FCPS TOACS · Ebstein Anomaly Mock Test

❓ Q1. Describe the ECG findings.

✅ Model Answer:
• RBBB (rsR' in V1-V2) – most common finding.
• Tall peaked P waves (right atrial enlargement) in II, III, aVF.
• WPW (25%) – short PR, delta wave, wide QRS.
• May have first-degree AV block, low voltage.

❓ Q2. Explain the pathophysiology of Ebstein anomaly. What is meant by 'atrialized right ventricle'?

✅ Model Answer:
• Apical displacement of tricuspid septal and posterior leaflets (≥8 mm/m²).
• Atrialized RV: thin-walled, dysfunctional portion of RV proximal to displaced valve → acts as part of RA.
• Consequences: TR, RA enlargement, right-to-left shunt (ASD/PFO), WPW (25%), reduced RV function.

❓ Q3. What are the associated cardiac defects in Ebstein anomaly? What is the incidence of WPW?

✅ Model Answer:
• ASD/PFO (80-90%) – allows right-to-left shunting → cyanosis.
• WPW (25%) – accessory pathways (right-sided).
• Pulmonary stenosis/atresia (functional or anatomic), VSD, mitral valve prolapse.

❓ Q4. This patient has a holosystolic murmur at the LLSB that increases with inspiration. What is this sign and what does it indicate?

✅ Model Answer:
• Carvallo sign: TR murmur increases with inspiration (increased venous return → more TR).
• Helps differentiate TR (increases with inspiration) from MR (does not change).

❓ Q5. What is the classic chest X-ray finding in Ebstein anomaly? Why does it occur?

✅ Model Answer:
• Massive cardiomegaly (box-shaped or water bottle heart) due to massive RA enlargement.
• Decreased pulmonary vascularity if pulmonary blood flow is reduced.

❓ Q6. This patient has WPW on ECG. What is the management of WPW in Ebstein anomaly?

✅ Model Answer:
• Radiofrequency ablation is treatment of choice for symptomatic WPW.
• Antiarrhythmics (flecainide, sotalol) if ablation not feasible.
• Avoid digoxin and verapamil (may accelerate conduction in AF).
• Many experts recommend ablation even in asymptomatic patients to prevent sudden death.

❓ Q7. What is the cone repair (Da Silva technique) for Ebstein anomaly? When is it performed?

✅ Model Answer:
• Surgical repair: detach leaflets, rotate to create cone-shaped valve, reattach to true annulus.
• ASD closure and atrialized RV plication may be performed.
• Indications: severe TR, symptoms, RV dysfunction, paradoxical embolism.
• Usually performed in older children/adolescents.

❓ Q8. What is the Starnes procedure? When is it indicated?

✅ Model Answer:
• Neonatal salvage procedure: closure of tricuspid valve + BT shunt + ASD creation.
• Indicated in severe neonatal Ebstein with functional pulmonary atresia, massive cardiomegaly, heart failure.
• Results in single ventricle physiology → eventual Fontan.

❓ Q9. A newborn presents with severe cyanosis and massive cardiomegaly. What is the initial management?

✅ Model Answer:
• PGE1 (0.05-0.1 mcg/kg/min IV) to maintain ductal patency (if pulmonary atresia).
• Supportive care: ventilation, correction of acidosis.
• Echocardiogram to confirm diagnosis.
• Starnes procedure for critically ill neonates.

❓ Q10. What are the indications for tricuspid valve surgery in Ebstein anomaly?

✅ Model Answer:
• Severe TR with RV dilation/dysfunction.
• Symptoms: heart failure, cyanosis (SpO2 <90%), arrhythmias.
• Paradoxical embolism.
• Progressive RV enlargement.
• Atrial arrhythmias (may require maze procedure).

❓ Q11. Should the ASD be closed in Ebstein anomaly? What are the risks and benefits?

✅ Model Answer:
• Usually closed during tricuspid valve surgery.
• Risks: If ASD is a "pop-off" valve, closure may worsen right heart failure.
• Generally closed if tricuspid valve is repaired and RV function is adequate.

❓ Q12. What is the long-term prognosis for a patient with Ebstein anomaly?

✅ Model Answer:
• Depends on severity, RV function, symptoms, arrhythmias.
• Mild forms: excellent prognosis.
• Cone repair: excellent results.
• Neonatal severe: guarded prognosis.
• Lifelong cardiology follow-up required.

❓ Q13. Is Ebstein anomaly associated with any genetic syndromes?

✅ Model Answer:
• Most cases are sporadic.
• Rarely associated with NKX2.5 mutations.
• Historical association with maternal lithium exposure.
• Noonan syndrome – occasionally.

⚕️ FCPS MCPS IMM MD Paediatrics TOACS · Mock Test