Congenital Heart Disease (CHD)

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Questions and Answers

Congenital Heart Disease (CHD) is best defined as:

  • Any heart condition requiring surgical intervention in infants.
  • Any cardiac condition that develops during childhood.
  • An abnormality in heart structure or function present at birth. (correct)
  • Heart disease acquired due to environmental factors after birth.

What is the estimated average incidence of Congenital Heart Disease (CHD) in live births?

  • 50 in 1000 live births
  • 1 in 100 live births
  • 8 in 1000 live births (correct)
  • 25 in 1000 live births

Which diagnostic modality is typically considered the primary method for evaluating most Congenital Heart Defects (CHDs)?

  • Cardiac Catheterization
  • Two-dimensional and Doppler echocardiography (correct)
  • Chest X-ray (CXR)
  • Electrocardiogram (ECG)

During fetal circulation, which of the following structures allows oxygenated blood from the placenta to bypass the liver and enter the inferior vena cava?

<p>Ductus venosus (C)</p> Signup and view all the answers

Prostaglandins play a crucial role in maintaining the patency of which fetal circulatory structure?

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

Which of the following is classified as a non-cyanotic, left-to-right shunt lesion?

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

Which genetic abnormality is most commonly associated with Atrioventricular septal defects?

<p>Down Syndrome (C)</p> Signup and view all the answers

Maternal rubella infection during pregnancy is a known environmental risk factor for which condition in the offspring?

<p>Congenital Heart Disease (CHD) (D)</p> Signup and view all the answers

An Atrial Septal Defect (ASD) is characterized by:

<p>An opening in the atrial septum allowing communication between the atria. (A)</p> Signup and view all the answers

What type of murmur is typically associated with an Atrial Septal Defect (ASD)?

<p>Systolic ejection murmur (D)</p> Signup and view all the answers

A 'fixed widely split S2' heart sound is a classic finding in which congenital heart defect?

<p>Atrial Septal Defect (ASD) (B)</p> Signup and view all the answers

When is the optimal time for elective surgical or catheterization closure of an Atrial Septal Defect (ASD) in an asymptomatic child?

<p>Between 2 and 5 years of age (C)</p> Signup and view all the answers

What is Eisenmenger's syndrome, a potential complication of untreated left-to-right shunts, characterized by?

<p>Reversal of shunt due to pulmonary hypertension, leading to cyanosis. (A)</p> Signup and view all the answers

Patent Foramen Ovale (PFO) differs from Atrial Septal Defect (ASD) in that PFO:

<p>Does not involve missing septal tissue. (B)</p> Signup and view all the answers

Which congenital heart defect is the most common in infancy and childhood?

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

A holosystolic murmur, best heard at the lower left sternal border or Erb's point, is most characteristic of:

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

In managing a large, symptomatic Ventricular Septal Defect (VSD) in an infant, which of the following is typically prioritized?

<p>Medical management of heart failure followed by early surgical repair. (C)</p> Signup and view all the answers

A continuous 'machinery-like' murmur is classically associated with which congenital heart defect?

<p>Patent Ductus Arteriosus (PDA) (A)</p> Signup and view all the answers

In preterm infants, Patent Ductus Arteriosus (PDA) can be medically closed using:

<p>Indomethacin (C)</p> Signup and view all the answers

Coarctation of the aorta primarily involves:

<p>A narrowing of the aorta, typically distal to the left subclavian artery. (A)</p> Signup and view all the answers

In a patient with coarctation of the aorta, blood pressure findings typically include:

<p>Higher BP in upper extremities than lower extremities. (B)</p> Signup and view all the answers

Diminished or delayed femoral pulses compared to brachial pulses (brachial-femoral delay) is a clinical sign suggestive of:

<p>Coarctation of the Aorta (A)</p> Signup and view all the answers

Rib notching on chest X-ray is a radiographic finding characteristic of:

<p>Coarctation of the Aorta (C)</p> Signup and view all the answers

What is the classic presentation of coarctation of the aorta in young adults?

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

Tetralogy of Fallot is characterized by a combination of four anatomical defects. Which of the following is NOT one of these defects?

<p>Atrial septal defect (ASD) (A)</p> Signup and view all the answers

What is the most common cause of cyanosis in infancy and childhood?

