Questions and Answers
What is the Van Praagh classification?
This classifies the heart into 3 segmental sets: atria/viscera situs, ventricular looping, and arterial situs.
What is the normal relationship of the aorta to the pulmonary artery?
The aorta is normally posterior and to the right of the pulmonary artery in arterial situs solitus.
What is Complete Transposition of the Great Arteries (TGA)?
Physiologic uncorrected ventriculoarterial discordance (the aorta arises from the RV, the PA arises from the LV).
What is the Van Praagh classification for TGA?
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What pathology is the second most common cyanotic lesion?
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What is the embryologic malformation that occurs with TGA?
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What patient population is more susceptible to TGA?
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Circulation in a patient with TGA can be described as which of the following?
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What happens to a baby when they have TGA and their PDA closes?
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List some commonly associated conditions with TGA.
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What are some typical features of TGA?
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What are the surgical treatments for TGA?
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What is the Le Compte maneuver?
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With transposition of the great arteries, which of the following increases systemic oxygen saturation?
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What does the Jatene procedure do?
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The clinical presentation of a child with congenitally corrected transposition of the great arteries is usually dependent on what factor?
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What is congenitally corrected transposition of the great arteries?
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What is the Van Praagh classification for congenitally corrected TGA?
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What is the embryologic malformation that occurs with congenitally corrected TGA?
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What is the most common associated defect with congenitally corrected TGA?
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What treatments are used for congenitally corrected TGA?
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What is double outlet right ventricle?
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What is the 50% rule?
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What is the embryologic malformation that occurs with DORV?
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What are the different great vessel relationships associated with DORV?
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The clinical presentation of DORV is dependent on which of the following?
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What are the different types of DORV? How are they classified?
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What is the most common type of VSD that is considered a remote VSD in DORV?
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What is the most common type of VSD with DORV? What is the least common?
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If a newborn presented with respiratory distress and cyanosis, which congenital abnormality would you presume?
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If a newborn presented with cyanosis but no respiratory distress, which congenital abnormality would you presume?
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What is hypoplastic left heart?
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What is the embryologic malformation that occurs to cause hypoplastic left heart?
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Which congenital abnormalities in this chapter are ductal dependent?
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Which congenital abnormality is the most common cause of death from heart disease during the first week of life?
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What are some associated defects with hypoplastic left heart?
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What is Hypoplastic left heart syndrome (HLHS)?
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How does HLHS differ from Hypoplastic left heart complex (HLHC)?
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What is Hypoplastic left heart complex (HLHC)?
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What is hypoplastic left heart syndrome's path of circulation?
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What is the direction of shunts in hypoplastic left heart syndrome?
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What is surgical treatment for hypoplastic left heart? What does it do?
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What is the progression and complications of hypoplastic left heart?
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What congenital abnormality would be implied if a baby presented with cyanosis at birth with a concave pulmonary artery segment and the apex tilted upward?
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What is the embryologic malformation that occurs with pulmonary atresia with intact IVS?
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What is the path of circulation for pulmonary atresia with intact IVS?
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What are sinusoids?
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What are some associated defects with pulmonary atresia with intact IVS?
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What is right ventricular dependent coronary artery circulation?
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If you have atretic PV with hypoplastic RV & abnormal TV, will you have a higher or lower incidence of sinusoids?
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If you have atretic PV with normal or enlarged RV & significant TR, will you have higher or lower incidence of sinusoids?
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Which direction will the shunt be for pulmonary atresia with intact IVS with the PDA and ASD/PFO?
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What is the Fontan procedure?
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Tricuspid atresia is considered a type of single ventricle.
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What is the embryologic malformation that occurs with tricuspid atresia?
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With tricuspid atresia, which ventricle is the main systemic ventricle?
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What is the embryologic malformation that occurs with single ventricle heart?
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What are some associated conditions with single ventricle heart?
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What is the most common type of single ventricle heart?
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What is heterotaxy?
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What is the variant of single ventricle heart with two separate AV valves and dominant morphologic LV?
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What is a single ventricle heart with one common AV valve and a dominant morphologic RV?
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What is the variant of single ventricle heart with two separate AV valves and a dominant morphologic RV?
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What is a restrictive bulboventricular foramen?
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A patient presents with pulmonary atresia (with intact IVS). The TV appears to be significantly hypoplastic. What is the likelihood you will find cardiac sinusoids?
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What is considered to be hypoplastic aorta?
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Study Notes
Van Praagh Classification
- Classifies hearts into three segmental sets: atria/viscera situs, ventricular looping, and arterial situs.
- A normal heart classification is S,D,S (situs solitus, d-looping, situs solitus).
Aorta and Pulmonary Artery Relationship
- In a normal heart, the aorta is posterior and to the right of the pulmonary artery.
Complete Transposition of the Great Arteries (TGA)
- Characterized by physiologic uncorrected ventriculoarterial discordance.
- Aorta arises from the right ventricle, pulmonary artery from the left ventricle; aorta typically anterior to the pulmonary artery.
Classification for TGA
- Van Praagh classification for TGA is SDD (situs solitus, d-looping, right-sided aorta).
Common Cyanotic Lesion
- TGA is the second most common cyanotic lesion affecting newborns.
Embryologic Malformation in TGA
- Caused by abnormal division of truncus arteriosus, leading to parallel vessel arrangement (0-degree rotation).
