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Arrythmogenic cardiomyopathy. Let's have a closer look to the left ventricle. Report of our experience
Journal of Cardiovascular Magnetic Resonance volume 17, Article number: Q74 (2015)
Background
The classic Arrythmogenic Cardiomyopathy (ARC) has predilection for the right ventricle (RV). However, recent studies have demonstrated another pattern with early and predominant left ventricular involvement, Left Dominant Arrythmogenic Cardiomyopathy (LDAC). In our centre the alarm went off when CMR studies in relatives of sudden death (SD) patients with autopsy proven ARC, demonstrated predominant LV involvement. These findings together with the literature, encouraged us to look for more cases with this particular pattern among our patients.
Our objective was to study clinical presentation of patients with CMR pattern of LDAC and try to find any prognostic parameters of clinical events.
Methods
With the finding of a diffuse mesocardial and subepicardial Late Gadolinium Enhancement (LGE) in SD patient's relatives as well as 3 patients with SD, we decided to review retrospectively the CMR and clinical database of our centre to obtain more cases with this pattern compatible with LDAC. We also included prospectively some more cases among patients scheduled for CMR during 2013-2014.
Results
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.
Conclusions
Until the quite recent widespread use of CMR, LDAC was an under-recognized entity. Meso-subepicardial hyperenhancement was recently described as one of the image criteria of LDAC. We found this pattern in patients with clinically suspected DCM, chest pain with normal coronary arteries or myocarditis, as well as in asymptomatic patients. However, its severity was not found to be related to development of clinical events. On the contrary, fatty infiltration was more frequently found in patients with VT and/or SD. Since the form of presentation can be very variable, and echocardiography can be normal, we should have it mind, because of its possible bad evolution.
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No fundings nor conflicts of interest.
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This article is published under license to BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/4.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.
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Feliu, E., Moscicki, R., Climent, V. et al. Arrythmogenic cardiomyopathy. Let's have a closer look to the left ventricle. Report of our experience. J Cardiovasc Magn Reson 17 (Suppl 1), Q74 (2015). https://doi.org/10.1186/1532-429X-17-S1-Q74
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DOI: https://doi.org/10.1186/1532-429X-17-S1-Q74
Keywords
- Sudden Death
- Myocarditis
- Dilate Cardiomyopathy
- Right Ventricle
- Late Gadolinium Enhancement