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

Excerpt	Reference
"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

Excerpt	Reference
"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)

Timeframe	Studies, This Condition (%)	All Conditions %
pre-1990	1 (5.26)	23.3326
1990's	2 (10.53)	12.5806
2000's	6 (31.58)	18.1394
2010's	5 (26.32)	28.8240
2020's	5 (26.32)	9.53

Drug	Relationship Strength	Studies
furosemide	low	1
iohexol	low	1
iopromide	low	1
methylprednisolone	low	1
gadolinium	low	2
barium sulfate	low	1
adenosine	low	1
xenon radioisotopes	low	1
bosentan anhydrous	low	1
tretinoin	low	1
propylthiouracil	low	1
methimazole	low	1
digoxin	low	1
gadolinium dtpa	low	1
gadofosveset trisodium	low	1
exudates	low	1
sildenafil citrate	low	1
eye	low	1

Protein Targets (319)

Protein	Potency Measurements	Inhibition Measurements	Activation Measurements	Drugs
Chain A, MAJOR APURINIC/APYRIMIDINIC ENDONUCLEASE	2	0	0	2
Chain A, Membrane lipoprotein tmpC	0	0	1	1
Chain A, Membrane lipoprotein tmpC	0	0	1	1
Chain A, Membrane lipoprotein tmpC	0	0	1	1
Chain A, Structure of PAE2307 in complex with adenosine	0	0	1	1
Chain B, Structure of PAE2307 in complex with adenosine	0	0	1	1
Chain A, ADENOSINE RECEPTOR A2A	0	1	0	1
Chain A, ADENOSINE RECEPTOR A2A	0	1	0	1
Chain A, tRNA (guanine-N(1)-)-methyltransferase	0	0	1	1
Chain A, Uncharacterized protein MJ0883	0	0	1	1
AR protein	8	0	0	8
signal transducer and activator of transcription 6, interleukin-4 induced	1	0	0	1
regulator of G-protein signaling 4	3	0	0	3
glucocorticoid receptor [Homo sapiens]	5	0	0	5
retinoid X nuclear receptor alpha	5	0	0	5
estrogen nuclear receptor alpha	8	0	0	8
NPYLR7B	0	0	1	1
arylsulfatase A	1	0	0	1
IDH1	3	0	0	3
Histone H2A.x	2	0	0	2
glycogen synthase kinase-3 beta isoform 1	0	0	1	1
mitogen-activated protein kinase 1	1	0	0	1
flap endonuclease 1	1	0	0	1
nuclear receptor ROR-gamma isoform 1	5	0	0	5
peripheral myelin protein 22	2	0	0	2
survival motor neuron protein isoform d	3	0	0	3
high affinity choline transporter 1 isoform a	0	1	0	1
Sodium/nucleoside cotransporter 1	0	2	0	2
Calcium dependent protein kinase	0	1	0	1
Mitogen-activated protein kinase kinase kinase 7	0	0	1	1
Sodium/nucleoside cotransporter 2	0	2	0	2
Voltage-dependent calcium channel gamma-2 subunit	3	0	0	3
Rap guanine nucleotide exchange factor 3	1	0	0	1
Purine nucleoside phosphorylase	0	1	0	1
Epidermal growth factor receptor	0	1	0	1
Phosphoglycerate kinase 1	0	1	0	1
Adenosine deaminase	0	0	0	1
Avidin	0	0	1	1
Adenosine deaminase	0	0	0	1
Glyceraldehyde-3-phosphate dehydrogenase	0	2	0	2
Receptor tyrosine-protein kinase erbB-2	0	1	0	1
Insulin receptor	0	0	0	1
Phosphoglycerate kinase 2	0	1	0	1
Heat shock protein HSP 90-alpha	0	1	1	2
Platelet-derived growth factor receptor beta	0	0	0	1
Adenosine receptor A3	0	4	2	7
Heat shock 70 kDa protein 1A	0	1	0	1
Heat shock cognate 71 kDa protein	0	0	1	1
