Does the standard ARVC risk calculator accurately predict ventricular arrhythmias in patients with Desmoplakin-associated arrhythmogenic right ventricular cardiomyopathy?
The standard ARVC risk calculator performs poorly in patients with Desmoplakin-associated ARVC, highlighting the need for a gene-specific risk algorithm and identifying nonsustained VT as a high-risk marker.
DSP-TFC+ patients are at substantial risk for VAs. The ARVC risk calculator performs poorly in DSP-TFC+ patients suggesting need for a gene-specific risk algorithm. Meanwhile, DSP-TFC+ patients with nonsustained VT should be considered as high-risk.
Gasperetti et al. (Thu,) studied this question.