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Prevalence and Prognostic Impact of Pathogenic Variants in Patients With Dilated Cardiomyopathy Referred for Ventricular Tachycardia Ablation

JACC: Clinical Electrophysiology, ISSN: 2405-500X, Vol: 6, Issue: 9, Page: 1103-1114
2020
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Article Description

This study aimed to assess the frequency of (likely) pathogenic variants (LP/P v ) among dilated cardiomyopathy (DCM) ventricular tachycardia (VT) patients referred for CA and their impact on procedural outcome and long-term prognosis. The prevalence of genetic variants associated with monomorphic VT among DCM is unknown. Ninety-eight consecutive patients (age 56 ± 15 years; 84% men, left ventricular ejection fraction [LVEF] 39 ± 12%) referred for DCM-VT ablation were included. Patients underwent electroanatomical mapping and testing of ≥55 cardiomyopathy-related genes. Mapping data were analyzed for low-voltage areas and abnormal potentials. LP/P v -positive (LP/P v +) patients were compared with LP/P v -negative (LP/P v– ) patients and followed for VT recurrence and mortality. In 37 (38%) patients, LP/P v were identified, most frequently LMNA (n = 11 of 37, [30%]), TTN (n = 6 of 37, [16%]), PLN (n = 6 of 37, [16%]), SCN5A (n = 3 of 37, [8%]), RBM20 (n = 2 of 37, [5%]) and DSP (n = 2 of 37, [5%]). LP/P v + carriers had lower LVEF (35 ± 13% vs. LP/P v– : 42 ± 11%; p = 0.005) and were less often men (n = 27 [73%] vs. n = 55 [90%]; p = 0.03). After a median follow-up of 2.4 years (interquartile range: 0.9 to 4.4 years), 63 (64%) patients had VT recurrence (LP/P v +: 30 of 37 [81%] vs. LP/P v– : 33 of 61 [54%]; p = 0.007). Twenty-eight patients (29%) died (LP/P v +: 19 of 37 [51%] vs. LP/P v– : 9 of 61 [15%]; p < 0.001). The cumulative 2-year VT-free survival was 41% in the total cohort (LP/P v +: 16% vs. LP/P v– : 54%; p = 0.001). The presence of LP/P v (hazard ratio: 1.9; 95% confidence interval: 1.1 to 3.4; p = 0.02) and unipolar low-voltage area size/cm 2 increase (hazard ratio: 2.5; 95% confidence interval: 1.6 to 4.0; p < 0.001) were associated with a decreased 2-year VT-free survival. In patients with DCM-VT, a genetic cause is frequently identified. LP/P v + patients have a lower LVEF and more extensive VT substrates, which, in combination with disease progression, may contribute to the poor prognosis. Genetic testing in patients with DCM-VT should therefore be recommended.

Bibliographic Details

Ebert, Micaela; Wijnmaalen, Adrianus P; de Riva, Marta; Trines, Serge A; Androulakis, Alexander F A; Glashan, Claire A; Schalij, Martin J; Peter van Tintelen, J; Jongbloed, Jan D H; Zeppenfeld, Katja

Elsevier BV

Medicine

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