Fibroblast growth factor receptor 1	0	0	0	1
Inosine-5'-monophosphate dehydrogenase 2	0	1	0	1
Endoplasmin	0	1	1	2
Glutamate receptor 2	3	0	0	3
Inosine-5'-monophosphate dehydrogenase 1	0	1	0	1
Fibroblast growth factor receptor 2	0	0	0	1
Fibroblast growth factor receptor 4	0	0	0	1
Fibroblast growth factor receptor 3	0	0	0	1
Streptavidin	0	0	1	1
Adenosylhomocysteinase	0	0	0	1
Adenosine receptor A1	0	1	1	2
Adenosine receptor A3	0	1	0	1
Adenosine receptor A2a	0	1	2	4
Adenosine receptor A2b	0	0	1	2
Adenosine receptor A2b	0	1	1	2
Adenylate kinase 2, mitochondrial	0	1	0	1
Adenosine receptor A1	0	2	2	5
Adenosine receptor A2a	0	1	1	2
Adenylate kinase isoenzyme 1	0	1	0	1
Phosphatidylinositol 4-kinase alpha	0	2	0	3
Adenosine receptor A1	0	1	1	2
Adenosine kinase	0	0	0	1
Adenosine deaminase	0	0	0	1
Equilibrative nucleoside transporter 2	0	2	0	2
Phosphatidylinositol 4-kinase type 2-beta	0	2	0	3
Histone-lysine N-methyltransferase, H3 lysine-79 specific	0	1	0	1
Equilibrative nucleoside transporter 1	0	2	0	2
Phosphatidylinositol 4-kinase type 2-alpha	0	2	0	3
Solute carrier family 28 member 3	0	2	0	2
Adenosine kinase	0	1	0	2
Phosphatidylinositol 4-kinase beta	0	2	0	3
5-methylthioadenosine/S-adenosylhomocysteine deaminase	0	0	0	1
Adenosine transporter 1	0	0	0	1
phosphopantetheinyl transferase	4	0	0	4
RAR-related orphan receptor gamma	4	0	0	4
GLI family zinc finger 3	6	0	0	6
estrogen receptor 2 (ER beta)	5	0	0	5
progesterone receptor	5	0	0	5
cytochrome P450 family 3 subfamily A polypeptide 4	4	0	0	4
retinoic acid nuclear receptor alpha variant 1	6	0	0	6
estrogen-related nuclear receptor alpha	6	0	0	6
pregnane X nuclear receptor	8	0	0	8
G	4	0	0	4
peroxisome proliferator activated receptor gamma	4	0	0	4
vitamin D (1,25- dihydroxyvitamin D3) receptor	4	0	0	4
activating transcription factor 6	3	0	0	3
v-jun sarcoma virus 17 oncogene homolog (avian)	3	0	0	3
vitamin D3 receptor isoform VDRA	2	0	0	2
heat shock protein beta-1	3	0	0	3
nuclear factor erythroid 2-related factor 2 isoform 1	6	0	0	6
ATP-binding cassette sub-family C member 3	0	8	0	8
Multidrug resistance-associated protein 4	0	8	0	8
Bile salt export pump	0	4	0	4
Nuclear receptor subfamily 1 group I member 2	0	0	2	2
Bile salt export pump	0	9	0	9
Interferon beta	5	0	0	5
HLA class I histocompatibility antigen, B alpha chain	4	0	0	4
Endothelin receptor type B	0	1	0	1
Endothelin receptor type B	0	2	1	3
Endothelin-1 receptor	0	2	0	2
Endothelin-1 receptor	0	2	1	3
Endothelin receptor type B	0	0	1	1
Deoxyhypusine synthase	0	1	0	1
Sodium/bile acid cotransporter	0	2	0	2
Endothelin-1 receptor	0	1	0	1
Inositol hexakisphosphate kinase 1	4	0	0	4
cytochrome P450 2C9, partial	4	0	0	4
Chain A, Beta-lactamase	2	0	0	2
Chain A, ATP-DEPENDENT DNA HELICASE Q1	2	0	0	2
Chain A, Cruzipain	2	0	0	2
interleukin 8	2	0	0	2
PAX8	0	0	0	1
SMAD family member 2	1	0	0	1
ATAD5 protein, partial	2	0	0	2
PPM1D protein	2	0	0	2
SMAD family member 3	1	0	0	1
