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Number 07-17 ARVC mimic – congenital partial absence of the pericardium

Case from: Arjun Ghosh, Andrew Flett, Martin Lowe, Perry Elliott. The Heart Hospital, University College Hospital, London, United Kingdom.

Clinical history: Family screening implemented for ARVC after a 1st degree relative died of cardiomyopathy with ventricular dilatation. In this individual, and abnormal ECG with right pre-cordial T wave inversion and a shift of the transition zone to V5 was found suggesting possible ARVC. Echo normal but with poor windows.

Cine CMR: The heart is heterotopic - displaced into the left side of the chest with the apex in the posterior axilliary line (a,b).  The pericardium is seen anteriorly but not posteriorly. LV and RV function are normal but the LV is enlarged. There are no RV wall motion abnormalities.     

Perspective: The ECG in this individual suggested gene carriage and ARVC by proposed familial criteria. The CMR highlights another potential mechanism for the abnormal ECG in this individual – that the changes were caused by cardiac heterotopia from congential partial absence of the pericardium.

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