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Questions and Answers
What is the primary characteristic of truncus arteriosus?
What is the primary characteristic of truncus arteriosus?
What defect is commonly associated with truncus arteriosus?
What defect is commonly associated with truncus arteriosus?
What structural abnormality can occur with the truncal valve in truncus arteriosus?
What structural abnormality can occur with the truncal valve in truncus arteriosus?
Where do the pulmonary arteries arise in truncus arteriosus?
Where do the pulmonary arteries arise in truncus arteriosus?
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Which classification system is based on the pulmonary artery origin in truncus arteriosus?
Which classification system is based on the pulmonary artery origin in truncus arteriosus?
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What embryological failure leads to truncus arteriosus?
What embryological failure leads to truncus arteriosus?
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Which echo finding is indicative of truncus arteriosus?
Which echo finding is indicative of truncus arteriosus?
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What is a common associated anomaly in patients with truncus arteriosus?
What is a common associated anomaly in patients with truncus arteriosus?
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What is the definition of Congenitally Corrected Transposition of the Great Arteries (CC-TGA)?
What is the definition of Congenitally Corrected Transposition of the Great Arteries (CC-TGA)?
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Which statement best describes the anatomy involved in CC-TGA?
Which statement best describes the anatomy involved in CC-TGA?
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Which is a key echocardiographic feature of CC-TGA?
Which is a key echocardiographic feature of CC-TGA?
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What is the primary treatment for heart failure associated with CC-TGA?
What is the primary treatment for heart failure associated with CC-TGA?
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What is the primary physiological consequence of d-Transposition of the Great Arteries (d-TGA)?
What is the primary physiological consequence of d-Transposition of the Great Arteries (d-TGA)?
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Which associated anomaly has the highest percentage in patients with CC-TGA?
Which associated anomaly has the highest percentage in patients with CC-TGA?
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Which of the following surgical procedures is the most preferred treatment for d-TGA?
Which of the following surgical procedures is the most preferred treatment for d-TGA?
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What is the most common presentation symptom in infants with d-TGA?
What is the most common presentation symptom in infants with d-TGA?
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What common feature is associated with the physiologic aspect of CC-TGA?
What common feature is associated with the physiologic aspect of CC-TGA?
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What is a potential surgical procedure for correcting CC-TGA?
What is a potential surgical procedure for correcting CC-TGA?
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Which shunt is necessary for survival in infants diagnosed with d-TGA?
Which shunt is necessary for survival in infants diagnosed with d-TGA?
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Which of the following statements about the incidence of CC-TGA is true?
Which of the following statements about the incidence of CC-TGA is true?
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Which anomaly is NOT typically associated with d-TGA?
Which anomaly is NOT typically associated with d-TGA?
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What condition may result from complications of the atrial switch procedure in d-TGA?
What condition may result from complications of the atrial switch procedure in d-TGA?
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What defines the anatomical abnormality in d-TGA?
What defines the anatomical abnormality in d-TGA?
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What is a common initial treatment to stabilize an infant with d-TGA?
What is a common initial treatment to stabilize an infant with d-TGA?
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Study Notes
Truncus Arteriosus
- Rare congenital heart defect (CHD) where a single artery (truncus) arises from the heart instead of separate pulmonary artery and aorta.
- Mixed oxygenated and deoxygenated blood is circulated to the systemic and pulmonary systems.
Key Features
- Single Great Artery: A single artery overrides both ventricles, supplying blood to coronary arteries, pulmonary arteries, and systemic circulation.
- Ventricular Septal Defect (VSD): Large, nonrestrictive VSD present, allowing communication between the right and left ventricles.
- Truncal Valve: A single valve, often with 3 cusps (can have 2 or 4), located at the root of the truncus arteriosus. This valve may have regurgitation or stenosis.
- Pulmonary Arteries: Pulmonary arteries arise from the truncus arteriosus, not a separate pulmonary trunk. Location and origin vary depending on the type of truncus.
- Aortic Arch: Aortic arch may have variations, including a right-sided aortic arch in 1/3 of patients.
Associated Defects
- Right aortic arch
- Secundum atrial septal defect (ASD)
- Coronary artery anomalies
- DiGeorge syndrome
- Interrupted aortic arch
Embryology
- Failure of the aorticopulmonary septum to fully develop and divide the common truncus arteriosus into the aorta and pulmonary trunk.
Echo Findings
- Dilated single great artery overriding both ventricles.
- Large, unrestricted VSD.
- Assessment of pulmonary artery anatomy and truncal valve morphology (cusp number, regurgitation, stenosis).
- Possible presence of a right aortic arch or coronary anomalies.