TDP1 protein	4	0	0	4
STAT3, partial	0	1	0	1
thioredoxin glutathione reductase	2	0	0	2
Smad3	1	0	0	1
nuclear receptor subfamily 1, group I, member 3	5	0	0	5
farnesoid X nuclear receptor	5	0	0	5
67.9K protein	1	0	0	1
peroxisome proliferator-activated receptor delta	3	0	0	3
alpha-galactosidase	1	0	0	1
cytochrome P450, family 19, subfamily A, polypeptide 1, isoform CRA_a	3	0	0	3
thyroid stimulating hormone receptor	2	0	0	2
nuclear factor of kappa light polypeptide gene enhancer in B-cells 1 (p105), isoform CRA_a	1	0	0	1
NPC intracellular cholesterol transporter 1 precursor	1	0	0	1
cellular tumor antigen p53 isoform a	1	0	0	1
tumor necrosis factor	1	0	0	1
thyroid hormone receptor beta isoform a	2	0	0	2
nuclear factor erythroid 2-related factor 2 isoform 2	3	0	0	3
parathyroid hormone/parathyroid hormone-related peptide receptor precursor	1	0	0	1
thyroid hormone receptor beta isoform 2	3	0	0	3
ras-related protein Rab-9A	1	0	0	1
signal transducer and activator of transcription 1-alpha/beta isoform alpha	0	1	0	1
geminin	6	0	0	6
lethal factor (plasmid)	1	0	0	1
lamin isoform A-delta10	3	0	0	3
Solute carrier organic anion transporter family member 1A4	0	1	0	2
Cellular tumor antigen p53	2	0	0	2
Sodium/potassium-transporting ATPase subunit alpha-1	0	2	0	2
Sodium/potassium-transporting ATPase subunit beta-1	0	2	0	2
Integrin beta-3	1	0	0	1
Sodium/potassium-transporting ATPase subunit alpha-2	0	1	0	1
Sodium/potassium-transporting ATPase subunit alpha-3	0	1	0	1
ATP-dependent translocase ABCB1	0	1	0	1
Sodium/potassium-transporting ATPase subunit beta-1	0	1	0	1
ATP-dependent translocase ABCB1	0	1	0	2
Integrin alpha-IIb	1	0	0	1
Cytochrome P450 3A4	0	1	1	2
Replicase polyprotein 1ab	0	1	0	1
Sodium/potassium-transporting ATPase subunit alpha-3	0	1	0	1
Sodium/potassium-transporting ATPase subunit beta-2	0	1	0	1
ATP-dependent translocase ABCB1	0	1	0	1
Sodium/potassium-transporting ATPase subunit alpha-2	0	2	0	2
Sodium/potassium-transporting ATPase subunit alpha-1	0	1	0	1
Nuclear receptor ROR-gamma	1	2	2	5
Sodium/potassium-transporting ATPase subunit beta-3	0	1	0	1
Sodium/potassium-transporting ATPase subunit gamma	0	1	0	1
Sodium/potassium-transporting ATPase subunit alpha-4	0	1	0	1
Sodium/potassium-transporting ATPase subunit alpha-4	0	1	0	1
Solute carrier organic anion transporter family member 4C1	0	0	0	1
Solute carrier organic anion transporter family member 4C1	0	0	0	1
ATPase family AAA domain-containing protein 5	3	0	0	3
Ataxin-2	3	0	0	3
Solute carrier organic anion transporter family member 1A4	0	0	0	1
Solute carrier organic anion transporter family member 1B3	0	1	0	1
Solute carrier organic anion transporter family member 1B1	0	1	0	1
aldehyde dehydrogenase 1 family, member A1	3	0	0	3
EWS/FLI fusion protein	2	0	0	2
aryl hydrocarbon receptor	2	0	0	2
thyrotropin-releasing hormone receptor	2	0	0	2
Solute carrier family 22 member 6	0	1	0	1
Carbonic anhydrase 12	0	1	0	3
