Heterotaxy Syndrome

"Heterotaxy Syndrome" is a descriptor in the National Library of Medicine's controlled vocabulary thesaurus, MeSH (Medical Subject Headings). Descriptors are arranged in a hierarchical structure, which enables searching at various levels of specificity.

MeSH information

Definition | Details | More General Concepts | Related Concepts | More Specific Concepts

Abnormal thoracoabdominal VISCERA arrangement (visceral heterotaxy) or malformation that involves additional CONGENITAL HEART DEFECTS (e.g., heart isomerism; DEXTROCARDIA) and/or abnormal SPLEEN (e.g., asplenia and polysplenia). Irregularities with the central nervous system, the skeleton and urinary tract are often associated with the syndrome.

Descriptor ID	D059446
MeSH Number(s)	C14.240.400.592 C14.280.400.592 C15.604.744.146 C16.131.077.401 C16.131.240.400.592
Concept/Terms	Heterotaxy Syndrome Heterotaxy Syndrome Heterotaxy Syndromes Syndrome, Heterotaxy Syndromes, Heterotaxy Visceral Heterotaxy Visceral Heterotaxy Heterotaxies, Visceral Heterotaxy, Visceral Visceral Heterotaxies Situs Ambiguus Viscerum Ambiguus Viscerum, Situs Ambiguus Viscerums, Situs Situs Ambiguus Viscerums Viscerum, Situs Ambiguus Viscerums, Situs Ambiguus Situs Ambiguus Ambiguus, Situs Polysplenia Syndrome Polysplenia Syndrome Polysplenia Syndromes Syndrome, Polysplenia Syndromes, Polysplenia Situs Ambiguus with Polysplenia Left Atrial Isomerism with Polysplenia Right Atrial Isomerism Right Atrial Isomerism Atrial Isomerism, Right Atrial Isomerisms, Right Isomerism, Right Atrial Isomerisms, Right Atrial Right Atrial Isomerisms Asplenia Syndrome Asplenia Syndrome Asplenia Syndromes Syndrome, Asplenia Syndromes, Asplenia Situs Ambiguus with Asplenia Right Atrial Isomerism with Asplenia Asplenia with Cardiovascular Anomalies Ivemark Syndrome Syndrome, Ivemark Left Atrial Isomerism Left Atrial Isomerism Atrial Isomerism, Left Atrial Isomerisms, Left Isomerism, Left Atrial Isomerisms, Left Atrial Left Atrial Isomerisms

Below are MeSH descriptors whose meaning is more general than "Heterotaxy Syndrome".

Diseases [C]
Cardiovascular Diseases [C14]
Cardiovascular Abnormalities [C14.240]
Heart Defects, Congenital [C14.240.400]
Heterotaxy Syndrome [C14.240.400.592]
Heart Diseases [C14.280]
Heart Defects, Congenital [C14.280.400]
Heterotaxy Syndrome [C14.280.400.592]
Hemic and Lymphatic Diseases [C15]
Lymphatic Diseases [C15.604]
Splenic Diseases [C15.604.744]
Heterotaxy Syndrome [C15.604.744.146]
Congenital, Hereditary, and Neonatal Diseases and Abnormalities [C16]
Congenital Abnormalities [C16.131]
Abnormalities, Multiple [C16.131.077]
Heterotaxy Syndrome [C16.131.077.401]
Cardiovascular Abnormalities [C16.131.240]
Heart Defects, Congenital [C16.131.240.400]
Heterotaxy Syndrome [C16.131.240.400.592]

Below are MeSH descriptors whose meaning is related to "Heterotaxy Syndrome".

Below are MeSH descriptors whose meaning is more specific than "Heterotaxy Syndrome".

publications

Timeline | Most Recent

This graph shows the total number of publications written about "Heterotaxy Syndrome" by people in this website by year, and whether "Heterotaxy Syndrome" was a major or minor topic of these publications.

Bar chart showing 18 publications over 9 distinct years, with a maximum of 4 publications in 2019

To see the data from this visualization as text, click here.

