C-0750
Lopac0_000228
MEGxp0_001350
MLS001055341
MLS001056714
MLS001066409
Mateina
Mateína
Maximum Strength Snapback Stimulant Powders
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
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
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
Spectrum2_001261
Spectrum3_000321
Spectrum4_001782
Spectrum5_000423
Spectrum_001301
Stim
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
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
12KavaNutraceutical1379000-38-8

Interventional clinical trials:

(show all 50)
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)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
32Global and Regional Myocardial Strain and Power Output In Patients With Single Ventricles Using Novel MRI TechniquesRecruitingNCT01107990
33Oxygen Consumption-based Assessments of Hemodynamics in Neonates Following Congenital Heart Surgery (Oxy-CAHN Study)RecruitingNCT02184169
34Cerebral Anatomy, Hemodynamics and MetabolismRecruitingNCT02919956
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
43National Collaborative to Improve Care of Children With Complex Congenital Heart DiseaseEnrolling by invitationNCT02852031
44SVR III: Brain Connectome and Neurodevelopmental OutcomesEnrolling by invitationNCT02692443
45Changes in Cerebral Oxygenation During Laparoscopy in Patients With Single Ventricle AnatomyEnrolling by invitationNCT02614664
46The Use of Optical Oximetry in Determining Gastrointestinal Complications After the Hybrid ProcedureEnrolling by invitationNCT02060825
47Diffuse Myocardial Fibrosis in Fontan PatientsEnrolling by invitationNCT01929174
48Examining Developmental Changes in Heart Contractions of Children With Congenital Heart DefectsTerminatedNCT00860327
49Aortic Arch ReconstructionTerminatedNCT00277940
50Sleep Disordered Breathing in Children With Single Ventricle PhysiologyWithdrawnNCT00156455

Search NIH Clinical Center for Hypoplastic Left Heart Syndrome

Inferred drug relations via UMLS66/NDF-RT44:

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 Syndrome25 23 GJA1

Anatomical Context for Hypoplastic Left Heart Syndrome

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MalaCards organs/tissues related to Hypoplastic Left Heart Syndrome:

34
Heart, Skin, Brain, Lung, Liver, Testes, Neutrophil

Animal Models for Hypoplastic Left Heart Syndrome or affiliated genes

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MGI Mouse Phenotypes related to Hypoplastic Left Heart Syndrome:

39 (show all 11)
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053888.3FOXC2, GJA1, HAND1, LEP, NKX2-5, NOTCH1
2MP:00053818.3FOXC2, FOXL1, GATA5, HAND1, LEP, NOTCH1
3MP:00053807.9CDH2, EHMT1, FOXC2, GJA1, HAND1, LEP
4MP:00053797.7FOXL1, GATA5, GJA1, LEP, NKX2-5, NOTCH1
5MP:00053677.4FOXC2, GATA5, LEP, NOTCH1, NPPA, XDH
6MP:00053847.4CDH2, FOXL1, GATA5, GJA1, HAND1, LEP
7MP:00053786.5CDH2, EHMT1, FOXC2, FOXL1, GJA1, HAND1
8MP:00053766.0CDH2, FOXC2, GATA5, GJA1, HAND1, LEP
9MP:00053696.0CDH2, FOXC2, GATA5, GJA1, HAND1, LEP
10MP:00053855.9CDH2, EHMT1, FOXC2, GATA5, GJA1, HAND1
11MP:00107685.8CDH2, 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 613)
idTitleAuthorsYear
1
Intraoperative Transesophageal Echocardiography in the Norwood-Sano Palliation of Hypoplastic Left Heart Syndrome. (27101490)
2016
2
Comparison of Feeding Strategies for Infants With Hypoplastic Left Heart Syndrome: A Randomized Controlled Trial. (27358299)
2016
3
EP07.03: Fetal intelligent navigation echocardiography (FINE) in a fetus affected by hypoplastic left heart syndrome and restrictive foramen ovale. (27646702)
2016
4
Hypoplastic Left Heart Syndrome: Exploring a Paradigm Shift in Favor of Surgery. (27567909)
2016
5
Preoperative Stabilization of Infants With Hypoplastic Left Heart Syndrome Before Stage I Palliation. (26830180)
2016
6
Application of the hybrid Stage 1 palliation concept to patients without hypoplastic left heart syndrome as a bridge to heart transplant. (27425401)
2016
7
Successful Repair of Hypoplastic Left Heart Syndrome With Intact Atrial Septum, Congenital Diaphragm Hernia, and Anomalous Origin of Coronary Artery: Defying the Odds. (27343534)
2016
8
Self-expanding stent implantation in arterial duct during hybrid palliation of hypoplastic left heart syndrome: midterm experience with a specially designed stent. (25631369)
2015
9
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
10
Frequent Dilatation of the Descending Aorta in Children With Hypoplastic Left Heart Syndrome Relates to Decreased Aortic Arch Elasticity. (26438562)
2015
11
Effect of Hybrid stage 1 procedure on ventricular function in infants with hypoplastic left heart syndrome. (26345472)
2015
12
Right ventricular systolic function in hypoplastic left heart syndrome: a comparison of velocity vector imaging and magnetic resonance imaging. (26323279)
2015
13
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
14
Hypoplastic left heart syndrome with dextrocardia and situs solitus. (24668985)
2014
15
Cardiac troponin I, cardiac troponin-specific autoantibodies and natriuretic peptides in children with hypoplastic left heart syndrome. (24101702)
2014
16
Psychosocial responses of parents to their infant's diagnosis of hypoplastic left heart syndrome. (25215886)
2014
17
Survey of How Pediatric Cardiologists Noninvasively Evaluate Patients with Hypoplastic Left Heart Syndrome. (25266754)
2014
18
Impaired cerebral perfusion after bilateral pulmonary arterial banding in patients with hypoplastic left heart syndrome. (23993895)
2013
19
Effects of pulmonary artery banding and retrograde aortic arch obstruction on the hybrid palliation of hypoplastic left heart syndrome. (23434295)
2013
20
QRS duration changes in patients with hypoplastic left heart syndrome undergoing hybrid palliation: prehybrid to post-Fontan. (23305551)
2013
21
Assessment of right ventricular volumes in hypoplastic left heart syndrome by real-time three-dimensional echocardiography: comparison with cardiac magnetic resonance imaging. (23946284)
2013
22
Fetal hypoplastic left heart syndrome and maternal liver transplantation for Wilson's disease: a case report. (24378118)
2013
23
Clinical outcomes and resource use for infants with hypoplastic left heart syndrome during bidirectional Glenn: summary from the Joint Council for Congenital Heart Disease National Pediatric Cardiology Quality Improvement Collaborative registry. (22673966)
2013
24
Heart for bonding: a new protocol of care for hypoplastic left heart syndrome. (22908051)
2012
25
Prenatal diagnosis of hypoplastic left heart syndrome: impact of counseling patterns on parental perceptions and decisions regarding termination of pregnancy. (22618584)
2012
26
Use of surveillance criteria reduces interstage mortality after the Norwood operation for hypoplastic left heart syndrome. (22219467)
2012
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
Outcomes of hypoplastic left heart syndrome in low-birth-weight patients. (21785995)
2011
30
Is "treat your child normally" helpful advice for parents of survivors of treatment of hypoplastic left heart syndrome? (19272201)
2009
31
Right ventricular performance in the fetus with hypoplastic left heart syndrome. (19324154)
2009
32
The current role of hybrid procedures in the stage 1 palliation of patients with hypoplastic left heart syndrome. (19278859)
2009
33
Impact of mitral stenosis and aortic atresia on survival in hypoplastic left heart syndrome. (18498820)
2008
34
Hybrid approach for hypoplastic left heart syndrome: intermediate results after the learning curve. (18498821)
2008
35
The effect of left ventricular size on right ventricular hemodynamics in pediatric survivors with hypoplastic left heart syndrome. (17961981)
2008
36
Hybrid therapy for hypoplastic left heart syndrome: reality check. (18404282)
2008
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
Hypoplastic left heart syndrome with intact or highly restrictive atrial septum: surgical experience from a single center. (17643639)
2007
40
Phenoxybenzamine in the treatment of hypoplastic left heart syndrome: a core review. (17646482)
2007
41
Successful surgical treatment of hypoplastic left heart syndrome associated with a divided left atrium that was diagnosed intraoperatively. (17320596)
2007
42
Anesthetic management of tracheoesophageal fistula repair in a newborn with hypoplastic left heart syndrome. (16677272)
2006
43
The management of hypoplastic left heart syndrome with a right aortic arch. (16984705)
2006
44
Birth weight and complexity are significant factors for the management of hypoplastic left heart syndrome. (16996917)
2006
45
Hypoplastic left heart syndrome and valvar pulmonary stenosis: presentation and management. (15565270)
2005
46
Cardiac magnetic resonance imaging after stage I Norwood operation for hypoplastic left heart syndrome. (16301358)
2005
47
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
48
Repair in infancy of right aortic arch with aberrant left brachiocephalic artery in the setting of a variant of the hypoplastic left heart syndrome. (15680052)
2004
49
Cardiac homeobox gene NKX2-5 mutations and congenital heart disease: associations with atrial septal defect and hypoplastic left heart syndrome. (12798584)
2003
50
Hypoplastic left heart syndrome with right aortic arch in a newborn. (8518070)
1993

