Congenital Heart Disease

Question 1

What is the prevalence of congenital heart diseases (CHDs) in the US?

Answer 1

Nearly 1% or about 40,000 births per year.

Question 2

What is the most common type of congenital heart defect?

Answer 2

Ventricular Septal Defect (VSD).

Question 3

What percentage of babies with non-critical CHDs survive to 18 years of age?

Answer 3

About 95%.

Question 4

What are the survival rates for babies with critical CHDs?

Answer 4

About 69% survive to 18 years of age.

Question 5

List common features of an atrial septal defect.

Answer 5

Right ventricular heave, widely split S2, grade I-III/VI systolic murmur at the upper left sternal border.

Question 6

When is treatment indicated for atrial septal defects?

Answer 6

Elective closure for large defects at 4-5 years of age.

Question 7

Describe the murmur in a ventricular septal defect (VSD).

Answer 7

Grade II-IV/VI harsh pansystolic murmur heard best at the lower left sternal border.

Question 8

What characterizes a patent ductus arteriosus (PDA)?

Answer 8

Bounding pulses, widened pulse pressure, and a continuous machinery murmur.

Question 9

When is treatment recommended for small PDAs?

Answer 9

If a murmur is present due to the risk of endarteritis.

Question 10

What are ‘tet spells’?

Answer 10

Sudden, potentially lethal episodes of severe cyanosis, often in Tetralogy of Fallot.

Question 11

What antenatal factors may suggest CHDs?

Answer 11

Cardiac malformations, fetal arrhythmias, hydrops on antenatal scans.

Question 12

What maternal illnesses are associated with CHDs?

Answer 12

Diabetes, rubella, and use of teratogenic medications.

Question 13

List key features on physical examination indicating CHDs.

Answer 13

Dysmorphism, central cyanosis, tachypnea, heart murmurs, hepatomegaly.

Question 14

What does the hyperoxia test involve?

Answer 14

Administering 100% oxygen, measuring ABG, with pO2 < 100 mmHg indicating cyanotic heart disease.

Question 15

What are the initial stabilization steps for managing CHDs?

Answer 15

Secure airway, ensure ventilation, provide circulatory support, and correct metabolic derangements.

Question 16

When is IV prostaglandin E infusion used?

Answer 16

For duct-dependent lesions to maintain ductus arteriosus patency.

Question 17

What are adverse effects of IV prostaglandin E?

Answer 17

Apnea, fever, hypotension.

Question 18

What conditions might present similarly to unresponsive duct-dependent lesions?

Answer 18

Transposition of great arteries, obstructed total anomalous pulmonary venous return.

Question 19

What is the characteristic murmur in PDA?

Answer 19

Continuous machinery murmur best heard at the upper left sternal border.

Question 20

What are common symptoms of critical CHDs in infants?

Answer 20

Cyanosis, dyspnea with feeding, poor growth, and hypercyanotic spells.

Question 21

What is the role of echocardiography in CHDs?

Answer 21

To confirm structural abnormalities and assess heart function.

Question 22

What percentage of under-five deaths in Malaysia in 2006 were related to CHDs?

Answer 22

0.1

Question 23

How are small atrial septal defects managed?

Answer 23

No treatment if asymptomatic.

Question 24

What is a common chest X-ray finding in CHDs?

Answer 24

Cardiac enlargement (cardiomegaly).

Question 25

What are the most common chromosomal syndromes associated with CHDs?

Answer 25

Trisomy 21, 18, 13, and Turner syndrome.

Question 26

Why is family history important in diagnosing CHDs?

Answer 26

CHDs often have a genetic component.

Question 27

How does the pulse pressure change in PDA?

Answer 27

It becomes widened.

Question 28

What are cyanotic heart diseases indicated by a hyperoxia test?

Answer 28

Conditions with a pO2 < 100 mmHg and minimal rise in pO2 after oxygen administration.

Question 29

What are the main goals of initial CHD management?

