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Case 020 - Young Athlete and Sudden Death Survivor

by Karl Loren

Ultrasound & Heart Disease

The ECHOcase of the Week
Case 020 - Young Athlete and Sudden Death Survivor

Contributing Editors: Raphael Rosenhek, MD and Thomas Binder, MD

ECHO Advisory Board: Helmut Baumgartner, MD and Gerald Maurer, MD

 


 

History

A 26-year-old male athlete suffered cardiac arrest while playing soccer. He was successfully resuscitated and brought to the hospital. The patient had previously noticed an "irregular heart beat" on several occasions but no chest pain. He had no family history of cardiac arrhythmia or sudden death.

Upon arrival at the hospital, the patient's blood pressure was 125/75 mm Hg. Oxygen saturation was 100% while he was breathing oxygen-enriched air. On examination, the patient was obese, pale, and sweaty. His lungs were clear. Heart sounds were normal but rapid, and jugular venous pressure was 12 cm of water. The ECG demonstrated ventricular tachycardia with a monomorphic left bundle-branch-block pattern.

Note:  You will need a video player installed on your browser to see any of the following four pictures in motion -- these are what "ultrasound" pictures look like to the technician (the moving versions).  You will probably get an "error message" saying something like "Runtime error."  If you do, click on "NO" -- do not try to debug the error -- it should then open the moving picture.

 
Click to zoom Click to zoom
Click to zoom Click to zoom
Click on the images to see the full Echocardiogram, then answer the questions below.

 

What is the most likely diagnosis?

A) Right ventricular dysplasia

B) Pulmonary stenosis

C) Aneurysm of the right ventricular outflow tract

D) Pulmonary embolism


 

A) Right ventricular dysplasia is correct!

Discussion

Diagnosis: Arrhythmogenic right ventricular dysplasia

Although it is rare, right ventricular dysplasia is one of the leading causes of sudden death in young people, particularly athletes. The disorder occurs predominantly in young men; a familial pattern has been suggested. Arrhythmogenic right ventricular dysplasia may cause sudden death without previous signs or symptoms. In some patients, arrhythmia, congestive heart failure, or pump dysfunction precedes sudden death.

Right ventricular dysplasia is characterized by thinning and fatty infiltration of the right ventricular wall and the formation of a small saccular aneurysm. The myocardium may be affected focally or diffusely. The disease is often associated with focal myocarditis.

Echocardiographic characteristics include right ventricular dilatation (in this case dilatation predominantly of the right ventricular outflow tract), diffuse or focal asynergy of the free right ventricular wall, and localized small aneurysms.

 

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