Congenital Heart Diseases (Acyanotic, Cyanotic) MCQs

Pediatrics · 50 free questions with answers & explanations.

1. A 2-month-old infant presents with poor feeding, excessive sweating during feeds, and failure to thrive. Examination reveals a harsh pansystolic murmur at the left lower sternal border, with a mid-diastolic rumble at the apex. What is the MOST likely diagnosis?
2. A cyanotic 4-day-old neonate develops worsening central cyanosis that improves with 100% oxygen administration (hyperoxia test PaO2 rises from 35 to 55 mmHg). Echocardiography shows the aorta arising from the right ventricle and pulmonary artery from the left ventricle. What is the DEFINITIVE treatment?
3. A 2-year-old boy has cyanosis since birth, squatting posture during play, and hypercyanotic spells relieved by knee-chest position. Echocardiography confirms Tetralogy of Fallot. Which of the following is NOT a component of this defect?
4. A 6-week-old preterm baby born at 30 weeks has persistent tachycardia, bounding pulses, and a continuous machinery murmur below the left clavicle. What is the FIRST-LINE medical treatment for this condition?
5. A 3-year-old child is found to have fixed splitting of the second heart sound (S2), a soft ejection systolic murmur at the upper left sternal border, and increased pulmonary vascular markings on chest X-ray. What is the MOST likely congenital heart defect?
6. A 3-year-old child has a loud pansystolic murmur at the lower left sternal border, no cyanosis, and echocardiography confirms a perimembranous VSD with left-to-right shunt and Qp:Qs of 2.8:1. The child has recurrent respiratory infections but no Eisenmenger changes. Regarding the risk of infective endocarditis (IE) prophylaxis, the CURRENT recommendation for this uncorrected VSD is:
7. A 6-hour-old neonate is deeply cyanotic with no respiratory distress. SpO2 is 55% in room air. CXR shows an 'egg-on-side' cardiac silhouette with narrow superior mediastinum and increased pulmonary vascularity. Prostaglandin E1 is started. The underlying anatomical anomaly responsible for parallel circulations in this defect is:
8. A 14-year-old boy presents with exertional dyspnea, clubbing, and central cyanosis that worsens over two years. He was diagnosed with a large VSD at birth but parents declined surgery. ECG shows right axis deviation and right ventricular hypertrophy. SpO2 is 82% on room air. The MOST important prognostic implication of this presentation is:
9. A neonate is diagnosed with total anomalous pulmonary venous connection (TAPVC) with obstruction. Which of the following variants is MOST commonly associated with obstruction?
10. A cyanotic neonate with tricuspid atresia, normally related great arteries, and a small VSD is being assessed. Which component of the cardiac catheterization data MOST accurately reflects the severity of pulmonary blood flow restriction?
11. A 3-year-old child presents with a harsh pansystolic murmur loudest at the lower left sternal border, with a thrill. Chest X-ray shows mildly increased pulmonary vascular markings. Echocardiography confirms a small perimembranous VSD with a left-to-right shunt and normal pulmonary artery pressure. What is the most appropriate next step in management?
12. A 6-month-old infant with Down syndrome presents with recurrent respiratory infections, poor feeding, and failure to thrive. Echocardiography reveals a complete atrioventricular septal defect (AVSD) with both atrial and ventricular components and a common AV valve. Which of the following is a cardinal feature distinguishing complete AVSD from partial AVSD on echocardiography?
13. A neonate presents with severe cyanosis at birth unresponsive to oxygen supplementation (hyperoxia test remains positive). ECG shows right axis deviation and right ventricular hypertrophy. Chest X-ray shows an 'egg on string' cardiac silhouette with a narrow superior mediastinum. Which of the following is the immediate management while awaiting surgery?
14. A 4-year-old child with Tetralogy of Fallot is brought to the emergency with severe cyanosis, deep breathing, and altered sensorium during a crying spell. Which of the following is NOT a component of the management of a hypercyanotic ('Tet') spell?
15. A neonate presents with shock-like symptoms, severe metabolic acidosis, and absent femoral pulses on day 2 of life as the ductus arteriosus starts to close. Chest X-ray shows cardiomegaly and pulmonary edema. Echocardiography reveals a hypoplastic left ventricle, severely hypoplastic aortic root, and critical coarctation. Which of the following is the most appropriate initial medical management?
16. A 3-month-old infant with Down syndrome presents with tachypnoea and poor feeding. Echocardiography reveals a complete atrioventricular septal defect (AVSD). Surgery is best planned at what age to prevent irreversible pulmonary hypertension?