<p>Tetralogy of Fallot (B)</p> Signup and view all the answers

A hypercyanotic 'tet spell' in Tetralogy of Fallot is typically caused by:

<p>Decreased systemic vascular resistance. (D)</p> Signup and view all the answers

Knee-to-chest position is a beneficial immediate intervention for a 'tet spell' because it:

<p>Increases systemic vascular resistance. (C)</p> Signup and view all the answers

A 'boot-shaped heart' on chest X-ray is a classic finding in:

<p>Tetralogy of Fallot (D)</p> Signup and view all the answers

Truncus arteriosus is a congenital heart defect where:

<p>A single arterial trunk arises from the heart instead of separate aorta and pulmonary artery. (B)</p> Signup and view all the answers

In Truncus Arteriosus, pulmonary circulation is exposed to very high pressures, leading to heart failure typically within:

<p>Weeks of birth. (A)</p> Signup and view all the answers

Transposition of the Great Arteries (TGA) is characterized by:

<p>The aorta arising from the right ventricle and the pulmonary artery from the left ventricle. (A)</p> Signup and view all the answers

Survival in Transposition of the Great Arteries (TGA) in the early days of life depends on:

<p>Patency of the Foramen ovale (PFO) or Patent Ductus Arteriosus (PDA). (C)</p> Signup and view all the answers

An 'egg-on-a-string' appearance on chest X-ray is highly suggestive of:

<p>Transposition of the Great Arteries (TGA) (A)</p> Signup and view all the answers

Tricuspid atresia is defined by:

<p>Absence of the tricuspid valve. (C)</p> Signup and view all the answers

In Tricuspid Atresia, blood flows from the right atrium to the left atrium through which structure?

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

Total Anomalous Pulmonary Venous Return (TAPVR) is a condition where:

<p>Pulmonary veins fail to connect to the left atrium and drain into the right atrium or its systemic venous tributaries. (C)</p> Signup and view all the answers

A 'snowman' or 'figure of 8' appearance on chest X-ray is characteristic of which type of Total Anomalous Pulmonary Venous Return (TAPVR)?

<p>Supracardiac TAPVR (B)</p> Signup and view all the answers

Ebstein's anomaly primarily affects which cardiac valve?

<p>Tricuspid valve (C)</p> Signup and view all the answers

Maternal lithium use during pregnancy is associated with an increased incidence of which congenital cardiac anomaly?

<p>Ebstein's anomaly (A)</p> Signup and view all the answers

Hypoplastic Left Heart Syndrome (HLHS) is characterized by:

<p>Underdevelopment of the left ventricle and aorta. (B)</p> Signup and view all the answers

In Hypoplastic Left Heart Syndrome (HLHS), which structure becomes the primary pumping chamber?

<p>Right ventricle (D)</p> Signup and view all the answers

Prostaglandin E1 (PGE1) is administered in Hypoplastic Left Heart Syndrome (HLHS) to:

<p>Maintain patency of the Patent Ductus Arteriosus (PDA). (B)</p> Signup and view all the answers

According to current guidelines, which of the following conditions necessitates endocarditis prophylaxis before dental procedures?

<p>Unrepaired cyanotic Congenital Heart Disease (CHD). (B)</p> Signup and view all the answers

Which of the following is NOT typically a 'red flag' symptom in pediatrics that may indicate underlying cardiac issues?

<p>Increased appetite (C)</p> Signup and view all the answers

Congenital Heart Disease (CHD) is primarily characterized by abnormalities in:

<p>Cardiac structure or function present at birth (A)</p> Signup and view all the answers

The majority of congenital heart defects are believed to originate from:

<p>Abnormal cardiac embryogenesis (B)</p> Signup and view all the answers

What is the approximate average incidence of Congenital Heart Disease (CHD) per 1000 live births?

<p>8 (D)</p> Signup and view all the answers

The clinical presentation of Congenital Heart Disease (CHD) is most directly dependent on:

<p>Hemodynamic changes resulting from the defect (D)</p> Signup and view all the answers

Which imaging modality is typically considered the gold standard for evaluating most types of Congenital Heart Disease (CHD)?

