Congenital Heart Defects Overview

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Questions and Answers

What is the primary issue associated with an atrial septal defect (ASD)?

  • Increased right ventricular pressure
  • Pulmonary artery stenosis
  • Decreased oxygen saturation in the right atrium
  • Left-to-right shunt of blood (correct)

Which type of atrial septal defect is most commonly isolated?

  • Ostium secundum atrial septal defect (correct)
  • Sinus venosus defect
  • Ostium primum atrial septal defect
  • Common atrium defect

What factor contributes to the increased oxygen saturation in the right atrium due to ASD?

  • Right ventricular hypertrophy
  • Shunting of deoxygenated blood to the left atrium
  • Shunting of oxygenated blood from the left atrium to the right atrium (correct)
  • Decreased pulmonary blood flow

What developmental issue is typically associated with ostium secundum atrial septal defects?

<p>Excessive resorption of the septum primum (A)</p> Signup and view all the answers

What is the primary consequence of atrial septal defect on the heart's anatomy?

<p>Right atrial and right ventricular volume overload (C)</p> Signup and view all the answers

What is the result of the anatomical reversal of the aorta and the pulmonary artery in transposition of the great vessels?

<p>Complete isolation of the pulmonary and systemic circuits (A)</p> Signup and view all the answers

Which statement correctly describes the survival mechanism following birth for infants with transposition of the great vessels?

<p>Survival is only possible with an intracardiac shunt (A)</p> Signup and view all the answers

In the context of cyanotic congenital heart defects, what is a characteristic consequence of the transposition of great vessels?

<p>Decreased oxygenated blood entering systemic circulation (A)</p> Signup and view all the answers

What developmental failure leads to the transposition of the great vessels in fetal heart formation?

<p>Failed spiraling of the aorticopulmonary septum (B)</p> Signup and view all the answers

Which physiological issue arises due to the significant isolation of the pulmonary and systemic circuits in transposition of great vessels?

<p>Insufficient oxygen levels in systemic blood (A)</p> Signup and view all the answers

What is a characteristic outcome of a patent foramen ovale (PFO) that persists beyond one year of age?

<p>Mild left-to-right shunt (B)</p> Signup and view all the answers

Which maneuver can induce a right-to-left shunt in individuals with a patent foramen ovale?

<p>Valsalva maneuver (D)</p> Signup and view all the answers

In coarctation of the aorta, where is stenosis most commonly located?

<p>At the aortic isthmus (D)</p> Signup and view all the answers

What symptom is associated with coarctation of the aorta in patients with stenosis distal to the left subclavian artery?

<p>Hypertension in the upper extremities (D)</p> Signup and view all the answers

What pathological change contributes to the narrowing of the aorta in coarctation?

<p>Tunica medial thickening and intimal hyperplasia (A)</p> Signup and view all the answers

What cardiovascular change occurs as a result of left ventricular outflow obstruction due to aortic coarctation?

<p>Myocardial hypertrophy (B)</p> Signup and view all the answers

Which of the following statements is true regarding hypertension in coarctation of the aorta?

<p>Hypertension is present in the upper extremities and head (B)</p> Signup and view all the answers

Which anatomical structure is primarily affected by the failure of the atrial septum primum to fuse with the septum secundum?

<p>Foramen ovale (B)</p> Signup and view all the answers

What proportion of babies born with congenital heart defects also have a chromosomal abnormality?

<p>12% (A)</p> Signup and view all the answers

Which of the following congenital heart defects is classified as an acyanotic heart defect?

<p>Ventricular septal defect (C)</p> Signup and view all the answers

What type of cardiac shunting occurs in cyanotic heart defects?

<p>Right-to-left shunt (C)</p> Signup and view all the answers

What is the estimated percentage of congenital heart defects attributed to environmental agents?

<p>2% (A)</p> Signup and view all the answers

Which congenital heart defect is most commonly associated with other major malformations?

