Arrhythmogenic Right Ventricular Cardiomyopathy
or ~Dysplasia
- usually Autosomal Dominant inherited myocardial disease associated with paroxysmal ventricular arrhythmias and sudden cardiac death.
- Characterized pathologically by fibro-fatty replacement of the RV myocardium.
- 2nd most common cause of sudden cardiac death in young people (after HOCM), causing up to20% of sudden cardiac deaths in patients < 35 yrs of age.
Clinical:
- typically presents with palpitations, syncope or cardiac arrest precipitated by exercise.
- 1st presenting symptom may be sudden cardiac death.
- Over time, develop features of RV failure, which may progress to severe biventricular failure and dilated cardiomyopathy.
- usually a FHx of sudden cardiac death.
Characteristic ECG abnormalities:
- Epsilon wave (most specific finding, seen in 30% of patients)
- T wave inversion in V1-3 (85% of patients)
- Prolonged S-wave upstroke of 55ms in V1-3 (95% of patients)
- Localised QRS widening of 110ms in V1-3
- Paroxysmal episodes of ventricular tachycardia with LBBB morphology (e.g. right ventricular VT).
Understanding athletes with ECG's with similarities to ARVC:
'Our study confirmed that the presence or absence of JPE did not reliably discriminate between pathological and physiological anterior TWI for ARVC evaluation in subjects of similar age, sex, and ethnicity. Rather, we identified 3 alternative ECG markers that were independent predictors of disease status. In athletes and ARVC subjects with TWI(V1V4), these 3 combined ECG features differentiated health from disease with 81% accuracy'
- extensive TWI [beyond lead V3 and/or inferior]
- the presence of PVCs, and
- Sokolow-Lyon LVH score <20 mm)
- has been reported that ARVC is 10x more common in white vs black South African populations
ECG features athletes vs AVRC - JACC 2018 https://www.sciencedirect.com/science/article/pii/S2405500X18307898#:~:text=Compared%20with%20healthy%20athletes%2C%20ARVC,lower%20voltage%20scores%20for%20LVH.