LVH: COCM in Tuberous Sclerosis

Report:

Sinus rhythm 148/min

SVEBs, some aberrant

Left ventricular hypertrophy with ST/T changes

Comment:

In epiloia – sclérose tuberéuse de Bourneville – LVH is mostly seen with evidence of CCF, like in this infant with fractional shortening of only 13% (normal >29%). He was started on carvedilol for COCM.

It is less well known that large intramural rhabdomyomata may have electrical activity and produce striking LVH with preserved LV function26. Most regress over time, with corresponding normalisation of the ECG.

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