Ebstein's Anomaly

Question 1

What is Ebstein’s Anomaly?

Answer 1

• A congenital heart condition where the tricuspid valve is set lower in the right side of the heart
• this causes a bigger right atrium and a smaller right ventricle
• This leads to poor flow from the right atrium to the right ventricle to the pulmonary vessels
• It is often associated with right to left shunt across the atria via a atrial septal defect
• When this happens blood bypasses the lungs leading to cyanosis
• It is associated with Wolff-Parkinson- White Syndrome

Question 2

How does Ebstein’s Anomaly normally present?

Answer 2

• Evidence of Heart Failure (oedema)
• Gallop rhythm heard on auscultation - addition of third and fourth heart sounds
• Cyanosis
• SOB and tachypnoea
• poor feeding
• Collapse of cardiac arrest

Question 3

When do symptoms normally present in Ebstein’s Anomaly?

Answer 3

• Symptoms are seen in patients with atrial septal defects a few days after birth when the ductus arteriosus closes
• The ductus arteriosus normally allows blood to flow from the aorta into the pulmonary vessels to get oxygenated
• This minimises the cyanosis
• When the duct closes the patient becomes cyanotic and symptomatic

Question 4

What is the gold standard investigation for Ebstein’s Anomaly?

Answer 4

• Echocardiogram is the investigation of choice for confirming the diagnosis and assessing the severity

Question 5

What is the Managment for Ebstein’s Anomaly?

Answer 5

• Treating arrhythmias and heart failure
• Prophylactic Abx to prevent Infective Endocarditis
• Definitive management by surgical correction of the underlying defect