Patient Population at Risk for TGA
- Increased susceptibility observed in males with diabetic mothers.
Circulation Description in TGA
- Circulation consists of two parallel circuits, dependent on PDA, VSD, and ASD for systemic oxygenation.
Prognosis with PDA Closure in TGA
- Closure of the PDA can result in sudden death due to lack of oxygenated blood delivery.
Associated Conditions with TGA
- Commonly associated defects include VSD, PDA, coarctation, left ventricular outflow obstruction, and coronary anomalies.
Typical Features of TGA
- More prevalent in males; newborns may exhibit cyanosis without respiratory distress.
Surgical Treatment Options for TGA
- Jatene procedure: arterial switch; Mustard/Senning procedure: atrial switch (less common).
- Palliative treatments include prostaglandins, balloon atrial septostomy, and PA banding.
Le Compte Maneuver
- A surgical procedure that repositions the pulmonary artery to anteriorly place it relative to the aorta in cases of TGA.
Increasing Systemic Oxygen Saturation in TGA
- A left-to-right PFO can enhance systemic oxygen saturation by promoting oxygenated blood flow from the lungs to systemic circulation.
Jatene Procedure
- Focuses on switching the outflows of the aorta and pulmonary artery, without rearranging valves.
Congenitally Corrected Transposition of the Great Arteries
- Exhibits atrioventricular and ventriculoarterial discordance with abnormal positioning of the aorta (anterior and to the left).
Van Praagh Classification for Congenitally Corrected TGA
- Classified as SLL (situs solitus, left looping, left-sided aorta).
Embryologic Malformation in Congenitally Corrected TGA
- Characterized by the primitive heart tube looping left instead of right.
Common Defects with Congenitally Corrected TGA
- Tricuspid abnormalities (~90%), VSD (~75%), and coarctation of the aorta are frequently associated.
Congenitally Corrected TGA Treatment
- Treatment focuses on correcting associated defects, including managing CHF and potential heart transplant.
Double Outlet Right Ventricle (DORV)
- Defined by both great vessels arising predominantly from the right ventricle.
50% Rule in DORV
- Indicates that both the pulmonary artery and over 50% of the aorta derive from the right ventricle.
Embryologic Malformation in DORV
- Stemming from failure to achieve conotruncal inversion and a leftward shift of conus.
Great Vessel Relationships in DORV
- Relationships include normal positioning, side-by-side, dextroposition, and levo positioning.
Clinical Presentation of DORV
- Depends on the location of VSD relative to great arteries and degree of left/right ventricular outflow tract obstruction.
Types of DORV
- Classified based on VSD type (subaortic, doubly committed) and specific circulatory requirements (with or without POTO).
Most Common Type of VSD in DORV
- The subaortic VSD is the most common; the doubly committed VSD is the rarest.
Hypoplastic Left Heart Syndrome (HLHS)
- Characterized by severe underdevelopment of left heart structures, leading to complicated hemodynamics at birth.
Embryologic Malformation in HLHS
- Caused by inadequate development of the left ventricle and aorta.
Ductal Dependent Conditions
- Conditions include HLHS, TGA, and pulmonary atresia with intact ventricular septum.
Leading Cause of Neonatal Death Due to Heart Disease
- HLHS is the most common cause during the first week of life.
Associated Abnormalities with HLHS
- Defects include aortic stenosis, coarctation, VSD, and mitral valve atresia.
Differentiating HLHS and HLHC
- HLHS is associated with valve atresia; HLHC encompasses cases without atresia but still shows significant ventricular hypoplasia.
Surgical Pathway for HLHS
- The Norwood procedure serves as a multistage reconstructive strategy to redirect systemic and pulmonary flow.
Complications of HLHS
- Potential complications include respiratory failure, metabolic acidosis, and multi-organ failure, leading to high mortality without intervention.
Implications of Cyanosis with Distinct Cardiac Features
- Cyanosis at birth with specific pulmonary artery appearances indicates conditions like pulmonary atresia with intact ventricular septum.
Sinusoids in Pulmonary Atresia with Intact IVS
- These are fistulas allowing RV blood to perfuse coronary arteries due to stenosis at the coronary origins.
Essential Components in Pulmonary Atresia with Intact IVS
- Requires a PDA and/or ASD for adequate systemic and pulmonary blood circulation.
Right Ventricular Dependent Coronary Artery Circulation
- Highlights risks associated with pressure fluctuations in the RV, affecting coronary artery perfusion.
Tricuspid Atresia Association
- Recognized as a form of single ventricle heart disease, primarily reliant on the left ventricle for systemic flow.
Single Ventricle Heart Anomalies
- Examples include LV type single ventricle and variant structures like double inlet left/right ventricle, depending on atrioventricular valve morphology.
Restrictive Bulboventricular Foramen
- Identified as a pressure gradient opening between main and outlet chambers in single ventricle hearts.
Hypoplastic Aorta Classification
- Defined as aorta measuring less than 5mm in diameter.
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Description
Explore the classification and implications of Complete Transposition of the Great Arteries (TGA) in this quiz. Understand the Van Praagh classification system, the normal aorta and pulmonary artery relationship, and the embryologic malformations involved. Test your knowledge on cyanotic lesions and their impact on newborns.