17. A 2-day-old neonate develops sudden severe cyanosis, tachycardia, and circulatory collapse. Echocardiography reveals transposition of the great arteries (TGA) with intact ventricular septum. Immediate stabilization is best achieved by:
18. A 6-year-old child with Ebstein anomaly undergoes cardiac evaluation. The hallmark ECG finding in Ebstein anomaly that distinguishes it from most other CHDs is:
19. A 6-month-old infant with Down syndrome presents with recurrent lower respiratory tract infections, failure to thrive, and a holosystolic murmur loudest at the lower left sternal border. Echocardiography reveals a complete atrioventricular septal defect (AVSD). At what age is surgical repair MOST appropriately planned?
20. A 4-year-old boy presents with progressive dyspnoea on exertion and cyanosis that is MORE pronounced in the lower extremities than the upper extremities. There is clubbing of the toes but not the fingers. Which defect BEST explains this presentation?
21. A newborn is found to have a bicuspid aortic valve with severe coarctation of the aorta. On day 3 of life, the neonate deteriorates acutely with poor femoral pulses. What is the immediate pharmacological intervention?
22. A child with Tetralogy of Fallot has a hypercyanotic spell (tet spell). Which combination of manoeuvres is MOST effective in terminating the spell?
23. A 3-year-old presents with recurrent squatting episodes, central cyanosis, and a harsh ejection systolic murmur at the left upper sternal border. Echocardiography confirms Tetralogy of Fallot with a hypoplastic pulmonary valve annulus. During a hypercyanotic (Tet) spell, which mechanism is primarily responsible for the acute worsening of cyanosis?
24. A full-term neonate presents at 5 days of age with sudden cardiovascular collapse. Prior to collapse, the infant fed well and had no cyanosis. On examination: mottled skin, absent femoral pulses, hepatomegaly, severe metabolic acidosis on blood gas. Echocardiography shows absent left ventricular cavity with aortic atresia. Which prostaglandin E1 dose and route is used as a bridge to surgical palliation?
25. An 8-year-old child is incidentally found to have a systolic murmur. Echocardiography reveals a secundum ASD with left-to-right shunt, Qp:Qs of 2.2:1, and mild right ventricular dilatation. There are no symptoms. Which statement about management of this secundum ASD is MOST accurate?
26. A 6-week-old infant has been in cardiac failure since birth. Echocardiography shows an L-transposition of the great arteries (congenitally corrected TGA, cc-TGA). The anatomical right ventricle is serving as the systemic ventricle. Which long-term complication is MOST characteristic of cc-TGA even without associated defects?
27. A 3-month-old infant has a prominent left precordium, harsh pansystolic murmur at the lower left sternal border, and a diastolic rumble at the apex. The ECG shows biventricular hypertrophy. Chest X-ray shows cardiomegaly with plethoric lung fields. The infant feeds poorly and sweats with feeds. Which of the following management decisions is CORRECT?
28. A 2-day-old neonate has cyanosis that does not improve with 100% oxygen administration (hyperoxia test: PaO2 increases from 40 to only 48 mmHg). Cardiac impulse is in the midline and heart sounds are best heard on the right. Echo confirms dextrocardia with situs inversus. The most likely underlying structural defect in the setting of cyanosis unresponsive to oxygen is:
29. A 5-year-old child with Down syndrome presents with increasing breathlessness and cyanosis. Examination reveals a right ventricular heave, absent second heart sound, loud P2, and peripheral oxygen saturation of 78%. Previously documented VSD murmur is now absent. Which best explains this clinical scenario?
30. A 3-month-old infant with Down syndrome presents with poor feeding, failure to thrive, and recurrent chest infections. On examination, there is a harsh pansystolic murmur at the lower left sternal border, and a soft mid-diastolic murmur at the apex. Echocardiography reveals a complete atrioventricular septal defect (AVSD). What is the MOST important reason for early surgical repair before 6 months?
31. A 6-week-old infant presents with sudden severe cyanosis, hyperpnea, and irritability. Cardiac examination reveals absent pulmonary component of S2, and a right ventricular heave. Echocardiography shows tetralogy of Fallot. What precipitates these 'tet spells' at this age?
