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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]

Term Hierarchy

GTR
MeSH

CClinical test, RResearch test, OOMIM, GGeneReviews, VClinVar

CROGVAtrial situs ambiguous

Disorder of cardiovascular system
- Abnormality of the cardiovascular system
  - Abnormal cardiovascular system morphology
    - Abnormal heart morphology
      - Abnormal spatial orientation of the cardiac segments
        Abnormal atrial arrangement
        Atrial situs ambiguous

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

Heterotaxy syndrome: Prenatal diagnosis, concomitant malformations and outcomes.

Akalın M, Demirci O, Kumru P, Yücel İK
Prenat Diagn 2022 Apr;42(4):435-446. Epub 2022 Feb 4 doi: 10.1002/pd.6110. PMID: 35102577

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

Human Laterality Disorders: Pathogenesis, Clinical Manifestations, Diagnosis, and Management.

Soofi M, Alpert MA, Barbadora J, Mukerji B, Mukerji V
Am J Med Sci 2021 Sep;362(3):233-242. Epub 2021 May 28 doi: 10.1016/j.amjms.2021.05.020. PMID: 34052215

See all (8)

Recent clinical studies

Etiology

Clinical implications of airway anomalies and stenosis in patients with heterotaxy syndrome.

Wang CC, Wu MH, Wu ET, Lu F, Chen SJ
Pediatr Pulmonol 2022 Sep;57(9):2074-2081. Epub 2022 Jun 2 doi: 10.1002/ppul.25981. PMID: 35582940

The surgical anatomy of hearts with isomeric atrial appendages-implications for surgical management.

Spicer DE, Chowdhury UK, Anderson RH, Pandey NN, Sankhyan LK, George N, Goja S, Malik V
Eur J Cardiothorac Surg 2022 Jun 15;62(1) doi: 10.1093/ejcts/ezac139. PMID: 35234855

Human Laterality Disorders: Pathogenesis, Clinical Manifestations, Diagnosis, and Management.

Soofi M, Alpert MA, Barbadora J, Mukerji B, Mukerji V
Am J Med Sci 2021 Sep;362(3):233-242. Epub 2021 May 28 doi: 10.1016/j.amjms.2021.05.020. PMID: 34052215

The Story of Dr Stella (1927-2006).

Van Praagh R
World J Pediatr Congenit Heart Surg 2019 Jan;10(1):125-128. doi: 10.1177/2150135117705639. PMID: 30799724

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

See all (108)

Diagnosis

Multimodality imaging of developmental splenic anomalies: tips and pitfalls.

Wong A, Fung KFK, Wong WC, Ng KK, Kung BT, Kan YLE
Clin Radiol 2022 May;77(5):319-325. Epub 2022 Jan 7 doi: 10.1016/j.crad.2021.12.014. PMID: 35000764

Prenatal Diagnosis and Postnatal Outcome of Eight Cases with Criss-Cross Heart - A Multicenter Case Series.

Human Laterality Disorders: Pathogenesis, Clinical Manifestations, Diagnosis, and Management.

Soofi M, Alpert MA, Barbadora J, Mukerji B, Mukerji V
Am J Med Sci 2021 Sep;362(3):233-242. Epub 2021 May 28 doi: 10.1016/j.amjms.2021.05.020. PMID: 34052215

The Story of Dr Stella (1927-2006).

Van Praagh R
World J Pediatr Congenit Heart Surg 2019 Jan;10(1):125-128. doi: 10.1177/2150135117705639. PMID: 30799724

Ivemark Syndrome.

Masiwal P, Chenthil KS, Priyadarsini B, Gnanaprakasam J, Srihari I
J Assoc Physicians India 2016 May;64(5):73-75. PMID: 27735157

See all (112)

Therapy

Congenital heart defects caused by FOXJ1.

Padua MB, Helm BM, Wells JR, Smith AM, Bellchambers HM, Sridhar A, Ware SM
Hum Mol Genet 2023 Jul 4;32(14):2335-2346. doi: 10.1093/hmg/ddad065. PMID: 37158461 Free PMC Article

Predictors and Outcomes of Arrhythmia on Stage I Palliation of Single Ventricle Patients.

Czosek RJ, Spar DS, Anderson JB, Khoury PR, Webster G
JACC Clin Electrophysiol 2022 Sep;8(9):1136-1144. Epub 2022 Aug 31 doi: 10.1016/j.jacep.2022.06.010. PMID: 36137719

Post-operative Morbidity and Mortality After Fontan Procedure in Patients with Heterotaxy and Other Situs Anomalies.

Duong SQ, Zaniletti I, Lopez L, Sutherland SM, Shin AY, Collins RT 2nd
Pediatr Cardiol 2022 Jun;43(5):952-959. Epub 2022 Jan 22 doi: 10.1007/s00246-021-02804-w. PMID: 35064275

Primary central pulmonary artery plasty for right atrial isomerism with pulmonary coarctation.

