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Chapter 62: Arrhythmogenic Cardiomyopathy

A 35-year-old man experiences a sudden-onset syncope while playing soccer, and you are consulted by the emergency department. All of the following represent major criteria from the updated Task Force criteria for arrhythmogenic right ventricular cardiomyopathy (ARVC) except:

A. Regional right ventricular akinesia on transthoracic echo

B. Inverted T waves in leads V1 and V2 in the absence of complete right bundle branch block

C. Nonsustained ventricular tachycardia with LBBB morphology and superior axis

D. Identification of a pathogenic mutation

E. Epsilon wave

The answer is B. (Hurst’s The Heart, 14th Edition, Chap. 62) Inverted T waves in the right precordial leads must be present past V2 to constitute a major criterion: including V1, V2, and V3 or beyond in patients > 14 years of age in the absence of complete right bundle branch block. Otherwise, inverted T waves in leads V1 and V2 only is a minor finding. Originally dating back to 1994, the Task Force criteria for ARVC were modified in 2010 to improve the diagnosis and management of ARVC. The criteria were aimed at facilitating the recognition and interpretation of the clinical and pathologic features of ARVC, and the modified criteria incorporated new knowledge on the genetic basis of the disease, improving diagnostic sensitivity and maintaining diagnostic specificity. The structural, histologic, ECG, arrhythmic, and genetic features are structured in major and minor criteria. The Task Force document formally introduced the biventricular variant and the left dominant variant.1 Options A and C through E represent major criteria from the updated Task Force criteria. A definite diagnosis requires two major, or one major and two minor, or four minor criteria from different categories to be fulfilled.

Which of the following regarding the prevalence of ARVC in the general population and the prevalence of clinical manifestations in men versus women respectively is correct?

A. 1/1000 – 1/5000; males > females 5:1

B. 1/5000 – 1/10,000; males > females 5:1

C. 1/1000 – 1/5000; males > females 3:1

D. 1/1000 – 1/5000; females > males 3:1

E. 1/5000 – 1/10,000; females > males 5:1

The answer is C. (Hurst’s The Heart, 14th Edition, Chap. 62) The prevalence of ARVC is estimated to range from 1 in 1000 to 1 in 5000 in the general population.2-5 A small proportion of patients progress to LV dysfunction; in these patients, the clinical hallmark remains ventricular arrhythmias.6 The prevalence of clinical manifestations of the ...

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