Classification Systems
Collet & Edwards
- Type 1: Main pulmonary artery (MPA) branches into left and right pulmonary arteries.
- Type 2: Separate pulmonary arteries branch close to the truncus.
Van Praagh
- Type 1: Portion of MPA present, branches into pulmonary arteries.
- Type 2: No MPA, separate branch pulmonary arteries arise from the truncus.
- Type 3: Only one pulmonary artery branch arises from the truncus.
d-Transposition of the Great Arteries (d-TGA)
- d-TGA is a congenital heart defect where the aorta arises from the right ventricle, and the pulmonary artery arises from the left ventricle.
- This results in two separate circulatory loops, one systemic and one pulmonary.
- The aorta is anterior and right, while the pulmonary artery is posterior and left.
- Requires a shunt (patent foramen ovale, atrial septal defect, patent ductus arteriosus) for life.
- Presents with cyanosis within the first day of life due to parallel circulatory systems.
- May hear systolic murmurs due to VSD or outflow tract obstruction.
Associated Anomalies in d-TGA
- Ventricular septal defect (VSD)
- Coronary artery anomalies
- Pulmonary stenosis
- Left ventricular outflow tract obstruction (LVOTO)
- Atrial septal defect (ASD)
- Patent ductus arteriosus (PDA)
Echo Features in d-TGA
- Abnormal ventriculo-arterial connections
- Parallel great arteries
- Associated shunts
Surgical Procedures for d-TGA
- Balloon septostomy
- Atrial switch (Mustard/Senning)
- Arterial switch (Jatene)
d-TGA Initial Stabilization
- Prostaglandin E1: keeps the ductus arteriosus open
- Balloon septostomy: creates or enlarges a shunt
d-TGA Atrial Switch
- Reroutes systemic and pulmonary venous return
- Corrects blood flow at the atrial level.
- Complications: right ventricular dysfunction, tricuspid regurgitation (TR), baffle leaks, arrhythmias.
d-TGA Arterial Switch
- Preferred surgery
- Switches the aorta and pulmonary artery to restore normal circulation
- Current preferred surgery
- Corrects ventriculo-arterial connections
- Complications: coronary artery obstruction and stenosis at anastomosis sites.
Incidence
- 3% of all congenital heart defects
- 20-22 per 100,000 live births
Key Features
- Parallel circulations with no oxygenation without a shunt.
- Profound cyanosis at birth.
- Echo: Parallel great arteries, rightward aorta, abnormal ventriculo-arterial connections.
Associated Lesions
- VSD
- Coronary artery anomalies
- Coarctation
- ASD.
Treatment
- Medical: Prostaglandin E1 to keep ductus arteriosus open, balloon septostomy
- Surgical: Atrial switch (Mustard or Senning), Arterial switch (Jatene), Lecompte maneuver.
Congenitally Corrected Transposition of the Great Arteries (CC-TGA)
- AV and VA discordance, but corrected circulation.
- Ventricles are inverted: right ventricle (on the left) is systemic, and the left ventricle (on the right) is subpulmonary.
CC-TGA Etiology
- Results from abnormal (L-looping) embryologic development.
CC-TGA Physiology
- Hemodynamically corrected, but structurally abnormal.
- May lead to right ventricular failure.
Associated Anomalies in CC-TGA
- VSD (60%)
- Tricuspid valve abnormalities (up to 90%)
- Left ventricular outflow tract obstruction.
Echocardiographic Features in CC-TGA
- Discordant atrioventricular and ventriculo-arterial connections.
- Systemic RV connected to the aorta.
Surgical Procedures for CC-TGA
- Double Switch: Combines atrial and arterial switch to redirect blood flow and make the left ventricle the systemic ventricle.
CC-TGA Incidence
- 0.5% of all congenital heart defects.
CC-TGA Key Features
- Asymptomatic early in life.
- Can be undiagnosed until adulthood.
- Echo: Discordant atrioventricular and ventriculo-arterial connections.
- Right ventricle becomes systemic and the left ventricle is subpulmonary.
Associated Lesions in CC-TGA
- VSD
- Systemic tricuspid valve abnormalities
- Left ventricular outflow tract obstruction.
CC-TGA Treatment
- Medical: Treat heart failure, reduce afterload
- Surgical: Tricuspid valve repair or replacement.
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Description
Explore the complex nature of truncus arteriosus, a rare congenital heart defect characterized by a single great artery supplying both systemic and pulmonary circulation. This quiz delves into its key features, including the presence of a ventricular septal defect and truncal valve abnormalities. Test your knowledge on this unique cardiovascular condition and its implications.