Ishidou M, Hirose K, Ikai A, Sakamoto K
Asian Cardiovasc Thorac Ann 2022 Jun;30(5):540-548. Epub 2021 Sep 15 doi: 10.1177/02184923211045216. PMID: 34524926

Survival into adulthood of patients with atrial isomerism undergoing cardiac surgery.

Bhaskar J, Galati JC, Brooks P, Oppido G, Konstantinov IE, Brizard CP, d'Udekem Y
J Thorac Cardiovasc Surg 2015 Jun;149(6):1509-13. Epub 2015 Jan 24 doi: 10.1016/j.jtcvs.2015.01.038. PMID: 25752370

See all (22)

Prognosis

Human Laterality Disorders: Pathogenesis, Clinical Manifestations, Diagnosis, and Management.

Soofi M, Alpert MA, Barbadora J, Mukerji B, Mukerji V
Am J Med Sci 2021 Sep;362(3):233-242. Epub 2021 May 28 doi: 10.1016/j.amjms.2021.05.020. PMID: 34052215

Isomerism of the atrial appendages: morphology and terminology.

Frescura C, Ho SY, Giordano M, Thiene G
Cardiovasc Pathol 2020 Jul-Aug;47:107205. Epub 2020 Jan 16 doi: 10.1016/j.carpath.2020.107205. PMID: 32179252

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

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

Atrial isomerism: a pictorial review.

Balan A, Lazoura O, Padley SP, Rubens M, Nicol ED
J Cardiovasc Comput Tomogr 2012 Mar-Apr;6(2):127-36. Epub 2012 Jan 28 doi: 10.1016/j.jcct.2011.10.019. PMID: 22398009

See all (83)

Clinical prediction guides

Clinical implications of airway anomalies and stenosis in patients with heterotaxy syndrome.

Wang CC, Wu MH, Wu ET, Lu F, Chen SJ
Pediatr Pulmonol 2022 Sep;57(9):2074-2081. Epub 2022 Jun 2 doi: 10.1002/ppul.25981. PMID: 35582940

The surgical anatomy of hearts with isomeric atrial appendages-implications for surgical management.

Spicer DE, Chowdhury UK, Anderson RH, Pandey NN, Sankhyan LK, George N, Goja S, Malik V
Eur J Cardiothorac Surg 2022 Jun 15;62(1) doi: 10.1093/ejcts/ezac139. PMID: 35234855

Prenatal Diagnosis and Postnatal Outcome of Eight Cases with Criss-Cross Heart - A Multicenter Case Series.

Isomerism of the atrial appendages: morphology and terminology.

Frescura C, Ho SY, Giordano M, Thiene G
Cardiovasc Pathol 2020 Jul-Aug;47:107205. Epub 2020 Jan 16 doi: 10.1016/j.carpath.2020.107205. PMID: 32179252

Atrial isomerism: a pictorial review.

Balan A, Lazoura O, Padley SP, Rubens M, Nicol ED
J Cardiovasc Comput Tomogr 2012 Mar-Apr;6(2):127-36. Epub 2012 Jan 28 doi: 10.1016/j.jcct.2011.10.019. PMID: 22398009

See all (47)

Recent systematic reviews

Outcomes of the Fontan Operation for Patients With Heterotaxy: A Meta-Analysis of 848 Patients.

Marathe SP, Cao JY, Celermajer D, Ayer J, Sholler GF, d'Udekem Y, Winlaw DS
Ann Thorac Surg 2020 Jul;110(1):307-315. Epub 2020 Jan 8 doi: 10.1016/j.athoracsur.2019.11.027. PMID: 31926157

Outcome of prenatally diagnosed fetal heterotaxy: systematic review and meta-analysis.

Buca DIP, Khalil A, Rizzo G, Familiari A, Di Giovanni S, Liberati M, Murgano D, Ricciardulli A, Fanfani F, Scambia G, D'Antonio F
Ultrasound Obstet Gynecol 2018 Mar;51(3):323-330. doi: 10.1002/uog.17546. PMID: 28603940

Chronic Arrhythmias in the Setting of Heterotaxy: Differences between Right and Left Isomerism.

Loomba RS, Willes RJ, Kovach JR, Anderson RH
Congenit Heart Dis 2016 Jan-Feb;11(1):7-18. Epub 2015 Jul 29 doi: 10.1111/chd.12288. PMID: 26219620

See all (3)

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Atrial situs ambiguous

Definition

Term Hierarchy

Conditions with this feature

Professional guidelines

PubMed

Recent clinical studies

Etiology

Diagnosis

Therapy

Prognosis

Clinical prediction guides

Recent systematic reviews

Supplemental Content

Table of contents

Clinical resources

Practice guidelines

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