Editor's Note: Commentary based on Finocchiaro G, Papadakis M, Dhutia H, et al. Electrocardiographic differentiation between 'benign T-wave inversion' and arrhythmogenic right ventricular cardiomyopathy. Europace 2019;21:332-8.

Study Questions:

• What are the characteristic ECG abnormalities in patients with confirmed arrhythmogenic right ventricular cardiomyopathy (ARVC)?
• Are there distinguishing features of anterior T-wave inversions (TWI) seen in patients with ARVC compared to the "benign" TWI seen in healthy athletic and sedentary controls?

Methods:
The cases in the study population consisted of 162 patients with ARVC, based on a definite diagnosis using the 2010 Task Force Criteria, at three cardiomyopathy centers located in the United Kingdom and Italy. The control subjects were 129 individuals between 14-35 years of age screened by Cardiac Risk in the Young (an established cardiac screening program in the United Kingdom) with anterior TWI on their ECG (confined to V2-V4). Of these control subjects, 53 (41%) were deemed sedentary and 76 (59%) were considered athletic, and the presence of a cardiomyopathy was excluded by comprehensive evaluation with echocardiography, cardiac MRI, exercise testing and Holter monitoring. Standard 12-lead ECGs from each subject were evaluated.

Results:
In the patients with ARVC, 131 (81%) had an abnormal ECG, and anterior TWI was the most common abnormality (51%), followed by QRS duration ratio V2:V5 >1.2 (31%) and prolonged terminal S wave >55ms in V2 (26%). In patients with ARVC and anterior TWI, the presence of at least one additional ECG abnormality was more common compared to controls (77% vs. 19% in controls). Additionally, deep anterior TWI, defined as T-wave deflection ≥-0.2 mV, was more common in patients with ARVC (73% vs. 26%), as were anterior TWI extending to V4 (65% vs. 25%). Prior studies have suggested that the absence of J-point elevation ≥0.1 mV prior to the anterior TWI could differentiate pathological versus benign TWI. In the present study, absence of J-point elevation ≥0.1 mV was seen in nearly all (98%) of the patients with ARVC with anterior TWI. However, it was also present in 76% of controls (including 66% of athletes).

Conclusions:
Anterior TWI are the most commonly seen ECG abnormality in patients with ARVC. In this study population, anterior TWI was more commonly accompanied by other ECG abnormalities in patients with ARVC. Additionally, the absence of J-point elevation ≥0.1 mV prior to anterior TWI was highly sensitive, but not specific, for the diagnosis of ARVC.

Perspective:

• Strengths: Relatively large number of patients with ARVC compared with similar prior studies, control cohort includes representation from both athletic and sedentary individuals, long-term follow-up in controls (39 ± 6 months) revealed no cardiac symptoms or events in this group.
• Weaknesses: Significantly older ages in ARVC cases with anterior TWI (39 ± 16 years) compared to controls (23 ± 6 years), mostly white population in both groups (96% and 84%, respectively).
• Impact: Differentiating pathological versus benign anterior TWI in young individuals, particularly athletes, remains a challenge. This study suggests that the presence of additional ECG abnormalities (including deep TWI and TWI extending to V4) may be helpful in identifying patients with ARVC. Further, in a predominantly white population, it suggests that the presence of J-point elevation ≥0.1 mV prior to anterior TWI makes the likelihood of ARVC very low, but its absence is not specific for ARVC pathology.

Keywords: Arrhythmogenic Right Ventricular Dysplasia, Electrocardiography, Ambulatory, Athletes, Follow-Up Studies, Electrocardiography, Echocardiography, Arrhythmias, Cardiac, Sports, Magnetic Resonance Imaging

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