Carbonic anhydrase 1	0	1	0	3
Carbonic anhydrase 2	0	1	0	3
Albumin	0	1	0	1
Carbonic anhydrase 3	0	1	0	2
Glucose-6-phosphate 1-dehydrogenase	0	1	0	1
Cytochrome P450 2C9	0	1	1	2
Carbonic anhydrase 4	0	1	0	3
Carbonic anhydrase 6	0	1	0	3
Serum paraoxonase/arylesterase 1	0	1	0	1
Corticosteroid 11-beta-dehydrogenase isozyme 1	0	1	0	1
Carbonic anhydrase 5A, mitochondrial	0	1	0	3
Carbonic anhydrase 7	0	1	0	3
Corticosteroid 11-beta-dehydrogenase isozyme 1	0	1	0	1
Cytochrome P450 2J2	0	1	0	1
6-phosphogluconate dehydrogenase, decarboxylating	0	1	0	1
Spike glycoprotein	2	0	0	2
Holo-[acyl-carrier-protein] synthase	0	1	0	1
Solute carrier organic anion transporter family member 2A1	0	1	0	1
Carbonic anhydrase 9	0	1	0	3
Canalicular multispecific organic anion transporter 1	0	7	0	7
Multidrug and toxin extrusion protein 1	0	1	0	1
Carbonic anhydrase 13	0	1	0	3
G-protein coupled receptor 35	0	1	1	2
CDGSH iron-sulfur domain-containing protein 1	0	2	0	2
Carbonic anhydrase 14	0	1	0	3
Broad substrate specificity ATP-binding cassette transporter ABCG2	0	1	0	1
Carbonic anhydrase 5B, mitochondrial	0	1	0	3
USP1 protein, partial	2	0	0	2
importin subunit beta-1 isoform 1	1	0	0	1
snurportin-1	1	0	0	1
GTP-binding nuclear protein Ran isoform 1	1	0	0	1
Chain A, JmjC domain-containing histone demethylation protein 3A	1	0	0	1
Luciferase	2	0	0	2
hypoxia-inducible factor 1 alpha subunit	3	0	0	3
euchromatic histone-lysine N-methyltransferase 2	3	0	0	3
Polyphenol oxidase 2	0	2	0	3
Dopamine beta-hydroxylase	0	2	0	2
Lactoperoxidase	0	1	0	1
Lanosterol 14-alpha demethylase	0	1	0	1
acetylcholinesterase	1	0	0	1
hypoxia-inducible factor 1, alpha subunit (basic helix-loop-helix transcription factor)	2	0	0	2
nonstructural protein 1	2	0	0	2
bromodomain adjacent to zinc finger domain 2B	2	0	0	2
nuclear receptor subfamily 1, group I, member 2	2	0	0	2
muscleblind-like protein 1 isoform 1	1	0	0	1
Glucocorticoid receptor	0	2	0	2
Glycine receptor subunit alpha-1	0	2	0	2
Androgen receptor	0	2	0	2
Glycine receptor subunit beta	0	2	0	2
Glycine receptor subunit alpha-2	0	2	0	2
Glycine receptor subunit alpha-3	0	2	0	2
Nuclear receptor subfamily 1 group I member 2	0	0	1	1
ATP-dependent phosphofructokinase	2	0	0	2
Chain A, Putative fructose-1,6-bisphosphate aldolase	1	0	0	1
thyroid stimulating hormone receptor	1	0	0	1
cytochrome P450 2C19 precursor	1	0	0	1
chromobox protein homolog 1	2	0	0	2
peptidyl-prolyl cis-trans isomerase NIMA-interacting 1	1	0	0	1
NADH-cytochrome b5 reductase 3	0	1	0	1
Myeloperoxidase	0	1	0	1
Thyroid peroxidase	0	1	0	1
Taste receptor type 2 member 38	0	0	1	2
potassium voltage-gated channel subfamily H member 2 isoform d	1	0	0	1
cGMP-specific 3',5'-cyclic phosphodiesterase	0	1	0	1
Chain A, Nuclear Receptor ROR-beta	0	1	0	1
Chain A, 2-oxoglutarate Oxygenase	1	0	0	1
Chain A, Ferritin light chain	1	0	0	1
thioredoxin reductase	1	0	0	1
pregnane X receptor	1	0	0	1