Answer 29

Stabilize the patient and prepare for transfer to a cardiac center if needed.

Question 30

What is the treatment for moderate to large PDAs?

Answer 30

Anti-failure therapy, surgical or transcatheter closure based on symptoms and size.

Question 31

What are bounding pulses indicative of in CHD?

Answer 31

Patent ductus arteriosus (PDA).

Question 32

What diagnostic measure confirms cyanotic heart disease in neonates?

Answer 32

Hyperoxia test with ABG analysis.

Question 33

What imaging is critical for assessing congenital heart defects?

Answer 33

Echocardiography.

Question 34

Which CHD is most commonly asymptomatic?

Answer 34

Atrial septal defect (ASD).

Question 35

What is the prognosis for most children with non-critical CHDs?

Answer 35

Survival to adulthood is expected in 95% of cases.

Question 36

What is the significance of a single second heart sound in CHD?

Answer 36

It is common in cyanotic conditions like Tetralogy of Fallot.

Question 37

How are heart murmurs in CHDs graded?

Answer 37

Based on intensity, from grade I (softest) to grade VI (loudest).

Question 38

What defines critical congenital heart disease?

Answer 38

Defects requiring intervention in the first year of life to prevent mortality.

Question 39

What physical exam findings suggest heart failure in CHDs?

Answer 39

Hepatomegaly, tachypnea, and weak or unequal pulses.

Question 40

Why is prompt cardiology consultation crucial in CHDs?

Answer 40

To ensure early diagnosis and intervention.

Question 41

What is the hallmark symptom of cyanotic heart disease in newborns?

Answer 41

Central cyanosis.

Question 42

What does a harsh systolic murmur at the left upper sternal border suggest?

Answer 42

Tetralogy of Fallot or other cyanotic heart defects.

Question 43

What conditions may be associated with dysmorphism in CHDs?

Answer 43

Genetic syndromes like Down syndrome or Turner syndrome.

Question 44

What general management is essential for duct-dependent lesions?

Answer 44

IV prostaglandin E infusion to maintain ductal patency.

Question 45

What investigation detects fetal cardiac malformations antenatally?

Answer 45

Ultrasound scans.

Question 46

Why might a cyanotic infant need immediate intervention?

Answer 46

To prevent hypoxia-related organ damage or mortality.

Question 47

What is the main treatment goal for large atrial septal defects?

Answer 47

Surgical closure to prevent complications like heart failure or arrhythmias.

Question 48

What symptom is commonly seen with feeding in cyanotic CHDs?

Answer 48

Dyspnea or difficulty feeding due to inadequate oxygenation.

Question 49

Which CHD is characterized by a machinery murmur?

Answer 49

Patent ductus arteriosus (PDA).

Question 50

What are 'tet spells' and how are they managed?

Answer 50

Episodes of cyanosis in Tetralogy of Fallot; managed with squatting, oxygen, or medications.

Question 51

What are common perinatal problems linked to CHDs?

Answer 51

Prematurity, meconium aspiration, and perinatal asphyxia.

Question 52

Why is metabolic acidosis corrected in CHDs?

Answer 52

To stabilize the patient and prevent further complications.

Question 53

What are general measures for stabilizing infants with CHDs?

Answer 53

Adequate ventilation, circulatory support, and preventing hypothermia.

Question 54

How is a ventricular septal defect murmur described?

Answer 54

A harsh, medium-to-high-pitched pansystolic murmur.

Question 55

What does hepatomegaly in a neonate suggest in CHDs?

Answer 55

Right heart failure or systemic venous congestion.

Question 56

What is the first-line medication for duct-dependent CHDs?

Answer 56

IV prostaglandin E infusion.

Question 57

What does a single second heart sound indicate?

Answer 57

Cyanotic heart disease like Tetralogy of Fallot.

Question 58

Additional question 58

Answer 58

Additional answer.

Question 59

Additional question 59

Answer 59

Additional answer.

Question 60

Additional question 60

Answer 60

Additional answer.