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Atrial situs ambiguous

MedGen UID:
756837
•Concept ID:
C3164429
•
Congenital Abnormality
Synonym: Atrial situs ambiguus
SNOMED CT: Atrial situs ambiguus (448681000); Indeterminate atrial arrangement (448681000)
 
HPO: HP:0011539

Definition

Common atrium without defining morphologic features. [from HPO]

Conditions with this feature

Heterotaxy, visceral, 8, autosomal
MedGen UID:
934635
•Concept ID:
C4310668
•
Disease or Syndrome
Autosomal visceral heterotaxy-8 (HTX8) is an autosomal recessive developmental disorder characterized by visceral situs inversus associated with complex congenital heart malformations caused by defects in the normal left-right asymmetric positioning of internal organs (summary by Vetrini et al., 2016). For a discussion of the genetic heterogeneity of visceral heterotaxy, see HTX1 (306955).
See: Condition Record
Heterotaxy, visceral, 8, autosomal

Professional guidelines

PubMed

Prenatal Diagnosis and Postnatal Outcome of Eight Cases with Criss-Cross Heart - A Multicenter Case Series.
Vorisek CN, Kurkevych A, Kuhn V, Stessig R, Ritgen J, Degenhardt J, Enzensberger C, Wolter A, Götte M, Khalil M, Akintürk H, Axt-Fliedner R
Ultraschall Med 2022 Dec;43(6):e90-e97. Epub 2020 Jul 16 doi: 10.1055/a-1205-0289. PMID: 32674186

Recent clinical studies

Etiology

Prenatal Diagnosis and Postnatal Outcome of Eight Cases with Criss-Cross Heart - A Multicenter Case Series.
Vorisek CN, Kurkevych A, Kuhn V, Stessig R, Ritgen J, Degenhardt J, Enzensberger C, Wolter A, Götte M, Khalil M, Akintürk H, Axt-Fliedner R
Ultraschall Med 2022 Dec;43(6):e90-e97. Epub 2020 Jul 16 doi: 10.1055/a-1205-0289. PMID: 32674186
Ductus arteriosus location in relation to aortic arch position, branching pattern, and viscero-atrial situs.
Sobh DM, Batouty NM, Abdelwahab RM, El-Badrawy A, Tawfik AM
Clin Radiol 2019 Sep;74(9):732.e1-732.e8. Epub 2019 Jun 12 doi: 10.1016/j.crad.2019.05.007. PMID: 31200931
Cardiac and Non-Cardiac Abnormalities in Heterotaxy Syndrome.
Mishra S
Indian J Pediatr 2015 Dec;82(12):1135-46. Epub 2015 Nov 26 doi: 10.1007/s12098-015-1925-x. PMID: 26612104
Efficacy of MRI in complicated congenital heart disease with visceral heterotaxy syndrome.
Hong YK, Park YW, Ryu SJ, Won JW, Choi JY, Sul JH, Lee SK, Cho BK, Choe KO
J Comput Assist Tomogr 2000 Sep-Oct;24(5):671-82. doi: 10.1097/00004728-200009000-00002. PMID: 11045684
Surgical experience with unroofed coronary sinus.
Quaegebeur J, Kirklin JW, Pacifico AD, Bargeron LM Jr
Ann Thorac Surg 1979 May;27(5):418-25. doi: 10.1016/s0003-4975(10)63339-5. PMID: 454016

