Congenital Heart Defect: Truncus Arteriosus
24 Questions
0 Views

Congenital Heart Defect: Truncus Arteriosus

Created by
@FlexibleExpressionism

Podcast Beta

Play an AI-generated podcast conversation about this lesson

Questions and Answers

What is the primary characteristic of truncus arteriosus?

  • Multiple distinct valves control blood flow from the heart
  • The presence of two pulmonary trunks
  • A single common artery arises from the heart (correct)
  • A separate pulmonary artery and aorta arise from the heart

    • What defect is commonly associated with truncus arteriosus?

  • Left ventricular hypertrophy
  • Patency of the ductus arteriosus
  • Atrial Septal Defect (ASD)
  • Ventricular Septal Defect (VSD) (correct)

    • What structural abnormality can occur with the truncal valve in truncus arteriosus?

  • It has only two cusps making it ineffective
  • It may have regurgitation or stenosis (correct)
  • It is always malformed leading to total atrioventricular block
  • It always remains normal without complications

    • Where do the pulmonary arteries arise in truncus arteriosus?

    <p>From the truncus arteriosus</p> Signup and view all the answers

    Which classification system is based on the pulmonary artery origin in truncus arteriosus?

    <p>Cellet &amp; Edwards classification</p> Signup and view all the answers

    What embryological failure leads to truncus arteriosus?

    <p>Failure of aorticopulmonary septum to develop</p> Signup and view all the answers

    Which echo finding is indicative of truncus arteriosus?

    <p>Dilated single great artery overriding both ventricles</p> Signup and view all the answers

    What is a common associated anomaly in patients with truncus arteriosus?

    <p>DiGeorge syndrome</p> Signup and view all the answers

    What is the definition of Congenitally Corrected Transposition of the Great Arteries (CC-TGA)?

    <p>A rare condition with switched ventricles and great arteries, but corrected blood flow.</p> Signup and view all the answers

    Which statement best describes the anatomy involved in CC-TGA?

    <p>Right ventricle is systemic and left ventricle is subpulmonary.</p> Signup and view all the answers

    Which is a key echocardiographic feature of CC-TGA?

    <p>Discordant atrioventricular and ventriculo-arterial connections.</p> Signup and view all the answers

    What is the primary treatment for heart failure associated with CC-TGA?

    <p>Emphasis on reducing afterload.</p> Signup and view all the answers

    What is the primary physiological consequence of d-Transposition of the Great Arteries (d-TGA)?

    <p>Cyanosis due to parallel circulation without oxygenation</p> Signup and view all the answers

    Which associated anomaly has the highest percentage in patients with CC-TGA?

    <p>Ventricular Septal Defect (VSD).</p> Signup and view all the answers

    Which of the following surgical procedures is the most preferred treatment for d-TGA?

    <p>Arterial switch (Jatene)</p> Signup and view all the answers

    What is the most common presentation symptom in infants with d-TGA?

    <p>Cyanosis on the first day of life</p> Signup and view all the answers

    What common feature is associated with the physiologic aspect of CC-TGA?

    <p>Hemodynamically corrected but structurally abnormal.</p> Signup and view all the answers

    What is a potential surgical procedure for correcting CC-TGA?

    <p>Double Switch Operation.</p> Signup and view all the answers

    Which shunt is necessary for survival in infants diagnosed with d-TGA?

    <p>Ductus arteriosus (PDA)</p> Signup and view all the answers

    Which of the following statements about the incidence of CC-TGA is true?

    <p>CC-TGA incidence is 0.5% of all congenital heart defects.</p> Signup and view all the answers

    Which anomaly is NOT typically associated with d-TGA?

    <p>Aortic coarctation</p> Signup and view all the answers

    What condition may result from complications of the atrial switch procedure in d-TGA?

    <p>All of the above</p> Signup and view all the answers

    What defines the anatomical abnormality in d-TGA?

    <p>Discordant vascular connections with normal AV connections</p> Signup and view all the answers

    What is a common initial treatment to stabilize an infant with d-TGA?

    <p>Prostaglandin E1 administration</p> Signup and view all the answers

    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.

    Studying That Suits You

    Use AI to generate personalized quizzes and flashcards to suit your learning preferences.

    Quiz Team

    Related Documents

    Truncus Arteriosus PDF
    d-TGA & CC-TGA PDF

    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.

    More Like This

    Use Quizgecko on...
    Browser
    Open
    Browser
    Browser