Page last updated: 2024-09-28

Anomalous Pulmonary Venous Return

An anomalous pulmonary venous return in which the right PULMONARY VEIN is not connected to the LEFT ATRIUM but to the INFERIOR VENA CAVA. Scimitar syndrome is named for the crescent- or Turkish sword-like shadow in the chest radiography and is often associated with hypoplasia of the right lung and right pulmonary artery, and dextroposition of the heart.

Synonyms(8)

Synonym
TAPVR
Scimitar Syndrome
Scimitar Anomaly
Total Anomalous Pulmonary Venous Return
TAPVR1
Total Anomalous Pulmonary Venous Return 1
Pulmonary Venous Return Anomaly
Anomalous Pulmonary Venous Return

Research Excerpts

Overview

ExcerptReference
"Scimitar syndrome is a rare congenital anomaly characterized by total or partial anomalous venous drainage of the right lung to the inferior vena cava."( Manoj, R; Muralidharan, J; Singhi, S; Sodhi, KS; Suri, D, 2008)
"Scimitar syndrome is a rare complex of congenital anomalies of the cardiopulmonary system."( Sergin, D; Tanatti, B; Ulukaya, S, 2018)
"Partial anomalous pulmonary venous return is a rare congenital aberrancy that involves oxygen-rich pulmonary venous drainage into the right atrium instead of into the systemic circulation."( Bierhals, AJ; Hachem, RR; Kreisel, D; Nava, RG; Pasque, MK; Patterson, GA; Puri, V; Shepherd, HM; Takahashi, T, 2022)

Context

ExcerptReference
"TAPVR has also been linked to the ANKRD1/CARP genes."( Arrington, CB; Bleyl, SB; Bowles, NE; Gruber, PJ; Jorde, LB; Kennedy, BJ; Nash, D; Ware, S; Wu, W; Yandell, M; Yost, HJ; Zhang, W, 2015)

Research

Studies (19)

TimeframeStudies, This Condition (%)All Conditions %
pre-19901 (5.26)23.3326
1990's2 (10.53)12.5806
2000's6 (31.58)18.1394
2010's5 (26.32)28.8240
2020's5 (26.32)9.53
DrugIndicatedRelationship StrengthStudiesTrials
furosemide0low10
iohexol0low10
iopromide0low10
methylprednisolone0low10
gadolinium0low20
barium sulfate0low10
adenosine0low10
xenon radioisotopes0low10
bosentan anhydrous0low10
tretinoin0low10
propylthiouracil0low10
methimazole0low10
digoxin0low10
gadolinium dtpa0low10
gadofosveset trisodium0low10
exudates0low10
sildenafil citrate0low10
eye0low10

Protein Targets (319)

