Dextrocardia pathophysiology: Difference between revisions
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{{Dextrocardia}} | {{Dextrocardia}} | ||
{{CMG}}; {{AE}} | {{CMG}}; {{AE}} {{Jose}} | ||
==Overview== | ==Overview== | ||
The exact pathogenesis of [ | 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== | ==Pathophysiology== | ||
===Pathogenesis=== | ===Pathogenesis=== | ||
* | * It may be caused by an abnormality in the cardiac formation, after the [[heart tube]] has been formed; | ||
* | * After its formation, there is the looping of the [[heart tube]] which will determine the position of the [[ventricle]] and the [[atria]].<ref name="pmid32310534">{{cite journal| author=| title=StatPearls | journal= | year= 2021 | volume= | issue= | pages= | pmid=32310534 | doi= | pmc= | url= }} </ref> | ||
* | * If it loops to the right (D-loop), the [[right ventricle]] is located to the right of the [[left ventricle]]. | ||
* If it loops to the left (L-loop), the [[right ventricle]] is located to the left of the [[left ventricle]]. | |||
* Another possibility is a normal D-looped [[heart]] failing to migrate to the [[left hemithorax]], which may cause a normal [[heart]] located in the [[right hemithorax]]. If an L-looped [[heart]] completes rotation in the [[right hemithorax]], it can become situated in the [[right hemithorax]]. | |||
* It may be associated with defect in [[dynein]] or [[cilia]].<ref name="pmid32310534">{{cite journal| author=| title=StatPearls | journal= | year= 2021 | volume= | issue= | pages= | pmid=32310534 | doi= | pmc= | url= }} </ref> | |||
==Genetics== | ==Genetics== | ||
* It may be associated with a genetic condition called [[primary ciliary dyskinesia]], which affects genes [[DNAI1]] and [[DNAH5]];<ref name="pmid19410201">{{cite journal| author=Escudier E, Duquesnoy P, Papon JF, Amselem S| title=Ciliary defects and genetics of primary ciliary dyskinesia. | journal=Paediatr Respir Rev | year= 2009 | volume= 10 | issue= 2 | pages= 51-4 | pmid=19410201 | doi=10.1016/j.prrv.2009.02.001 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19410201 }} </ref> | |||
* | * Other genes that may be involved in the pathogenesis of dextrocardia include: [[ZIC3]], [[ACVR2B]], [[NODAL]].<ref name="pmid21864452">{{cite journal| author=Ma L, Selamet Tierney ES, Lee T, Lanzano P, Chung WK| title=Mutations in ZIC3 and ACVR2B are a common cause of heterotaxy and associated cardiovascular anomalies. | journal=Cardiol Young | year= 2012 | volume= 22 | issue= 2 | pages= 194-201 | pmid=21864452 | doi=10.1017/S1047951111001181 | pmc=3678527 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21864452 }} </ref> | ||
* In many instances other genetic causes are unknown. | |||
==Associated Conditions== | ==Associated Conditions== | ||
Conditions associated with [ | Conditions associated with [[dextrocardia]] include:<ref name="pmid27330607">{{cite journal| author=Khoury M, Harbieh B, Heriopian A| title=Isolated dextrocardia and congenital heart blocking. | journal=Radiol Case Rep | year= 2013 | volume= 8 | issue= 1 | pages= 521 | pmid=27330607 | doi=10.2484/rcr.v8i1.521 | pmc=4900208 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27330607 }} </ref><ref name="pmid22605582">{{cite journal| author=Panneerselvam A, Subbiahnadar P| title=Is it dextrocardia or dextroversion? | journal=BMJ Case Rep | year= 2012 | volume= 2012 | issue= | pages= | pmid=22605582 | doi=10.1136/bcr.01.2012.5493 | pmc=3316784 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22605582 }} </ref><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="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> | ||
*Tricuspid atresia | *[[Tricuspid atresia]] | ||
*Transposition of the great vessels | *[[Transposition of the great vessels]] | ||
*Pulmonary stenosis | *[[Pulmonary stenosis]] | ||
*Double-outlet double-inlet ventricle | *Double-outlet double-inlet ventricle | ||
*Single ventricle | *[[Single ventricle]] | ||
*Sick sinus syndrome | *[[Sick sinus syndrome]] | ||
*Situs inversus | *[[Situs inversus]] | ||
*Situs solitus | *[[Situs solitus]] | ||
*Situs ambiguous | *[[Situs ambiguous]] | ||
*Kartagener syndrome | *[[Kartagener syndrome]] | ||
*Double-outlet or double-inlet ventricles | *Double-outlet or double-inlet ventricles | ||
Latest revision as of 21:04, 3 July 2021
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: José Eduardo Riceto Loyola Junior, M.D.[2]
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
Pathogenesis
- It may be caused by an abnormality in the cardiac formation, after the heart tube has been formed;
- After its formation, there is the looping of the heart tube which will determine the position of the ventricle and the atria.[1]
- If it loops to the right (D-loop), the right ventricle is located to the right of the left ventricle.
- If it loops to the left (L-loop), the right ventricle is located to the left of the left ventricle.
- Another possibility is a normal D-looped heart failing to migrate to the left hemithorax, which may cause a normal heart located in the right hemithorax. If an L-looped heart completes rotation in the right hemithorax, it can become situated in the right hemithorax.
- It may be associated with defect in dynein or cilia.[1]
Genetics
- It may be associated with a genetic condition called primary ciliary dyskinesia, which affects genes DNAI1 and DNAH5;[2]
- Other genes that may be involved in the pathogenesis of dextrocardia include: ZIC3, ACVR2B, NODAL.[3]
- In many instances other genetic causes are unknown.
Associated Conditions
Conditions associated with dextrocardia include:[4][5][6][7]
- 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
References
- ↑ 1.0 1.1 "StatPearls". 2021. PMID 32310534 Check
|pmid=value (help). - ↑ Escudier E, Duquesnoy P, Papon JF, Amselem S (2009). "Ciliary defects and genetics of primary ciliary dyskinesia". Paediatr Respir Rev. 10 (2): 51–4. doi:10.1016/j.prrv.2009.02.001. PMID 19410201.
- ↑ Ma L, Selamet Tierney ES, Lee T, Lanzano P, Chung WK (2012). "Mutations in ZIC3 and ACVR2B are a common cause of heterotaxy and associated cardiovascular anomalies". Cardiol Young. 22 (2): 194–201. doi:10.1017/S1047951111001181. PMC 3678527. PMID 21864452.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.