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Background Contemporary studies in hypertrophic cardiomyopathy (HCM) suggest that moderate intensity exercise can improve cardiorespiratory fitness without raising significant safety concerns. Although low/moderate intensity exercise may be appropriate for older HCM patients, it is unlikely to attract younger, often asymptomatic patients, who wish to engage in higher intensity regimes. Purpose To assess the feasibility, safety and outcomes of an individually tailored, high intensity exercise programme in young patients with HCM. Methods In this RCT, 80 patients with HCM, aged 16–60 (mean 45.7, [SD8.6]) underwent baseline testing with ECG, echocardiography, blood testing, exercise testing, 48-hour ECG and psychological assessment. Individuals were randomised to a 12-week supervised exercise programme (HRR increased from 70–85%) (n=40) or usual activity (n=40). Baseline investigations were repeated at 12 weeks. Feasibility was assessed by a) recruitment, adherence and retention rates; b) staffing ratios and logistics; c) acceptability of the intervention/educational materials. Safety was assessed as a composite of 1) cardiovascular death, 2) cardiac arrest, 3) device therapy, 4) exercise induced syncope, 5) sustained/non-sustained (NS) ventricular tachycardia (VT) or 6) sustained atrial arrhythmias. Secondary outcomes included health and psychological benefits. Results 67 individuals (82.5%) completed the study. Reasons for refusal included travel, work and family commitments. The majority (64.7%) of exercising individuals progressed to 85%HRR. Resource requirements were similar to other programmes. All individuals felt supported, more confident to exercise, and found educational materials clear and informative. There was no significant difference between groups for the composite safety outcome. One individual experienced exercise induced syncope due to ventricular standstill (exercise) and another sustained VT (control). Both required device implantation. There was no significant difference between groups in episodes of NSVT (p=0.573) or ectopic burden (p=0.729). At 12 weeks, exercise group participants demonstrated greater activity levels (+1.1 hours [CI 0.2–2.1], p=0.024). The change in peak aerobic capacity (+255.2ml/min [CI 93.2–417.1], p=0.003), time to anaerobic threshold (AT) (+115s [CI 54.2–176.0], p Conclusions A high intensity exercise programme is feasible in young patients with HCM, with considerable gains in cardiorespiratory fitness and psychological outcomes. Importantly, arrhythmia burden was not increased in the exercise group. Further research is still required to assess the long-term safety of high intensity exercise in the HCM population. Funding Acknowledgement Type of funding source: Other. Main funding source(s): Cardiac Risk in the Young

Background The diagnosis of arrhythmogenic right ventricular cardiomyopathy (ARVC) is often challenging and some structural abnormalities typical of the disease may overlap with changes reflective of cardiac adaptation to exercise. Purpose The aim of the study was to assess the performance of the cardiac magnetic resonance (CMR) 2010 Task Force Criteria (TFC) in a cohort of patients with definite diagnosis of ARVC and define the overlap with a cohort of highly trained athletes of similar age and sex. Methods We compared the CMR features of 43 patients (mean age 49±17 years, 49% males, 32 (74%) genotyped) with a definite diagnosis of ARVC according to the revised TFC to 97 (mean age 45±16 years, 61% males) highly-trained athletes of similar age and sex, in whom cardiac disease was excluded after comprehensive work-up. Results The CMR was abnormal in 37 (86%) patients. The right ventricle (RV) was affected in isolation in 17 (39%) patients, with 18 (42%) patients exhibiting biventricular involvement and 2 (5%) patients showing isolated left ventricular (LV) involvement. The most common RV abnormalities were regional wall motion abnormalities (RWMA) (n=34; 79%), RV dilatation fulfilling a major or minor volume TFC (n=18; 42%), impaired systolic function (RV ejection fraction (EF) ≤45%: n=17; 40%) and myocardial fibrosis (n=13; 30%). The predominant LV abnormality was myocardial fibrosis (n=20; 47%), with a small proportion of patients exhibiting RWMA (n=6; 14%) and impaired systolic function (LVEF Conclusions The great majority (86%) of patients with ARVC demonstrates structural abnormalities suggestive of cardiomyopathy on CMR but only 53% fulfills any of the CMR TFC. Only a small proportion (16%) of older athletes demonstrate significant RV dilatation that overlaps with the volume criteria for ARVC, in juxtaposition to younger athletes who exhibit a greater degree of overlap. The emergence of ARVC as a biventricular disease provides an opportunity to re-evaluate the diagnostic criteria and include LV involvement in conjunction with RV involvement to improve diagnostic accuracy. Acknowledgement/Funding CRY (Cardiac Risk in the Young) charity

