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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 To compare an echocardiographic method for detecting abnormal cardiac function before development of overt cardiomyopathy with a recently validated technique of quantifying myocardial iron load. Methods and results We examined thalassaemia patients whose myocardial iron load had been evaluated with magnetic resonance imaging (MRI). By tissue Doppler echocardiography, myocardial velocities were sampled continuously from base to apex in the RV and LV free wall, and the septum in 52 patients aged 29.2 (14.2–43.1) years and 52 age-matched controls. Ninety-six percent of patients had normal LV ejection fraction by MRI. Thirty-eight (73%) had abnormal iron loading of the myocardium, and 33 of those had regional wall motion abnormalities detected in the septum (n=29), LV (n=2), RV (n=1), and septum plus LV (n=1). The incidence of wall motion abnormalities was significantly higher ( P

Aims To develop and validate a non-invasive method for measuring myocardial iron in order to allow diagnosis and treatment before overt cardiomyopathy and failure develops.Methods and Results We have developed a new magnetic resonance T2-star (T2*) technique for the measurement of tissue iron, with validation to chemical estimation of iron in patients undergoing liver biopsy. To assess the clinical value of this technique, we subsequently correlated myocardial iron measured by this T2* technique with ventricular function in 106 patients with thalassaemia major. There was a significant, curvilinear, inverse correlation between iron concentration by biopsy and liver T2* (r=0(.)93, P

Purpose: 4% of African Americans possess the transthyretin (TTR) V122I polymorphism, which is associated with late onset cardiac amyloidosis. However, the incidence of clinically significant disease is not known and the diagnosis is readily missed due to clues on echocardiography being attributed to hypertensive heart disease, along with the misconception that the ECG always demonstrates low voltage complexes in amyloidosis. We previously demonstrated that 10% of Afro-Caribbean patients attending a heart failure clinic in South London have ATTR V122I cardiac amyloidosis, and we report here a simple diagnostic algorithm that we applied to an Afro-Caribbean patient population at another large tertiary cardiac centre in London. Methods: We prospectively reviewed the echocardiograms of Afro-Caribbean patients with non-ischaemic cardiomyopathy over a 6 month period. Patients with increased ventricular wall thickness (>12 mm) and no clinical diagnosis of hypertrophic cardiomyopathy were referred for cardiovascular magnetic resonance (CMR) and 99mTc-DPD scintigraphy. Patients in whom cardiac imaging suggested amyloidosis were further investigated at the National Amyloidosis Centre. Results: 49 Afro-Caribbean patients presented during the screening period, 11 of whom (22%) met the criteria for further investigation (median age 67 (55-77); 72% males). DPD scintigraphy was performed in all 11 patients and CMR in the 6 patients without a contraindication. A diagnosis of amyloidosis was made in 3 patients (27% of those referred for specialist review and 6% of the total number screened), two of whom had the TTR V122I variant and one had wild-type ATTR amyloidosis. Conclusions: Cardiac amyloidosis is an important cause of heart failure in the elderly Afro-Caribbean population and the diagnosis should be considered when echocardiography suggests "LVH". DPD and CMR imaging are valuable diagnostic tools. Several specific therapies for ATTR amyloidosis are currently in clinical development, underscoring the need for accurate diagnosis.

May we draw your attention to the ESC/AHA/ACC scientific statement published in the December issue of the European Heart Journal .1 Scenario 10 discusses the indication for endomyocardial biopsy in patients with suspected myocardial siderosis (due to hereditary or acquired haemochromatosis). This states that ‘cardiac involvement …