Subgroup of PTS. With HCM has a high risk of sudden death. According to US and EU guidelines do exist two different systems in prediction of this risk and indication to ICD implantation. Both systems lead to entirely different results.
- yes, quite sure, and therefore I consider them both, and finally use a kind of "personal/institutional" risk stratification.
The reasons for this are the following
1) At least in symptomatic HCM patients I consider a high LVOT gradient as something that can and should be taken away by appropiate treatment (drugs, ablation, myectomy) rather than a risk marker. In the occasional asymptomatic pt. with significant obstruction this may be different, but usually obstruction is a target of therapy and not just a marker.
2) I include marked fibrosis (- as assessed by cardiac MRI) into the risk assessment. in contrast to obstruction, this disease feature is far less treatable, and there is good evidence that fibrotic areas (- apart from a nice subaortic ablation lesion...) is a bad thing to have.
What if the LVOT is non significant or normal, and no symptoms but HCM. Is Cardiac MRI indicated?
In my hospital we have started to use the european system these year, but including for some decisions the information provided by MRI
Incorporate MRI measures of fibrosis. They are very helpful and have high odds ratio for prediction of VT/SCD. I incorporate all the usual metrics for clinical decison making for AICD's but, if there is >10gms of LGE it is an independent predictor of VT/SCD. Unfortunately the guidelines haven't completely caught up with current data. It is still a bit controversial but I would submit to you: "Can you name one CV condition in which any amount of LGE has been shown to be a favorable prognosis?"
In our hospital all HCM patients are considered on case to case basis with European guidelines as primary focus. But from the electrophysiological point of view, fibrosis (LGE on MRI) is always considered as a basic arrhythmigenic substrate and therefore should be taken into account for best decisions regarding ICD therapy.
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