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
- 29Citations
- 38Captures
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Metrics Details
- Citations29
- Citation Indexes29
- 29
- CrossRef6
- Captures38
- Readers38
- 38
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
http://www.sciencedirect.com/science/article/pii/S2405500X20303443; http://dx.doi.org/10.1016/j.jacep.2020.04.025; http://www.scopus.com/inward/record.url?partnerID=HzOxMe3b&scp=85089376577&origin=inward; http://www.ncbi.nlm.nih.gov/pubmed/32972544; https://linkinghub.elsevier.com/retrieve/pii/S2405500X20303443; https://dx.doi.org/10.1016/j.jacep.2020.04.025
Elsevier BV
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