<p>Ventricular septal defect (D)</p> Signup and view all the answers

In which condition does deoxygenated blood enter systemic circulation due to a congenital defect?

<p>Cyanotic heart defect (D)</p> Signup and view all the answers

Which of the following statements correctly describes a characteristic feature of left-to-right shunting in congenital heart defects?

<p>It causes heart failure due to volume overload. (B)</p> Signup and view all the answers

What anatomical change occurs in transposition of the great vessels?

<p>Reversal of the pulmonary artery and the aorta (D)</p> Signup and view all the answers

What is a critical survival mechanism for infants with transposition of the great vessels after birth?

<p>Open connection through a patent ductus arteriosus (D)</p> Signup and view all the answers

What is the main consequence of failed spiraling of the aorticopulmonary septum?

<p>Deoxygenated blood entering systemic circulation (A)</p> Signup and view all the answers

What percentage of cyanotic congenital heart defects does transposition of the great vessels account for?

<p>20% (A)</p> Signup and view all the answers

What primary effect does transposition of the great vessels have on blood circulation?

<p>Complete isolation of the pulmonary and systemic circuits (D)</p> Signup and view all the answers

What is the most common congenital heart defect?

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

Which part of the ventricular septum is VSD most commonly located?

<p>Membranous part (A)</p> Signup and view all the answers

What leads to right ventricular (RV) hypertrophy in patients with VSD?

<p>Increased pulmonary artery pressure (D)</p> Signup and view all the answers

What is a secondary effect of the left-to-right shunt caused by VSD?

<p>Pulmonary hypertension (D)</p> Signup and view all the answers

How does the size of the ventricular septal defect affect the volume of shunting?

<p>Larger defects lead to greater left-to-right shunting. (D)</p> Signup and view all the answers

What condition arises due to excessive blood flow to the pulmonary circulation from VSD?

<p>Pulmonary hypertension (D)</p> Signup and view all the answers

What is the consequence of left ventricular volume overload due to VSD?

<p>Left ventricular hypertrophy (B)</p> Signup and view all the answers

What type of shunting occurs with a defect in the ventricular septum?

<p>Left-to-right shunting (B)</p> Signup and view all the answers

What is a potential consequence of increased pulmonary artery pressure caused by VSD?

<p>Right heart failure (B)</p> Signup and view all the answers

What physiological condition is indicated by a mild left-to-right shunt in the presence of a patent foramen ovale?

<p>Persistence of foramen ovale (B)</p> Signup and view all the answers

What maneuver can potentially reverse the shunt direction in a patent foramen ovale?

<p>Valsalva maneuver (B)</p> Signup and view all the answers

What symptom is most commonly observed in patients with coarctation of the aorta distal to the left subclavian artery?

<p>Lower blood pressure in the upper extremities (B)</p> Signup and view all the answers

What is the most common anatomical site of coarctation in the aorta?

<p>Distal to the left subclavian artery (B)</p> Signup and view all the answers

What hemodynamic effect is associated with coarctation of the aorta?

<p>Increased collateral blood flow (D)</p> Signup and view all the answers

Which consequence is likely due to the narrowing of the aorta in coarctation?

<p>Myocardial hypertrophy (B)</p> Signup and view all the answers

What change occurs in blood flow due to coarctation of the aorta?

<p>Decreased flow to the lower body (A)</p> Signup and view all the answers

What is one of the physiological changes associated with the persistence of a patent foramen ovale?

<p>Mild right-to-left shunt under stress (D)</p> Signup and view all the answers

What structural change in the aorta is a result of coarctation leading to decreased flow?

<p>Tunica medial thickening (B)</p> Signup and view all the answers

What vascular adaptation may develop as a result of coarctation of the aorta?

<p>Collateral circulation via the intercostals (C)</p> Signup and view all the answers

What occurs in patent ductus arteriosus after birth?

