Procedures done for the management of congenital heart diseases
🫀 Congenital Heart Disease — 40 Interactive MCQs
Click an option — correct answer will be highlighted in green; wrong choices will turn red. Explanations show immediately.
Q1
Which is the most common congenital heart defect overall?
A. Ventricular septal defect (VSD)
B. Atrial septal defect (ASD)
C. Patent ductus arteriosus (PDA)
D. Tetralogy of Fallot (TOF)
Correct: VSD. VSD is the most common congenital heart defect across all age groups.
Q2
Which lesion is classically associated with a ‘boot-shaped’ heart on chest X-ray?
A. Transposition of great arteries
B. Total anomalous pulmonary venous connection
C. Patent ductus arteriosus
D. Tetralogy of Fallot
Correct: TOF. Boot-shaped heart is due to right ventricular hypertrophy and small pulmonary arteries.
Q3
Which screening test is recommended for critical CHD in newborns?
A. Routine chest X-ray
B. Pulse oximetry screening
C. Routine ECG
D. Cardiac MRI
Correct: Pulse oximetry. It’s a simple screening tool to detect hypoxemia from critical CHD in neonates.
Q4
Which drug is used to keep the ductus arteriosus open in duct-dependent lesions?
A. Indomethacin
B. Ibuprofen
C. Prostaglandin E1 (alprostadil)
D. Acetaminophen
Correct: Prostaglandin E1. It maintains ductal patency in neonates with duct-dependent circulation.
Q5
A continuous ‘machinery’ murmur at the left infraclavicular area suggests:
A. Ventricular septal defect
B. Patent ductus arteriosus (PDA)
C. Atrial septal defect
D. Pulmonary stenosis
Correct: PDA. The continuous shunt from aorta to pulmonary artery produces a continuous ‘machinery’ murmur.
Q6
The gold-standard initial imaging for most CHD is:
A. Transthoracic echocardiography
B. Cardiac CT
C. Cardiac MRI
D. Cardiac catheterization
Correct: Echocardiography. It’s the primary, noninvasive diagnostic modality for structural heart disease.
Q7
Eisenmenger syndrome is best described as:
A. Early correction of VSD
B. A cyanotic lesion present at birth
C. Pulmonary embolism causing right-to-left shunt
D. Reversal of a long-standing left-to-right shunt due to pulmonary vascular disease
Correct: Reversal of shunt from pulmonary vascular remodeling, leading to cyanosis and clubbing.
Q8
The first-stage palliation for hypoplastic left heart syndrome (HLHS) is:
A. Glenn procedure
B. Fontan completion
C. Norwood procedure
D. Rastelli operation
Correct: Norwood. It reconstructs the aorta and provides systemic output using the right ventricle.
Q9
Which lesion is most commonly closed by transcatheter device?
A. Large perimembranous VSD
B. Secundum type ASD
C. Complete AVSD
D. Coarctation of aorta
Correct: Secundum ASD. It’s the most common lesion amenable to device closure (eg Amplatzer).
Q10
Balloon atrial septostomy (Rashkind) is primarily performed in neonates with:
A. Transposition of the great arteries (TGA)
B. Tetralogy of Fallot
C. Coarctation of aorta
D. PDA requiring closure
Correct: TGA. It improves interatrial mixing and systemic oxygenation prior to definitive repair.
Q11
A modified Blalock–Taussig shunt connects which vessels?
A. Pulmonary artery to left atrium
B. Left ventricle to pulmonary artery
C. Aorta to right ventricle
D. Subclavian artery (or graft) to pulmonary artery
Correct: Modified BT shunt connects the subclavian artery (via graft) to the pulmonary artery to augment pulmonary blood flow.
Q12
Tetralogy of Fallot includes all EXCEPT:
A. Ventricular septal defect
B. Overriding aorta
C. Patent ductus arteriosus
D. Right ventricular outflow tract obstruction
Correct: PDA is not part of the classic TOF tetrad (VSD, overriding aorta, RVOT obstruction, RVH).
Q13
Which chromosomal abnormality is strongly associated with endocardial cushion defects (AVSD)?
