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Joseph Junewick, MD FACR
over 4 years ago
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ARSt Case Repository

Arrythmogenic Right Heart Syndrome

Case Detail

Anatomy: Chest
Junewick
Joseph Junewick, MD FACR
Diagnostic Category: Genetic or Congenital
Created: over 5 years ago
Updated: over 4 years ago
Tags: PEDS
Modality/Study Types: MR
Activities:
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History

Teenager with tachycardia


Case Images


Diagnosis

Arrythmogenic Right Heart Syndrome (ARHS)

Findings

MRI reveals decreased right ventricular free wall thickness and T1 hyperintensity of the infundibulum. Poor right ventricular contractility was noted on cinegraphic imaging.

Discussion

ARHS presents in young and middle age patients with ventricular dysrhythmia and left bundle branch block and occasionally sudden death. Males are three times more often affected than females and there is usually a strong family history. MR is the preferred imaging modality to confirm the diagnosis. T1 hyperintensity in the right ventricular outlet tract corresponds to fat and is associated with a higher incidence of inducible tachycardia. Cinegraphic imaging helps determine right ventricular function and wall motion abnormalities. Treatment includes anti-arrhythmic drugs, ablation, defibrillator and/or ventriculotomy. The risk of death is 1% per year.



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