<p>Two-dimensional and Doppler echocardiography (B)</p> Signup and view all the answers

During fetal circulation, the ductus arteriosus primarily shunts blood away from the:

<p>Lungs (C)</p> Signup and view all the answers

Prostaglandins are essential during fetal life to maintain the patency of the ductus arteriosus. Which of the following is the primary effect of prostaglandins on the ductus arteriosus?

<p>Maintaining vasodilation (D)</p> Signup and view all the answers

Which of the following is classified as a cyanotic congenital heart defect?

<p>Tetralogy of Fallot (TOF) (D)</p> Signup and view all the answers

Down syndrome is most strongly associated with which type of congenital heart defect?

<p>Atrioventricular septal defect (AV canal defect) (B)</p> Signup and view all the answers

Maternal rubella infection during the first trimester is a known risk factor for congenital heart defects, especially:

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

An Atrial Septal Defect (ASD) is best described as:

<p>An opening in the atrial septum allowing communication between atria (B)</p> Signup and view all the answers

What type of heart murmur is most typically auscultated in a patient with an Atrial Septal Defect (ASD)?

<p>Systolic ejection murmur at the left sternal border (A)</p> Signup and view all the answers

A 'fixed widely split S2' heart sound is a hallmark finding in which congenital cardiac anomaly?

<p>Atrial Septal Defect (ASD) (C)</p> Signup and view all the answers

In an asymptomatic child with an Atrial Septal Defect (ASD), when is the generally recommended timeframe for elective surgical or catheterization closure?

<p>Between 2 and 5 years of age (D)</p> Signup and view all the answers

Eisenmenger's syndrome, a severe complication of untreated left-to-right shunts, is characterized by:

<p>Reversal of shunt to right-to-left due to pulmonary hypertension (C)</p> Signup and view all the answers

How does a Patent Foramen Ovale (PFO) differ fundamentally from an Atrial Septal Defect (ASD)?

<p>PFO is a flap-like opening that can open under increased right atrial pressure, while ASD is a true defect in the septal tissue (D)</p> Signup and view all the answers

Which congenital heart defect is recognized as the most prevalent in infancy and childhood?

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

A holosystolic murmur, best heard at the lower left sternal border or Erb's point, is most indicative of:

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

In managing a symptomatic infant with a large Ventricular Septal Defect (VSD), what is usually the initial priority?

<p>Medical management of heart failure (D)</p> Signup and view all the answers

In preterm infants, Patent Ductus Arteriosus (PDA) can often be medically closed using:

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

Coarctation of the aorta is characterized by:

<p>Narrowing of the aorta, typically distal to the left subclavian artery (D)</p> Signup and view all the answers

In a patient diagnosed with coarctation of the aorta, typical blood pressure findings would include:

<p>Lower blood pressure in the lower extremities compared to the upper extremities (A)</p> Signup and view all the answers

Diminished or delayed femoral pulses when compared to brachial pulses (brachial-femoral delay) is a crucial clinical sign suggesting:

<p>Coarctation of the aorta (C)</p> Signup and view all the answers

Rib notching observed on a chest X-ray is a radiographic finding classically associated with:

<p>Coarctation of the aorta (C)</p> Signup and view all the answers

What is the classic presentation of coarctation of the aorta in young adults, often leading to its diagnosis?

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

Which of the following anatomical defects is NOT a component of Tetralogy of Fallot?

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

In infants and children, what is the most common cause of cyanotic congenital heart disease?

<p>Tetralogy of Fallot (TOF) (D)</p> Signup and view all the answers

A hypercyanotic 'tet spell' in Tetralogy of Fallot is typically triggered by a sudden:

<p>Decrease in systemic vascular resistance (C)</p> Signup and view all the answers

The knee-to-chest position is a beneficial immediate intervention for a 'tet spell' because it primarily aims to:

<p>Increase systemic vascular resistance (A)</p> Signup and view all the answers

A 'boot-shaped heart' configuration on a chest X-ray is a classic radiographic finding in:

<p>Tetralogy of Fallot (TOF) (D)</p> Signup and view all the answers

In Truncus Arteriosus, heart failure typically develops within weeks of birth due to:

<p>Excessive pulmonary blood flow and volume overload (A)</p> Signup and view all the answers

In Transposition of the Great Arteries (TGA), survival in the neonatal period is critically dependent on:

<p>Patency of the ductus arteriosus or foramen ovale (D)</p> Signup and view all the answers

In Tricuspid Atresia, how does blood typically flow from the right atrium to the left atrium?

