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Number 07-14: ARVC with LV involvement

Case from: Dee Ward, Antonis Pantazis, The Heart Hospital, London, UK

Clinical history: A 59 year old with an 8 year history of asymptomatic ARVC diagnosed through family screening. A disease causing mutation in plakophilin was found. Type 1 diabetes since childhood. Recent echo showed a possible new LV apical aneurysm. Coronary angiography normal.

Cine CMR:
RV moderate impairment with multiple wall motion abnormalities (micro aneuysms). Discreet areas of LV thinning (apex, mid antero-lateral wall)

Tissue characterisation – intrinsic contrast:
(top right); T1W TSE: Fatty infiltration of the RV side of the septum, parts of the moderator band and RV trabeculae, also the lateral wall of the LV, nulling with fat sat, (bottom left T1W TSE with fatsat).

Tissue characterisation – extrinsic contrast:
(bottom right) Late gadolinium enhancement (LGE) matching the fatty infiltration (possibly explained by the shorter T1 of fat) but additional RV free wall LGE and in the LV not explained by the fat.

Interpretation:
ARVC with LV involvement is present here with both fibrous and fatty infiltration. LV involvement may be more common than once thought.

Scanner: Siemens avanto.

References: Sen-Chowdhry S et al. Circulation 2007;115:1710-20; Sen-chowdhry S et al. JACC 2006;48:2132-40

Further views (sa):

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