Imaging the “Hot Phase” of a Familiar Left-Dominant Arrhythmogenic Cardiomyopathy

Figures

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  Figure 1. 12-leads ECG showing T wave inversion in the inferolateral leads.

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  Figure 2. (A) STIR T2 weighted short-axis mid-ventricular image showing an area of high signal intensity in the interventricular septum (arrows). (B) LGE short-axis mid-ventricular image showing mid-wall enhancement in the interventricular septum (arrowheads).

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  Figure 3. 2-chamber (A) and mid-ventricular short-axis (B) LGE images showing subepicardial circumferential enhancement involving the entire left ventricle (arrows).

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  Figure 4. LGE 2 chamber (A) and short-axis (B) images of the patient’s sister and mother (C,D) showing subepicardial circumferential late enhancement similar to that found in the proband.

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  Figure 5. Pedigree of the family of propositus. Genotype and phenotype are defined according to the legend inset. Open symbols represent subjects with a negative genotype and phenotype. II-2, III-1, III-2: Arrhythmogenic cardiomyopathy; I-1, I-2: Unknown phenotype.