Year	Major Topic	Minor Topic	Total
2011	1	1	2
2012	1	2	3
2014	0	1	1
2015	2	0	2
2016	2	1	3
2017	1	0	1
2018	1	0	1
2019	4	0	4
2021	1	0	1

Below are the most recent publications written about "Heterotaxy Syndrome" by people in Profiles.

Spigel ZA, Edmunds EE, Caldarone CA, Hickey EJ, Binsalamah ZM, Heinle JS. Total anomalous pulmonary venous connection: Influence of heterotaxy and venous obstruction on outcomes. J Thorac Cardiovasc Surg. 2022 Feb; 163(2):387-395.e3.

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Lahiri S, Wang Y, Caldarone CA, Morris SA. Trends in Infant Mortality After TAPVR Repair over 18?Years in Texas and Impact of Hospital Surgical Volume. Pediatr Cardiol. 2020 Jan; 41(1):77-87.

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Broda CR, Salciccioli KB, Lopez KN, Ermis PR, Moodie DS, Dickerson HA. Outcomes in adults with congenital heart disease and heterotaxy syndrome: A single-center experience. Congenit Heart Dis. 2019 Nov; 14(6):885-894.

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Loomba RS, Frommelt PC, Anderson RH, Flores S, Tweddell JS. Timing of Fontan Completion in Children with Functionally Univentricular Hearts and Isomerism: The Impact of Age, Weight, and Pre-Fontan Arterial Oxygen Saturation. Pediatr Cardiol. 2019 Apr; 40(4):753-761.

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Li AH, Hanchard NA, Azamian M, D'Alessandro LCA, Coban-Akdemir Z, Lopez KN, Hall NJ, Dickerson H, Nicosia A, Fernbach S, Boone PM, Gambin T, Karaca E, Gu S, Yuan B, Jhangiani SN, Doddapaneni H, Hu J, Dinh H, Jayaseelan J, Muzny D, Lalani S, Towbin J, Penny D, Fraser C, Martin J, Lupski JR, Gibbs RA, Boerwinkle E, Ware SM, Belmont JW. Genetic architecture of laterality defects revealed by whole exome sequencing. Eur J Hum Genet. 2019 04; 27(4):563-573.

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Bove KE, Thrasher AD, Anders R, Chung CT, Cummings OW, Finegold MJ, Finn L, Ranganathan S, Kim GE, Lovell M, Magid MS, Melin-Aldana H, Russo P, Shehata B, Wang L, White F, Chen Z, Spino C, Magee JC. Inflammation, Active Fibroplasia, and End-stage Fibrosis in 172 Biliary Atresia Remnants Correlate Poorly With Age at Kasai Portoenterostomy, Visceral Heterotaxy, and Outcome. Am J Surg Pathol. 2018 12; 42(12):1625-1635.

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Niu MC, Dickerson HA, Moore JA, de la Uz C, Valdés SO, Kim JJ, Bard DE, Morris SA, Miyake CY. Heterotaxy syndrome and associated arrhythmias in pediatric patients. Heart Rhythm. 2018 04; 15(4):548-554.

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Cowan JR, Tariq M, Shaw C, Rao M, Belmont JW, Lalani SR, Smolarek TA, Ware SM. Copy number variation as a genetic basis for heterotaxy and heterotaxy-spectrum congenital heart defects. Philos Trans R Soc Lond B Biol Sci. 2016 12 19; 371(1710).

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Vetrini F, D'Alessandro LC, Akdemir ZC, Braxton A, Azamian MS, Eldomery MK, Miller K, Kois C, Sack V, Shur N, Rijhsinghani A, Chandarana J, Ding Y, Holtzman J, Jhangiani SN, Muzny DM, Gibbs RA, Eng CM, Hanchard NA, Harel T, Rosenfeld JA, Belmont JW, Lupski JR, Yang Y. Bi-allelic Mutations in PKD1L1 Are Associated with Laterality Defects in Humans. Am J Hum Genet. 2016 Oct 06; 99(4):886-893.

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Loomba RS, Geddes GC, Basel D, Benson DW, Leuthner SR, Hehir DA, Ghanayem N, Shillingford AJ. Bacteremia in Patients with Heterotaxy: A Review and Implications for Management. Congenit Heart Dis. 2016 Dec; 11(6):537-547.

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