Article ID: CJ-21-0571
A 69-year-old woman was admitted to hospital with symptomatic heart failure accompanied by ventricular tachycardia (VT). The ECG displayed a sinus rhythm (Supplementary Figure, Upper panel); negative T waves were observed in the lateral precordial V5 and V6 leads. An epsilon wave was detected in leads II, III, and aVF, and the signal-averaged ECG showed positive late potentials (Figure A). The VT was characterized by a right bundle branch block (Supplementary Figure, Lower panel). Echocardiography showed left ventricular (LV) asynergy, with a 28% ejection fraction (Supplementary Movie). Late gadolinium enhancement-magnetic resonance imaging (LGE-MRI) was detected on the anterior wall, interventricular septum (IVS), and inferior-lateral wall (Figure B). An ECG-gated contrast computed tomography (CT) scan indicated linear hypointensity in the LV wall and the middle layer of the IVS, suggesting fatty degeneration (Figure C,D, arrows), and a scalloped appearance in the LV free wall.1 123I-β-methyl-p-iodophenyl-pentadecanoic acid single-photon emission CT (BMIPP-SPECT) revealed a partial defect within the LV inferior-posterior-lateral wall (Figure F, arrows), and 18F-fluorodeoxyglucose positron emission tomography (FDG-PET) displayed mild uptake around the same lesion (Figure E, arrows). Electroanatomical voltage mapping demonstrated that LV polarity was disrupted, but not right ventricular polarity (Figure G). Histopathological findings revealed moderate interstitial fibrosis in <50% of the residual myocardium (Figure H). Furthermore, genetic tests disclosed a desmoplakin mutation (c.944G>C, p.R315P) related to arrhythmogenic cardiomyopathy (ACM) with LV involvement. The patient was diagnosed with ACM based on the Task Force Criteria,2 but the LV lesions were insufficient for diagnosis. Therefore, specific characteristics of LV involvement and diagnostic strategies for ACM are required.
(A) ECG findings. (B) Late gadolinium enhancement-magnetic resonance imaging. (C,D) Contrast-enhanced computed tomography (CT). (E) 123I-β-methyl-p-iodophenylpentadecanoic acid single-photon emission CT. (F) 18F-fluorodeoxyglucose positron emission tomography. (G) Voltage mapping: LV, left ventricle; RV, right ventricle. (H) Histopathology (Masson’s trichrome staining): IVS, interventricular septum.
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N.H. is a member of Circulation Journal’s Editorial Team.
Supplementary Movie. Echocardiogram.
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http://dx.doi.org/10.1253/circj.CJ-21-0571
