Ebstein's anomaly

Question 1

What is the congenital condition involving the tricuspid valve?

Answer 1

• a congenital condition where the tricuspid valve is set lower in the right side of the heart (towards the apex), causing a bigger RA and smaller RV
• this leads to poor flow from the RA to the RV and therefore poor flow to the pulmonary vessels
• it’s often associated with R→L shunt across the atria via an ASD
• when this happens, blood bypasses the lungs ⇒ cyanosis

It’s often associated with a right-to-left shunt across the atria via an ASD, leading to cyanosis.

Question 2

What causes this congenital condition?

Answer 2

Use of lithium during pregnancy, e.g., in bipolar disorder.

Question 3

What can this condition be associated with?

Answer 3

Wolff-Parkinson-White syndrome.

Question 4

What are the presentations of this condition?

Answer 4

• evidence of heart failure (oedema)
• gallop rhythm heard on auscultation characterised by the addition of the 3rd and 4th heart sounds
• cyanosis
• SOB and tachypnoea
• poor feeding
• collapse or cardiac arrest

Question 5

When do symptoms present in patients with an associated ASD?

Answer 5

• a few days after birth when the ductus arteriosus closes
• when there is a right to left shunt across an ASD, the ductus arteriosus 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 6

What is the diagnosis for this condition?

Answer 6

Echocardiogram.

Question 7

What is the management for this condition?

Answer 7

• medical management includes treating arrhythmias and heart failure
• prophylactic antibiotics may be used to prevent infective endocarditis
• definitive management is by surgical correction