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{{DiseaseDisorder infobox |
__NOTOC__
  Name          = Transposition of the great vessels |
{{Transposition of the great vessels}}
  ICD10          = {{ICD10|Q|21|3|q|20}} |
  ICD9          = {{ICD9|745.2}} |
  ICDO          = |
  Image          = Transposition of the great vessels svg.png |
  Caption        = diagram of a healthy heart and one suffering from Transposition of the great vessels |
  OMIM          = 187500 |
  MedlinePlus    = 001567 |
  eMedicineSubj  = emerg |
  eMedicineTopic = 575 |
  DiseasesDB    = 4660 |
  MeshID        = D013771 |
}}


'''For patient information click [[{{PAGENAME}} (patient information)|here]]'''


'''For patient information click [[Transposition of the great vessels(patient information)|here]]'''
{{CMG}}; '''Associate Editor(s)-In-Chief:''' [[Priyamvada Singh|Priyamvada Singh, M.B.B.S.]] [mailto:psingh13579@gmail.com]; {{CZ}}; [[Kristin Feeney|Kristin Feeney, B.S.]] [mailto:kfeeney@elon.edu] {{ADG}}


{{CMG}}
==[[Transposition of the great vessels overview | Overview ]]==


'''Associate Editors-In-Chief:''' [[User:KeriShafer|Keri Shafer, M.D.]] [mailto:kshafer@bidmc.harvard.edu]; Atif Mohammad, M.D., [[Priyamvada Singh]], [[MBBS]]
==[[Transposition of the great vessels historical perspective | Historical Perspective ]]==
 
===[[Transposition of the great vessels diagram | Diagram ]]===
 
===[[Transposition of the great vessels overview | Overview ]]===
 
==[[Transposition of the great vessels epidemiology and demographics|Epidemiology and demographics]]==
 
==[[Transposition of the great vessels causes|Causes]]==
 
===Transposition of the great arteries===  


==Classification==
*[[Dextro-transposition of the great arteries/complete transposition of the great arteries]]
*[[Dextro-transposition of the great arteries/complete transposition of the great arteries]]
*[[L-transposition of the great arteries or congenitally corrected transposition of the great arteries]]
*[[L-transposition of the great arteries or congenitally corrected transposition of the great arteries]]


==[[Transposition of the great vessels pathophysiology | Pathophysiology ]]==


==[[Transposition of the great vessels causes|Causes ]]==


==[[Transposition of the great arteries differential diagnosis |Differentiating Transposition of the great vessels from other Diseases]]==


==[[Transposition of the great vessels epidemiology and demographics|Epidemiology and Demographics]]==


==[[Transposition of the great vessels risk factors | Risk Factors  ]]==


== L-TGA or Congenitally Corrected Transposition of the Great Arteries (CCTGA)==
==[[Transposition of the great vessels screening| Screening ]]==
{{Infobox_Disease |
  Name          = Levo-Transposition of the great arteries |
  Image          = |
  Caption        = |
  DiseasesDB    = 13259 |
  ICD10          = {{ICD10|Q|20|5|q|20}} |
  ICD9          = {{ICD9|745.12}} |
  ICDO          = |
  OMIM          = |
  MedlinePlus    = |
  eMedicineSubj  = |
  eMedicineTopic = |
  MeshName      = |
  MeshNumber    = |
}}
===Overview===
'''[[wiktionary:laevus|levo]]-Transposition of the great arteries''' ('''l-Transposition of the great arteries''', '''levo-TGA''', or '''l-TGA'''), also commonly referred to as congenitally corrected transposition of the great arteries ('''CC-TGA'''), is an [[acyanotic heart defect|acyanotic]] [[congenital]] [[congenital heart defect|heart defect]] ('''CHD''') in which the primary [[arteries]] (the [[aorta]] and the [[pulmonary artery]]) are [[wiktionary:transpose|transpose]]d, with the aorta anterior and to the left of the pulmonary artery; and the [[wiktionary:morphology|morphological]] left and right [[ventricle (heart)|ventricles]] are also transposed.