A. Turner syndrome
B. Down syndrome (Trisomy 21)
C. Marfan syndrome
D. Klinefelter syndrome
Correct: Down syndrome. AVSDs (endocardial cushion defects) are common in Trisomy 21.
Q14
Coarctation of the aorta classically causes which blood pressure finding?
A. Higher BP in arms than legs (brachial-femoral delay)
B. Higher BP in legs than arms
C. Equal BP in all limbs
D. Isolated hypotension
Correct: Blood pressure is higher in the arms with diminished pulses in the legs due to the narrowed aortic segment.
Q15
Which drug is used pharmacologically to close a PDA in preterm neonates?
A. Prostaglandin E1
B. Indomethacin or ibuprofen
C. Warfarin
D. Dopamine
Correct: Indomethacin or ibuprofen inhibit prostaglandin synthesis and promote ductal closure in preterms.
Q16
Which chest X-ray description is classic for transposition of the great arteries (TGA)?
A. Boot-shaped heart
B. Water bottle heart
C. Egg-on-a-string (narrow mediastinum)
D. Cardiomegaly with pulmonary edema
Correct: Egg-on-a-string reflects a narrow mediastinum and convex cardiac silhouette in TGA.
Q17
Which device is commonly used for secundum ASD closure?
A. Amplatzer septal occluder
B. Coil embolization
C. Mitral clip
D. Pacemaker lead
Correct: Amplatzer devices are widely used for percutaneous secundum ASD closure.
Q18
Balloon valvuloplasty is first-line for which congenital valvular lesion in children?
A. Mitral stenosis
B. Pulmonary valve stenosis
C. Aortic regurgitation
D. Tricuspid stenosis
Correct: Pulmonary valvuloplasty is commonly performed for valvular pulmonary stenosis in children.
Q19
A 22q11.2 deletion (DiGeorge) is classically associated with which CHD group?
A. Isolated VSDs only
B. Complex left-sided obstructive lesions
C. Purely valvular defects
D. Conotruncal anomalies (eg truncus arteriosus, interrupted arch)
Correct: DiGeorge is linked to conotruncal anomalies due to neural crest developmental issues.
Q20
Which staged operation sequence is typical for single-ventricle palliation?
A. Norwood (or PDA-dependent palliation) → Glenn → Fontan
B. Fontan → Glenn → Norwood
C. Rastelli → Norwood → Fontan
D. Arterial switch → Glenn → Fontan
Correct: The staged approach progresses from neonatal palliation (Norwood) to Glenn and then Fontan completion.
Q21
Rastelli operation is used in which combination?
A. Isolated ASD
B. Coarctation without VSD
C. TGA with VSD and pulmonary stenosis
D. PDA closure
Correct: Rastelli connects LV to aorta via VSD patch and places an RV-PA conduit for TGA with VSD+PS.
Q22
Which CHD patients may need endocarditis prophylaxis for dental procedures?
A. All patients with small unrepaired ASD
B. Patients with prosthetic material in the heart or prior endocarditis
C. All adults with repaired VSD
D. No CHD patients require prophylaxis
Correct: Patients with prosthetic valves, prosthetic material, or prior endocarditis are higher-risk and may need prophylaxis per guidelines.
Q23
Pulmonary artery banding is used primarily to:
A. Control pulmonary overcirculation in large left-to-right shunts
B. Increase pulmonary blood flow in cyanotic neonates
C. Close a PDA
D. Repair coarctation
Correct: Banding reduces pulmonary blood flow to protect pulmonary vasculature prior to definitive repair.
Q24
Which post-operative complication is particularly important after atrial switch (Mustard/Senning)?
A. Pulmonary hypertension
B. RVOT obstruction
C. Ventricular septal rupture
D. Atrial arrhythmias and baffle obstruction
Correct: Atrial arrhythmias and baffle obstruction are long-term issues after atrial switch procedures.
Q25
Cardiac MRI is particularly useful in CHD for:
A. Routine neonatal screening
B. Detailed anatomy and ventricular function quantification in complex CHD
C”>C. Replacing echocardiography in all cases
D. Immediate bedside diagnostics
Correct: MRI provides high-resolution anatomical detail and functional assessment — valuable in complex or repaired CHD.
