MCQ Quiz: Congenital Heart Disease

Congenital heart disease (CHD) represents a diverse group of structural abnormalities of the heart and great vessels present at birth. As one of the most common types of birth defects, CHD management requires a deep, interdisciplinary understanding of complex pathophysiology and targeted pharmacotherapy. For PharmD students, knowledge of this area is critical, as medications play a vital role in managing symptoms, preventing complications, and providing support before and after surgical interventions. This quiz covers advanced topics in pediatric cardiology, including the classification of acyanotic and cyanotic defects, the use of prostaglandins, diuretics, and agents for pulmonary hypertension, and the crucial role of the pharmacist in optimizing care for these vulnerable patients.

1. Which of the following is an example of an acyanotic congenital heart defect with a left-to-right shunt?

• a) Tetralogy of Fallot
• b) Transposition of the Great Arteries
• c) Ventricular Septal Defect (VSD)
• d) Tricuspid Atresia

Answer: c) Ventricular Septal Defect (VSD)

2. A neonate with a ductal-dependent systemic circulation (e.g., severe coarctation of the aorta) requires which medication to maintain patency of the ductus arteriosus?

• a) Indomethacin
• b) Ibuprofen
• c) Prostaglandin E1 (Alprostadil)
• d) Furosemide

Answer: c) Prostaglandin E1 (Alprostadil)

3. What is the primary mechanism of action of indomethacin when used for pharmacologic closure of a patent ductus arteriosus (PDA) in a premature infant?

• a) It stimulates prostaglandin synthesis.
• b) It is a potent vasodilator.
• c) It inhibits prostaglandin synthesis, which is responsible for keeping the ductus open.
• d) It directly constricts vascular smooth muscle.

Answer: c) It inhibits prostaglandin synthesis, which is responsible for keeping the ductus open.

4. A key feature of Tetralogy of Fallot (TOF) that contributes to cyanosis is:

• a) A large atrial septal defect.
• b) An overriding aorta that receives blood from both ventricles.
• c) Pulmonary artery stenosis.
• d) Both B and C are correct.

Answer: d) Both B and C are correct.

5. What is a “tet spell,” often seen in infants with unrepaired Tetralogy of Fallot?

• a) A type of seizure caused by low blood oxygen.
• b) An episode of profound cyanosis and hyperpnea, often triggered by crying or feeding.
• c) A sudden drop in blood pressure.
• d) A period of asymptomatic heart murmur.

Answer: b) An episode of profound cyanosis and hyperpnea, often triggered by crying or feeding.

6. Which of the following congenital heart defects is characterized by the aorta arising from the right ventricle and the pulmonary artery from the left ventricle?

• a) Atrial Septal Defect (ASD)
• b) Coarctation of the Aorta
• c) Transposition of the Great Arteries (TGA)
• d) Patent Ductus Arteriosus (PDA)

Answer: c) Transposition of the Great Arteries (TGA)

7. A child with a large, un-repaired VSD is at risk for developing which long-term complication due to chronic pulmonary overcirculation?

• a) Systemic hypertension
• b) Aortic stenosis
• c) Eisenmenger syndrome (pulmonary hypertension with shunt reversal)
• d) Coronary artery disease

Answer: c) Eisenmenger syndrome (pulmonary hypertension with shunt reversal)

8. Which diuretic is commonly used to manage symptoms of fluid overload and heart failure in infants with large left-to-right shunts?

• a) Hydrochlorothiazide
• b) Furosemide
• c) Amiloride
• d) Acetazolamide

Answer: b) Furosemide

9. In pediatric patients with heart failure secondary to CHD, an ACE inhibitor like captopril is used primarily to:

• a) Increase the heart rate.
• b) Reduce afterload and promote systemic blood flow.
• c) Increase pulmonary blood flow.
• d) Constrict the pulmonary arteries.

Answer: b) Reduce afterload and promote systemic blood flow.

10. According to the latest guidelines, antibiotic prophylaxis to prevent infective endocarditis before a dental procedure is recommended for which patient?

• a) A patient with a repaired secundum atrial septal defect without residual shunt.
• b) A patient with a history of rheumatic fever without valvular disease.
• c) A patient with a prosthetic heart valve.
• d) All patients with any form of congenital heart disease.

Answer: c) A patient with a prosthetic heart valve.

11. What is the standard oral antibiotic regimen for infective endocarditis prophylaxis in a child who can take oral medication and has no penicillin allergy?

