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Aims Arrhythmogenic right ventricular cardiomyopathy (ARVC) is characterized by ventricular dysfunction and ventricular arrhythmias (VA). Adequate arrhythmic risk assessment is important to prevent sudden cardiac death. We aimed to study the incremental value of strain by feature-tracking cardiac magnetic resonance imaging (FT-CMR) in predicting sustained VA in ARVC patients. Methods and results CMR images of 132 ARVC patients (43% male, 40.6 ± 16.0 years) without prior VA were analysed for global and regional right and left ventricular (RV, LV) strain. Primary outcome was sustained VA during follow-up. We performed multivariable regression assessing strain, in combination with (i) RV ejection fraction (EF); (ii) LVEF; and (iii) the ARVC risk calculator. False discovery rate adjusted P-values were given to correct for multiple comparisons and c-statistics were calculated for each model. During 4.3 (2.0–7.9) years of follow-up, 19% of patients experienced sustained VA. Compared to patients without VA, those with VA had significantly reduced RV longitudinal (P ≤ 0.03) and LV circumferential (P ≤ 0.04) strain. In addition, patients with VA had significantly reduced biventricular EF (P ≤ 0.02). After correcting for RVEF, LVEF, and the ARVC risk calculator separately in multivariable analysis, both RV and LV strain lost their significance [hazard ratio 1.03–1.18, P > 0.05]. Likewise, while strain improved the c-statistic in combination with RVEF, LVEF, and the ARVC risk calculator separately, this did not reach statistical significance (P ≥ 0.18). Conclusion Both RV longitudinal and LV circumferential strain are reduced in ARVC patients with sustained VA during follow-up. However, strain does not have incremental value over RVEF, LVEF, and the ARVC VA risk calculator.

Background/Introduction Specific athlete reference values for cardiac volumes and function using cardiac magnetic resonance (CMR) parameters have been reported and are used in clinical practice. Elite athletes are conventionally thought to demonstrate the highest levels of physiological adaptation to sports and therefore dictate the upper limit of normal for biventricular size and function in the athlete population. Purpose To compare volumetric parameters in a cohort of both male and female elite-level athletes to previously published reference values. Methods We used data from the prospective ELITE-cohort, which collects pre-participation data of elite athletes (national-, international-, Olympic-, Paralympic-level), including CMR imaging. Athletes suspected of pathology based on comprehensively investigated electrocardiograph, exercise test, echocardiogram and late gadolinium enhancement were excluded. Biventricular volumes were derived from short-axis cine images using Cvi42 (v5.1.2.) and indexed for body surface area (BSA). Volumetric parameters were compared to the previously reported gender- and Mitchell sports classification specific cardiac 95th percentile (95%tile) reference values, as reported by Luijkx et al.: left-ventricle (LV) end-diastolic volume (EDV) / BSA for high-static and high-dynamic sports (HS/HD; ♀ 142 ♂ 158); low-static and high-dynamic (LS/HD; ♀ 127 ♂ 149); high-static and low-dynamic (HS/LD; ♀ 114 ♂ 140); right ventricle (RV) EDV / BSA HS/HD (♀ 154 ♂ 184), LS/HD (♀ 136 ♂ 163), and HS/LD (♀ 120 ♂ 157). Results We analysed a total of 221 athletes (38% female), with a median age (IQR) of 26.0 (22.5–29.8) years, and mean ±SD BSA of 1.96±0.22 m2. Athletes were classified according to the Mitchell sports classification: HS/HD 116 (54%); LS/HD 61 (29%); moderate-static and high-dynamic (MS/HD) 20 (9.4%); HS/LD 16 (7.5%) (Table). We found smaller mean ±SD (95%tile) RV EDV/BSA in athletes participating in HS/HD sports (♀: 117±20 (148) and ♂: 133±20 (160)) and LS/HD sports (♀: 109±11 (129) and ♂: 119±15 (142)) and smaller LV EDV/BSA in male athletes in LS/HD sports (116±15 (138)) compared to reference values, (Figure). When using conventional cut-offs in our population we found 10 (4.5%) and 2 (0.9%) athletes above 95%tile for LVEDV/BSA and RVEDV/BSA, respectively (Figure). Conclusion In a cohort consisting of healthy, elite athletes, volumetric adaptations were less outspoken as compared with current reference ranges, specifically in the RV. Only 1 percent of our athletes exceeded conventional 95%tile references for the RV. Potentially, current athlete reference values could overestimate healthy upper limits of cardiac volumes in elite athletes. Our study could indicate that a stricter definition of volumetric reference values, including 95%tile, might be of added value in the specific group of elite athletes to help differentiate between pathology and sports adaptation. Funding Acknowledgement Type of funding sources: Public grant(s) – National budget only. Main funding source(s): Amsterdam Movement Sciences (P1A210AMC2018)

Additional file 2: Figure 1. Weighted mean T2* values and weighted standard deviations (SD) of the sub-analysis in patients with myocardial infarction and iron overload measured at 1.5 T (A) and 3 T (B). In myocardial infarction, T2* values of remote myocardium (r) (grey square), infarct core (c) (black square) and infarct zone (z) (black triangle) are presented. In iron overload, the T2* value of iron overload patients (p) with cardiac involvement is presented. The number of included measurements for each population is reported above the graph. MI myocardial infarction, IO iron overload. Figure 2. Weighted mean T2 values and weighted standard deviations (SD) of the sub-analysis in patients with myocardial infarction, heart transplantation and myocarditis measured at 1.5 T (A) and 3 T (B). In myocardial infarction, T2 values of remote myocardium (r) (grey square), infarct core (c) (black square) and infarct zone (z) (black triangle) are presented. In heart transplantation, T2 values of heart transplant recipients with negative rejection (n) (grey square) and positive rejection (p) (black square) are presented. In myocarditis, T2 values of populations scanned in the non-acute phase (n) (grey square) and in the acute phase (a) (black square) are presented. The number of included subjects for each population is reported above the graph. MI myocardial infarction, Trans heart transplantation, MC myocarditis.

Additional file 1.