Key result
Clinical risk factors (NSVT, LVEF <45%, male sex, non-missense mutations) predicted malignant ventricular arrhythmias in LMNA mutation carriers (C-index 0.76; 95% CI 0.72-0.80; P<0.001).
Why the study?
The prognostic model underlying international ICD recommendations for LMNA mutation carriers had not been validated in an external cohort or in a population composed solely of relatives.
Population
77 LMNA mutation carriers in an external independent validation cohort
Comparison
Stratification by number of clinical risk factors (0 vs 1 vs ≥ 2 risk factors)
Design
Single-centre observational cohort external validation study
Follow-up
Median 3.4 years
Authors
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Supports current ICD recommendations in LMNA cardiomyopathy; leaves open refined risk models for prospective validation.
Cohort (n=77)
No
Effect estimate: C-index 0.76 (95% CI 0.72-0.80)
Absolute Event Rate: 75% vs 100%
p-value: p=<0.001
This study successfully externally validates a prognostic model using four risk factors (NSVT, LVEF < 45%, male sex, non-missense mutations) to predict malignant ventricular arrhythmias in LMNA mutation carriers.
Thuillot et al. (2019) conducted a cohort in LMNA mutation carriers (n=77). Clinical risk factors (NSVT, LVEF < 45%, male sex, non-missense mutations) vs. No risk factors was evaluated on MVA-free survival at 3 years (C-index 0.76, 95% CI 0.72-0.80, p=<0.001). Clinical risk factors (NSVT, LVEF <45%, male sex, non-missense mutations) predicted malignant ventricular arrhythmias in LMNA mutation carriers (C-index 0.76; 95% CI 0.72-0.80; P<0.001).
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