<p>There is a left-to-right shunt. (B)</p> Signup and view all the answers

Which of the following is a characteristic of Tetralogy of Fallot?

<p>It involves a ventricular septal defect. (D)</p> Signup and view all the answers

What is primarily responsible for the persistent left-to-right shunt in patent ductus arteriosus?

<p>Failure of the ductus arteriosus to close. (A)</p> Signup and view all the answers

What is a potential consequence of a left-to-right shunt in patent ductus arteriosus?

<p>Heart failure due to volume overload in pulmonary vessels. (D)</p> Signup and view all the answers

Which defect is NOT part of the Tetralogy of Fallot?

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

How does pulmonary vascular resistance change after birth in relation to patent ductus arteriosus?

<p>It decreases, allowing left-to-right shunting. (A)</p> Signup and view all the answers

What is the primary consequence of a right-to-left shunt in congenital heart defects?

<p>Cyanosis due to mixing of oxygenated and deoxygenated blood. (B)</p> Signup and view all the answers

What effect does a left-to-right shunt have on the right ventricle in patent ductus arteriosus?

<p>Increases workload and can lead to deterioration of function. (B)</p> Signup and view all the answers

Which condition is associated with a continuous communication between the aorta and pulmonary artery?

<p>Patent ductus arteriosus (B)</p> Signup and view all the answers

Flashcards

Atrial Septal Defect (ASD)

A defect in the interatrial septum that causes a left-to-right shunt of blood, increasing blood volume in the right atrium and ventricle.

Left-to-Right Shunt (ASD)

Blood flows from the left atrium to the right atrium through the defect.

Ostium Primum ASD

A type of ASD, usually accompanied by other heart defects, where the defect is near the bottom of the septum.

Ostium Secundum ASD

A common isolated type of ASD located in the fossa ovalis (a normal fetal opening in the heart).

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Impaired Growth

Incomplete development of heart structures, in the context of ASD, leading to an opening in the atrial septum.

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Excessive Resorption

A process where heart tissues break down in a more rapid way than normal.

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Atrial Septum

The wall that separates the two atria of the heart.

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Fossa Ovalis

A small depression in the atrial septum, marking where the fetal foramen ovale was.

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Transposition of Great Vessels

A heart defect where the aorta and pulmonary artery are switched, resulting in complete separation of oxygenated and deoxygenated blood flow.

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Aorta-Pulmonary swap

The aorta carries deoxygenated blood, and the pulmonary artery carries oxygenated blood in a transposition of great vessels.

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Cyanotic CHD

A type of congenital heart disease characterized by a bluish discoloration of the skin due to low oxygen levels in the blood.

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Intracardiac shunt

An abnormal passage between chambers of the heart that allows blood to bypass normal circulation.

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Patent Ductus Arteriosus

A persistent connection between the aorta and pulmonary artery, often present in newborns and causing a shunt.

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Patent Foramen Ovale

A persistent opening between the atria in the heart, normally present in fetal development but should close.

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Patent Foramen Ovale (PFO)

Foramen ovale remains open after birth, causing a small blood flow from right to left atrium.

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Atrial Septal Defect

Failure of the atrial septum primum and secundum to fuse, resulting in an opening causing mild left to right shunt.

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Coarctation of Aorta

Narrowing of the aorta, often distal to the left subclavian artery, leading to high blood pressure in the upper body and low blood pressure in the lower body.

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Aortic Isthmus

Specific part of the aorta where narrowing often occurs in coarctation.

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Collateral Vessels

Blood vessels that develop to compensate for reduced blood flow due to coarctation, often around the narrowed part of the aorta.

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Shunt Reversal

Change in the direction of blood flow through an abnormal opening, such as in a patient with PFO during certain maneuvers.

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Valsalva Maneuver

A maneuver involving forceful exhalation against a closed glottis, increasing right atrial pressure. It's possibly a trigger for shunt reversal.