Diagnosis

Prenatal Diagnosis and Postnatal Outcome of Eight Cases with Criss-Cross Heart - A Multicenter Case Series.
Vorisek CN, Kurkevych A, Kuhn V, Stessig R, Ritgen J, Degenhardt J, Enzensberger C, Wolter A, Götte M, Khalil M, Akintürk H, Axt-Fliedner R
Ultraschall Med 2022 Dec;43(6):e90-e97. Epub 2020 Jul 16 doi: 10.1055/a-1205-0289. PMID: 32674186
Abnormalities of Fetal Situs: An Overview and Literature Review.
Lambert TE, Kuller J, Small M, Rhee E, Barker P
Obstet Gynecol Surv 2016 Jan;71(1):33-8. doi: 10.1097/OGX.0000000000000262. PMID: 26819133
Cardiac and Non-Cardiac Abnormalities in Heterotaxy Syndrome.
Mishra S
Indian J Pediatr 2015 Dec;82(12):1135-46. Epub 2015 Nov 26 doi: 10.1007/s12098-015-1925-x. PMID: 26612104
Segmental approach to imaging of congenital heart disease.
Lapierre C, Déry J, Guérin R, Viremouneix L, Dubois J, Garel L
Radiographics 2010 Mar;30(2):397-411. doi: 10.1148/rg.302095112. PMID: 20228325
Clinical implications of atrial isomerism.
Chiu IS, How SW, Wang JK, Wu MH, Chu SH, Lue HC, Hung CR
Br Heart J 1988 Jul;60(1):72-7. doi: 10.1136/hrt.60.1.72. PMID: 3408620Free PMC Article

Prognosis

Prenatal Diagnosis and Postnatal Outcome of Eight Cases with Criss-Cross Heart - A Multicenter Case Series.
Vorisek CN, Kurkevych A, Kuhn V, Stessig R, Ritgen J, Degenhardt J, Enzensberger C, Wolter A, Götte M, Khalil M, Akintürk H, Axt-Fliedner R
Ultraschall Med 2022 Dec;43(6):e90-e97. Epub 2020 Jul 16 doi: 10.1055/a-1205-0289. PMID: 32674186
Left superior vena cava: cross-sectional imaging overview.
Batouty NM, Sobh DM, Gadelhak B, Sobh HM, Mahmoud W, Tawfik AM
Radiol Med 2020 Mar;125(3):237-246. Epub 2019 Dec 10 doi: 10.1007/s11547-019-01114-9. PMID: 31823296
Abnormalities of Fetal Situs: An Overview and Literature Review.
Lambert TE, Kuller J, Small M, Rhee E, Barker P
Obstet Gynecol Surv 2016 Jan;71(1):33-8. doi: 10.1097/OGX.0000000000000262. PMID: 26819133
Cardiac and Non-Cardiac Abnormalities in Heterotaxy Syndrome.
Mishra S
Indian J Pediatr 2015 Dec;82(12):1135-46. Epub 2015 Nov 26 doi: 10.1007/s12098-015-1925-x. PMID: 26612104
Clinical implications of atrial isomerism.
Chiu IS, How SW, Wang JK, Wu MH, Chu SH, Lue HC, Hung CR
Br Heart J 1988 Jul;60(1):72-7. doi: 10.1136/hrt.60.1.72. PMID: 3408620Free PMC Article

Clinical prediction guides

Prenatal Diagnosis and Postnatal Outcome of Eight Cases with Criss-Cross Heart - A Multicenter Case Series.
Vorisek CN, Kurkevych A, Kuhn V, Stessig R, Ritgen J, Degenhardt J, Enzensberger C, Wolter A, Götte M, Khalil M, Akintürk H, Axt-Fliedner R
Ultraschall Med 2022 Dec;43(6):e90-e97. Epub 2020 Jul 16 doi: 10.1055/a-1205-0289. PMID: 32674186
Ductus arteriosus location in relation to aortic arch position, branching pattern, and viscero-atrial situs.
Sobh DM, Batouty NM, Abdelwahab RM, El-Badrawy A, Tawfik AM
Clin Radiol 2019 Sep;74(9):732.e1-732.e8. Epub 2019 Jun 12 doi: 10.1016/j.crad.2019.05.007. PMID: 31200931
Segmental approach to imaging of congenital heart disease.
Lapierre C, Déry J, Guérin R, Viremouneix L, Dubois J, Garel L
Radiographics 2010 Mar;30(2):397-411. doi: 10.1148/rg.302095112. PMID: 20228325
Two-dimensional echocardiographic assessment of dextrocardia: a segmental approach.
Huhta JC, Hagler DJ, Seward JB, Tajik AJ, Julsrud PR, Ritter DG
Am J Cardiol 1982 Dec;50(6):1351-60. doi: 10.1016/0002-9149(82)90474-x. PMID: 7148713

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