<p>Through an Atrial Septal Defect (ASD) or Patent Foramen Ovale (PFO) (A)</p> Signup and view all the answers

Maternal use of lithium during pregnancy has been associated with an increased incidence of which specific congenital cardiac anomaly?

<p>Ebstein's anomaly (D)</p> Signup and view all the answers

Hypoplastic Left Heart Syndrome (HLHS) is fundamentally characterized by:

<p>Underdevelopment of the left ventricle and aorta (C)</p> Signup and view all the answers

In Hypoplastic Left Heart Syndrome (HLHS), which cardiac chamber becomes the primary pumping chamber for systemic circulation?

<p>Right ventricle (D)</p> Signup and view all the answers

Prostaglandin E1 (PGE1) is administered in Hypoplastic Left Heart Syndrome (HLHS) primarily to:

<p>Maintain patency of the ductus arteriosus (A)</p> Signup and view all the answers

According to current guidelines, which condition definitively necessitates endocarditis prophylaxis before dental procedures?

<p>Unrepaired cyanotic Congenital Heart Disease (CHD) (C)</p> Signup and view all the answers

Which of the following symptoms is LEAST likely to be a 'red flag' indicating underlying cardiac issues in a pediatric patient?

<p>Intermittent abdominal pain (B)</p> Signup and view all the answers

A 2-year-old child presents with frequent respiratory infections and poor weight gain. On auscultation, a holosystolic murmur is heard best at the lower left sternal border with a palpable thrill. The murmur does not radiate and is not affected by position. Which of the following is the most likely diagnosis?

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

A newborn presents with severe cyanosis shortly after birth. CXR shows a narrow mediastinum and an "egg-on-a-string" appearance. The infant is stabilized with IV prostaglandin. Which of the following conditions is most consistent with these findings?

<p>Transposition of the Great Arteries (C)</p> Signup and view all the answers

A 3-month-old infant has a continuous "machinery-like" murmur and signs of poor feeding and respiratory distress. Which of the following would be the first-line treatment in a preterm infant?

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

A 7-year-old with known ASD is found to have a fixed widely split S2 and a II/VI systolic ejection murmur at the 2nd LICS. The child is asymptomatic. Which of the following is the most likely cause of the murmur?

<p>Pulmonary valve flow murmur (A)</p> Signup and view all the answers

A 14-year-old girl presents with hypertension, headaches, and cold legs with diminished pulses. BP is 148/90 mmHg in the arms and 92/62 mmHg in the legs. CXR shows rib notching and a 3-sign on the aortic silhouette. What is the most likely diagnosis?

<p>Coarctation of the aorta (D)</p> Signup and view all the answers

A child with a history of Tetralogy of Fallot develops sudden cyanosis and becomes unresponsive while crying. The mother states he "turned blue fast." What is the first priority in management?

<p>Place child in knee-chest position (A)</p> Signup and view all the answers

A neonate with hypoplastic left heart syndrome is critically ill in the first few days of life. What is the essential initial medical therapy before surgical correction?

<p>Prostaglandin E1 (B)</p> Signup and view all the answers

A newborn with cyanosis is found to have pulmonary veins draining into the superior vena cava, with oxygenation maintained only through a PFO. CXR shows a snowman sign. What is the most likely diagnosis?

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

A cyanotic infant is diagnosed with Ebstein's anomaly. Which maternal history detail would raise suspicion for this defect?

<p>Lithium therapy (C)</p> Signup and view all the answers

Which of the following congenital heart defects becomes inoperable if pulmonary hypertension with shunt reversal develops?

<p>ASD (C)</p> Signup and view all the answers

A 3-year-old child is noted to have a fixed wide split of S2 and a systolic ejection murmur at the 2nd left intercostal space. The child is otherwise asymptomatic and thriving well. Which of the following is the most likely pathophysiologic mechanism?

