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Arrythmogenic Right Heart Syndrome
Case Detail
| Anatomy: Chest |
 Joseph Junewick, MD FACR |
| Diagnostic Category: Genetic or Congenital |
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| Created: over 4 years ago |
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| Updated: over 3 years ago |
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| Tags:
PEDS
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| Modality/Study Types:
MR
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Activities:  PDF  ImageJA |
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.