Ebstein’s anomaly

Published on 02/04/2015 by admin

Filed under Internal Medicine

Last modified 02/04/2015

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35 Ebstein’s anomaly

Instruction

Listen to this patient’s heart. He was told he had an innocent murmur during a school medical examination many years ago but now has a large globular heart on chest radiography.

Salient features

History

• An incidental cardiac murmur

• Ask the patient about palpitations (paroxysmal supraventricular tachycardia)

• Symptoms of right-sided heart failure

• History of maternal lithium ingestion.

Examination

• Raised jugular venous pulse; the large ‘v’ of tricuspid regurgitation is absent because the giant right atrium absorbs most of the regurgitant volume

• Left parasternal heave

• Loud first heart sound produced by the sail-like anterior tricuspid leaflet

• Pansystolic murmur which increases on inspiration

• Hepatomegaly

• Proceed to:

• ascertain whether the patient has exertional cyanosis or dyspnoea
• exclude an atrial septal defect.

Diagnosis

This patient has isolated tricuspid regurgitation (lesion) that is probably of congenital aetiology as there is no pulmonary hypertension. He has Ebstein’s anomaly with cardiomegaly and cardiac failure (functional status).

Advanced-level questions

What is the pathology in Ebstein’s anomaly?

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