<p>Increased pulmonary blood flow across the pulmonic valve (D)</p> Signup and view all the answers

A 1-month-old infant is found to have a holosystolic murmur at the lower left sternal border, a palpable thrill, and frequent respiratory infections. Which of the following physical exam findings is most consistent with the diagnosis?

<p>Hyperactive precordium (A)</p> Signup and view all the answers

A neonate is born with cyanosis immediately after birth. Echocardiography confirms transposition of the great arteries (TGA). Which of the following immediate treatments is most crucial to survival?

<p>Prostaglandin E1 (B)</p> Signup and view all the answers

A child has a known Tetralogy of Fallot and presents with sudden worsening cyanosis after exercise. The child instinctively squats. Which of the following best explains the improvement in symptoms with squatting?

<p>Increased systemic vascular resistance (B)</p> Signup and view all the answers

Which of the following radiographic findings is most classically associated with Total Anomalous Pulmonary Venous Return (TAPVR)?

<p>Figure-of-eight (snowman) (B)</p> Signup and view all the answers

A 2-week-old infant with hypoplastic left heart syndrome (HLHS) presents with severe cyanosis. Which of the following best explains the initial survival in these patients?

<p>Prostaglandin-mediated maintenance of the PDA (A)</p> Signup and view all the answers

A teenager with hypertension is noted to have diminished femoral pulses, a 3/6 systolic murmur, and rib notching on chest X-ray. Which of the following is the most likely diagnosis?

<p>Coarctation of the aorta (A)</p> Signup and view all the answers

Which cyanotic congenital heart disease classically presents with an "egg on a string" appearance on CXR and requires arterial switch surgery in the first 1-2 weeks of life?

<p>Transposition of the great arteries (B)</p> Signup and view all the answers

A neonate presents with cyanosis, clubbing, and a single S2. Imaging shows a boot-shaped heart. What is the most likely diagnosis?

<p>Tetralogy of Fallot (D)</p> Signup and view all the answers

Which of the following is NOT a component of Tetralogy of Fallot?

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

A 3-week-old infant is brought in for evaluation of cyanosis. CXR reveals cardiomegaly with increased pulmonary vascular markings. Echo shows a single arterial trunk overriding a large VSD. Which of the following is the most likely diagnosis?

<p>Truncus arteriosus (D)</p> Signup and view all the answers

A newborn has profound cyanosis shortly after birth. You suspect TGA. What finding would allow for survival until surgery?

<p>VSD or PDA (B)</p> Signup and view all the answers

Which of the following best explains why a "tet spell" becomes more severe when a child is agitated or in a warm bath?

<p>Decreased systemic vascular resistance (D)</p> Signup and view all the answers

A 5-year-old with known VSD is now presenting with cyanosis and digital clubbing. Echo shows elevated pulmonary artery pressures and bidirectional shunting. Which of the following diagnoses is now most appropriate?

<p>Eisenmenger's syndrome (D)</p> Signup and view all the answers

Which of the following is a non-cyanotic heart lesion that often presents in young adults with headache, hypertension, and delayed femoral pulses?

<p>Coarctation of the aorta (C)</p> Signup and view all the answers

An infant presents with severe cyanosis. Echo reveals no communication between the right atrium and right ventricle, a large PFO, and a small RV. Which of the following is the most likely diagnosis?

<p>Tricuspid atresia (D)</p> Signup and view all the answers

Which cyanotic congenital heart disease is associated with a snowman sign on CXR, PFO-dependent oxygenation, and pulmonary veins draining to the SVC or IVC?

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

Which CHD is commonly associated with maternal lithium exposure and frequently involves ASD or VSD?

<p>Ebstein's anomaly (A)</p> Signup and view all the answers

Which condition features a "boot-shaped heart" on CXR due to right ventricular hypertrophy?

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

Which of the following requires endocarditis prophylaxis?

<p>Unrepaired cyanotic congenital heart disease (C)</p> Signup and view all the answers

Flashcards

Congenital Heart Disease (CHD)

Abnormality of cardiac structure/function, present at birth, arising from abnormal embryogenesis.

