Paediatrics: Ebstein's Anomaly: Free MSRA Podcast

🩺 FREE MSRA PODCAST – Ebstein’s Anomaly: Right-Sided Heart, Real-World ImpactIn this revision-focused episode, we break down everything you need to know about Ebstein’s anomaly for the MSRA – covering the classic anatomical features, clinical presentation, diagnosis, management, and essential complications. Whether you’re revising or want a quick clinical refresher, this episode makes it stick.🧠 Key Learning Points📌 Definition• Ebstein’s anomaly is a rare congenital heart defect• Key feature: Apical displacement of the tricuspid valve, causing “atrialisation” of part of the right ventricle• Leads to a smaller functioning right ventricle and an enlarged right atrium📌 Causes & Associations• Congenital: Due to abnormal fetal heart development• Most cases are sporadic, but genetic and environmental factors (e.g. maternal benzodiazepines or lithium) may play a role• Associated with: – Tricuspid regurgitation (almost universal) – Wolff-Parkinson-White syndrome (WPW) in ~15% (⚡ mnemonic: “Ebstein’s & Extras!”) – Right bundle branch block (ECG) – Other defects (e.g. ASD, VSD, pulmonary stenosis)📌 Clinical Features• Huge variability – from severe neonatal heart failure/cyanosis to mild/asymptomatic cases in adults• Classic findings: – Cyanosis – Fatigue, breathlessness, palpitations (arrhythmias) – Right heart failure: peripheral oedema, hepatomegaly – Atrialisation = large right atrium• Murmur: Pansystolic murmur (tricuspid regurgitation), widely split S2, S3/S4 possible📌 Diagnosis• Echocardiography – gold standard (shows valve displacement, atrialised RV, regurgitation severity)• ECG: Right atrial enlargement, RBBB, WPW pattern• CXR: Box-shaped or globular heart, possible cardiomegaly• EP studies: For mapping accessory pathways in WPW📌 Management• Mild/asymptomatic: Monitoring only• Medical: – Heart failure treatment (diuretics, ACE inhibitors, etc.) – Arrhythmia management (medication, ablation) – Endocarditis prophylaxis• Surgical: – Valve repair preferred (if possible); replacement (bioprosthetic/tissue) if needed – Associated ASD/PFO closure, resection of atrialised RV if required – Ablation for arrhythmia if indicated• Severe/end-stage: Palliative shunt or heart transplant📌 Complications• Right heart failure• Arrhythmias (incl. WPW, risk of sudden death)• Paradoxical embolism (stroke/TIA with ASD/PFO)• Infective endocarditis• Brain abscess (rare, via right-to-left shunt)📌 Prognosis• Highly variable – depends on severity and management• Mild cases can live to old age; severe untreated: <5% survive beyond 50• Modern surgical advances improving outcomes even in neonates📎 More MSRA Resources for Ebstein’s Anomaly:📝 Revision Notes: https://www.passthemsra.com/topic/ebsteins-anomaly-revision-notes/🧠 Flashcards: https://www.passthemsra.com/topic/ebsteins-anomaly-flashcards/💬 Accordion Q&A: https://www.passthemsra.com/topic/ebsteins-anomaly-accordion-qa-notes/🚀 Rapid Quiz: https://www.passthemsra.com/topic/ebsteins-anomaly-rapid-quiz/🎓 Paediatrics Course: https://www.passthemsra.com/courses/paediatrics-for-the-msra/#MSRA #EbsteinsAnomaly #Paediatrics #MSRARevision #MSRATextbook #MSRAQuiz #MSRAFlashcards #MSRAAccordions #PaediatricCardiology #CongenitalHeartDisease