• a) A single dose of Azithromycin 1 hour before the procedure.
• b) A single dose of Amoxicillin 1 hour before the procedure.
• c) A 7-day course of Penicillin VK starting the day of the procedure.
• d) A single dose of Clindamycin 1 hour before the procedure.

Answer: b) A single dose of Amoxicillin 1 hour before the procedure.

12. Coarctation of the Aorta is best described as:

• a) A hole between the atria.
• a) A narrowing of the aorta.
• c) A connection between the aorta and pulmonary artery.
• d) A misplaced coronary artery.

Answer: a) A narrowing of the aorta.

13. A classic clinical finding in an infant with significant coarctation of the aorta is:

• a) Equal blood pressures in the arms and legs.
• b) Higher blood pressure in the lower extremities compared to the upper extremities.
• c) Bounding pulses in the feet.
• d) Higher blood pressure in the upper extremities compared to the lower extremities.

Answer: d) Higher blood pressure in the upper extremities compared to the lower extremities.

14. Digoxin therapy in an infant with CHD requires careful monitoring due to its narrow therapeutic index. A sign of toxicity could be:

• a) Tachycardia
• b) Vomiting and poor feeding
• c) Increased alertness
• d) High fever

Answer: b) Vomiting and poor feeding

15. A common side effect of prostaglandin E1 infusion is:

• a) Hypertension
• b) Apnea (cessation of breathing)
• c) Constipation
• d) Hypothermia

Answer: b) Apnea (cessation of breathing)

16. Which type of atrial septal defect (ASD) is the most common and often closes spontaneously in the first year of life?

• a) Primum ASD
• b) Secundum ASD
• c) Sinus venosus ASD
• d) Coronary sinus ASD

Answer: b) Secundum ASD

17. What is the primary role of a pharmacist when a premature infant is started on ibuprofen for PDA closure?

• a) To administer the IV dose.
• b) To confirm the correct dose based on weight and monitor for adverse effects like renal dysfunction and GI bleeding.
• c) To perform an echocardiogram to confirm closure.
• d) To decide if the infant is a surgical candidate.

Answer: b) To confirm the correct dose based on weight and monitor for adverse effects like renal dysfunction and GI bleeding.

18. In a patient with Eisenmenger syndrome, a right-to-left shunt develops, leading to:

• a) Increased oxygen saturation.
• b) Chronic cyanosis and polycythemia.
• c) Left ventricular hypertrophy.
• d) Systemic hypotension.

Answer: b) Chronic cyanosis and polycythemia.

19. Which medication class is commonly used to manage pulmonary arterial hypertension (PAH) in patients with CHD?

• a) Beta-blockers
• b) Calcium channel blockers
• c) Phosphodiesterase-5 inhibitors (e.g., sildenafil)
• d) Statins

Answer: c) Phosphodiesterase-5 inhibitors (e.g., sildenafil)

20. A child with a complex single-ventricle physiology (e.g., Hypoplastic Left Heart Syndrome) may be prescribed what medication post-surgically to prevent thrombosis?

• a) Ibuprofen
• b) Acetaminophen
• c) Aspirin or other anticoagulants
• d) Propranolol

Answer: c) Aspirin or other anticoagulants

21. The “lub-dub” sound of a normal heartbeat is produced by:

• a) The flow of blood through the aorta.
• b) The contraction of the atria.
• c) The closing of the heart valves.
• d) The firing of the sinoatrial (SA) node.

Answer: c) The closing of the heart valves.

22. Which acyanotic defect is often described as causing a “machine-like” continuous murmur?

• a) Atrial Septal Defect (ASD)
• b) Ventricular Septal Defect (VSD)
• c) Patent Ductus Arteriosus (PDA)
• d) Coarctation of the Aorta

Answer: c) Patent Ductus Arteriosus (PDA)

23. When counseling parents of a child on furosemide, it is important to mention monitoring for:

• a) Hyperkalemia
• b) Hypokalemia and dehydration
• c) Weight gain
• d) Hypertension

Answer: b) Hypokalemia and dehydration

24. A cyanotic heart defect is one that involves:

• a) A shunt of oxygenated blood from the left side of the heart to the right.
• b) A narrowing of the aortic valve.
• c) A shunt of deoxygenated blood from the right side of the heart to the left, bypassing the lungs.
• d) An abnormally slow heart rate.

