RV Functional Abnormalities in Arrhythmogenic Cardiomyopathy
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This study aimed to perform an external validation of the value of right ventricular (RV) deformation patterns and RV mechanical dispersion in patients with arrhythmogenic cardiomyopathy (AC). Secondly, this study assessed the association of these parameters with life-threatening ventricular arrhythmia (VA).

Subtle RV dysfunction assessed by echocardiographic deformation imaging is valuable in AC diagnosis and risk prediction. Two different methods have emerged, the RV deformation pattern recognition and RV mechanical dispersion, but these have neither been externally validated nor compared.

Researchers analyzed AC probands and mutation-positive family members, matched from 2 large European referral centers. They performed speckle tracking echocardiography, whereby they classified the subtricuspid deformation patterns from normal to abnormal and assessed RV mechanical dispersion from 6 segments. They defined VA as sustained ventricular tachycardia, appropriate implantable cardioverter-defibrillator therapy, or aborted cardiac arrest.

Results:
-- They included 160 subjects, 80 from each center (43% proband, 55% women, age 41 ± 17 years).

-- VA had occurred in 47 (29%) subjects. In both cohorts, patients with a history of VA showed abnormal deformation patterns (96% and 100%) and had greater RV mechanical dispersion.

-- Both parameters were independently associated to VA.

-- The association with VA significantly improved when adding RV mechanical dispersion to pattern recognition.

Conclusively, researchers externally validated 2 RV dysfunction parameters in AC. Adding RV mechanical dispersion to RV deformation patterns significantly improved the association with life-threatening VA, indicating incremental value.

Source: https://www.jacc.org/doi/10.1016/j.jcmg.2020.12.028?_ga=2.139224883.1260721703.1620203202-777820309.1584504539
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