OBJECTIVE

To evaluate the incidence and predictors of adverse outcomes in the Russian population of patients with arrhythmogenic right ventricular dysplasia (ARVD).

MATERIAL AND METHODS

There were 54 patients with ARVD. Mean age was 38.7±14.1 years. Men made up 42.6%, follow-up period — 21 [6; 60] months, period from the onset of disease to the last contact with a patient — 6.5 [2.9; 10.3] years. All patients underwent ECG, 24-hour Holter monitoring, echocardiography. Survey also included MRI (n=49) and signal-averaged ECG (n=18).

RESULTS

Overall mortality was 13%. Predictors of mortality were reduced left ventricular (LV) ejection fraction <35%, left atrium enlargement >120 ml and right atrium (RA) dilatation >115 ml, right ventricular dimension over 3.5 cm, male gender and increased C-reactive protein (CRP) level ≥0.6 mg/dL. Resistant ventricular tachycardia was registered in 35.2% of patients. Predictors of this event were previous syncope, RA volume >80 ml and RV diameter >3.5 cm. Cardioverter-defibrillator (ICD) was implanted in 13 patients (26%). The appropriate shocks were recorded in 9 (69.2%) patients. The predictor of appropriate ICD shocks was LV end-diastolic dimension ≥5 cm. The incidence of chronic heart failure (CHF) in our cohort was 39%. The main predictors of CHF were RA volume ≥70 ml, duration of disease ≥5.5 years and CRP level ≥0.2 mg/dL.

CONCLUSION

We have studied the predictors of adverse outcomes in the Russian population of ARVD patients for the first time. Mathematical models and calculators of adverse outcome risk have been developed and can be used in clinical practice.