Q26
In infants, signs of heart failure from CHD include all EXCEPT:
A. Tachypnea
B. Hepatomegaly
C. Polyuria
D. Poor feeding and failure to thrive
Correct: Polyuria is not a typical sign — respiratory distress, hepatomegaly, poor feeding are common in infant CHF.
Q27
For duct-dependent systemic circulation, the immediate medical management includes:
A. Start prostaglandin E1 infusion
B. Immediate PDA ligation
C. High-dose indomethacin
D. Immediate balloon valvuloplasty
Correct: Prostaglandin E1 maintains ductal patency to preserve systemic perfusion until definitive intervention.
Q28
Which operation re-implants the coronary arteries as part of the repair?
A. Fontan procedure
B. Arterial switch (Jatene) for TGA
C. Blalock–Taussig shunt
D. Pulmonary artery banding
Correct: During an arterial switch, coronaries are transferred to the new aorta (neoaorta).
Q29
Which repaired CHD patients generally require lifelong cardiology follow-up?
A. Isolated small VSD closed in childhood and asymptomatic
B. Patients with simple PDA ligation with no sequelae
C. Repaired TOF, arterial switch, and Fontan patients
D. All patients cured with a single minor procedure
Correct: Complex repairs require lifelong specialized follow-up due to arrhythmia, RV function, conduit issues, etc.
Q30
Untreated large VSDs are most likely to cause:
A. Pulmonary hypertension and Eisenmenger physiology
B. Isolated atrial fibrillation in infancy
C. Immediate cyanosis at birth
D. Left ventricular outflow tract obstruction
Correct: Large left-to-right shunts lead to increased pulmonary flow and pulmonary vascular disease if uncorrected.
Q31
Which interventional option is commonly used for recoarctation in adolescents/adults?
A. Surgical banding
B. Balloon angioplasty with/without stenting
C. PDA device closure
D. ASD device closure
Correct: Balloon angioplasty ± stent is often used for re-coarctation in older children and adults.
Q32
Which congenital heart lesion typically requires immediate prostaglandin infusion to survive until repair?
A. Small ASD
B. Repaired VSD
C. Mild pulmonary stenosis
D. Duct-dependent lesions like critical coarctation or pulmonary atresia
Correct: Duct-dependent lesions need prostaglandin to maintain systemic or pulmonary flow until intervention.
Q33
Which symptom is least likely to be present in cyanotic CHD?
A. Clubbing
B. Polycythemia
C. Wide, fixed split of second heart sound
D. Exertional cyanosis
Correct: Wide fixed S2 split is typical of ASD (acyanotic); cyanotic lesions cause clubbing and polycythemia.
Q34
Turner syndrome is commonly associated with which cardiac lesion?
A. Tetralogy of Fallot
B. Coarctation of the aorta and bicuspid aortic valve
C. PDA
D. Truncus arteriosus
Correct: Turner patients often have left-sided obstructive lesions like coarctation and bicuspid aortic valve.
Q35
Which percutaneous valve is commonly used for failing RV-PA conduits?
A. Melody valve (transcatheter pulmonary valve)
B. TAVR for aortic valve in children
C. MitraClip in RV conduits
D. Amplatzer device
Correct: The Melody valve is used transcatheter for dysfunctional RV-PA conduits.
Q36
A child with cyanosis, boot-shaped heart, and harsh systolic murmur likely has:
A. PDA
B. TGA
C. ASD
D. Tetralogy of Fallot
Correct: TOF presents with cyanosis, systolic murmur from RVOT obstruction, and boot-shaped heart.
Q37
A ‘hybrid’ procedure in CHD usually refers to:
A. Two-stage surgical repair only
B. Combined surgical and catheter-based treatment in the same patient
C. Drug therapy plus physiotherapy
D. Two simultaneous catheter procedures
Correct: Hybrid approaches (eg hybrid Norwood) combine surgery and catheter interventions to minimize neonatal risk.
Q38
Which complication is commonly monitored long-term after TOF repair?
A. Left ventricular failure
B. Rapid coronary artery disease
C. Pulmonary regurgitation and RV dilatation
D. Immediate sternal wound infection only
Correct: Pulmonary regurgitation after TOF repair can cause progressive RV dilation and dysfunction over years.