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Congenital Heart Defects

Heart and vascular abnormalities present in approximately 1% of live-born infants, often linked to genetic and environmental factors.

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Acyanotic CHD

Congenital heart defects causing left-to-right shunting of blood.

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Cyanotic CHD

Congenital heart defects characterized by right-to-left shunting of blood, leading to deoxygenated blood entering systemic circulation.

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Left-to-Right Shunt

Blood flowing from the left side of the heart to the right side through an abnormal opening.

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Atrial Septal Defect

An opening in the wall separating the atria, causing a left-to-right shunt.

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Ventricular Septal Defect

An opening in the wall separating the ventricles, causing a left-to-right shunt.

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Patent Ductus Arteriosus

Persistence of a fetal blood vessel connection between the aorta and pulmonary artery.

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Coarctation of Aorta

Narrowing of the aorta, often impacting blood flow.

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Patent Ductus Arteriosus

A persistent connection between the aorta and pulmonary artery after birth, causing a left-to-right shunt.

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Left-to-Right Shunt (PDA)

Blood flow from the aorta to the pulmonary arteries in patent ductus arteriosus.

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Tetralogy of Fallot (TOF)

A combination of four heart defects: ventricular septal defect, pulmonary stenosis, overriding aorta, and right ventricular hypertrophy.

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Cyanotic Heart Defects

Congenital heart conditions causing low oxygen levels in blood, leading to bluish skin.

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Heart Failure (CHD)

Condition resulting from excessive strain on the right and/or left ventricles due to circulatory imbalance in congenital heart disease.

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Patent Foramen Ovale (PFO)

Foramen ovale remaining open after birth, causing a small blood flow from right to left atrium.

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Atrial Septal Defect

Imperfect fusion of atrial septum primum and secundum leading to a hole, allowing mild left-to-right shunt.

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Coarctation of Aorta

Narrowing of the aorta, typically distal to the left subclavian artery, leading to high BP in upper body and low BP in lower body.

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Shunt Reversal

Blood flow direction change through an abnormal opening, often triggered by maneuvers increasing right atrial pressure.

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Valsalva Maneuver

Forcibly exhaling against a closed glottis, increasing right atrial pressure and potentially causing shunt reversal.

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Aortic Isthmus

Specific part of the aorta where coarctation frequently happens.

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Collateral Vessels

Blood vessels emerging around narrowed aorta compensating for reduced blood flow.

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Ventricular Septal Defect

An abnormal opening between the left and right ventricles of the heart, creating a left-to-right shunt.

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Left-to-Right Shunt

Blood flows from the left side of the heart to the right side, usually due to a heart defect.

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Ventricular Septal Defect Location

Most commonly found in the membranous part of the ventricular septum.

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VSD and RV Volume Overload

VSD creates increased volume in the right ventricle, causing it to enlarge.

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Pulmonary Hypertension

Increased blood pressure in the pulmonary artery, caused by excessive pulmonary blood flow.

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Cardiac Output Reduction

The amount of blood pumped by the heart decreases.

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VSD and LV Hypertrophy

The left ventricle also enlarges due to extra blood volume.

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Congenital Heart Defect

A heart condition present at birth.

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Common CHD

Ventricular septal defect is a frequently occurring congenital heart disease.

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Transposition of Great Vessels

A heart defect where the aorta and pulmonary artery are switched, completely separating the oxygenated and deoxygenated blood flow.

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Cyanotic CHD

Congenital heart disease causing low oxygen levels in the blood, leading to bluish skin.

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Intracardiac shunt

An abnormal passage between heart chambers that allows blood to bypass normal circulation.

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Oxygenated blood in systemic circulation

Normal blood flow where oxygen-rich blood reaches the body tissues.

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Deoxygenated blood in systemic circulation

Abnormal blood flow where deoxygenated blood is pumped to the body's tissues.

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VSD and truncus arteriosus

Ventricular Septal Defect (VSD) mixes blood flows making truncus arteriosus pump both oxygenated and deoxygenated blood.

