Wolff Parkinson white pattern in Danon disease: When preexcitation is not what it seems

Abstract

Danondisease is a rare genetic disorder with an X-linked dominant inheritance affecting both skeletal and cardiac muscle. Its characteristic cardiac phenotype consists on a severe, non-obstructive and concentric hypertrophic cardiomyopathy (HCM) usually associated with a Wolff-Parkinson-White (WPW) type preexcitation pattern. Whether this corresponds to the presence of an AV or another type of accessory pathways, such as fasciculoventricular pathways (FVP) remains controversial in the literature. However, we describe the case of a teenager with Danon disease and preexcitation who develops a first degree AV block without any change in his QRS morphology, fact that favors the hypothesis of the presence of a FVP. This finding has important clinical implications in the management and prognosis of these complex patients. The absence of an AV accessory pathway decreases their risk of potential SCD in the context of a fast atrial arrhythmia and their chances of having a reentrant AV tachycardia.

Keywords: AV accessory pathway; Adenosine test; Arrhythmia; Danon disease; Electrophysiological study; Fasciculoventricular pathway; Hypertrophic cardiomyopathy; Preexcitation; WPW syndrome.