Wednesday, October 31, 2012

Arrhythmogenic Right Ventricular Dysplasia

In young Emergency Department patients with syncope, most of the time, testing is minimal.  Generally, the only universal testing is a pregnancy test and/or an electrocardiogram.

We've gotten pretty good at understanding the "life-threatening" causes of syncope in young adults diagnosed by electrocardiography, including:
 - Wolff-Parkinson-White Syndrome
 - Hypertrophic Obstructive Cardiomyopathy
 - Brugada Syndrome
 - Congenital Long QT

But there's always more, and Arrhythmogenic Right Ventricular Dysplasia is one of those "more" that seems not to be on everyone's lists.  ARVD is a genetically-inherited abnormality in cardiac desmosomes that leads to fibrofatty deposition in the right ventricle.  It is currently estimated to result in ~5% of the sudden cardiac deaths in adults under age 65, secondary to sustained monomorphic ventricular tachycardia.  The characteristic EKG finding to look out for is, unfortunately, quite subtle – the "epsilon wave".  These waves are most prominent in V1-V3, and manifest as sharp upward deflections from baseline at the conclusion of the QRS complex.

Very few Emergency Department presentations mix the high-risk needle-in-the-haystack with the low-risk like young adults with syncope, so it's important to stay alert for these rare ECG findings.

"Impact of new electrocardiographic criteria in arrhythmogenic cardiomyopathy"
www.ncbi.nlm.nih.gov/pubmed/23015790

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