Q39
Which measure reduces risk of CHD related to maternal infection?
A. Rubella immunization prior to pregnancy
B. Giving live rubella vaccine during pregnancy
C. Avoiding all vaccines ever
D. Routine antibiotic prophylaxis in pregnancy
Correct: Maternal rubella immunization before pregnancy reduces the risk of congenital rubella syndrome and CHD.
Q40
Modern outcomes after most CHD repairs in tertiary centres are:
A. Usually poor with <50% survival
B. Excellent with many repairs showing >90% early survival
C. Unknown for all lesions
D. No need for follow-up after repair
Correct: Advances in surgical and perioperative care provide high early survival for many CHD repairs; long-term follow-up remains important.
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🩺 I. PALLIATIVE PROCEDURES
(Aim: to improve oxygenation or pulmonary/systemic flow before definitive repair)
A. Systemic–Pulmonary Shunts (to increase pulmonary blood flow)
- Blalock–Taussig (BT) shunt — subclavian artery to pulmonary artery anastomosis
- Modified BT shunt — using Gore-Tex tube graft
- Waterston–Cooley shunt — ascending aorta to right pulmonary artery (obsolete)
- Potts shunt — descending aorta to left pulmonary artery (obsolete)
- Central shunt — ascending aorta to main pulmonary artery
B. Pulmonary Artery Banding (PAB)
- Restricts excessive pulmonary flow (e.g., in large VSD, AV canal defects) to prevent pulmonary hypertension
C. Atrial Septostomy / Fenestration (to improve mixing or decompression)
- Balloon atrial septostomy (Rashkind procedure) — in transposition of great arteries (TGA)
- Blade atrial septostomy — for thick atrial septum
- Atrial septal stent placement — for maintaining interatrial communication
⚙️ II. CORRECTIVE SURGICAL PROCEDURES
(Aim: to anatomically repair the defect and restore normal circulation)
A. Septal Defect Repairs
- Ventricular Septal Defect (VSD) closure — with patch (Dacron or pericardial)
- Atrial Septal Defect (ASD) closure — direct suture or patch closure
- Atrioventricular Septal Defect (AVSD) repair — patch closure + AV valve repair
B. Outflow Tract and Valvular Lesions
- Pulmonary valvotomy / valvectomy — for pulmonary stenosis
- Aortic valvotomy / valvuloplasty — for congenital aortic stenosis
- Resection of subaortic stenosis / myectomy
- Relief of supravalvular aortic or pulmonary stenosis
C. Conotruncal Anomalies
- Arterial switch operation (Jatene procedure) — for TGA
- Atrial switch operations — Mustard or Senning procedure (for TGA, now rarely done)
- Rastelli operation — VSD closure + RV-PA conduit (TGA with VSD + PS)
- Fallot’s tetralogy (TOF) repair — VSD closure + RVOT reconstruction
- Truncus arteriosus repair — VSD closure + RV-PA conduit
D. Left Heart Obstructive Lesions
- Coarctation repair — end-to-end anastomosis, patch aortoplasty, or subclavian flap aortoplasty
- Interrupted aortic arch repair — end-to-end anastomosis
- Norwood operation (Stage I) — for hypoplastic left heart syndrome
- Glenn shunt (bidirectional cavopulmonary anastomosis) — second-stage single ventricle palliation
- Fontan procedure / total cavopulmonary connection (TCPC) — final stage single ventricle palliation
💉 III. CATHETER-BASED INTERVENTIONAL PROCEDURES
(Minimally invasive transcatheter techniques)
A. Balloon Dilatation Procedures
- Balloon pulmonary valvuloplasty — for valvular pulmonary stenosis
- Balloon aortic valvuloplasty — for congenital aortic stenosis
- Balloon coarctoplasty — for coarctation of aorta
- Balloon atrial septostomy — for TGA (as above)
B. Device Closure Procedures
- ASD device closure — Amplatzer septal occluder
- VSD device closure — muscular or perimembranous occluder
- Patent ductus arteriosus (PDA) closure — coil or device occluder
C. Stent Placement
- Pulmonary artery or branch stenting — for stenotic segments
- Aortic coarctation stent — for recoarctation or native coarctation
