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Development and validation of a new risk prediction score for life-threatening ventricular tachyarrhythmias in laminopathies

Circulation Jul 28, 2019

Wahbi K, et al. - Researchers sought to develop and validate a prediction model that may assist in determining the absolute 5-year risk of life-threatening (LT) ventricular tachyarrhythmia (VTA) in patients with LMNA mutations and compared its contribution with current clinical practice guidelines. This study included 839 adult patients with LMNA mutations; the development sample comprised 660 patients from a French nationwide registry, and the validation sample comprised 179 patients from other countries, referred to five tertiary centers for cardiomyopathies. Male sex, nonmissense LMNA mutation, first degree and higher atrioventricular block, nonsustained ventricular tachycardia, and left ventricular ejection fraction were the identified predictors of LTVTA in the derivation sample. A 5-year estimated risk threshold ≥7% could predict 96.2% of LTVTA and net reclassification of 28.8% of patients with LTVTA vs the guidelines-based approach. The choice of candidates for implantable cardioverter defibrillators was significantly facilitated using this risk prediction model for LTVTA in laminopathies vs the current standard of care.

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