Best diagnostic test for CHD

Two-dimensional and Doppler echocardiography

Fetal Circulation Communications

Ductus arteriosus, foramen ovale, and ductus venosus

Acyanotic: Left-to-Right Shunt Lesions

Atrial Septal Defect (ASD), Ventricular Septal Defect (VSD), and Patent Ductus Arteriosus (PDA)

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Cyanotic Congenital Heart Defects

Truncus Arteriosus (TA), Transposition of the Great Vessels (TGV), Tetralogy of Fallot (TOF)

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CHD: Risk Factors

Risk is 10-15%. Risk factors: genetic abnormalities, environmental factors (alcohol, rubella), medications, smoking.

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Atrial Septal Defect (ASD)

Opening in the atrial septum permitting free communication of blood between the atria, causing a left-to-right shunt

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ASD: Clinical Signs

Fixed widely split S2, systolic ejection murmur at LSB

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Atrial Septal Defect Treatment

Surgical or catheterization closure is done electively ages 2-5

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ASD: Complications

Pulmonary HTN with shunt reversal, known as Eisenmenger's syndrome.

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Patent Foramen Ovale (PFO)

An open communication between the left and right atria, but with no missing septal tissue.

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Ventricular Septal Defect (VSD)

An opening in the ventricular septum permitting free communication of blood between the ventricles, causing a left-to-right shunt.

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VSD: Clinical Signs

Holosystolic murmur, best heard over the tricuspid area (Erb's point)

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VSD: Complications

Pulmonary hypertension with shunt reversal (Eisenmenger's syndrome).

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Patent Ductus Arteriosus (PDA)

Continuous murmur heard in systole and diastole

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PDA: Medical Treatment

Indomethacin

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Eisenmenger's Syndrome

Redirection of blood flow through VSD, ASD, or PDA, from L-R to R-L shunt.

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Classic Findings in Eisenmenger's

Cyanosis, pulmonary HTN, and erythrocytosis

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Coarctation of the Aorta

Narrowing of the aorta

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Coarctation of the Aorta: Young Adults

Classic presentation is hypertension.

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Coarctation of the Aorta Classic Symptoms

Diminished or delayed femoral pulses

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Coarctation of the Aorta CXR

Notching of the ribs

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Cyanotic Heart Disease: Hypoplastic Left Heart Syndrome (HLHS)

Rare, but One of the most severe cyanotic congenital heart defects.

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Tetralogy of Fallot

One of the five 'T's, most common cause of cyanosis in infancy/childhood

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4 Defects of TOF

Pulmonary stenosis, large VSD, overriding aorta, right ventricular hypertrophy

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Hypercyanotic "tet" spell

Positioning infant knee-to-chest

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Treatment of 'tet spell'

  1. Volume(kneechest squat) 2) PVR 3) SUR
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Tetralogy of Fallot CXR

Boot shaped heart

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Truncus Arteriosus

Pulmonary and aorta form 1 trunk instead of 2 separate vessels. Blood from both ventricles passes across a VSD into the 1 trunk leading to heart failure.

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Transposition of the Great Arteries (TGA)

Arteries are switched, and blood in deoxygenated, causing cyanosis that birth that requires immediate treatment

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Tricuspid Atresia (absent)

No connection between RA and RV. Blood goes from RA to LA through PFO and RV is small, requiring VSD for blood to reach the lungs.

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Total Anomalous Pulmonary Venous Return

The pulmonary veins are attached to SUC or IVC that lead to cyanosis and signs of heart failure.

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Abnormalities in the tricuspid valve and right ventricle

Ebstein anomaly

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Infant Treatment Hypoplastic Left Heart Syndrome

Very cyanotic and ill in the first few days of life and give PGE1 supportive therapy

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Unrepaired cyanotic heart

Dental cleaning

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Radiology Findings

Rib Notching = Coarctation of the aorta, Boot shaped heart = TOF, Egg on a string appearance = TGA, Snowman or Figure of 8 appearance = TAPVR.