- Ductus arteriosus stent — to maintain ductal patency (e.g., duct-dependent lesions)
❤️ IV. HYBRID & SPECIALIZED PROCEDURES
- Hybrid Norwood (Stage I) — combination of surgical banding and ductal stent
- Melody valve implantation (percutaneous pulmonary valve)
- Transcatheter aortic valve implantation (TAVI) — in selected congenital aortic stenosis cases
- Extracardiac conduit replacements — using homograft or prosthetic material
✅ Summary Table:
| Type | Purpose | Common Examples |
|---|---|---|
| Palliative | Improve oxygenation/flow | BT shunt, PAB, atrial septostomy |
| Corrective | Definitive anatomical repair | TOF repair, ASD/VSD closure, Arterial switch |
| Interventional | Catheter-based correction | Device closures, valvuloplasties, stents |
| Hybrid | Combine surgery + catheter | Hybrid Norwood, ductal stenting |
🫀 Procedures in Congenital Heart Disease Management
| Category | Purpose | Examples / Procedures |
|---|---|---|
| Palliative Procedures | To improve oxygenation or pulmonary/systemic blood flow before definitive repair |
• Blalock–Taussig (BT) shunt, Modified BT shunt • Waterston–Cooley or Potts shunt (obsolete) • Pulmonary artery banding (PAB) • Balloon atrial septostomy (Rashkind), Blade septostomy • Atrial septal stent placement |
| Corrective Surgical Procedures | Definitive anatomical repair to restore normal circulation |
• ASD/VSD/AVSD closure (patch or suture) • Pulmonary or aortic valvotomy/valvuloplasty • TOF repair, Rastelli operation • Arterial switch (Jatene), Mustard/Senning (TGA) • Coarctation repair, Interrupted arch repair • Norwood, Glenn, Fontan procedures |
| Catheter-Based Interventional Procedures | Minimally invasive, transcatheter correction of defects |
• Balloon pulmonary/aortic valvuloplasty • Balloon coarctoplasty • ASD, VSD, PDA device closure • Pulmonary artery or aortic stenting • Ductus arteriosus stent (to maintain patency) |
| Hybrid & Specialized Procedures | Combination of surgery and catheter-based interventions |
• Hybrid Norwood procedure • Melody valve (percutaneous pulmonary valve) • Transcatheter aortic valve implantation (TAVI) • Extracardiac conduit replacements |
Summary: Congenital heart disease (CHD) management includes palliative procedures to stabilize the patient, corrective surgeries for definitive anatomical repair, catheter-based interventions for minimally invasive correction, and hybrid approaches combining both surgical and interventional methods.
🫀 Congenital Heart Disease (CHD) — 50 High-Yield FAQs
1. What is congenital heart disease (CHD)?
Congenital heart disease (CHD) refers to structural or functional abnormalities of the heart or great vessels present at birth.
2. What are the common types of CHD?
Common types include ASD, VSD, PDA, TOF, coarctation of the aorta, and transposition of the great arteries (TGA).
3. What causes congenital heart disease?
Causes include genetic mutations, chromosomal abnormalities, maternal infections like rubella, diabetes, and teratogenic drug exposure.
4. How is CHD classified?
It is classified as acyanotic (left-to-right shunts or obstructive) and cyanotic (right-to-left shunts or complex lesions).
5. Which CHDs are acyanotic?
ASD, VSD, PDA, AVSD, and coarctation of the aorta.
6. Which CHDs are cyanotic?
TOF, TGA, tricuspid atresia, truncus arteriosus, and TAPVC.
7. What is the most common CHD overall?
Ventricular septal defect (VSD) is the most common congenital heart disease.
8. What is the most common cyanotic CHD?
Tetralogy of Fallot (TOF) is the most common cyanotic CHD after infancy.
9. How is CHD diagnosed in newborns?
By clinical exam, pulse oximetry, echocardiography, ECG, and chest X-ray.
10. What is the role of echocardiography in CHD?
It is the primary non-invasive diagnostic tool for defining cardiac structure and function.
11. What is a palliative procedure in CHD?
A temporary operation that improves oxygenation or blood flow before definitive repair.