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Study Notes

Congenital Heart Defects

  • Congenital heart defects (CHDs) are the largest category of human birth defects, involving heart and vascular abnormalities.
  • Approximately 1% of live born infants have CHDs.
  • CHDs can be caused by a complex interplay of genetic and environmental factors (multifactorial causes).
  • 12% of infants with CHDs have chromosomal abnormalities.
  • 33% of infants with chromosomal abnormalities have a CHD.
  • 30% of CHDs occur with other major malformations.
  • 2% of CHDs are due to environmental agents.

Learning Outcomes

  • Understand the steps in normal heart chamber development and relate them to CHDs.

Types of Congenital Heart Defects

  • Acyanotic defects: These defects result in left-to-right shunting, where oxygenated blood flows from the left side of the heart to the right side, causing increased blood flow to the lungs.

  • Common examples include:

  • Ventricular septal defect (VSD): An abnormal opening between the ventricles.

  • Atrial septal defect (ASD): An abnormal opening between the atria.

  • Patent ductus arteriosus (PDA): Failure of the fetal vessel to close after birth.

  • Coarctation of the aorta: Narrowing of the aorta.

  • Cyanotic defects: These defects lead to right-to-left shunting, causing deoxygenated blood to enter the systemic circulation. This results in reduced oxygen levels in the blood.

  • Common examples include:

  • Tetralogy of Fallot (TOF): A combination of four defects (pulmonary stenosis, VSD, overriding aorta, right ventricular hypertrophy).

  • Transposition of the great arteries (TGA): The aorta and pulmonary artery are switched.

  • Persistent truncus arteriosus: A single blood vessel emerges from both ventricles.

  • Tricuspid atresia: The tricuspid valve is missing or severely malformed.

  • Ebstein anomaly: The tricuspid valve is abnormally positioned in the right ventricle.

Specific Defects:

  • Ventricular Septal Defect (VSD): A hole in the ventricular septum, leading to left-to-right shunting, causing increased blood flow to the lungs. Commonly occurring in the membranous part of the ventricular septum. Resulting in RV volume overload and hypertrophy. Excessive pulmonary blood flow can lead to pulmonary hypertension.

  • Atrial Septal Defect (ASD): A hole in the atrial septum that causes left-to-right shunting, overloading the right side of the heart and increasing the workload on the lungs. Different types, including ostium primum (often accompanied by additional defects) and ostium secundum (more common on its own) have varying effects.

  • Patent Foramen Ovale (PFO): The flap of the foramen ovale (an opening between the atria in the fetus) does not fully close after birth, causing a left-to-right shunt. Right to left shunting can occur under stress or in certain maneuvers that increase right atrial pressure.

  • Coarctation of the Aorta: Narrowing of the aorta, causing higher blood pressure in the upper extremities compared to the lower extremities. Blockage or narrowing of the aorta that occurs distal to the left subclavian artery in particular causes issues with blood flow to the lower body. This can lead to hypoperfusion in the lower extremities.

  • Patent Ductus Arteriosus (PDA): Failure of the fetal vessel to close after birth, creating a left-to-right shunt, and increasing blood flow to the lungs.

  • Holt-Oram syndrome: Genetic disorder affecting heart and limb development, often associated with abnormalities in heart septal formation.

  • Ebstein anomaly: The tricuspid valve is displaced towards the apex of the right ventricle, resulting in incomplete closure of the right atrioventricular valve and potentially causing a right-to-left shunt.

  • Persistent Truncus Arteriosus: The single blood vessel connecting both ventricles to the aortic arch does not separate, resulting in blood from both ventricles being mixed in a single vessel.

  • Transposition of the Great Vessels: The aorta and pulmonary artery switch positions, and blood will flow to the wrong circulation. This is almost always accompanied by a VSD or a PFO allowing oxygenated blood into the systemic circulation.

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