FCPS TOACS Mock Test · Ebstein Anomaly

❓ Q1. Describe the radiographic findings. What is the most likely diagnosis?

✅ Model Answer:
• Massive cardiomegaly (box‑shaped or water bottle heart).
• Markedly enlarged right atrium (dominant chamber).
• Decreased pulmonary vascularity (if pulmonary flow reduced).
• Normal or small pulmonary artery segment.
• Clear lung fields (no pulmonary edema).
• Diagnosis: Ebstein anomaly of the tricuspid valve.

❓ Q2. Describe the pathophysiology of Ebstein anomaly. What is the "atrialized right ventricle"?

✅ Model Answer:
• Ebstein anomaly: Congenital malformation with apical displacement of the tricuspid septal and posterior leaflets into the RV (≥8 mm/m²).
• Displacement Index: Measured from the tricuspid annulus.
• Atrialized right ventricle: The portion of RV proximal to the displaced valve becomes thin-walled, dysfunctional, and electrically silent – it functions as part of the RA.
• Consequences: Severe TR, massive RA enlargement, right-to-left shunt (ASD/PFO) → cyanosis, reduced RV function, WPW (25%).

❓ Q3. What are the clinical features of Ebstein anomaly in the neonatal period?

✅ Model Answer:
• Cyanosis – due to right-to-left shunting through ASD/PFO.
• Tachypnoea and respiratory distress.
• Tachycardia and heart failure (in severe cases).
• Auscultation: Widely split S1 (delayed tricuspid closure), systolic murmur at LLSB (TR), multiple systolic clicks, single S2 (due to PS or absent pulmonary component).
• Massive cardiomegaly on CXR.
• ECG: RBBB, tall P waves (RA enlargement), WPW (25%).

❓ Q4. What is the Carvallo sign and what does it indicate in Ebstein anomaly?

✅ Model Answer:
• Carvallo sign: The murmur of tricuspid regurgitation (holosystolic, best heard at the LLSB) increases in intensity with inspiration.
• Mechanism: Inspiration increases venous return to the right heart → increased RV volume → increased TR → louder murmur.
• Significance: Helps distinguish TR (increases with inspiration) from MR (does not change or decreases with inspiration).
• In Ebstein, TR is a hallmark feature, and the Carvallo sign is often present.

❓ Q5. What are the associated cardiac defects in Ebstein anomaly? What is the incidence of WPW?

✅ Model Answer:
• ASD or PFO – present in 80-90% of patients → right-to-left shunting → cyanosis.
• WPW (Wolff-Parkinson-White) syndrome – occurs in 25% of patients (right-sided accessory pathways).
• Pulmonary stenosis or atresia – functional (due to RV dysfunction) or anatomic.
• VSD – less common.
• Mitral valve prolapse – may be seen.

❓ Q6. What are the classic ECG findings in Ebstein anomaly?

✅ Model Answer:
• Right bundle branch block (RBBB) – rsR' pattern in V1-V2 with wide QRS (≥0.12 sec). Present in most patients.
• Tall peaked P waves – right atrial enlargement (P pulmonale) – tall P waves (>2.5 mm) in leads II, III, aVF.
• WPW syndrome (25%) – short PR interval (<0.08 sec), delta wave, wide QRS.
• First-degree AV block – prolonged PR interval may be present.
• Low voltage – may be seen in some cases.

❓ Q7. What is the management of WPW in Ebstein anomaly?

✅ Model Answer:
• Electrophysiology study (EPS) and radiofrequency ablation – treatment of choice for symptomatic WPW (palpitations, SVT, syncope). Curative.
• If ablation not feasible: Antiarrhythmics (flecainide, propafenone, sotalol).
• Avoid digoxin and verapamil – can accelerate conduction over accessory pathway during AF → VF.
• Asymptomatic WPW: Many experts recommend ablation in Ebstein due to high risk of SVT and AF (risk of sudden death).

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

✅ Model Answer:
• Starnes procedure: Neonatal salvage procedure for severe Ebstein anomaly.
• Procedure: Closure of the tricuspid valve orifice, BT shunt (Blalock-Taussig) to provide pulmonary blood flow, and ASD creation to decompress the right heart.
• Indications: Severe neonatal Ebstein with functional pulmonary atresia, massive cardiomegaly, severe cyanosis, and heart failure.
• Outcome: Results in single ventricle physiology; the patient becomes a Fontan candidate.
• High-risk procedure (mortality ~30-50%) but life-saving.

❓ Q9. What is the cone repair (Da Silva technique) for Ebstein anomaly? When is it performed?

✅ Model Answer:
• Cone repair (Da Silva technique): Surgical tricuspid valve repair.
• Procedure: Detach leaflets, rotate to create a cone-shaped valve, reattach to true annulus. ASD closure and atrialized RV plication may be performed.
• Indications: Severe TR with RV dilation/dysfunction, symptoms (heart failure, cyanosis, exercise intolerance), paradoxical embolism.
• Timing: Usually performed in older children, adolescents, or adults (>3-5 years).
• Excellent results: avoids valve replacement, improves RV function.

❓ Q10. What is the long-term prognosis for a patient with Ebstein anomaly?

✅ Model Answer:
• Depends on severity, RV function, symptoms, and arrhythmias.
• Mild forms: excellent prognosis, normal life expectancy.
• With cone repair: excellent results – most patients have good valve function and improved symptoms.
• Severe neonatal form (Starnes): guarded prognosis (30-50% mortality).
• Long-term follow-up: Lifelong cardiology follow-up. Annual echocardiograms to monitor TR severity, RV function, and valve repair integrity. Monitor for arrhythmias (WPW, atrial flutter).
• Pregnancy: High-risk, requires multidisciplinary care.

❓ Q11. A newborn with Ebstein anomaly presents with severe cyanosis and massive cardiomegaly. What is your initial management?

✅ Model Answer:
1. PGE1 (Prostaglandin E1) – 0.05-0.1 mcg/kg/min IV to maintain ductal patency if functional pulmonary atresia (duct-dependent pulmonary blood flow).
2. Supportive care: Ventilatory support, correction of acidosis, treatment of heart failure.
3. Echocardiogram to confirm diagnosis, assess TR severity, RV function, and pulmonary valve.
4. Starnes procedure – for critically ill neonates with severe cyanosis, massive cardiomegaly, and heart failure.
5. If stable: May be managed medically (PGE1 weaned, watchful waiting) and cone repair deferred to later childhood.

❓ Q12. Should the ASD be closed in Ebstein anomaly? What are the risks and benefits?

✅ Model Answer:
• ASD is present in 80-90% of patients.
• Usually closed during tricuspid valve surgery (cone repair or valve replacement).
• Risks of closure: If the ASD is a "pop-off" valve for the right heart, closing it may worsen heart failure (increases right-sided pressures).
• Recommendation: ASD closure is generally performed only if the tricuspid valve is repaired and the right heart can handle the volume. In patients with severe RV dysfunction, ASD closure may be delayed or not performed.

⚕️ FCPS MCPS IMM MD Paediatrics TOACS · Mock Test