Transposition of the great vessels
Template:DiseaseDisorder infobox
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Associate Editors-In-Chief: Keri Shafer, M.D. [2]; Atif Mohammad, M.D., Priyamvada Singh, MBBS
Diagram
Overview
Epidemiology and demographics
Causes
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-TGA or Congenitally Corrected Transposition of the Great Arteries (CCTGA)
| Levo-Transposition of the great arteries | |
| ICD-10 | Q20.5 |
|---|---|
| ICD-9 | 745.12 |
| DiseasesDB | 13259 |
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 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 diagnosed in utero with an ultrasound after 18 weeks gestation.
The following links demonstrate the findings on echocardiography in ccTGA:
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
Acknowledgements and Initial Contributors to Page
Leida Perez, M.D.
External links
- Diagram at kumc.edu
- Diagram and description at umich.edu
- Overview at pediheart.org
- Royal Children's Hospital, Melbourne
- Mayo Clinic, Arizona - Florida - Minnesota, USA
