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Abnormal atrial arrangement

MedGen UID:
488996
•Concept ID:
C2959688
•
Congenital Abnormality
Synonym: Abnormal location of heart atrium
SNOMED CT: Abnormal atrial arrangement (445898001)
 
HPO: HP:0011535

Definition

Abnormality of the spatial relationship of the atria to other components of the heart. [from HPO]

Professional guidelines

PubMed

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
Prenatal diagnosis of criss-cross heart: sonographical and pathological features of five cases.
Li S, Luo G, Norwitz ER, Wang C, Ouyang S, Yao Y, Wen H, Chen C, Fu Q, Xia X, Bi J, Zhu J
J Perinatol 2013 Feb;33(2):98-102. Epub 2012 May 3 doi: 10.1038/jp.2012.56. PMID: 22555779
Prenatal diagnosis of topsy-turvy heart.
Jaeggi E, Chitayat D, Golding F, Kim P, Yoo SJ
Cardiol Young 2008 Jun;18(3):337-42. Epub 2008 May 6 doi: 10.1017/S1047951108002308. PMID: 18460225

Recent clinical studies

Etiology

Situs Ambiguus Is Associated With Adverse Clinical Outcomes in Children With Primary Ciliary Dyskinesia.
Kaspy KR, Dell SD, Davis SD, Ferkol TW, Rosenfeld M, Sagel SD, Milla C, Olivier KN, Barber AT, Wee W, Lin FC, Li L, Rampakakis E, Zariwala MA, Knowles MR, Leigh MW, Shapiro AJ
Chest 2024 May;165(5):1070-1081. Epub 2023 Dec 9 doi: 10.1016/j.chest.2023.12.005. PMID: 38072392Free PMC Article
Comparing Long-Term Sequelae of the Systemic Right Ventricle: An Overview of Single Versus Biventricular Arrangements.
Fuller S
Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2022;25:2-10. doi: 10.1053/j.pcsu.2022.05.002. PMID: 35835513
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
Atrial septal defects, supravalvular aortic stenosis and syndromes predisposing to aneurysm of large vessels.
Baglivo M, Dassati S, Krasi G, Fanelli F, Kurti D, Bonelli A, Arabia G, Fabbicatore D, Muneretto C, Bertelli M
Acta Biomed 2019 Sep 30;90(10-S):53-57. doi: 10.23750/abm.v90i10-S.8760. PMID: 31577255Free PMC Article

Diagnosis

Situs Ambiguus Is Associated With Adverse Clinical Outcomes in Children With Primary Ciliary Dyskinesia.
Kaspy KR, Dell SD, Davis SD, Ferkol TW, Rosenfeld M, Sagel SD, Milla C, Olivier KN, Barber AT, Wee W, Lin FC, Li L, Rampakakis E, Zariwala MA, Knowles MR, Leigh MW, Shapiro AJ
Chest 2024 May;165(5):1070-1081. Epub 2023 Dec 9 doi: 10.1016/j.chest.2023.12.005. PMID: 38072392Free PMC Article
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
Atrial septal defects, supravalvular aortic stenosis and syndromes predisposing to aneurysm of large vessels.
Baglivo M, Dassati S, Krasi G, Fanelli F, Kurti D, Bonelli A, Arabia G, Fabbicatore D, Muneretto C, Bertelli M
Acta Biomed 2019 Sep 30;90(10-S):53-57. doi: 10.23750/abm.v90i10-S.8760. PMID: 31577255Free PMC Article
Ivemark Syndrome.
Masiwal P, Chenthil KS, Priyadarsini B, Gnanaprakasam J, Srihari I
J Assoc Physicians India 2016 May;64(5):73-75. PMID: 27735157
Isomerism or heterotaxy: which term leads to better understanding?
Loomba RS, Hlavacek AM, Spicer DE, Anderson RH
Cardiol Young 2015 Aug;25(6):1037-43. Epub 2015 Jun 19 doi: 10.1017/S1047951115001122. PMID: 26088959