Variations for Hypoplastic Left Heart Syndrome

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Copy number variations for Hypoplastic Left Heart Syndrome from CNVD:

6 (show all 21)
id CNVD ID Chromosom Start End Type Gene Symbol CNVD Disease
1140681103830000103950000DeletionHypoplastic left heart syndrome
2215891159750000159870000DuplicationHypoplastic left heart syndrome
3258021194970000195150000DeletionHypoplastic left heart syndrome
442872104197000042150000DeletionHypoplastic left heart syndrome
543118104455000044670000DuplicationHypoplastic left heart syndrome
6664811229999149999DuplicationHypoplastic left heart syndrome
767101123633000036690000DuplicationHypoplastic left heart syndrome
875411131971000019950000DeletionHypoplastic left heart syndrome
986813146579000065910000DeletionHypoplastic left heart syndrome
1089821151911000020070000DuplicationHypoplastic left heart syndrome
1190056151989000020010000DuplicationHypoplastic left heart syndrome
1290977152673000026910000DuplicationHypoplastic left heart syndrome
13105401167946999980189999DuplicationHypoplastic left heart syndrome
14113091174173000041910000DuplicationHypoplastic left heart syndrome
151353322113909999114089999DeletionHypoplastic left heart syndrome
16157162211353000013650000DeletionHypoplastic left heart syndrome
17157211211394999914129999DeletionHypoplastic left heart syndrome
18157986212817000028230000DeletionHypoplastic left heart syndrome
191600772197500009870000DeletionHypoplastic left heart syndrome
2021365864994999950009999DuplicationHypoplastic left heart syndrome
212175267101789999101909999DuplicationHypoplastic left heart syndrome

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
1fascia adherensGO:00059169.9CDH2, GJA1
2contractile fiberGO:00432929.6GJA1, TNNI3

