MCID: HYP055
MIFTS: 59

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

About this section
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 sclerosing cholangitis and endogenous depression, 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 brain, 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 412)
idRelated DiseaseScoreTop Affiliating Genes
1sclerosing cholangitis30.0NKX2-6, NPPB
2endogenous depression30.0NPPA, NPPB
3hepatitis c29.8NKX2-5, NKX2-6, NPPB
4hypoplastic left heart syndrome 212.9
5hypoplastic left heart syndrome 112.8
6hemophagocytic lymphohistiocytosis11.9
7left ventricular outflow tract obstruction11.6
8endotheliitis10.6
9aortic valve disease 110.6
10hepatitis10.6
11leukemia10.5
12cerebritis10.5
13fbln1-related developmental delay-central nervous system anomaly-syndactyly syndrome10.4NOTCH1, SMAD6
14ventricular septal defect 310.4
15aortic valve disease 210.4
16hypoplastic right heart syndrome10.4
17lung cancer10.4
18breast cancer10.4
19arthritis10.4
20lymphoma10.4
21ischemia10.4
22tuberculosis10.4
23thyroiditis10.4
24hypotrichosis10.4GJA1, NOTCH1
25mucopolysaccharidosis iva10.4FOXC2, FOXL1
26brachydactyly10.4FOXC2, FOXL1
27rheumatoid arthritis10.3
28colorectal cancer10.3
29renal cell carcinoma10.3
30atherosclerosis10.3
31chronic lymphocytic leukemia10.3
32esophagitis10.3
33prostatitis10.3
34melanoma10.3
35adenocarcinoma10.3
36neuronitis10.3
37lupus erythematosus10.3
38leishmaniasis10.3
39aneurysm10.3
40mental retardation, autosomal dominant 810.3EHMT1, NOTCH1
41hemophagocytic lymphohistiocytosis, familial, 110.3
42lymphoproliferative syndrome, x-linked, 110.3
43macrophage activation syndrome10.3
44schizophrenia10.3
45neuroblastoma10.3
46systemic lupus erythematosus10.3
47prostate cancer10.3
48hepatocellular carcinoma10.3
49asthma10.3
50attention deficit-hyperactivity disorder10.3

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

About this section

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, Brain, Breast, T cells, Liver, Lung