Answer: c) A shunt of deoxygenated blood from the right side of the heart to the left, bypassing the lungs.

25. Spironolactone is often used in combination with furosemide in pediatric heart failure because it:

• a) Is a more potent diuretic.
• b) Helps to counteract potassium loss.
• c) Works faster than furosemide.
• d) Lowers the heart rate.

Answer: b) Helps to counteract potassium loss.

26. A key counseling point for a child taking an ACE inhibitor is to report:

• a) Increased energy levels.
• b) A persistent, dry cough.
• c) Improved appetite.
• d) Darker urine.

Answer: b) A persistent, dry cough.

27. The four classic components of Tetralogy of Fallot are VSD, pulmonary stenosis, overriding aorta, and:

• a) Left ventricular hypertrophy
• b) Mitral valve regurgitation
• c) Aortic stenosis
• d) Right ventricular hypertrophy

Answer: d) Right ventricular hypertrophy

28. An infant with TGA must have mixing of oxygenated and deoxygenated blood to survive. This mixing can occur through a PDA, VSD, or:

• a) A coarctation of the aorta
• b) An atrial septal defect (ASD)
• c) A normal foramen ovale
• d) The coronary arteries

Answer: b) An atrial septal defect (ASD)

29. What is a significant challenge in providing nutritional support to infants with symptomatic CHD?

• a) They have a very low metabolic rate.
• b) They often have poor feeding and increased caloric needs due to increased work of breathing.
• c) They cannot tolerate protein.
• d) They require a low-fat diet.

Answer: b) They often have poor feeding and increased caloric needs due to increased work of breathing.

30. Which of these is NOT a cyanotic congenital heart defect?

• a) Tetralogy of Fallot
• b) Transposition of the Great Arteries
• c) Atrial Septal Defect
• d) Truncus Arteriosus

Answer: c) Atrial Septal Defect

31. The primary risk of a large, un-repaired atrial septal defect in adulthood is:

• a) Aortic aneurysm
• b) Paradoxical embolism and stroke
• c) Myocardial infarction
• d) Systemic hypertension

Answer: b) Paradoxical embolism and stroke

32. The immediate management of a hypercyanotic “tet spell” includes:

• a) Administering a diuretic.
• b) Placing the infant in a knee-chest position and providing supplemental oxygen.
• c) Encouraging the infant to cry to open up the lungs.
• d) Administering a dose of aspirin.

Answer: b) Placing the infant in a knee-chest position and providing supplemental oxygen.

33. What is the role of captopril in a pediatric patient with a large VSD and signs of heart failure?

• a) To close the VSD.
• b) To decrease systemic vascular resistance, making it easier for the left ventricle to pump blood to the body instead of through the VSD.
• c) To increase the heart rate.
• d) To increase pulmonary vascular resistance.

Answer: b) To decrease systemic vascular resistance, making it easier for the left ventricle to pump blood to the body instead of through the VSD.

34. For a child with CHD who is prescribed warfarin, what is a critical counseling point for the family?

• a) The importance of a diet with highly variable vitamin K intake.
• b) The need for regular INR monitoring to ensure therapeutic anticoagulation.
• c) That the medication can be stopped if the child feels well.
• d) That bruising is not a concern.

Answer: b) The need for regular INR monitoring to ensure therapeutic anticoagulation.

35. A “shunt” in cardiology refers to:

• a) A type of heart valve.
• b) An abnormal pathway for blood flow between heart chambers or blood vessels.
• c) The electrical conduction system of the heart.
• d) A surgical instrument.

Answer: b) An abnormal pathway for blood flow between heart chambers or blood vessels.

36. A child with a history of CHD and a prosthetic valve repair requires antibiotic prophylaxis for a routine dental cleaning. They have a history of anaphylaxis to penicillin. What is an appropriate alternative?

• a) Amoxicillin
• b) Cephalexin
• c) Clindamycin
• d) Vancomycin

Answer: c) Clindamycin

37. Which aspect of a pharmacist’s role is crucial in managing medications for children with CHD?

• a) Diagnosing the specific type of CHD.
• b) Recommending surgical procedures.
• c) Ensuring appropriate dosing, preparing special formulations if needed, and monitoring for adverse effects.
• d) Interpreting echocardiogram results.

Answer: c) Ensuring appropriate dosing, preparing special formulations if needed, and monitoring for adverse effects.