GLS protein	1	0	0	1
Microtubule-associated protein tau	1	0	0	1
Parkin	1	0	0	1
Caspase-7	1	0	0	1
Bloom syndrome protein isoform 1	1	0	0	1
caspase-3	1	0	0	1
DNA polymerase kappa isoform 1	1	0	0	1
cytochrome P450 3A4 isoform 1	1	0	0	1
M-phase phosphoprotein 8	1	0	0	1
histone acetyltransferase KAT2A isoform 1	1	0	0	1
Gamma-aminobutyric acid receptor subunit pi	1	0	0	1
Ornithine decarboxylase	0	0	0	1
Amyloid-beta precursor protein	0	1	0	1
Cytochrome P450 1A2	0	0	1	1
Ornithine decarboxylase	0	0	0	1
60 kDa chaperonin	0	1	0	1
Retinoic acid receptor alpha	0	2	2	7
Cytochrome P450 2D6	0	0	1	1
60 kDa heat shock protein, mitochondrial	0	1	0	1
Retinoic acid receptor beta	0	2	2	7
Retinoic acid receptor alpha	0	1	2	3
Retinoic acid receptor gamma	0	2	2	7
Gamma-aminobutyric acid receptor subunit beta-1	1	0	0	1
Alpha-1B adrenergic receptor	0	2	0	2
Alpha-1A adrenergic receptor	0	1	0	1
Gamma-aminobutyric acid receptor subunit delta	1	0	0	1
Gamma-aminobutyric acid receptor subunit gamma-2	1	0	0	1
Retinoic acid receptor gamma	0	1	2	3
5-hydroxytryptamine receptor 1A	0	1	0	1
Retinoic acid receptor RXR-alpha	0	2	2	4
Gamma-aminobutyric acid receptor subunit alpha-5	1	0	0	1
Gamma-aminobutyric acid receptor subunit alpha-3	1	0	0	1
Retinoic acid receptor beta	0	1	2	3
Cellular retinoic acid-binding protein 2	0	0	1	1
Gamma-aminobutyric acid receptor subunit gamma-1	1	0	0	1
Gamma-aminobutyric acid receptor subunit alpha-2	1	0	0	1
Alpha-1D adrenergic receptor	0	1	0	1
Gamma-aminobutyric acid receptor subunit alpha-4	1	0	0	1
Gamma-aminobutyric acid receptor subunit gamma-3	1	0	0	1
Mitogen-activated protein kinase 1	0	1	0	1
Retinoic acid receptor RXR-alpha	0	1	2	3
Retinoic acid receptor RXR-beta	0	2	2	4
Retinoic acid receptor RXR-beta	0	1	2	3
Retinoic acid receptor RXR-gamma	0	1	2	3
Indoleamine 2,3-dioxygenase 1	0	1	0	1
Gamma-aminobutyric acid receptor subunit alpha-6	1	0	0	1
Cytochrome P450 2C19	0	0	1	1
Nuclear receptor ROR-alpha	0	1	0	1
Alpha-synuclein	0	1	0	1
Cellular retinoic acid-binding protein 1	0	1	0	2
5-hydroxytryptamine receptor 2B	0	2	0	2
Alpha-1A adrenergic receptor	0	2	0	2
Retinoic acid receptor RXR-gamma	0	2	2	4
10 kDa heat shock protein, mitochondrial	0	1	0	1
Gamma-aminobutyric acid receptor subunit alpha-1	1	0	0	1
Cellular retinoic acid-binding protein 1	0	0	1	1
Gamma-aminobutyric acid receptor subunit beta-3	1	0	0	1
Gamma-aminobutyric acid receptor subunit beta-2	1	0	0	1
Peptidyl-prolyl cis-trans isomerase NIMA-interacting 1	0	2	1	3
Thiosulfate sulfurtransferase	0	1	0	1
60 kDa chaperonin	0	1	0	1
10 kDa chaperonin	0	1	0	1
Indoleamine 2,3-dioxygenase 2	0	1	0	1
GABA theta subunit	1	0	0	1
Nuclear receptor ROR-beta	0	1	0	1
Gamma-aminobutyric acid receptor subunit epsilon	1	0	0	1
[prepared from compound, protein, and bioassay information from National Library of Medicine (NLM), extracted Dec-2023]

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