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 571)
idTitleAuthorsYear
1
Gender differences in the clinical features of hypertrophic cardiomyopathy caused by cardiac myosin-binding protein C gene mutations. (25123604)
2015
2
The Natural History of Antisocial Personality Disorder. (26175389)
2015
3
Motor progression of Parkinson's disease with the leucine-rich repeat kinase 2 G2019S mutation. (24903616)
2014
4
Primary knee arthrodesis for severe crystalline arthropathy. (24278911)
2013
5
HDAC inhibitor trichostatin A suppresses esophageal squamous cell carcinoma metastasis through HADC2 reduced MMP-2/9. (23544610)
2013
6
A focal domain of extreme demethylation within D4Z4 in FSHD2. (23284062)
2013
7
Spontaneous coronary artery dissection: case report and review of literature. (23439852)
2012
8
Efficacy of Early Infliximab Treatment for Pediatric Crohn's Disease: A Three-year Follow-up. (24010094)
2012
9
Tyrosinase-expressing neuronal cell line as in vitro model of Parkinson's disease. (20480001)
2010
10
Phosphorylation status of the NR2B subunit of NMDA receptor regulates its interaction with calcium/calmodulin-dependent protein kinase II. (19453375)
2009
11
Differential cytokine/chemokines and KL-6 profiles in patients with different forms of tuberculosis. (19570688)
2009
12
A new mechanism for bile acid diarrhea: defective feedback inhibition of bile acid biosynthesis. (19426836)
2009
13
Fasudil inhibits lysophosphatidic acid-induced invasiveness of human ovarian cancer cells. (19955921)
2009
14
Molecular design guided by a local map of sequence space: DNA aptamers that inhibit cathepsin E. (18321158)
2008
15
Mosaic tetraploidy and transient GFI1 mutation in a patient with severe chronic neutropenia. (17096407)
2008
16
Relation of haptoglobin phenotype to early vascular changes in patients with diabetes mellitus. (18082523)
2007
17
Treatment of airway inflammation in cystic fibrosis]. (17702549)
2007
18
Fibroblast growth factors 2, 4, and 8 exert both negative and positive effects on limb, frontonasal, and mandibular chondrogenesis via MEK-ERK activation. (17167778)
2007
19
No association between polymorphisms in the BDNF gene and age at onset in Huntington disease. (17096834)
2006
20
EGFR and HER2 gene expression status and their correlation in non-small cell lung cancer]. (17069674)
2006
21
Cerebellar ataxia with coenzyme Q10 deficiency: diagnosis and follow-up after coenzyme Q10 supplementation. (16677673)
2006
22
Elevated cysteine-rich 61 mediates aberrant collagen homeostasis in chronologically aged and photoaged human skin. (16877350)
2006
23
Inflammatory markers in women with a recent history of gestational diabetes mellitus. (15816369)
2005
24
Involvement of poly(ADP-ribose) polymerase-1 and XRCC1/DNA ligase III in an alternative route for DNA double-strand breaks rejoining. (15498778)
2004
25
Bilateral deep keratitis caused by systemic lupus erythematosus. (15075893)
2004
26
Bile acid reduces the secretion of very low density lipoprotein by repressing microsomal triglyceride transfer protein gene expression mediated by hepatocyte nuclear factor-4. (15337761)
2004
27
Vascular complications of homocystinuria: a retrospective multicenter study]. (11928374)
2002
28
CYP2C19 genotype and S-mephenytoin 4'-hydroxylation phenotype in a Chinese Dai population. (11956668)
2002
29
Intermolecular interactions between cholecystokinin-8 and the third extracellular loop of the cholecystokinin-2 receptor. (11926817)
2002
30
Characterization of a mammalian Golgi-localized protein complex, COG, that is required for normal Golgi morphology and function. (11980916)
2002
31
Proteomics characterization of abundant Golgi membrane proteins. (11042173)
2001
32
Relationship between FIGO grade and AgNOR, S100-positive langerhans cells in endometrial adenocarcinoma. (10972859)
2000
33
Chromogranin A, a significant prognostic factor in small cell lung cancer. (10574253)
1999
34
Modulation of immune responses to DNA vaccines by codelivery of cytokine genes. (10705687)
1999
35
p53 alteration in regional lymph node metastases from prostate carcinoma: a marker for progression? (10357418)
1999
36
Differential expression of cell adhesion molecules in inflamed appendix: correlation with clinical stage. (10209493)
1998
37
Chemopreventive agent resveratrol, a natural product derived from grapes, triggers CD95 signaling-dependent apoptosis in human tumor cells. (9680369)
1998
38
Bacterial and mammalian DNA alkyltransferases sensitize Escherichia coli to the lethal and mutagenic effects of dibromoalkanes. (9363995)
1997
39
Human T-cell leukemia virus type 1 Tax transactivates the human proliferating cell nuclear antigen promoter. (8995640)
1997
40
Inhibitor-stimulated non-parallel pancreatic secretion in man: hormonal and neural regulation? (7701255)
1995
41
Structure and evolution of opossum, guinea pig, and porcupine cytochrome b genes. (8315653)
1993
42
Abnormal lymph drainage in patients with chronic venous leg ulcers. (8463459)
1993
43
Isolated hypoglossal nerve palsy and Horner's syndrome with benign course. (2030364)
1991
44
Delineation of the intronless nature of the genes for the human and hamster beta 2-adrenergic receptor and their putative promoter regions. (3034889)
1987
45
Sparganosis: a clinical and pathologic observation of the first observed case in a child. (3601496)
1987
46
Ultrasound evaluation of amniotic fluid: outcome of pregnancies with severe oligohydramnios. (3515948)
1986
47
Hb Etobicoke, alpha 85(F5) Ser leads to Arg found in a newborn of French-Indian-English descent. (6874377)
1983
48
Angiocardiography in congenital subvalvular aortic stenosis: prognosis and operative indications. (556931)
1977
49
Hemarthrosis in an heparinized patient. (856220)
1977
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