Case of the week 07-17 ARVC mimic

1
Case of the week 07-17 ARVC mimic congenital
partial absence of the pericardium
a
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. 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.        Conclusion 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.  
b
c
Arjun Ghosh, Andrew Flett, Martin Lowe, Perry
Elliott. The Heart Hospital, University College
Hospital, London, United Kingdom.