ProteinPotency MeasurementsInhibition MeasurementsActivation MeasurementsDrugs
Chain A, MAJOR APURINIC/APYRIMIDINIC ENDONUCLEASE2002
Chain A, Membrane lipoprotein tmpC0011
Chain A, Membrane lipoprotein tmpC0011
Chain A, Membrane lipoprotein tmpC0011
Chain A, Structure of PAE2307 in complex with adenosine0011
Chain B, Structure of PAE2307 in complex with adenosine0011
Chain A, ADENOSINE RECEPTOR A2A0101
Chain A, ADENOSINE RECEPTOR A2A0101
Chain A, tRNA (guanine-N(1)-)-methyltransferase0011
Chain A, Uncharacterized protein MJ08830011
AR protein8008
signal transducer and activator of transcription 6, interleukin-4 induced1001
regulator of G-protein signaling 43003
glucocorticoid receptor [Homo sapiens]5005
retinoid X nuclear receptor alpha5005
estrogen nuclear receptor alpha8008
NPYLR7B0011
arylsulfatase A1001
IDH13003
Histone H2A.x2002
glycogen synthase kinase-3 beta isoform 10011
mitogen-activated protein kinase 11001
flap endonuclease 11001
nuclear receptor ROR-gamma isoform 15005
peripheral myelin protein 222002
survival motor neuron protein isoform d3003
high affinity choline transporter 1 isoform a0101
Sodium/nucleoside cotransporter 10202
Calcium dependent protein kinase0101
Mitogen-activated protein kinase kinase kinase 70011
Sodium/nucleoside cotransporter 20202
Voltage-dependent calcium channel gamma-2 subunit3003
Rap guanine nucleotide exchange factor 31001
Purine nucleoside phosphorylase0101
Epidermal growth factor receptor0101
Phosphoglycerate kinase 1 0101
Adenosine deaminase0001
Avidin0011
Adenosine deaminase0001
Glyceraldehyde-3-phosphate dehydrogenase0202
Receptor tyrosine-protein kinase erbB-20101
Insulin receptor0001
Phosphoglycerate kinase 20101
Heat shock protein HSP 90-alpha0112
Platelet-derived growth factor receptor beta0001
Adenosine receptor A30427
Heat shock 70 kDa protein 1A 0101
Heat shock cognate 71 kDa protein0011
Fibroblast growth factor receptor 10001
Inosine-5'-monophosphate dehydrogenase 20101
Endoplasmin0112
Glutamate receptor 23003
Inosine-5'-monophosphate dehydrogenase 1 0101
Fibroblast growth factor receptor 20001
Fibroblast growth factor receptor 40001
Fibroblast growth factor receptor 30001
Streptavidin0011
Adenosylhomocysteinase0001
Adenosine receptor A10112
Adenosine receptor A30101
Adenosine receptor A2a0124
Adenosine receptor A2b0012
Adenosine receptor A2b0112
Adenylate kinase 2, mitochondrial0101
Adenosine receptor A10225
Adenosine receptor A2a0112
Adenylate kinase isoenzyme 1 0101
Phosphatidylinositol 4-kinase alpha0203
Adenosine receptor A10112
Adenosine kinase0001
Adenosine deaminase 0001
Equilibrative nucleoside transporter 20202
Phosphatidylinositol 4-kinase type 2-beta0203
Histone-lysine N-methyltransferase, H3 lysine-79 specific0101
Equilibrative nucleoside transporter 10202
Phosphatidylinositol 4-kinase type 2-alpha0203
Solute carrier family 28 member 30202
Adenosine kinase0102
Phosphatidylinositol 4-kinase beta0203
5-methylthioadenosine/S-adenosylhomocysteine deaminase0001
Adenosine transporter 10001
phosphopantetheinyl transferase4004
RAR-related orphan receptor gamma4004
GLI family zinc finger 36006
estrogen receptor 2 (ER beta)5005
progesterone receptor5005
cytochrome P450 family 3 subfamily A polypeptide 44004
retinoic acid nuclear receptor alpha variant 16006
estrogen-related nuclear receptor alpha6006
pregnane X nuclear receptor8008
G4004
peroxisome proliferator activated receptor gamma4004
vitamin D (1,25- dihydroxyvitamin D3) receptor4004
activating transcription factor 63003
v-jun sarcoma virus 17 oncogene homolog (avian)3003
vitamin D3 receptor isoform VDRA2002
heat shock protein beta-13003
nuclear factor erythroid 2-related factor 2 isoform 16006
ATP-binding cassette sub-family C member 30808
Multidrug resistance-associated protein 40808
Bile salt export pump0404
Nuclear receptor subfamily 1 group I member 20022
Bile salt export pump0909
Interferon beta5005
HLA class I histocompatibility antigen, B alpha chain 4004
Endothelin receptor type B0101
Endothelin receptor type B0213
Endothelin-1 receptor0202
Endothelin-1 receptor0213
Endothelin receptor type B0011
