Long-Term Arrhythmic Follow-Up and Risk Stratification of Patients With Desmoplakin-Associated Arrhythmogenic Right Ventricular Cardiomyopathy.
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Gasperetti Alessio, Carrick Richard, Protonotarios Alexandros, Laredo Mikael, van der Schaaf Iris, Syrris Petros, Murray Brittney, Tichnell Crystal, Cappelletto Chiara, Gigli Marta, Medo Kristen, Crabtree Peter, Saguner Ardan M, Duru Firat, Hylind Robyn, Abrams Dominic, Lakdawala Neal K, Massie Charles, Cadrin-Tourigny Julia, Targetti Mattia, Olivotto Iacopo, Graziosi Maddalena, Cox Moniek, Biagini Elena, Charron Philippe, Casella Michela, Tondo Claudio, Yazdani Momina, Ware James S, Prasad Sanjay, Calò Leonardo, Smith Eric, Helms Adam, Hespe Sophie, Ingles Jodie, Tandri Harikrishna, Ader Flavie, Mestroni Luisa, Wilde Arthur, Merlo Marco, Gandjbakhch Estelle, Calkins Hugh, Te Riele Anneline S J M, Peter van Tintelen J, Elliot Perry, James Cynthia A
Аннотация
OBJECTIVES: The purpose of this study was to characterize arrhythmic outcomes and to test the performance of the recently validated ARVC risk calculator in patients with DSP likely pathogenic/pathogenic variants fulfilling definite 2010 ARVC Task Force Criteria (DSP-TFC+).
METHODS: DSP-TFC+ patients were enrolled from 20 institutions across 3 continents. Ventricular arrhythmias (VA), defined as a composite of sustained ventricular tachycardia (VT), appropriate implantable cardioverter defibrillator therapies, and ventricular fibrillation/sudden cardiac death events in follow-up, were reported as the primary outcome. We tested the performance of the ARVC risk calculator for VA prediction, reporting c-statistics.
RESULTS: Among 252 DSP-TFC+ patients (age 39.6 ± 16.9 years, 35.3% male), 94 (37.3%) experienced VA over 44.5 [IQR: 19.6-78.3] months. Patients with left ventricle involvement (n = 194) were at higher VA risk (log-rank P = 0.0239). History of nonsustained VT (aHR 2.097; P = 0.004) showed the strongest association with VA occurrence during the first 5-year follow-up. Neither age (P = 0.723) nor male sex (P = 0.200) was associated with VAs at follow-up. In 204 patients without VA at diagnosis, incident VA rate was high (32.8%; 7.37%/y). The ARVC risk calculator performed poorly overall (c-statistic 0.604 [0.594-0.614]) and very poorly in patients with left ventricular disease (c-statistic 0.558 [0.556-0.560]).
CONCLUSIONS: 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.