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Polysplenia heterotaxy syndrome

MedGen UID:
Concept ID:
Disease or Syndrome
Synonym: Heterotaxy Syndrome with Polysplenia
SNOMED CT: Polysplenia heterotaxy syndrome (8641000119101)


A congenital defect in which there is an abnormal spatial arrangement of the internal thoraco-abdominal organs; this manifestation of the disorder presents with many accessory spleens instead of one. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPolysplenia heterotaxy syndrome

Professional guidelines


Napolitano M, Franchi-Abella S, Damasio MB, Augdal TA, Avni FE, Bruno C, Darge K, Ključevšek D, Littooij AS, Lobo L, Mentzel HJ, Riccabona M, Stafrace S, Toso S, Woźniak MM, Di Leo G, Sardanelli F, Ording Müller LS, Petit P
Pediatr Radiol 2021 Feb;51(2):314-331. Epub 2020 Nov 17 doi: 10.1007/s00247-020-04840-9. PMID: 33201318
Loomba RS, Geddes GC, Basel D, Benson DW, Leuthner SR, Hehir DA, Ghanayem N, Shillingford AJ
Congenit Heart Dis 2016 Dec;11(6):537-547. Epub 2016 Jul 18 doi: 10.1111/chd.12395. PMID: 27425254
Escobar-Diaz MC, Friedman K, Salem Y, Marx GR, Kalish BT, Lafranchi T, Rathod RH, Emani S, Geva T, Tworetzky W
Am J Cardiol 2014 Aug 15;114(4):612-7. Epub 2014 Jun 6 doi: 10.1016/j.amjcard.2014.05.042. PMID: 24996551Free PMC Article

Recent clinical studies


Duarte R, Morais H
Rev Port Cardiol 2015 Jan;34(1):73.e1-3. Epub 2015 Jan 10 doi: 10.1016/j.repc.2014.08.012. PMID: 25583627
Kapa S, Gleeson FC, Vege SS
JOP 2007 Jul 9;8(4):433-7. PMID: 17625295

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