12. Examples of palliative procedures?
Blalock-Tausig shunt, pulmonary artery banding, and balloon atrial septostomy.
13. What is the Blalock-Tausig shunt used for?
It increases pulmonary blood flow in cyanotic CHDs like TOF.
14. What is pulmonary artery banding?
It reduces excessive pulmonary flow in large left-to-right shunts to prevent vascular damage.
15. What are corrective surgical procedures?
Definitive anatomical repairs: ASD/VSD closure, TOF repair, arterial switch, or coarctation repair.
16. What is the arterial switch operation?
Jatene procedure — reconnects great arteries and coronary arteries correctly in TGA.
17. What is the Fontan procedure?
It routes systemic venous blood directly to pulmonary arteries for single-ventricle physiology.
18. What is the Glenn shunt?
Connects SVC to right pulmonary artery as a stage toward Fontan circulation.
19. What are catheter-based interventions?
Device closures, balloon valvuloplasty, coarctation angioplasty, and stent placement.
20. What is balloon atrial septostomy?
Creates/enlarges atrial communication to improve mixing, used in neonatal TGA.
21. What is the role of stenting in CHD?
To maintain ductal or vessel patency or relieve stenosis in vessels.
22. Which procedure treats coarctation of aorta?
Surgical repair (end-to-end, patch, flap) or balloon angioplasty ± stent.
23. What is a hybrid procedure?
Combines surgery and catheter techniques, e.g. hybrid Norwood (PA banding + ductal stent).
24. Which procedure treats PDA?
Surgical ligation or catheter device occlusion.
25. Most common lesion for device closure?
Secundum ASD.
26. Signs of heart failure in infants?
Poor feeding, tachypnea, sweating with feeds, hepatomegaly, failure to thrive.
27. Which cyanotic CHD has ↑ pulmonary flow?
TGA and truncus arteriosus.
28. Which cyanotic CHD has ↓ pulmonary flow?
TOF and tricuspid atresia.
29. What is Eisenmenger syndrome?
Reversal of left-to-right shunt to right-to-left from pulmonary hypertension.
30. Which drug maintains ductal patency?
Prostaglandin E1 (alprostadil).
31. Which drug closes PDA in preterms?
Indomethacin, ibuprofen, or paracetamol.
32. First-stage surgery for HLHS?
Norwood procedure.
33. Which imaging is definitive?
Echocardiography; MRI/CT for complex lesions.
34. Complications of untreated VSD?
CHF, pulmonary hypertension, Eisenmenger syndrome.
35. Murmur in VSD?
Harsh pansystolic murmur at left lower sternal border.
36. Murmur in PDA?
Continuous “machinery” murmur at left infraclavicular area.
37. What is a boot-shaped heart?
Seen in TOF due to right ventricular hypertrophy.
38. What is an egg-on-a-string appearance?
Chest X-ray finding in TGA with narrow mediastinum.
39. What is the Coanda effect?
Jet flow adheres to vessel wall, affects flow patterns across stenoses.
40. Genetic syndromes linked to CHD?
Down, Turner, Marfan, and DiGeorge (22q11.2 deletion) syndromes.
41. CHD in Down syndrome?
Atrioventricular septal defect (AVSD).
42. CHD in Turner syndrome?
Coarctation of aorta and bicuspid aortic valve.
43. CHD in DiGeorge syndrome?
Conotruncal defects like truncus arteriosus and interrupted aortic arch.
44. Long-term follow-up for CHD?
Regular cardiology review, imaging, arrhythmia monitoring, exercise advice, endocarditis prophylaxis.
45. Can CHD be prevented?
Complete rubella immunization, diabetes control, avoid teratogens, genetic counseling.
46. Survival after CHD surgery?
Most modern CHD surgeries have >90% early survival and good long-term outcomes.
47. What is the role of genetic counseling?
Helps families understand recurrence risk and options for prenatal testing.
48. Which CHD needs lifelong follow-up?
Repaired TOF, TGA, and Fontan patients need lifelong specialized cardiology care.
49. What are hybrid cardiac procedures?
Combined surgical and interventional techniques for high-risk or neonatal cases.
50. What are future directions in CHD management?
Advances in fetal intervention, 3D printing for planning, and stem-cell-based tissue repair.
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