Dextrocardia pathophysiology: Difference between revisions

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==Pathophysiology==
==Pathophysiology==
===Embryology===
===Embryology===
*The fetal heart forms from an [[embryonic heart tub]]e. The [[heart tube]] is formed by fusion of the [[endocardial tubes]].<ref name="pmid21861958">{{cite journal| author=Perloff JK| title=The cardiac malpositions. | journal=Am J Cardiol | year= 2011 | volume= 108 | issue= 9 | pages= 1352-61 | pmid=21861958 | doi=10.1016/j.amjcard.2011.06.055 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21861958  }} </ref><ref name="pmid5120229">{{cite journal| author=De la Cruz MV, Anselmi G, Munos-Castellanos L, Nadal-Ginard B, Munoz-Armas S| title=Systematization and embryological and anatomical study of mirror-image dextrocardias, dextroversions, and laevoversions. | journal=Br Heart J | year= 1971 | volume= 33 | issue= 6 | pages= 841-53 | pmid=5120229 | doi=10.1136/hrt.33.6.841 | pmc=458437 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=5120229  }} </ref><ref name="pmid7787459">{{cite journal| author=Angelini P| title=Embryology and congenital heart disease. | journal=Tex Heart Inst J | year= 1995 | volume= 22 | issue= 1 | pages= 1-12 | pmid=7787459 | doi= | pmc=325204 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=7787459  }} </ref>
 
*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]].


===Pathogenesis===
===Pathogenesis===
*[[Dextrocardia]] is a cardiac anomaly in which the major axis of the [[hear]]t 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]].<ref name="pmid26411880">{{cite journal| author=Ogunlade O, Ayoka AO, Akomolafe RO, Akinsomisoye OS, Irinoye AI, Ajao A | display-authors=etal| title=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. | journal=J Med Case Rep | year= 2015 | volume= 9 | issue=  | pages= 222 | pmid=26411880 | doi=10.1186/s13256-015-0695-4 | pmc=4584464 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26411880  }} </ref><ref name="pmid16287744">{{cite journal| author=Bernasconi A, Azancot A, Simpson JM, Jones A, Sharland GK| title=Fetal dextrocardia: diagnosis and outcome in two tertiary centres. | journal=Heart | year= 2005 | volume= 91 | issue= 12 | pages= 1590-4 | pmid=16287744 | doi=10.1136/hrt.2004.048330 | pmc=1769217 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16287744  }} </ref>
 
*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.<ref name="MaldjianSaric2007">{{cite journal|last1=Maldjian|first1=Pierre D.|last2=Saric|first2=Muhamed|title=Approach to Dextrocardia in Adults:Review|journal=American Journal of Roentgenology|volume=188|issue=6_supplement|year=2007|pages=S39–S49|issn=0361-803X|doi=10.2214/AJR.06.1179}}</ref>
*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.<ref name="KumarSingh2014">{{cite journal|last1=Kumar|first1=Abnish|last2=Singh|first2=Manoj Kumar|last3=Yadav|first3=Neeraj|title=Dextrocardia and asplenia in situs inversus totalis in a baby: a case report|journal=Journal of Medical Case Reports|volume=8|issue=1|year=2014|issn=1752-1947|doi=10.1186/1752-1947-8-408}}</ref>


==Genetics==
==Genetics==
Genes involved in the pathogenesis of dextrocardia include:<ref name="FahedGelb2013">{{cite journal|last1=Fahed|first1=Akl C.|last2=Gelb|first2=Bruce D.|last3=Seidman|first3=J. G.|last4=Seidman|first4=Christine E.|title=Genetics of Congenital Heart Disease|journal=Circulation Research|volume=112|issue=4|year=2013|pages=707–720|issn=0009-7330|doi=10.1161/CIRCRESAHA.112.300853}}</ref>
 
*[[ZIC3]]
*[[ACVR2B]]
*[[NODAL]]


==Associated Conditions==
==Associated Conditions==

Revision as of 21:57, 29 June 2021

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

Overview

The exact pathogenesis of dextrocardia is not fully understood. 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. Genes involved in the pathogenesis of dextrocardia include: ZIC3, ACVR2B, NODAL.

Pathophysiology

Embryology

Pathogenesis

Genetics

Associated Conditions

Conditions associated with [disease name] include:[1][2][3][4]

References

  1. 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.
  2. 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.
  3. 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.
  4. 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.

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