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Number 08-02: Apical HCM, fibrosis and sustained VT
Case from: Davies, EJ, Bellenger NG. Royal Devon and Exeter
NHS Foundation Trust, Exeter, UK
Clinical history: An 82 yr man admitted with palpitations,
presyncope and sustained monomorphic VT. Family history, his brother died age
6 of unknown cause.
Cine CMR: Mid cavity hypertrophy (max LVWT 19mm) with obliteration
and an apical aneurysm without thrombus
LGE CMR: Patchy late gadolinium enhancement in the mid LV but
full thickness enhancement of the distal thinned aneurysm. (compare the LGE
with the cine – look for LGE but also the absence of normal dark myocardium
Perspective:This patient has apical HCM with scarring distal to mid cavity
obstrucion forming an apical aneurysm. This is the substrate for sustained monomorphic
VT which is not common in other forms of HCM.
In this case, treatment was with warfarin, beta blockade, amiodarone and an ICD.
Family screening is planned.
Scanner: Siemens avanto.
References: References: here(full
text)
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