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Pulmonary Valve Flow Murmur

A systolic ejection murmur heard best at the 2nd left intercostal space alongside a fixed split S2

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Management of Tet Spell

Administer 100% oxygen, place the child in the knee-chest position, and consider morphine to decrease agitation and pulmonary vascular resistance

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Hypoplastic Left Heart Syndrome (HLHS) Initial Management

RV supports all circulation; maintain ductus patency with Prostaglandin E1 (PGE1)

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Tetralogy of Fallot Diagnosis

Cyanosis and single S2 sound, imaging reveals a boot-shaped heart

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Truncus Arteriosus on CXR

Single arterial trunk overriding a large VSD with cardiomegaly and increased pulmonary vascular markings

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Ebstein's Anomaly

Maternal lithium exposure, malformed tricuspid valve, ASD/VSD

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Tricuspid Atresia

No communication between the right atrium and right ventricle, a large PFO, and a small RV

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Endocarditis Prophylaxis

Requires prophylaxis for unrepaired cyanotic CHD, repair with prosthetic material within 6 months, or residual defect after repair

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ASD (Atrial Septal Defect) Murmur

Fixed, widely split S2 and a systolic ejection murmur at the 2nd left intercostal space.

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PGE1 for TGA (Transposition of the Great Arteries)

Give PGE1 to keep the ductus arteriosus open in order to allow for systemic blood flow

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Coarctation of the Aorta Symptoms

Hypertension in arms, diminished/delayed femoral pulses, rib notching and 3 sign on CXR

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VSD Characteristics

Holosystolic murmur at LLSB with palpable thrill that does not radiate; associated with frequent respiratory infections and poor weight gain

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TGA Diagnostic Findings

Narrow mediastinum/"egg-on-a-string" on CXR; cyanosis shortly after birth, requiring PDA/PFO maintenance with PGE1

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PDA Characteristics

Continuous "machinery-like" murmur, poor feeding, and respiratory distress; treat with indomethacin in preterm infants to close the PDA

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Study Notes

Non-Cyanotic CHD: Atrial Septal Defect (ASD)

  • Increased blood flow across the pulmonic valve due to increased flow across the atrial septal defect causes a systolic murmur at the 2nd left intercostal space.
  • Fixed splitting of S2 hearts sound is heard with ASD.

Non-Cyanotic CHD: Ventricular Septal Defect (VSD)

  • VSD causes a holosystolic murmur, heard best at the lower left sternal border, and a palpable thrill.
  • Murmur does not radiate and is not affected by position.
  • Frequent respiratory infections and poor weight gain can occur.
  • Hyperdynamic heart from volume overload is present.

Non-Cyanotic CHD: Patent Ductus Arteriosus (PDA)

  • Indomethacin is a prostaglandin E1 inhibitor and is the first-line treatment in preterm infants to close a PDA.

Cyanotic CHD: Transposition of the Great Arteries (TGA)

  • TGA presents with cyanosis at birth as well as narrow mediastinum and "egg-on-a-string" appearance on CXR.
  • PDA/PFO is needed for survival, and prostaglandin E1 (PGE1) is administered.

Cyanotic CHD: Tetralogy of Fallot (TOF)

  • Place child in knee-chest position as first priority to manage "tet spell".
  • "Tet spell" is associated with increased systemic vascular resistance, increased right-to-left shunt, and cyanosis.
  • Squatting or knee-to-chest position increases preload and systemic vascular resistance, helping to reverse the shunt and improve oxygenation.

Cyanotic CHD: Total Anomalous Pulmonary Venous Return (TAPVR)

  • TAPVR is associated with pulmonary veins draining into the superior vena cava, oxygenation maintained only through a patent foramen ovale (PFO), and a snowman sign on CXR.
  • Pulmonary veins drain into the SVC/IVC, snowman sign present, PFO needed to survive.

Cyanotic CHD: Ebstein's Anomaly

  • Maternal lithium exposure is a risk factor for Ebstein's anomaly.
  • Ebstein's anomaly includes malformed tricuspid valve and ASD/VSD.

Eisenmenger Syndrome

  • Eisenmenger syndrome develops when pulmonary hypertension and right-to-left shunt occur due to ASD/VSD/PDA.
  • Once Eisenmenger syndrome develops closure is too late.

Non-Cyanotic CHD: Coarctation of the Aorta (CoA)

  • Coarctation of the aorta is associated with hypertension, headaches, cold legs, and diminished pulses.
  • Rib notching and a 3-sign can be seen on chest X-ray (CXR).
  • Hypertension with leg claudication, rib notching, and 3 sign are signs of coarctation.

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