Deoxyhypusine synthase0101
Sodium/bile acid cotransporter0202
Endothelin-1 receptor0101
Inositol hexakisphosphate kinase 14004
cytochrome P450 2C9, partial4004
Chain A, Beta-lactamase2002
Chain A, ATP-DEPENDENT DNA HELICASE Q12002
Chain A, Cruzipain2002
interleukin 82002
PAX80001
SMAD family member 21001
ATAD5 protein, partial2002
PPM1D protein2002
SMAD family member 31001
TDP1 protein4004
STAT3, partial0101
thioredoxin glutathione reductase2002
Smad31001
nuclear receptor subfamily 1, group I, member 35005
farnesoid X nuclear receptor5005
67.9K protein1001
peroxisome proliferator-activated receptor delta3003
alpha-galactosidase1001
cytochrome P450, family 19, subfamily A, polypeptide 1, isoform CRA_a3003
thyroid stimulating hormone receptor2002
nuclear factor of kappa light polypeptide gene enhancer in B-cells 1 (p105), isoform CRA_a1001
NPC intracellular cholesterol transporter 1 precursor1001
cellular tumor antigen p53 isoform a1001
tumor necrosis factor1001
thyroid hormone receptor beta isoform a2002
nuclear factor erythroid 2-related factor 2 isoform 23003
parathyroid hormone/parathyroid hormone-related peptide receptor precursor1001
thyroid hormone receptor beta isoform 23003
ras-related protein Rab-9A1001
signal transducer and activator of transcription 1-alpha/beta isoform alpha0101
geminin6006
lethal factor (plasmid)1001
lamin isoform A-delta103003
Solute carrier organic anion transporter family member 1A40102
Cellular tumor antigen p532002
Sodium/potassium-transporting ATPase subunit alpha-1 0202
Sodium/potassium-transporting ATPase subunit beta-10202
Integrin beta-31001
Sodium/potassium-transporting ATPase subunit alpha-20101
Sodium/potassium-transporting ATPase subunit alpha-3 0101
ATP-dependent translocase ABCB10101
Sodium/potassium-transporting ATPase subunit beta-1 0101
ATP-dependent translocase ABCB10102
Integrin alpha-IIb1001
Cytochrome P450 3A40112
Replicase polyprotein 1ab0101
Sodium/potassium-transporting ATPase subunit alpha-30101
Sodium/potassium-transporting ATPase subunit beta-20101
ATP-dependent translocase ABCB10101
Sodium/potassium-transporting ATPase subunit alpha-20202
Sodium/potassium-transporting ATPase subunit alpha-10101
Nuclear receptor ROR-gamma1225
Sodium/potassium-transporting ATPase subunit beta-30101
Sodium/potassium-transporting ATPase subunit gamma0101
Sodium/potassium-transporting ATPase subunit alpha-40101
Sodium/potassium-transporting ATPase subunit alpha-40101
Solute carrier organic anion transporter family member 4C10001
Solute carrier organic anion transporter family member 4C10001
ATPase family AAA domain-containing protein 53003
Ataxin-23003
Solute carrier organic anion transporter family member 1A40001
Solute carrier organic anion transporter family member 1B30101
Solute carrier organic anion transporter family member 1B10101
aldehyde dehydrogenase 1 family, member A13003
EWS/FLI fusion protein2002
aryl hydrocarbon receptor2002
thyrotropin-releasing hormone receptor2002
Solute carrier family 22 member 60101
Carbonic anhydrase 120103
Carbonic anhydrase 10103
Carbonic anhydrase 20103
Albumin0101
Carbonic anhydrase 30102
Glucose-6-phosphate 1-dehydrogenase0101
Cytochrome P450 2C9 0112
Carbonic anhydrase 40103
Carbonic anhydrase 60103
Serum paraoxonase/arylesterase 10101
Corticosteroid 11-beta-dehydrogenase isozyme 10101
Carbonic anhydrase 5A, mitochondrial0103
Carbonic anhydrase 70103
Corticosteroid 11-beta-dehydrogenase isozyme 10101
Cytochrome P450 2J20101
6-phosphogluconate dehydrogenase, decarboxylating0101
Spike glycoprotein2002
Holo-[acyl-carrier-protein] synthase0101
Solute carrier organic anion transporter family member 2A10101
Carbonic anhydrase 90103
Canalicular multispecific organic anion transporter 10707
Multidrug and toxin extrusion protein 10101
Carbonic anhydrase 130103
G-protein coupled receptor 350112
CDGSH iron-sulfur domain-containing protein 10202
Carbonic anhydrase 140103
Broad substrate specificity ATP-binding cassette transporter ABCG20101
Carbonic anhydrase 5B, mitochondrial0103
USP1 protein, partial2002
importin subunit beta-1 isoform 11001
snurportin-11001