38. The term “acyanotic” means:

• a) The patient appears blue due to low oxygen levels.
• b) There is no structural defect in the heart.
• c) There is no significant mixing of deoxygenated blood into the systemic circulation.
• d) The heart rate is abnormally fast.

Answer: c) There is no significant mixing of deoxygenated blood into the systemic circulation.

39. Long-term use of high-dose furosemide in infants can lead to which of the following complications?

• a) Hypercalcemia
• b) Nephrocalcinosis (calcium deposits in the kidneys)
• c) Improved bone density
• d) Weight gain

Answer: b) Nephrocalcinosis (calcium deposits in the kidneys)

40. A small, muscular VSD is most likely to:

• a) Require immediate surgical intervention.
• b) Cause severe cyanosis at birth.
• c) Close spontaneously over time.
• d) Lead to rapid development of pulmonary hypertension.

Answer: c) Close spontaneously over time.

41. Why might a child with significant CHD require higher caloric density formula?

• a) They are often less active than other children.
• b) To promote rapid weight gain and obesity.
• c) To meet their increased metabolic demands with less fluid volume.
• d) Because standard formula tastes bad to them.

Answer: c) To meet their increased metabolic demands with less fluid volume.

42. Which diagnostic tool is the primary method for visualizing and diagnosing structural congenital heart defects?

• a) Chest X-ray
• b) Electrocardiogram (ECG)
• c) Echocardiogram
• d) Cardiac catheterization

Answer: c) Echocardiogram

43. A pharmacist is dispensing sildenafil for a child with pulmonary arterial hypertension. What is a key counseling point?

• a) This medication is used to treat erectile dysfunction.
• b) This medication helps relax the blood vessels in the lungs to lower pressure.
• c) This medication should only be taken when the child feels short of breath.
• d) The medication will cure the pulmonary hypertension.

Answer: b) This medication helps relax the blood vessels in the lungs to lower pressure.

44. A child with complete atrioventricular (AV) canal defect has defects in both the atrial and ventricular septa, as well as abnormalities of the AV valves. This condition is most commonly associated with:

• a) Turner Syndrome
• b) Marfan Syndrome
• c) Trisomy 21 (Down Syndrome)
• d) Cystic Fibrosis

Answer: c) Trisomy 21 (Down Syndrome)

45. Before dispensing a prescription for digoxin for an infant, the pharmacist should confirm that:

• a) The dose is prescribed in milligrams (mg), not micrograms (mcg).
• b) The dose is appropriate for the infant’s weight and renal function.
• c) The family understands how to administer the exact dose with an oral syringe.
• d) All of the above are correct.

Answer: d) All of the above are correct.

46. Which of the following is considered a “single ventricle” lesion?

• a) Atrial Septal Defect
• b) Hypoplastic Left Heart Syndrome (HLHS)
• c) Patent Ductus Arteriosus
• d) Small Ventricular Septal Defect

Answer: b) Hypoplastic Left Heart Syndrome (HLHS)

47. A common goal for pharmacotherapy in a child with a large VSD causing heart failure is to:

• a) Increase the size of the VSD.
• b) Medically manage the symptoms of heart failure until the child is big enough for surgical repair.
• c) Increase the pressure in the pulmonary artery.
• d) Avoid surgery altogether.

Answer: b) Medically manage the symptoms of heart failure until the child is big enough for surgical repair.

48. Patients who have undergone surgical repair for coarctation of the aorta need lifelong monitoring for what common complication?

• a) Hypotension
• b) Bradycardia
• c) Recoarctation or late hypertension
• d) Aortic valve regurgitation

Answer: c) Recoarctation or late hypertension

49. An infant with a cyanotic heart defect would likely present with an oxygen saturation that is:

• a) 98-100% on room air.
• b) Persistently low (e.g., 75-85%) and does not improve significantly with supplemental oxygen.
• c) Abnormally high (greater than 100%).
• d) Fluctuating wildly between 50% and 100%.

Answer: b) Persistently low (e.g., 75-85%) and does not improve significantly with supplemental oxygen.

50. The pharmacist’s role in the transition of care for an adolescent with CHD moving to adult care includes:

• a) Ensuring the patient understands their condition and the importance of lifelong medication adherence and follow-up.
• b) Performing the final surgical repair.
• c) Deciding which adult cardiologist the patient should see.
• d) Discontinuing all of the patient’s medications.

Answer: a) Ensuring the patient understands their condition and the importance of lifelong medication adherence and follow-up.