Dextrocardia pathophysiology

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief:


The exact pathogenesis of [disease name] is not fully understood.


It is thought that [disease name] is the result of / is mediated by / is produced by / is caused by either [hypothesis 1], [hypothesis 2], or [hypothesis 3].


[Pathogen name] is usually transmitted via the [transmission route] route to the human host.


Following transmission/ingestion, the [pathogen] uses the [entry site] to invade the [cell name] cell.


[Disease or malignancy name] arises from [cell name]s, which are [cell type] cells that are normally involved in [function of cells].


The progression to [disease name] usually involves the [molecular pathway].


The pathophysiology of [disease/malignancy] depends on the histological subtype.



  • The fetal heart forms from an embryonic heart tube. The heart tube is formed by fusion of the endocardial tubes.[1][2][3]
  • The cranial portion of the heart tube attaches to the arterial trunk and the caudal connects to the venous channels.
  • The next step after the formation of the heart tube is looping which dictates the position of the ventricle in relationship to the atria. If the heart tube loops to the right (D-loop), the morphologic right ventricle is positioned to the right of the left ventricle. If it loops to the left (L-loop), the morphologic right ventricle is positioned to the left of the left ventricle.


  • Dextrocardia is a cardiac anomaly in which the major axis of the heart from base to apex points to the right side, in contrast to the normal orientation of the heart where the apex points to the left side. The term dextrocardia outlines the position of the cardiac axis only and not the chamber organisation and structural anatomy of the heart.[4][5]
  • The malposition is not as a result of any extracardiac abnormalities but intrinsic to the heart. It is as a result of embryological abnormalities that occur during the development of the heart.[6]
  • Failure of migration of the D-bulboventricular loop into the left hemithorax can result in dextrocardia with the heart in the right hemithorax. Also, the complete rotation of the L-bulboventricular loop in the right hemithorax can result in the heart situated in the right hemithorax.
  • Dextrocardia with a normal abdominal situs has a 90 to 95% chance of associated congenital cardiac anomalies including atrial septal defect, transposition of the great vessels, and ventricular septal defect. It has a lower incidence (0 to 10%) in the presence of situs inversus.[7]


Genes involved in the pathogenesis of dextrocrdia include:[8]

  • ZIC3
  • ACVR2B

Associated Conditions

Conditions associated with [disease name] include:[9][10][4][6]

  • Tricuspid atresia
  • Transposition of the great vessels
  • Pulmonary stenosis
  • Double-outlet double-inlet ventricle
  • Single ventricle
  • Sick sinus syndrome
  • Situs inversus
  • Situs solitus
  • Situs ambiguous
  • Kartagener syndrome
  • Double-outlet or double-inlet ventricles


  1. Perloff JK (2011). "The cardiac malpositions". Am J Cardiol. 108 (9): 1352–61. doi:10.1016/j.amjcard.2011.06.055. PMID 21861958.
  2. De la Cruz MV, Anselmi G, Munos-Castellanos L, Nadal-Ginard B, Munoz-Armas S (1971). "Systematization and embryological and anatomical study of mirror-image dextrocardias, dextroversions, and laevoversions". Br Heart J. 33 (6): 841–53. doi:10.1136/hrt.33.6.841. PMC 458437. PMID 5120229.
  3. Angelini P (1995). "Embryology and congenital heart disease". Tex Heart Inst J. 22 (1): 1–12. PMC 325204. PMID 7787459.
  4. 4.0 4.1 Ogunlade O, Ayoka AO, Akomolafe RO, Akinsomisoye OS, Irinoye AI, Ajao A; et al. (2015). "The role of electrocardiogram in the diagnosis of dextrocardia with mirror image atrial arrangement and ventricular position in a young adult Nigerian in Ile-Ife: a case report". J Med Case Rep. 9: 222. doi:10.1186/s13256-015-0695-4. PMC 4584464. PMID 26411880.
  5. Bernasconi A, Azancot A, Simpson JM, Jones A, Sharland GK (2005). "Fetal dextrocardia: diagnosis and outcome in two tertiary centres". Heart. 91 (12): 1590–4. doi:10.1136/hrt.2004.048330. PMC 1769217. PMID 16287744.
  6. 6.0 6.1 Maldjian, Pierre D.; Saric, Muhamed (2007). "Approach to Dextrocardia in Adults:Review". American Journal of Roentgenology. 188 (6_supplement): S39–S49. doi:10.2214/AJR.06.1179. ISSN 0361-803X.
  7. Kumar, Abnish; Singh, Manoj Kumar; Yadav, Neeraj (2014). "Dextrocardia and asplenia in situs inversus totalis in a baby: a case report". Journal of Medical Case Reports. 8 (1). doi:10.1186/1752-1947-8-408. ISSN 1752-1947.
  8. Fahed, Akl C.; Gelb, Bruce D.; Seidman, J. G.; Seidman, Christine E. (2013). "Genetics of Congenital Heart Disease". Circulation Research. 112 (4): 707–720. doi:10.1161/CIRCRESAHA.112.300853. ISSN 0009-7330.
  9. Khoury M, Harbieh B, Heriopian A (2013). "Isolated dextrocardia and congenital heart blocking". Radiol Case Rep. 8 (1): 521. doi:10.2484/rcr.v8i1.521. PMC 4900208. PMID 27330607.
  10. Panneerselvam A, Subbiahnadar P (2012). "Is it dextrocardia or dextroversion?". BMJ Case Rep. 2012. doi:10.1136/bcr.01.2012.5493. PMC 3316784. PMID 22605582.

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