Therapy

Situs Ambiguus Is Associated With Adverse Clinical Outcomes in Children With Primary Ciliary Dyskinesia.
Kaspy KR, Dell SD, Davis SD, Ferkol TW, Rosenfeld M, Sagel SD, Milla C, Olivier KN, Barber AT, Wee W, Lin FC, Li L, Rampakakis E, Zariwala MA, Knowles MR, Leigh MW, Shapiro AJ
Chest 2024 May;165(5):1070-1081. Epub 2023 Dec 9 doi: 10.1016/j.chest.2023.12.005. PMID: 38072392Free PMC Article
Conduction velocity is reduced in the posterior wall of hypertrophic cardiomyopathy patients with normal bipolar voltage undergoing ablation for paroxysmal atrial fibrillation.
Zahid S, Malik T, Peterson C, Tarabanis C, Dai M, Katz M, Bernstein SA, Barbhaiya C, Park DS, Knotts RJ, Holmes DS, Kushnir A, Aizer A, Chinitz LA, Jankelson L
J Interv Card Electrophysiol 2024 Jan;67(1):203-210. Epub 2023 Mar 23 doi: 10.1007/s10840-023-01533-9. PMID: 36952090
The Need for Surgery After Vascular or Cardiac Trauma, or Technical Adverse Events in the Congenital Cardiac Catheterization Laboratory.
Holzer R, Batlivala SP, Boe B, Goldstein B, Gudausky T, Hasan B, O'Byrne M, Quinn B, Sathanadam S, Shahanavaz S, Trucco SM, Zampi JD, Bergersen L
Pediatr Cardiol 2023 Apr;44(4):795-805. Epub 2023 Feb 20 doi: 10.1007/s00246-023-03126-9. PMID: 36806971
Unusual long survival in a case of heterotaxy and polysplenia.
Doello K, Conde V, Perez MC, Mendoza I, Mesas C, Prados J
Surg Radiol Anat 2021 Apr;43(4):607-611. Epub 2020 Sep 29 doi: 10.1007/s00276-020-02586-5. PMID: 32995936
Double-decker repair of partial anomalous pulmonary venous return into the superior vena cava.
Hongu H, Yamagishi M, Maeda Y, Itatani K, Asada S, Fujita S, Yaku H
J Thorac Cardiovasc Surg 2019 May;157(5):1970-1977. Epub 2019 Jan 26 doi: 10.1016/j.jtcvs.2019.01.057. PMID: 30824350

Prognosis

Situs Ambiguus Is Associated With Adverse Clinical Outcomes in Children With Primary Ciliary Dyskinesia.
Kaspy KR, Dell SD, Davis SD, Ferkol TW, Rosenfeld M, Sagel SD, Milla C, Olivier KN, Barber AT, Wee W, Lin FC, Li L, Rampakakis E, Zariwala MA, Knowles MR, Leigh MW, Shapiro AJ
Chest 2024 May;165(5):1070-1081. Epub 2023 Dec 9 doi: 10.1016/j.chest.2023.12.005. PMID: 38072392Free PMC Article
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
Atrial septal defects, supravalvular aortic stenosis and syndromes predisposing to aneurysm of large vessels.
Baglivo M, Dassati S, Krasi G, Fanelli F, Kurti D, Bonelli A, Arabia G, Fabbicatore D, Muneretto C, Bertelli M
Acta Biomed 2019 Sep 30;90(10-S):53-57. doi: 10.23750/abm.v90i10-S.8760. PMID: 31577255Free PMC Article
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
Right heart and pulmonary vessels structure and function.
D'Alto M, Scognamiglio G, Dimopoulos K, Bossone E, Vizza D, Romeo E, Vonk-Noordergraaf A, Gaine S, Peacock A, Naeije R
Echocardiography 2015 Jan;32 Suppl 1:S3-10. Epub 2014 Sep 19 doi: 10.1111/echo.12227. PMID: 25244348

Clinical prediction guides

Situs Ambiguus Is Associated With Adverse Clinical Outcomes in Children With Primary Ciliary Dyskinesia.
Kaspy KR, Dell SD, Davis SD, Ferkol TW, Rosenfeld M, Sagel SD, Milla C, Olivier KN, Barber AT, Wee W, Lin FC, Li L, Rampakakis E, Zariwala MA, Knowles MR, Leigh MW, Shapiro AJ
Chest 2024 May;165(5):1070-1081. Epub 2023 Dec 9 doi: 10.1016/j.chest.2023.12.005. PMID: 38072392Free PMC Article
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
Long-term survival and phenotypic spectrum in heterotaxy syndrome: A 25-year follow-up experience.
Baban A, Cantarutti N, Adorisio R, Lombardi R, Calcagni G, Piano Mortari E, Dallapiccola B, Marino B, Iorio FS, Carsetti R, Digilio MC, Giannico S, Drago F, Carotti A
Int J Cardiol 2018 Oct 1;268:100-105. doi: 10.1016/j.ijcard.2018.02.050. PMID: 30041775
Right heart and pulmonary vessels structure and function.
D'Alto M, Scognamiglio G, Dimopoulos K, Bossone E, Vizza D, Romeo E, Vonk-Noordergraaf A, Gaine S, Peacock A, Naeije R
Echocardiography 2015 Jan;32 Suppl 1:S3-10. Epub 2014 Sep 19 doi: 10.1111/echo.12227. PMID: 25244348
Echocardiographic characteristics of the criss-cross heart.
Yang YL, Wang XF, Cheng TO, Xie MX, Lü Q, He L, Lu XF, Wang J, Li L, Anderson RH
Int J Cardiol 2010 Apr 15;140(2):133-7. Epub 2009 Sep 16 doi: 10.1016/j.ijcard.2009.08.039. PMID: 19762095

Recent systematic reviews

Lead-I ECG for detecting atrial fibrillation in patients with an irregular pulse using single time point testing: a systematic review and economic evaluation.
Duarte R, Stainthorpe A, Greenhalgh J, Richardson M, Nevitt S, Mahon J, Kotas E, Boland A, Thom H, Marshall T, Hall M, Takwoingi Y
Health Technol Assess 2020 Jan;24(3):1-164. doi: 10.3310/hta24030. PMID: 31933471Free PMC Article

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