Congenital Heart Disease Flashcards by Jacob Thorn

What is Eisenmenger syndrome?

A condition where left-to-right shunts shift to right-to-left shunts due to increased pulmonary vascular resistance, leading to cyanosis and other symptoms.

Signs include cyanosis, digital clubbing, and polycythemia.

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What are the initial characteristics of left-to-right shunts?

They are initially acyanotic but may develop cyanosis later due to shunt reversal.

This condition is known as cyanosis tardive.

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What are the signs of Eisenmenger syndrome?

Cyanosis late, digital clubbing, and polycythemia.

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What is an atrial septal defect?

A left-to-right shunt characterized by an early-peaking midsystolic ejection murmur and wide, fixed splitting of S2.

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What is the most common type of congenital heart disease that presents in adults?

Atrial septal defects.

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What causes paradoxical embolism?

A thrombus from the venous system crosses into arterial circulation via an abnormal connection between the right and left cardiac chambers.

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What is a bubble study?

A test to identify right-to-left intracardiac shunts by injecting agitated normal saline and observing for microbubbles in the left heart.

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What does a ventricular septal defect (VSD) murmur sound like?

A harsh, holosystolic murmur at the left lower sternal border.

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What happens to the VSD murmur after birth?

It may not be audible immediately due to high pulmonary vascular resistance but typically develops over days.

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What is the characteristic of a patent ductus arteriosus (PDA)?

A continuous, machine-like murmur over the left infraclavicular region.

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What is the role of indomethacin in the context of PDA?

It can be used to close a PDA in premature infants.

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What is differential cyanosis?

Cyanosis affecting only the lower body, suggesting right-to-left shunting across the ductus arteriosus.

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What is truncus arteriosus?

A congenital defect characterized by a single large vessel that receives output from both ventricles.

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What syndrome is truncus arteriosus associated with?

DiGeorge syndrome.

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What is transposition of the great arteries?

A condition where the aorta originates from the right ventricle and the pulmonary artery from the left ventricle, causing cyanosis at birth.

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What is the initial management for transposition of the great arteries?

Enlarging the patent foramen ovale to allow partially oxygenated blood to enter systemic circulation.

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What are the four components of Tetralogy of Fallot?

Right ventricular outflow tract obstruction
Ventricular septal defect
Overriding aorta
Right ventricular hypertrophy

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What is the most common cause of early childhood cyanosis?

Tetralogy of Fallot.

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What is a characteristic feature of an atrioventricular septal defect?

A complete atrioventricular canal defect, which includes an atrial septal defect, a ventricular septal defect, and a common AV valve.

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What is coarctation of the aorta associated with?

Chronic hypertension and an increased risk of life-threatening aneurysms.

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What is Ebstein anomaly?

A condition characterized by downward displacement of the tricuspid valve into the right ventricle, leading to an enlarged right atrium.

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What is a characteristic feature of Williams syndrome?

Supravalvular aortic stenosis and dysmorphic facial features.

What is Eisenmenger syndrome?

A condition where left-to-right shunts develop cyanosis later due to increased pulmonary vascular resistance, leading to right-to-left shunting.

It results from uncorrected congenital heart defects causing pulmonary hypertension.

What are the signs of Eisenmenger syndrome?

Signs include:
* Late cyanosis (‘blue kids’)
* Digital clubbing
* Polycythemia

These signs indicate chronic hypoxemia and increased red blood cell production.

What characterizes an atrial septal defect?

Early-peaking midsystolic ejection murmur over the left upper sternal border and a wide, fixed split S2. ## Footnote It is the most common congenital heart disease in adults.

What is paradoxical embolism?

Occurs when a thrombus crosses from the venous to the arterial circulation via a patent foramen ovale or septal defect. ## Footnote This can happen during straining or coughing.

What is a bubble study?

A diagnostic method that injects agitated saline intravenously to detect right-to-left intracardiac shunts. ## Footnote It's useful in identifying atrial septal defects.

What murmur is associated with ventricular septal defect?

A harsh, holosystolic murmur at the left lower sternal border. ## Footnote The murmur increases in intensity with increased left ventricular afterload.

What is the typical presentation of patent ductus arteriosus?

A continuous, machine-like murmur over the left infraclavicular region and isolated pulmonary artery SpO2 elevation. ## Footnote It is commonly associated with premature infants or congenital rubella.

What can close a patent ductus arteriosus in premature infants?

Indomethacin, a PGE2 synthesis inhibitor. ## Footnote Surgical ligation may be needed in older patients.

What are the 5 T's associated with right-to-left shunts?

The 5 T's are: * Truncus arteriosus * Transposition of great arteries * Tricuspid atresia * Tetralogy of Fallot * Total anomalous pulmonary venous return (TAPVR) ## Footnote These conditions are characterized by right-to-left shunting.

What is the cause of transposition of the great arteries?

Failure of the aorticopulmonary septum spiraling. ## Footnote This results in an anterior aorta from the right ventricle and a posterior pulmonary artery from the left ventricle.

What are the four components of Tetralogy of Fallot?

Tetralogy of Fallot is characterized by: * Right ventricular outflow tract obstruction * Large ventricular septal defect * Overriding aorta * Right ventricular hypertrophy ## Footnote Severity depends on the degree of right ventricular outflow obstruction.

What triggers Tet spells in patients with Tetralogy of Fallot?

Tet spells are triggered by increased right ventricular outflow obstruction during crying, feeding, or exertion. ## Footnote Squatting can help alleviate these spells.

What is an atrioventricular septal defect?

Also known as an endocardial cushion defect, characterized by: * Atrial septal defect * Ventricular septal defect * Common atrioventricular valve ## Footnote It is the most common congenital heart defect in Down syndrome.

What is coarctation of the aorta associated with?

Chronic hypertension and increased risk of aortic dissection, rupture, and intracranial hemorrhage. ## Footnote It is caused by abnormal development of the fourth aortic arch on the left.

What is Ebstein anomaly?

A condition where the tricuspid valve is displaced downward into the right ventricle, causing an enlarged right atrium and small right ventricle. ## Footnote It is strongly associated with maternal lithium use during pregnancy.

What are the features of Williams syndrome?

Features include: * Supravalvular aortic stenosis * Elfin-like facial features * Friendly demeanor (hypersociability) * Musical talent and strong verbal skills ## Footnote It is caused by a microdeletion on chromosome 7.

A 4-month-old child is being evaluated for episodes of cyanosis when crying. Physical examination finds a harsh, systolic murmur heard best over the left upper sternal border. A chest x-ray shows a boot-shaped heart due to right ventricular hypertrophy. What is the most likely cause of these abnormalities?

Tetralogy of Fallot ## Footnote Tetralogy of Fallot is characterized by right ventricular outflow tract obstruction, a large ventricular septal defect, an overriding aorta, and right ventricular hypertrophy (boot-shaped heart). Tetralogy of Fallot with severe right ventricular outflow tract obstruction typically presents shortly after birth with cyanosis; moderate obstruction may present during childhood.

Differential clubbing (aka differential cyanosis) refers to cyanosis and clubbing of the lower extremities with normal upper extremities (normal peripheral pulses). This clinical sign is most specific for Eisenmenger syndrome developing in an individual with?

Patent ductus arteriosus

Murmur associated w/ atrial septal defect

Atrial septal defect, which is a left-to-right shunt, is characterized by an early-peaking midsystolic ejection murmur over the left upper sternal border and wide, fixed split S2. Atrial septal defects are the most common type of congenital heart disease that present in adults.

Six causes of cyanotic heart disease