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

(show all 35)
idNameGO IDScoreTop Affiliating Genes
1atrioventricular node developmentGO:000316210.7NKX2-5, NOTCH1
2cardiac right ventricle formationGO:000321910.6HAND1, NOTCH1
3glomerular mesangial cell developmentGO:007214410.6FOXC2, NOTCH1
4cardiac muscle tissue morphogenesisGO:005500810.6NKX2-5, NOTCH1
5adult heart developmentGO:000751210.6GJA1, NKX2-5
6ventricular septum morphogenesisGO:006041210.5NKX2-5, NOTCH1
7negative regulation of myotube differentiationGO:001083210.5NKX2-5, NOTCH1
8outflow tract septum morphogenesisGO:000314810.4NKX2-5, SMAD6
9pulmonary valve morphogenesisGO:000318410.4NOTCH1, SMAD6
10cardiac septum morphogenesisGO:006041110.4HAND1, NOTCH1
11negative regulation of ossificationGO:003027910.4NOTCH1, SMAD6
12cardiac ventricle morphogenesisGO:000320810.4NKX2-5, NOTCH1
13ventricular trabecula myocardium morphogenesisGO:000322210.4NKX2-5, NOTCH1
14blood vessel morphogenesisGO:004851410.3CDH2, GJA1
15negative regulation of endothelial cell proliferationGO:000193710.3GJA1, XDH
16cardiac muscle cell proliferationGO:006003810.3FOXC2, NKX2-5, NOTCH1
17regulation of blood vessel sizeGO:005088010.3FOXC2, NPPA
18neuronal stem cell population maintenanceGO:009715010.3CDH2, NOTCH1
19glial cell differentiationGO:001000110.2CDH2, NOTCH1
20ventricular cardiac muscle tissue morphogenesisGO:005501010.1FOXC2, HAND1, TNNI3
21heart contractionGO:006004710.1NKX2-5, TNNI3
22receptor guanylyl cyclase signaling pathwayGO:000716810.0NPPA, NPPB
23regulation of bone remodelingGO:004685010.0GJA1, LEP
24cGMP biosynthetic processGO:000618210.0NPPA, NPPB
25embryonic heart tube developmentGO:00350509.9FOXC2, GJA1, HAND1, NKX2-5
26negative regulation of canonical Wnt signaling pathwayGO:00900909.8CDH2, NKX2-5, NOTCH1
27positive regulation of JAK-STAT cascadeGO:00464279.8LEP, NOTCH1
28heart loopingGO:00019479.8GJA1, HAND1, NKX2-5, NOTCH1
29positive regulation of p38MAPK cascadeGO:19007459.7LEP, XDH
30negative regulation of cell growthGO:00303089.5GJA1, NPPA, NPPB
31regulation of cardiac conductionGO:19037799.4NKX2-5, NPPA, TNNI3
32positive regulation of transcription, DNA-templatedGO:00458939.2FOXC2, GATA5, HAND1, NKX2-5, NOTCH1
33regulation of blood pressureGO:00082178.9LEP, NPPA, NPPB
34negative regulation of transcription from RNA polymerase II promoterGO:00001228.2EHMT1, FOXC2, HAND1, LEP, NKX2-5, NOTCH1
35heart developmentGO:00075078.0FOXC2, FOXL1, GATA5, GJA1, HAND1, NKX2-5

Molecular functions related to Hypoplastic Left Heart Syndrome according to GeneCards Suite gene sharing:

(show all 10)
idNameGO IDScoreTop Affiliating Genes
1transcriptional activator activity, RNA polymerase II transcription factor bindingGO:000119010.6NKX2-5, NOTCH1
2transcription factor activity, RNA polymerase II distal enhancer sequence-specific bindingGO:000370510.1FOXC2, GATA5, NKX2-5
3peptide hormone receptor bindingGO:00514289.8LEP, NPPA
4chromatin bindingGO:00036829.4GATA5, NKX2-5, NOTCH1, SMAD6
5transcription regulatory region DNA bindingGO:00442129.3FOXC2, GATA5, HAND1, NKX2-5, SMAD6
6hormone activityGO:00051799.2LEP, NPPA, NPPB
7sequence-specific DNA bindingGO:00435659.1FOXC2, FOXL1, HAND1, NKX2-5, NOTCH1
8transcription factor activity, sequence-specific DNA bindingGO:00037009.0FOXC2, FOXL1, GATA5, NKX2-5, NOTCH1, SMAD6
9receptor bindingGO:00051028.5GJA1, LEP, NPPA, NPPB
10protein bindingGO:00055154.5CDH2, EHMT1, FOXC2, GATA5, GJA1, HAND1

Sources for Hypoplastic Left Heart Syndrome

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2CDC
6CNVD
10DGIdb
15ExPASy
16FDA
17FMA
25GTR
26HGMD
27HMDB
28ICD10
29ICD10 via Orphanet
30ICD9CM
31IUPHAR
32KEGG
35MedGen
37MeSH
38MESH via Orphanet
39MGI
42NCI
43NCIt
44NDF-RT
47NINDS
48Novoseek
50OMIM
51OMIM via Orphanet
55PubMed
56QIAGEN
61SNOMED-CT via Orphanet
65Tumor Gene Family of Databases
66UMLS
67UMLS via Orphanet