GTP-binding nuclear protein Ran isoform 11001
Chain A, JmjC domain-containing histone demethylation protein 3A1001
Luciferase2002
hypoxia-inducible factor 1 alpha subunit3003
euchromatic histone-lysine N-methyltransferase 23003
Polyphenol oxidase 20203
Dopamine beta-hydroxylase 0202
Lactoperoxidase0101
Lanosterol 14-alpha demethylase0101
acetylcholinesterase1001
hypoxia-inducible factor 1, alpha subunit (basic helix-loop-helix transcription factor)2002
nonstructural protein 12002
bromodomain adjacent to zinc finger domain 2B2002
nuclear receptor subfamily 1, group I, member 22002
muscleblind-like protein 1 isoform 11001
Glucocorticoid receptor0202
Glycine receptor subunit alpha-10202
Androgen receptor0202
Glycine receptor subunit beta0202
Glycine receptor subunit alpha-20202
Glycine receptor subunit alpha-30202
Nuclear receptor subfamily 1 group I member 20011
ATP-dependent phosphofructokinase2002
Chain A, Putative fructose-1,6-bisphosphate aldolase1001
thyroid stimulating hormone receptor1001
cytochrome P450 2C19 precursor1001
chromobox protein homolog 12002
peptidyl-prolyl cis-trans isomerase NIMA-interacting 11001
NADH-cytochrome b5 reductase 3 0101
Myeloperoxidase0101
Thyroid peroxidase0101
Taste receptor type 2 member 380012
potassium voltage-gated channel subfamily H member 2 isoform d1001
cGMP-specific 3',5'-cyclic phosphodiesterase0101
Chain A, Nuclear Receptor ROR-beta0101
Chain A, 2-oxoglutarate Oxygenase1001
Chain A, Ferritin light chain1001
thioredoxin reductase1001
pregnane X receptor1001
GLS protein1001
Microtubule-associated protein tau1001
Parkin1001
Caspase-71001
Bloom syndrome protein isoform 11001
caspase-31001
DNA polymerase kappa isoform 11001
cytochrome P450 3A4 isoform 11001
M-phase phosphoprotein 81001
histone acetyltransferase KAT2A isoform 11001
Gamma-aminobutyric acid receptor subunit pi1001
Ornithine decarboxylase0001
Amyloid-beta precursor protein0101
Cytochrome P450 1A20011
Ornithine decarboxylase0001
60 kDa chaperonin0101
Retinoic acid receptor alpha0227
Cytochrome P450 2D60011
60 kDa heat shock protein, mitochondrial0101
Retinoic acid receptor beta0227
Retinoic acid receptor alpha0123
Retinoic acid receptor gamma 0227
Gamma-aminobutyric acid receptor subunit beta-11001
Alpha-1B adrenergic receptor0202
Alpha-1A adrenergic receptor0101
Gamma-aminobutyric acid receptor subunit delta1001
Gamma-aminobutyric acid receptor subunit gamma-21001
Retinoic acid receptor gamma0123
5-hydroxytryptamine receptor 1A0101
Retinoic acid receptor RXR-alpha0224
Gamma-aminobutyric acid receptor subunit alpha-51001
Gamma-aminobutyric acid receptor subunit alpha-31001
Retinoic acid receptor beta0123
Cellular retinoic acid-binding protein 20011
Gamma-aminobutyric acid receptor subunit gamma-11001
Gamma-aminobutyric acid receptor subunit alpha-21001
Alpha-1D adrenergic receptor0101
Gamma-aminobutyric acid receptor subunit alpha-41001
Gamma-aminobutyric acid receptor subunit gamma-31001
Mitogen-activated protein kinase 10101
Retinoic acid receptor RXR-alpha0123
Retinoic acid receptor RXR-beta0224
Retinoic acid receptor RXR-beta0123
Retinoic acid receptor RXR-gamma0123
Indoleamine 2,3-dioxygenase 10101
Gamma-aminobutyric acid receptor subunit alpha-61001
Cytochrome P450 2C190011
Nuclear receptor ROR-alpha0101
Alpha-synuclein0101
Cellular retinoic acid-binding protein 10102
5-hydroxytryptamine receptor 2B0202
Alpha-1A adrenergic receptor0202
Retinoic acid receptor RXR-gamma0224
10 kDa heat shock protein, mitochondrial0101
Gamma-aminobutyric acid receptor subunit alpha-11001
Cellular retinoic acid-binding protein 10011
Gamma-aminobutyric acid receptor subunit beta-31001
Gamma-aminobutyric acid receptor subunit beta-21001
Peptidyl-prolyl cis-trans isomerase NIMA-interacting 10213
Thiosulfate sulfurtransferase0101
60 kDa chaperonin 0101
10 kDa chaperonin 0101
Indoleamine 2,3-dioxygenase 20101
GABA theta subunit1001
Nuclear receptor ROR-beta0101
Gamma-aminobutyric acid receptor subunit epsilon1001
[prepared from compound, protein, and bioassay information from National Library of Medicine (NLM), extracted Dec-2023]