32. A newborn is diagnosed with transposition of the great arteries (TGA) on echocardiography. The baby is severely cyanotic with an O2 saturation of 55%. The most critical intervention to maintain life until corrective surgery is:
33. A 5-year-old child with known unrepaired VSD presents with increasing cyanosis and clubbing over the past year. Echocardiography now shows right-to-left shunting across the VSD and severe pulmonary hypertension. PVR/SVR ratio is 0.8. This child's condition is best characterised as:
34. A newborn with d-Transposition of Great Arteries (d-TGA) and an intact ventricular septum presents with severe cyanosis (SpO2 52%) within hours of birth. Prostaglandin E1 infusion is started. The definitive anatomical correction performed in the neonatal period is:
35. A hypercyanotic 'tet spell' is occurring in a 6-month-old child with Tetralogy of Fallot. The child is squatting-equivalent (placed prone by the nurse) and is given 100% oxygen by mask. SpO2 remains 65% despite these measures. The most appropriate pharmacological intervention is:
36. A 6-month-old infant presents with failure to thrive, recurrent lower respiratory tract infections, and a harsh pansystolic murmur at the left sternal border. Echocardiography confirms a large perimembranous VSD with Qp:Qs ratio of 3:1. Which hemodynamic change is MOST responsible for the recurrent respiratory symptoms?
37. A 2-year-old child with known Tetralogy of Fallot suddenly develops paroxysmal hypercyanosis, irritability, and loss of consciousness during crying. Which of the following is the LEAST appropriate initial intervention for this hypercyanotic spell?
38. A neonate presents on day 5 of life with sudden cardiovascular collapse, absent femoral pulses, and severe metabolic acidosis. Echocardiography shows hypoplastic ascending aorta, mitral stenosis, and aortic valve stenosis. Which of the following interventions is CRITICAL to prevent immediate death in this condition?
39. A 6-month-old infant with a known large VSD develops increasing tachypnoea, poor feeding, and growth failure. The harsh pansystolic murmur is now softer. A new soft, mid-diastolic murmur appears at the apex. Chest X-ray shows cardiomegaly with increased pulmonary vascularity. What does the decrease in VSD murmur indicate?
40. A 3-year-old child with tetralogy of Fallot is found cyanosed, distressed, squatting and crying inconsolably after waking up. SpO2 is 60%. Heart murmur is now absent. This is a hypercyanotic spell. The FIRST pharmacological intervention is:
41. A cyanotic neonate on the first day of life has severe hypoxia (PaO2 25 mmHg) unresponsive to 100% oxygen (hyperoxia test negative) and a chest X-ray showing an 'egg on a string' (egg on its side) cardiac silhouette with narrow superior mediastinum. What is the underlying anatomical defect?
42. A 3-day-old neonate on prostaglandin E1 (PGE1) infusion becomes dependent on ductal patency for systemic circulation. Echocardiography shows no flow across the aortic valve. Which lesion REQUIRES ductus-dependent SYSTEMIC circulation (not pulmonary) from PGE1?
43. A cyanotic 2-month-old baby has a single loud S2, no murmur, and CXR shows an 'egg on side' cardiac silhouette with narrow mediastinum. ECG shows right-axis deviation and right ventricular hypertrophy. Echocardiography is ordered. Which diagnosis does this presentation most strongly suggest?
44. A 4-day-old neonate develops progressive cyanosis, tachypnea, and poor feeding. Chest X-ray shows an 'egg on a string' appearance with a narrow mediastinum. Echocardiography confirms the diagnosis. Prostaglandin E1 infusion is started. Which intervention is performed as an emergency palliation before definitive repair?
45. A 6-month-old with known large ventricular septal defect presents with increasing respiratory distress, failure to thrive, and frequent lower respiratory tract infections. On examination, there is a pansystolic murmur at the lower left sternal border, with a mid-diastolic rumble at the apex, and a hepatomegaly. The murmur intensity has recently decreased. What does the decreasing murmur intensity suggest in this context?
46. A 5-day-old neonate develops sudden cardiovascular collapse. On examination, femoral pulses are absent, BP is 90/60 in the right arm but unobtainable in the legs. The neonate had been discharged from the nursery at day 2. The MOST likely diagnosis and immediate treatment is:
47. An 18-month-old child with unrepaired VSD is found to have reversal of the cardiac shunt — now right-to-left — causing central cyanosis, finger clubbing, and pulmonary hypertension. The pulmonary vascular resistance now exceeds systemic vascular resistance. This pathophysiological syndrome is:
48. A cyanotic neonate is found on chest radiograph to have a narrow mediastinum (egg-on-a-string appearance) with increased pulmonary vascular markings. Echocardiography confirms d-transposition of the great arteries (d-TGA). The immediate intervention to improve oxygenation until definitive arterial switch operation is:
49. A 4-year-old cyanotic child squats frequently after exertion. He has a parasternal heave, single S2, and grade 3/6 ejection systolic murmur. Chest X-ray shows boot-shaped heart. This picture of Tetralogy of Fallot. What is the mechanism of squatting benefit?
50. Tet spells (hypercyanotic spells) in TOF are treated in a stepwise fashion. Which combination correctly represents the correct management sequence?