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Left dominant arrhythmogenic cardiomyopathy: more specific MRI findings

Session Poster session 1

Speaker Ilaria Dentamaro

Congress : EuroCMR 2019

  • Topic : imaging
  • Sub-topic : Cardiac Magnetic Resonance: Myocardium
  • Session type : Poster Session
  • FP Number : P182

Authors : I Dentamaro (Bari,IT), V Pineda (Barcelona,ES), A Guala (Barcelona,ES), F Valente (Barcelona,ES), G Teixido (Barcelona,ES), I Silveira (Barcelona,ES), R Fernandez-Galera (Barcelona,ES), L Galian (Barcelona,ES), T Gonzalez-Alujas (Barcelona,ES), H Cuellar (Barcelona,ES), A Evangelista (Barcelona,ES), J Rodriguez-Palomares (Barcelona,ES)

I Dentamaro1 , V Pineda2 , A Guala2 , F Valente2 , G Teixido2 , I Silveira2 , R Fernandez-Galera2 , L Galian2 , T Gonzalez-Alujas2 , H Cuellar2 , A Evangelista2 , J Rodriguez-Palomares2 , 1Polyclinic Hospital of Bari, Cardiology Department, DETO - Bari - Italy , 2University Hospital Vall d'Hebron - Barcelona - Spain ,

European Heart Journal - Cardiovascular Imaging ( 2019 ) 20 ( Supplement 2 ), ii141

INTRODUCTION: Subepicardial late gadolinium enhancement (LGE) is hallmark finding of left ventricle dominant arrhythmogenic cardiomyopathy variant (LDAC) but it is not pathognomonic. LDAC is a recognized AC variant, probably remains underdiagnosed due to its wide clinical spectrum and common imaging finding with inflammatory myocardiopathy. Specific diagnostic criteria are not available. The aim of this study was to analyze the discriminative capacity of different cardiac magnetic resonance (CMR) findings in patients with LDAC versus myocarditis. METHODS: After reviewing CMR exams of patients referred to our unit from 2014 until 2017, 42 patients were included with subepicardial LGE and no left ventricular dysfunction. 15 patients (mean age 32.9 ± 11.0) selected for the LDAC with a pathogenic mutation and/or family history of premature sudden death (<35 years of age) due to suspected AC. 27 (mean age 38.8 ± 15.6)  patients with  diagnosis of acute myocarditis (fulfilled Lake Louise Criteria). All the CMR exams were analysed by a certified operator blinded to patients clinical conditions. We collected CMR parameters and some specifics features like the presence of a scalloped LVW epicardial borders, and late LGE distribution (continuous or patchy) in long axis view [Figure1]. RESULTS: The two groups didn’t showed any differences regarding LV volumes or ejection fraction. In the univariate analysis long-axis LGE continuous pattern (p value <0.001) and scalloped LV epicardial border (p value <0.001) were statistically significantly different between the two groups [Table 1]. In multivariable analysis corrected for differences in weight and height, long-axis LGE continuous pattern resulted in an AUC of 0.96, sensitivity of 92.6 %, specificity of 86.7 %, PPV of 7% and NPV of 0.1%. and scalloped LV epicardial border resulted in an AUC of 0.98, sensitivity of 92.9 %, specificity of 92.9 %, PPV of 13% and NPV of 0.1% [Figure 2]. CONCLUSIONS:  LDCA and myocarditis  are difficult to distinguish. CMR is the imaging technique of choice to make the differential diagnosis,  but often common imaging finding are observed.  This study provides  new CMR parameters that can help the differential diagnosis.

Myocarditis LDCA Univariate
N 27 15 p-value
LVEF [%] 57.3 ± 12.1 54.4±11.2 0.450
LVEDV [ml] 158.9 ± 29.8 177.0 ± 55.4 0.176
long-axis LGE continuous pattern 11% 87% <0.001
Scalloped LV epicardial borders 4 % 87 % <0.001

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