Use of the term "corrected" has been disputed by many due to the frequent occurrence of other abnormalities and or [[acquired disorder]]s in l-TGA patients.
==[[Transposition of the great vessels natural History, complications & prognosis | Natural History, Complications and Prognosis]]==


In [[segmental analysis (biology)|segmental analysis]], this condition is described as '''[[wiktionary:atrioventricular|atrioventricular]] discordance''' ('''ventricular inversion''') '''with [[wiktionary:ventriculoarterial|ventriculoarterial]] discordance'''.
==Diagnosis==
[[Transposition of the great vessels history and symptoms|History and Symptoms]] | [[Transposition of the great vessels physical examination|Physical Examination]] | [[Transposition of the great vessels laboratory findings|Laboratory Findings]] | [[Transposition of the great vessels electrocardiogram|Electrocardiogram]] | [[Transposition of the great vessels chest x ray|Chest X Ray]] | [[Transposition of the great vessels CT|CT]] | [[Transposition of the great vessels MRI|MRI]] | [[Transposition of the great vessels echocardiography or ultrasound|Echocardiography or Ultrasound]] | [[Transposition of the great vessels other diagnostic studies|Other Diagnostic Studies]] |


l-TGA is often referred to simply as '''[[transposition of the great arteries]]''' ('''TGA'''); however, TGA is a more general term which may also refer to '''[[dextro-transposition of the great arteries]]''' ('''d-TGA''').
==Treatment==
[[Transposition of the great vessels medical therapy|Medical Therapy]] | [[Transposition of the great vessels palliative care]] | [[Transposition of the great vessels corrective surgery]] | [[Transposition of the great vessels post-operative care]] | [[Transposition of the great vessels follow up]] | [[Transposition of the great vessels prevention|Prevention]]


Another term commonly used to refer to both l-TGA and d-TGA is '''[[transposition of the great vessels]]''' ('''TGV'''), although this term can have an even broader meaning than TGA.
==Case Studies==
[[Transposition of the great vessels case study one|Case #1]]


The letter L in the terms l-TGA or L-TGA refers to the a leftward or Levo [[aorta]], versus the normal dextro or rightward [[aorta]].
==Related Chapters==
*[[Levo-Transposition of the great arteries]]
*[[Transposition of the great arteries]]
*[[Dextro-transposition of the great arteries]]


===Subclassification of TGA===
====Simple and complex l-TGA====
l-TGA is often accompanied by other heart defects, the most common type being [[wiktionary:intracardiac|intracardiac]] [[shunt (medical)|shunts]] such as [[atrial septal defect]] ('''ASD''') including [[patent foramen ovale]] ('''PFO'''), [[ventricular septal defect]] ('''VSD'''), and [[patent ductus arteriosus]] ('''PDA'''). [[Stenosis]] of valves such as [[pulmonary stenosis]] or atresia may also be present. [[Tricuspid regurgitation]] may be present as well.


When no other heart defects are present it is called 'simple' l-TGA; when other defects are present it is called 'complex' l-TGA.
{{Congenital malformations and deformations of circulatory system}}
 
[[Category:Congenital heart disease]]
===Pathophysiology===
[[Category:Cardiology]]
Levo-transposition of the great arteries is a defect in which [[atrial]] and ventricular morphologies are discordant, and also the morphology of each ventricle is discordant with the great artery that comes from it. In other words this anomaly is a "double discordance" with both atrioventricular  and ventriculoarterial discordance, which essentially "corrects" the physiologic abnormality. The atria are in normal position and received appropriate venous return, but the atria are connected to the opposite ventricle ([[RA]] to the [[LV]] and [[LA]] to the [[RV]]). In addition the ventricles are inversely connected to the wrong great artery.
[[Category:Disease]]
 
In a normal [[heart]], [[oxygen]]-depleted ("blue") blood is pumped from the right [[Atrium (heart)|atrium]] into the right ventricle, then through the pulmonary artery to the [[lung]]s where it is oxygenated. The oxygen-rich ("red") blood then returns, via the [[pulmonary veins]], to the left atrium from which it is pumped into the [[left ventricle]], then through the aorta to the rest of the body, including the heart muscle itself.
 
With l-TGA, blue blood is pumped from the [[right atrium]] into the morphological [[left ventricle]] (which lies on the right side of the heart), then through the [[pulmonary artery]] to the [[lungs]]. The red blood then returns, via the [[pulmonary veins]], to the [[left atrium]] from which it is pumped into the morphological [[right ventricle]], then ejected into the [[aorta]].
 
===History===
Congenitally corrected transposition of the great arteries (CCTGA), was first described by Von Rokitansky in 1875.
 
===Epidemiology===
Among patients with congenital heart disease, CCTGA has an incidence of 0.5%, with a slight male predominance.  95% of CCTGA occurs in "situs solitus".
 
===Genetics===
An increased prevalence in families has been reported.
 
===Diagnosis===
'''Simple l-TGA''' may be "accidentally" diagnosed in adulthood, as an incidental finding as part of the evaluation or treatment of other conditions.
 
