Abstract
Objectives: Left ventricular (LV) involvement has been associated with unfavorable prognosis in arrhythmogenic cardiomyopathy (ACM). We aim to evaluate LV mechanics by cardiovascular magnetic resonance-feature tracking (CMR-FT) in ACM patients with right ventricular (RV) dysfunction.Methods: We retrospectively recruited ACM patients diagnosed according to the revised Task Force Criteria (rTFC) from January 2015 to July 2017. All patients underwent CMR examinations and collections of clinical, electrocardiographic data. The strain and dyssynchrony parameters of LV and RV were analyzed. These patients were followed, and primary study outcome was defined as a composite of cardiovascular events (arrhythmic events and heart transplantation), secondary study outcome included arrhythmic events.Results: Eighty-nine ACM patients (40.40 ± 13.98 years, 67.42% male) were included. LV and RV ejection fractions were 49.12 ± 12.02% and 22.28 ± 10.11%, respectively. During a median (IQR) follow-up for 18.20 (11.60-30.04) months, 30 patients experienced cardiovascular events which included 22 patients who experienced arrhythmic events. Patients with cardiovascular events had impaired LV global longitudinal strain (−10.82 ± 2.77 vs. −12.61 ± 3.18%, p = 0.010), impaired LV global circumferential strain (−11.81 ± 2.40 vs. −13.04 ± 2.83%, p = 0.044), and greater LV longitudinal dyssynchrony (LVLD) (80.98 ± 30.98 vs. 64.23 ± 25.51 ms, p = 0.012) than those without. After adjusting for age, sex, and other confounding factors, LVLD ≥89.15 ms was an independent risk factor for cardiovascular events (HR: 4.50, 95% CI: 1.94 to 10.42; p = 0.001) and for arrhythmic events (HR: 4.79, 95% CI: 1.74 to 13.20; p = 0.003).Conclusions: LVLD by CMR-FT was an independent risk factor for cardiovascular and arrhythmic events in ACM patients in advanced stage, which could provide prognostic value for this subtype.
Highlights
Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inheritable myocardial disease with potential high risk of malignant ventricular arrhythmias and progressive heart failure at end-stage [1, 2]
It was shown that Left ventricular longitudinal dyssynchrony (LVLD) was significantly associated with cardiovascular events (HR: 1.02, 95% confidence intervals (CI): 1.00-1.04; p = 0.002) and arrhythmic events (HR: 1.03, 95% CI: 1.01-1.05; p = 0.002) even after adjusting for confounding variables
We introduced Cardiac magnetic resonance (CMR)-Feature tracking (FT) technique in evaluating left ventricular (LV) and right ventricle (RV) mechanics in a sizable definite arrhythmogenic cardiomyopathy (ACM) cohort, in which all patients were recruited in a tertiary referral center and had advanced RV dysfunction
Summary
Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inheritable myocardial disease with potential high risk of malignant ventricular arrhythmias and progressive heart failure at end-stage [1, 2]. It is classically characterized by fibro-fatty myocardial replacement predominantly at the right ventricle (RV) [3]. All ACM subcategories are characterized by similar fibro-fatty alteration and life-threatening ventricular arrhythmias, usually already in the early disease stage [6]. The substantially predictive value of LV involvement could not be fully illustrated in the setting of ACM patients with heterogeneous severities of biventricular dysfunction
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