부산대학교 도서관

Introduction:We sought to evaluate the impact of left ventricular dysfunction (LVDfx) in children with a definite arrhythmogenic right ventricular cardiomyopathy (ARVC) diagnosis.Methods:We retrospectively reviewed patients 0-18 years of age diagnosed with ARVC at our institution between 2001-2018. Patients with LVDfx, defined as an ejection fraction of <55% on echocardiogram, were identified and their outcomes compared to ARVC patients without LVDfx. Kaplan-Meier transplant-free survival curves were utilized.Results:Thirty-five children with a diagnosis of ARVC were identified. Twelve patients (34%) had LVDfx, of which 7 (20%) had moderate-severe LVDfx. The median age at diagnosis was 15 years (IQR 13.2 - 15.8) with a median follow-up time of 2.1 years (IQR 1.1 - 4.3). There was no association with increased left ventricular dimension or mitral regurgitation in either group. Cardiac magnetic resonance imaging demonstrated that LVDfx was associated with moderate to severe right ventricular dysfunction (41.6% vs 17.4% p = 0.009) and late gadolinium enhancement (58.3% vs 8.7% p = 0.004). Arrhythmia burden was greater in the LVDfx group with a higher incidence of ectopy (83% vs 50% p = 0.04) and non-sustained ventricular tachycardia (83% vs 22% p = 0.0009). The LVDfx group was significantly more likely to receive an angiotensin converting enzyme inhibitor (41.6% vs 0% p = 0.002) or a beta-blocker (75% vs 8.7% p = 0.0001). Total length of hospital stay was significantly longer in the LVDfx cohort (median 2 days vs 0 days p = 0.001). The two-year transplant free survival was significantly worse in the LVDfx group (88% vs. 100%, p=0.04, figure 1).Conclusions:ARVC is not always confined to a single chamber and LVDfx confers a greater risk to the healthcare trajectory of children with ARVC. Therapeutic strategies for LVDfx may have a substantial impact on outcomes in this population.