Transposition of the great arteries: Difference between revisions
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==Background== | ==Background== | ||
[[File:D-tga-575px.jpg|thumb|Dextro-transposition of the great arteries.]] | |||
[[File:D-TGA.jpg|thumb|]] | |||
*Type of cyanotic [[congenital heart disease]] | *Type of cyanotic [[congenital heart disease]] | ||
*Aorta arises from the right ventricle and the pulmonary artery from the left ventricle | *Aorta arises from the right ventricle and the pulmonary artery from the left ventricle | ||
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**The second sends oxygenated pulmonary venous blood to the left atrium and back to the lungs via the left ventricle and pulmonary artery | **The second sends oxygenated pulmonary venous blood to the left atrium and back to the lungs via the left ventricle and pulmonary artery | ||
*Incompatible with life unless there is communication between the two parallel circuits | *Incompatible with life unless there is communication between the two parallel circuits | ||
**Mixing occurs either intracardiac (patent foramen ovale, [[VSD]] or [[ASD]]) | **Mixing occurs either intracardiac (patent foramen ovale, [[VSD]] or [[ASD]]) '''OR''' extracardiac connections ([[patent ductus arteriosus]] or bronchopulmonary collateral circulation) | ||
*Often associated with other cardiac abnormalities | *Often associated with other cardiac abnormalities | ||
**Ventricular septal | **[[Ventricular septal defect]]s (VSD) | ||
**Left ventricular outflow tract obstruction (dynamic or anatomical) | **Left ventricular outflow tract obstruction (dynamic or anatomical) | ||
**Mitral and tricuspid valve abnormalities | **Mitral and tricuspid valve abnormalities | ||
**Coronary artery variations | **[[coronary artery anomalies|Coronary artery variations]] | ||
*Prevalence in the United States is estimated to be 4.7 per 10,000 live births<ref>Improved national prevalence estimates for 18 selected major birth defects--United States, 1999-2001. MMWR Morb Mortal Wkly Rep. 2006;54(51):1301-5.</ref> | *Prevalence in the United States is estimated to be 4.7 per 10,000 live births<ref>Improved national prevalence estimates for 18 selected major birth defects--United States, 1999-2001. MMWR Morb Mortal Wkly Rep. 2006;54(51):1301-5.</ref> | ||
*Accounts for 3% of all [[congenital heart disease]] and almost 20% of all cyanotic CHD defects <ref>Reller MD. Prevalence of congenital heart defects in metropolitan Atlanta, 1998-2005. J Pediatr. 2008;153(6):807-13.</ref> | *Accounts for 3% of all [[congenital heart disease]] and almost 20% of all cyanotic CHD defects <ref>Reller MD. Prevalence of congenital heart defects in metropolitan Atlanta, 1998-2005. J Pediatr. 2008;153(6):807-13.</ref> | ||
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==Clinical Features== | ==Clinical Features== | ||
*Cyanosis, determined by amount of intercirculatory mixing | *Cyanosis, determined by amount of intercirculatory mixing | ||
*Tachypnea | *[[shortness of breath (peds)|Tachypnea]] | ||
* | *[[Murmur]]s | ||
**Pansystolic murmur at lower left sternal border if there is a VSD | **Pansystolic murmur at lower left sternal border if there is a VSD | ||
**Systolic ejection murmur along the upper left sternal border in patients with left ventricular outflow obstruction | **Systolic ejection murmur along the upper left sternal border in patients with left ventricular outflow obstruction | ||
*Diminished pulses in patients who also have coarctation of the aorta or interruption of the aortic arch | *Diminished pulses in patients who also have [[coarctation of the aorta]] or interruption of the aortic arch | ||
==Differential Diagnosis== | ==Differential Diagnosis== | ||
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==Evaluation== | ==Evaluation== | ||
*Echocardiography | [[File:Transposition-of-great-vessels.jpg|thumb|"Egg on side/string" sign]] | ||
[[File:Transposition great arteries Orphanet 1750-1172-3-27-2.jpg|thumb|Subcostal echo view showing discordant ventriculoarterial connections together with the presence of parallel, rather than crossing, great arteries arising from the ventricles.]] | |||
[[File:Transposition great arteries Orphanet 1750-1172-3-27-1.jpg|thumb|Echocardiogram in transposition of the great arteries. This subcostal view shows the left ventricle giving rise to a vessel that bifurcates, which is thus identified as the pulmonary artery. | |||
Abbreviations: RA=right atrium, RV=right ventricle, LV=left ventricle, PT=pulmonary trunk, LPA and RPA=left and right pulmonary artery.]] | |||
*[[Echocardiography]] | |||
*[[ECG]] | *[[ECG]] | ||
**Initial ECG often normal | **Initial ECG often normal | ||
* | *[[CXR]] | ||
**Classically with “egg on a string” appearance, thought to be a result of the great arteries forming a narrowed vascular pedicle when transposed | **Classically with “egg on a string” appearance, thought to be a result of the great arteries forming a narrowed vascular pedicle when transposed | ||
*Cardiac | *Cardiac catheterization | ||
