Korean Circ J.  2017 Mar;47(2):282-285. 10.4070/kcj.2016.0328.

Catheter Ablation of Ventricular Tachycardia/Fibrillation in a Patient with Right Ventricular Amyloidosis with Initial Manifestations Mimicking Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy

• Chung FP
• Lin YJ
• Kuo L
• Chen SA

• KMID: 2377471
• DOI: http://doi.org/10.4070/kcj.2016.0328

Abstract

Differentiating arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) from other cardiomyopathies is clinically important but challenging. Although the modified Task Force Criteria can facilitate diagnosis of ARVD/C according to clinical manifestations, histopathological examination plays a pivotal role in excluding other diseases that can mimic ARVD/C. Here, we report a patient with amyloidosis that initially presented similarly to ARVD/C. The diagnosis was confirmed by endomyocardial biopsy, and catheter ablation eliminated the ventricular tachyarrhythmias through an epicardial approach.

Keyword

Amyloidosis; Arrhythmogenic right ventricular cardiomyopathy-dysplasia; Catheter ablation; Biopsy; Ventricular tachycardia

MeSH Terms

Advisory Committees
Amyloidosis*
Arrhythmogenic Right Ventricular Dysplasia
Biopsy
Cardiomyopathies
Catheter Ablation*
Catheters*
Diagnosis
Humans
Tachycardia
Tachycardia, Ventricular

Figure

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  Fig. 1 Electrocardiograms of the patient. (A) 12-lead ECG morphology showed a wide QRS complex tachycardia with LBBB morphology and superior axis. Intracardiac tracing demonstrated VA dissociation, which rendered the diagnosis of supraventricular tachycardia less likely. (B) ECG during sinus rhythm showed T wave inversion of precordial leads from V1-V4. (C) SAECG showed positive depolarization abnormalities, including fQRS, duration of terminal QRS <40 µV, and RMS voltage in terminal 40 ms. ECG: electrocardiogram, LBBB: left bundle branch block, VA: ventricular arrhythmia, SAECG: signal-averaged electrocardiogram, RMS: root means square.

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  Fig. 2 Endocardial and epicardial RV voltage mapping. (A) Bipolar voltage mapping demonstrated the scar/low voltage zone extending from RVOT to RV free wall. (B) Unipolar voltage mapping showed a larger abnormal substrate extending from RVOT to RV free wall compared to those identified by bipolar voltage mapping. (C) Epicardial voltage mapping also revealed extensive scarring involving RVOT, basal RV free wall and RV apex. Examples of abnormal electrograms, including fractionated and isolated late potentials, were recorded surrounding the scar/LVZ. RV: right ventricular, RVOT: right ventricular outflow tract, LVZ: low voltage zone.

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  Fig. 3 Histopathological examination of amyloid heart tissue by endomyocardial biopsy. (A) H&E staining (×400) of endomyocardial biopsy obtained from right ventricular free wall. (B) Congo red staining showed light-chain right ventricular amyloidosis (yellow and green arrows).

Reference

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