Arrythmogenic cardiomyopathy. Let’s have a closer look to the left ventricle. Report of our experience

We found 33 patients (median age 56 years, 76% males) with images suggestive of LDAC.
The main indications for a CMR study were arrhythmias (43%), Dilated Cardiomyopathy
(DCM)(21%), family history of SD (15%), chest pain with normal coronary arteries and
myocarditis (12%). Four patients, studied because of family history of SD, had LV
myocardial LGE and were asymptomatic. Midwall and/or subepicardial pattern of LGE
(100%), fatty epicardial infiltration (66.7%) and LV segmental contractility abnormalities
(59%) were the most common findings. Echocardiography was normal in most of the cases.

Thirteen patients presented VT and/or SD. In 9 cases ICD implantation was indicated.
Autopsy performed in one case of SD was positive for LDAC, and the genetic study found
a mutation of FLNC gene responsible for filamin synthesis.

We have found that fatty infiltration (p= 0.022) and not severity of LGE (p= 0.46)
was associated with the development of adverse clinical events.