Ebsteins_Anomaly_Flashcards
What is Ebstein’s anomaly?
Ebstein’s anomaly is a congenital heart defect characterized by a low insertion of the tricuspid valve, resulting in a large atrium and small ventricle, often referred to as ‘atrialisation’ of the right ventricle.
What potential in-utero exposure is associated with Ebstein’s anomaly?
Ebstein’s anomaly may be caused by exposure to lithium in-utero.
What are common associations with Ebstein’s anomaly?
Common associations include patent foramen ovale (PFO) or atrial septal defect (ASD) in at least 80% of patients, and Wolff-Parkinson White syndrome.
What are the clinical features of Ebstein’s anomaly?
Clinical features include cyanosis, a prominent ‘a’ wave in the jugular venous pulse, hepatomegaly, tricuspid regurgitation, a pansystolic murmur worsening on inspiration, and a right bundle branch block with a widely split S1 and S2.
summarise
Ebstein’s anomaly
Ebstein’s anomaly is a congenital heart defect characterised by low insertion of the tricuspid valve resulting in a large atrium and small ventricle. It is sometimes referred to as ‘atrialisation’ of the right ventricle.
Ebstein’s anomaly may be caused by exposure to lithium in-utero.
Associations
patent foramen ovale (PFO) or atrial septal defect (ASD) is seen in at least 80% of patients, resulting in a shunt between the right and left atria
Wolff-Parkinson White syndrome
Clinical features
cyanosis
prominent ‘a’ wave in the distended jugular venous pulse,
hepatomegaly
tricuspid regurgitation
pansystolic murmur, worse on inspiration
right bundle branch block → widely split S1 and S2
A 34-year-old woman who is 38 weeks pregnant overdoses on lithium. Once she is stabilised, the baby is safely delivered. During routine neonatal checks, a pan-systolic murmur is heard. Cardiac echocardiogram reveals a low insertion of the tricuspid valve with a large right atrium, a small right ventricle and associated tricuspid incompetence. What is the most likely diagnosis?
Fallot’s tetralogy
Coarctation of the aorta
Transposition of the great arteries
Ebstein’s anomaly
Patent ductus arteriosus
Ebstein’s anomaly - ‘atrialisation’ of the right ventricle
Important for meLess important
1 - Incorrect. Fallot’s tetralogy is a cyanotic congenital heart disease and presents with ventricular septal defect, right ventricular hypertrophy, overriding aorta, and pulmonary stenosis.
2 - Incorrect. This is a congenital narrowing of the aorta and would present with absent/weak femoral pulses; hypertension in arms but hypotension in legs.
3 - Incorrect. Transposition of the great arteries is where the aorta arises from the right ventricle and the pulmonary artery arises from the left ventricle. It presents mainly as cyanosis in children.
4 - Correct. Ebstein’s anomaly results in low insertion of the tricuspid valve resulting in a large right atrium and small right ventricle causing tricuspid incompetence.
5 - Incorrect. Patent ductus arteriosus is where there is a failure of the ductus arteriosus to close after birth causing a left-right shunting and would present as a loud continuous murmur with bounding pulses and a wide pulse pressure.