Background Structural cardiac adaptations due to hypertension (HTN) present a diagnostic challenge when differentiating from hypertrophic cardiomyopathy (HCM), using traditional imaging techniques such as echocardiography (echo). Cardiac magnetic resonance imaging (CMR) offers reproducible anatomical, functional quantification and myocardial tissue characterisation which discriminates between hypertension and HCM. Purpose To identify hypertensive individuals with undiagnosed HCM using CMR imaging. Methods 100 consecutive hypertensive patients underwent CMR at a tertiary centre dedicated blood pressure clinic (55% male, mean age 51 years). In keeping with ESC guidelines, end diastolic wall thickness (EDWT) ≥15mm identified individuals within the “grey zone” between hypertension and with a potential HCM diagnosis. 19 individuals were referred on to the dedicated inherited cardiac conditions clinic for further evaluation. Four patients expressed a definitive LV phenotype and were diagnosed with HCM. CMR parameters were compared in three groups: Hypertensive (HTN), “grey zone” Hypertensive (GZH) and HCM. Results CMR demonstrated end diastolic wall thickness (EDWT) >11 mm in 50% of hypertensives. 73% of the referred patients were Afro-Caribbean (AC) and all 4 HCM patients were AC. All referrals demonstrated EDWTs ≥14mm, 9 (47%) demonstrated late gadolinium enhancement of which 3 (16%) had HCM. Three had asymmetrical septal hypertrophy – 2 were in the HCM cohort and one underwent endomyocardial biopsy confirming HTN. Left ventricular mass index (LVMI) was significantly higher in GZH compared to HTN (p Table 1. P-values for CMR data in hypertensive (HTN), gray zone hypertensive (GZH) and hypertrophic cardiomyopathy (HCM) cohorts CMR Parameter HCM vs. HTN GZH vs. HTN GZH vs. HCM EDVI NS p=0.025 NS EDWT p=0.0002 p Conclusion This study reports a 4% prevalence of HCM among hypertensive patients - 20 x greater than in the general population - which would be left undiagnosed using echo alone. Screening hypertensive individuals with CMR is not routine but we advocate its use in these individuals especially in Afro Caribbeans and in those in the “grey zone”, to identify undiagnosed HCM, which has significant implications for lifestyle modification and family screening. Acknowledgement/Funding Cardiac Risk in the Young

Aims Anterior T-wave inversion (TWI) is a recognized variant in athletes of African/Afro Caribbean origin and some endurance athletes; however, the presence of this specific repolarization anomaly also raises the possibility of cardiomyopathy. The differentiation between physiological adaptation and cardiomyopathy may be facilitated by examining other repolarization parameters, notably the J-point and the ST-segment. Methods and results We compared the electrocardiogram pattern of anterior TWI in a series of 80 healthy athletes (median age 21 years, 75% males); 95 patients with hypertrophic cardiomyopathy (HCM) (median age 46 years, 75% males), including 26 affected athletes; and 58 patients with arrhythmogenic right ventricular cardiomyopathy (ARVC) (median age 32 years, 71% males), including 9 affected athletes. Athletes and patients were of either white/Caucasian or black/Afro Caribbean descent and showed TWI ≥1 mm in ≥2 contiguous anterior leads (V1–V4). We aimed to identify repolarization patterns for differentiating physiologic from pathologic TWI. After adjustment for age, gender, and ethnicity, J-point elevation