====Symptoms====
'''Simple l-TGA''' may not yield symptoms in infancy. However, since the morphologic [[right ventricle]] normally functions in a low pressure system, the [[right ventricle]] may eventually hypertrophy due to increased pressure of ejecting into the systemic circulation of the aorta, and produce symptoms such as [[dyspnea]] or [[fatigue (physical)|fatigue]] may develop.
 
'''Complex l-TGA''' in contrast, may be associated with symptoms earlier in the natural history of the disase depending on the nature, degree and number of accompanying defect(s). If a right-to-left or bidirectional shunt is present, the list of signs and symptoms may include mild [[cyanosis]]. Infants and children can present with congestive heart failure [[CHF]], due to a large [[VSD]] or severe [[tricuspid regurgitation]].
 
====Physical Examination====
=====Heart=====
If there is an associated large [[VSD]], or severe [[tricuspid regurgitation]], these may be detected on physical examination.
 
====Laboratory Findings====
=====Chest X ray=====
Depending upon underlying associated defects such as a [[VSD]], or severe [[tricuspid regurgitation]], the chest x ray may be abnormal and consistent with these associated defects.
 
=====Electrocardiogram=====
Premature development of [[heart block]] may be present.
 
=====Echocardiography=====
l-TGA can sometimes be [[diagnose]]d in utero with an [[ultrasound]] after 18 weeks [[gestation]].
 
The following links demonstrate the findings on echocardiography in ccTGA:
 
[http://www.medscape.com/content/2002/00/43/31/433155/case042.l07.answ.html LA-RV RA-LV connections]
 
[http://www.medscape.com/content/2002/00/43/31/433155/case042.l13.answ.html RV-aorta LV-PA connections]
 
===Prognosis===
Simple l-TGA has a very good prognosis. Most patients are [[asymptomatic]] and not require surgical correction.
 
===Treatment===
In some cases, the technically challenging "double switch operation" has been successfully performed to restore the normal blood flow through the appropriate morphologic ventricles.
 
==References==
{{reflist|2}}
 
==Acknowledgements and Initial Contributors to Page==
Leida Perez, M.D.
 
==External links==
*[http://www.kumc.edu/instruction/medicine/pedcard/cardiology/pedcardio/dtgadiagram.gif Diagram at kumc.edu]
*[http://www.med.umich.edu/cvc/mchc/partran.htm Diagram and description at umich.edu]
*[http://www.pediheart.org/practitioners/defects/ventriculoarterial/l-TGA.htm Overview at pediheart.org]
*[http://www.rch.org.au/cardiology/defects.cfm?doc_id=5098 Royal Children's Hospital, Melbourne]
*[http://www.mayoclinic.org/corrected-transposition-great-arteries Mayo Clinic, Arizona - Florida - Minnesota, USA]


[[de:Transposition der großen Arterien]]
[[fr:Transposition des gros vaisseaux]]
[[fr:Transposition des gros vaisseaux]]
[[nl:Transpositie van de grote vaten]]
[[zh:大血管轉位]]
[[Category:DiseaseState]]
[[Category:Cardiology]]
[[Category:Congenital heart disease]]


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Latest revision as of 07:47, 21 October 2020

Transposition of the great vessels Microchapters

Home

Patient Information

Overview

Historical perspective

Classification

Dextro-transposition of the great arteries
L-transposition of the great arteries

Pathophysiology

Causes

Differentiating Transposition of the great vessels from other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications and Prognosis

Diagnosis

History and Symptoms

Physical Examination

Laboratory Findings

Electrocardiogram

Chest X Ray

MRI

CT

Echocardiography

Other Diagnostic Studies

Treatment

Medical Therapy

Surgery

Palliative care
Corrective surgery
Post-operative care
Follow up

Prevention

Reproduction

Case Studies

Case #1

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-In-Chief: Priyamvada Singh, M.B.B.S. [2]; Cafer Zorkun, M.D., Ph.D. [3]; Kristin Feeney, B.S. [4] Aditya Ganti M.B.B.S. [5]

Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Transposition of the great vessels from other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications and Prognosis

Diagnosis

History and Symptoms | Physical Examination | Laboratory Findings | Electrocardiogram | Chest X Ray | CT | MRI | Echocardiography or Ultrasound | Other Diagnostic Studies |

Treatment

Medical Therapy | Transposition of the great vessels palliative care | Transposition of the great vessels corrective surgery | Transposition of the great vessels post-operative care | Transposition of the great vessels follow up | Prevention

Case Studies

Case #1

Related Chapters


de:Transposition der großen Arterien


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