**Remains the gold standard, but seldom required to make the diagnosis | **Remains the gold standard, but seldom required to make the diagnosis | ||
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**Balloon is placed across the atrial septum into the left atrium, inflated and then pulled vigorously back across the septum to produce an [[ASD]] | **Balloon is placed across the atrial septum into the left atrium, inflated and then pulled vigorously back across the septum to produce an [[ASD]] | ||
*Surgery, Arterial switch operation | *Surgery, Arterial switch operation | ||
**In rare instances, atrial switch operation when coronary artery anatomy makes arterial switch infeasible | |||
**Typically performed within the first two weeks of life | **Typically performed within the first two weeks of life | ||
*Most will die within the first year of life without treatment | *Most will die within the first year of life without treatment | ||
Latest revision as of 16:38, 13 November 2024
Background
- Type of cyanotic congenital heart disease
- Aorta arises from the right ventricle and the pulmonary artery from the left ventricle
- Results in two parallel circulations
- The first sends deoxygenated systemic venous blood to the right atrium and back to the systemic circulation via the right ventricle and aorta
- The second sends oxygenated pulmonary venous blood to the left atrium and back to the lungs via the left ventricle and pulmonary artery
- Incompatible with life unless there is communication between the two parallel circuits
- Mixing occurs either intracardiac (patent foramen ovale, VSD or ASD) OR extracardiac connections (patent ductus arteriosus or bronchopulmonary collateral circulation)
- Often associated with other cardiac abnormalities
- Ventricular septal defects (VSD)
- Left ventricular outflow tract obstruction (dynamic or anatomical)
- Mitral and tricuspid valve abnormalities
- Coronary artery variations
- Prevalence in the United States is estimated to be 4.7 per 10,000 live births[1]
- Accounts for 3% of all congenital heart disease and almost 20% of all cyanotic CHD defects [2]
Clinical Features
- Cyanosis, determined by amount of intercirculatory mixing
- Tachypnea
- Murmurs
- Pansystolic murmur at lower left sternal border if there is a VSD
- Systolic ejection murmur along the upper left sternal border in patients with left ventricular outflow obstruction
- Diminished pulses in patients who also have coarctation of the aorta or interruption of the aortic arch
Differential Diagnosis
Congenital Heart Disease Types
- Cyanotic
- Acyanotic
- AV canal defect
- Atrial septal defect (ASD)
- Ventricular septal defect (VSD)
- Cor triatriatum
- Patent ductus arteriosus (PDA)
- Pulmonary/aortic stenosis
- Coarctation of the aorta
- Differentiation by pulmonary vascularity on CXR[3]
- Increased pulmonary vascularity
- Decreased pulmonary vascularity
- Tetralogy of fallot
- Rare heart diseases with pulmonic stenosis
Evaluation
Echocardiogram in transposition of the great arteries. This subcostal view shows the left ventricle giving rise to a vessel that bifurcates, which is thus identified as the pulmonary artery. Abbreviations: RA=right atrium, RV=right ventricle, LV=left ventricle, PT=pulmonary trunk, LPA and RPA=left and right pulmonary artery.
- Echocardiography
- ECG
- Initial ECG often normal
- CXR
- Classically with “egg on a string” appearance, thought to be a result of the great arteries forming a narrowed vascular pedicle when transposed
- Cardiac catheterization
- Remains the gold standard, but seldom required to make the diagnosis
Management
- Stabilization of cardiac and pulmonary function and ensure adequate systemic oxygenation
- Prostaglandin E1
- Provide sufficient intercirculatory mixing between the two parallel circulations by maintaining patency of the ductus arteriosus
- Start infusion at 0.05 mcg/kg/min IV and titrate up to 0.1 mcg/kg/min, monitoring for hypotension (and apnea)
- Maintains the ductus (which completely seals by ~3 wks)
- Side Effects: Hypotension, Bradycardia, Seizures and Apnea
- Balloon atrial septostomy
- Performed to stabilize patients with severe hypoxemia due to inadequate mixing between the two parallel circuits
- Balloon is placed across the atrial septum into the left atrium, inflated and then pulled vigorously back across the septum to produce an ASD
- Surgery, Arterial switch operation
- In rare instances, atrial switch operation when coronary artery anatomy makes arterial switch infeasible
- Typically performed within the first two weeks of life
- Most will die within the first year of life without treatment
Disposition
- Admit
See Also
External Links
References
- ↑ Improved national prevalence estimates for 18 selected major birth defects--United States, 1999-2001. MMWR Morb Mortal Wkly Rep. 2006;54(51):1301-5.
- ↑ Reller MD. Prevalence of congenital heart defects in metropolitan Atlanta, 1998-2005. J Pediatr. 2008;153(6):807-13.
- ↑ Knipe K et al. Cyanotic congenital heart diseases. Radiopaedia. http://radiopaedia.org/articles/cyanotic-congenital-heart-disease