July 03, 2019
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Gene variant tied to elevated arrhythmia risk in dilated cardiomyopathy

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Among patients with dilated cardiomyopathy on implantable cardioverter defibrillator or cardiac resynchronization defibrillator therapies, titin-truncating variants in the TTN gene were associated with elevated risk for ventricular tachycardia and other arrhythmias, researchers reported.

The researchers retrospectively analyzed 117 patients (mean age, 57 years; 65% men) who had nonischemic dilated cardiomyopathy and an ICD or CRT defibrillator.

The primary outcome was time to first device-treated ventricular tachycardia or ventricular fibrillation episode. Median follow-up was 4.2 years.

Patients with a titin-truncating variant in the TTN gene had higher risk for the primary outcome compared with those who did not (HR = 4.9; 95% CI, 2.2-10.7), Ben Corden, PhD, National Institute for Health Research (NIHR) postdoctoral academic clinical fellow at National Heart and Lung Institute, Imperial College London, and colleagues wrote.

The association persisted after adjustment for midwall fibrosis as measured by late gadolinium enhancement on cardiac MRI (HR = 7.9; 95% CI, 2.6-24) and midwall fibrosis plus age, sex, left bundle branch block, device type, BMI and left ventricular ejection fraction (HR = 8.3; 95% CI, 1.8-37.6), according to the researchers.

Titin-truncating variants were also associated with having any shock (HR = 3.6; 95% CI, 1.1-11.6), the researchers wrote.

Compared with those who had neither, patients who had both titin-truncating variants and midwall fibrosis had more than 16-fold risk for receiving appropriate device therapy (HR = 16.6; 95% CI, 3.5-79.3), Corden and colleagues found.

Among patients with dilated cardiomyopathy on implantable cardioverter defibrillator or cardiac resynchronization defibrillator therapies, titin-truncating variants in the TTN gene were associated with elevated risk for ventricular tachycardia and other arrhythmias, researchers reported.
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In addition, titin-truncating variants were associated with development of new persistent atrial fibrillation (HR = 3.9; 95% CI, 1.3-12), according to the researchers.

“Knowledge of a patient’s [titin-truncating variant] status may be complementary to midwall fibrosis imaging by [CV MRI] in predicting arrhythmic risk in patients with [dilated cardiomyopathy],” Corden and colleagues wrote. – by Erik Swain

Disclosures: Corden reports no relevant financial disclosures. Please see the study for all other authors’ relevant financial disclosures.