Abstract Funding Acknowledgements Type of funding sources: None. Background The apical variant of hypertrophic cardiomyopathy (ApHCM) has male predominance and is a relatively rare phenotype in Western population. Characteristics of female and black patients diagnosed with ApHCM are very limited in the existing literature. Purpose We aimed to investigate whether gender and race are associated with a different clinical presentation and CMR findings in apical HCM. Methods We retrospectively analysed 150 patients (113 males and 37 females) with a diagnosis of apical HCM who have been followed in our inherited cardiac conditions (ICC) clinic between 2010 and 2020. Only patients with a CMR study and apical hypertrophy defined as ≥ 13mm at the time of diagnosis were included. Demographics and clinical characteristics were obtained from electronic records. Volumetric CMR data were taken from confirmed reports while other parameters were measured by standard protocol. "Pure" ApHCM was defined as isolated apical hypertrophy and "mixed" with both apical and septal hypertrophy but with the apex thickest (1). Apical displacement of papillary muscles (PM) was defined when the base of PM originated from the apical one-third of the left ventricle (LV) in the apical 4- or 2-chamber views. Giant T-wave inversion was defined as T-wave inversion that is equal or greater than 10 mm (1 mV) in any electrocardiogram lead. Results Our study population included patients of White (55, 37%), Black (37, 25%), Asian (36, 24%) and Mixed/Others (22, 15%) ethnicity. Black patients were more likely to have a diagnosis of hypertension at presentation when compared to White (70% vs 40%, p = 0.01) and to Asian and Mixed/Other patients (70% vs 48%, p = 0.03). Similarly, they were more likely to have "mixed" ApHCM than White (49% vs 20%, p = 0.003) and Asian and Mixed/Other (49% vs 26%, p = 0.02) patients. Females were diagnosed at an older age (63 ± 12 vs 52 ± 14, p < 0.001) and were less likely to have deep T-wave inversion on their ECG at presentation (14% vs 32%, p = 0.03) compared to their male counterparts. Females in this cohort also had higher representation of black ethnicity and were more likely to have hypertension (68% vs 47%, p = 0.03). Apart from the expected gender related differences in volumes and LV mass, there were no differences in cardiomyopathy-specific parameters we investigated. Conclusions In our cohort, females with ApHCM presented at an older age and were less likely to have giant T-wave inversion on ECG. Black patients with ApHCM were more likely to have hypertension and the "mixed" type of the disease.

Background: Hypertrophic cardiomyopathy (HCM) has a variable phenotypic expression.Numerous studies have noted that individuals of African/Afro-Caribbean origin (black) and Caucasians (white) demonstrate marked differences on ECG and echocardiography.However, there is a paucity of literature on cardiac MRI (CMR) findings of ethnic differences in HCM.Purpose: Assess if phenotypic differences on CMR exist between black and white individuals with HCM.Methods: Patients with HCM were recruited from a dedicated inherited cardiac conditions clinic if they were NYHA class 1, had a blood pressure ≤140/90mmHg, no evidence of LV outflow tract obstruction and were not known to have ischemic heart disease (n = 119, males = 99, white = 76, aged 16-65 years).Sequences were performed on a 3T Phillips scanner, using standard techniques and analysed using semi-automated software (MRI extended workspace 2.6.3.3,Phillips and Circle, Cardiovascular Imaging Inc, release 5.6.1,Calgary, Canada).Native T1 was calculated using ShMOLLI sequences for the basal, mid and apical septal segments and the mid-ventricular short axis slice.Results: Comparison of left ventricular (LV) mass between ethnicities demonstrated higher LV mass in black compared to white HCM patients (p < 0.001, absolute values and normalised for body surface area).Mean LV mass (normalised) for black and white HCM patients was 91.21g/m² and 74.29g/m² respectively.Strain analysis demonstrated a 7% lower peak global radial strain percentage and a 3% lower peak global circumferential strain percentage in black HCM patients compared to white HCM patients (p = 0.010, p = 0.001 respectively).Black HCM patients demonstrated less late gadolinium enhancement (LGE) than white HCM patients by mass (grams) and by percentage of myocardium (auto-threshold using 6 standard deviations) (p = 0.005, p < 0.001 respectively).Analysis of native T1 demonstrated that black HCM patients had a lower native T1 in the basal septum compared to white HCM patients (p = 0.006).There were no statistically significant ethnic differences in maximal LV wall thickness, LV end diastolic volume, left atrial area, LV stroke volume, LV ejection fraction, or peak global longitudinal strain percentage. Conclusion:This is one of the first CMR studies assessing for ethnic differences in tissue characterisation in HCM between black and white patients.Findings from this study found black HCM patients had greater LV mass, reduced peak global radial and circumferential strain percentage, lower quantity of LGE, and lower native T1 values compared to white HCM patients.

INTRODUCTION:Cardiovascular manifestations are common in Marfan syndrome (MFS) patients.The presence of cardiomyopathy without significant valve disease or previous aortic or cardiac surgery has been identified before.Our aim was to investigate the prevalence of Left ventricular disfunction in a selected sub cohort of our MFS patients using cardiac magnetic resonance (CMR). METHODS:We retrospectively evaluated 90 consecutive patients seen in the Aortopathy clinic who had a CMR.Patient with significant valve dysfunction, previous cardiac or aortic surgery and age ≤17 years old were excluded.49 MFS patients who met the Ghent criteria were included.Using CMR Left ventricular (LV) volumes, ejection fraction (EF), stroke volume (SV) and mass were assessed with the standard protocols as well aortic dimensions, presence of valve disease and Late Gadolinium Enhancement (LGE).LV dysfunction was defined as left ventricular ejection fraction ≤56%.RESULTS: 49 patients were studied, 27 male (55.1%) with a mean population age of 39.9 ± 14 years old.The LV end-diastolic volume (LVEDV) mean was 154.1 ± 36.3 ml with a LVEDV indexed of 78 ± 15.7 ml/m2, LVESV 58.8 ± 18.6 ml, LVESV index 48.5 ± 10 ml/m2, LVEF was 62.8 ± 6.9%, SV 95.77 ± 22.3 ml, SV index 48.5 ± 10 ml/m2, and aortic root diameter was 40 ± 4.7 mm.LV dysfunction was present in 6 patients (12.2%), which was associated with lower SV and SV index compared with 43 patients (87%) with normal LVEF (SV 71.2 ± 15.5 ml vs 99.3 ± 21.1 ml (p = 0.003); SV index 36.7 ± 10.3 ml/m2 vs 50.3 ± 8.7 ml/m2 (p = 0.001)).There were no differences found in gender, age, LV or atrial volumes or AoR dimension between the 2 groups.11 Patients (22.4%) had LVEDV indexed ≥75th percentile (≥182 ml/m2).None of them had LV dysfunction (fig1).Comparing patients with LVEDV indexed ≥75th percentile to those with <75th percentile the SV was 126.8 ± 10.2ml vs 86.5 ± 15.6ml (p < 0.001) and SV indexed was 58.3 ± 6.9 vs 45.46 ± 8.9 ml/m2 (p < 0.001) respectively. CONCLUSION:In our subgroup of MFS with mildly dilated AoR the presence of Left ventricular dysfunction was 12.2%.This was associated with lower SV and SV index.None of the patients with LVEDV indexed ≥75th percentile (≥182 ml/m2) had LVEF dysfunction.No myocardial enhancement was seen in patients who had LGE study.

Abstract 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 <50%: n=6; 14%). Twenty-three (53%) patients fulfilled a major (n=14; 32%) or a minor (n=9; 21%) CMR TFC. Sixteen (16%) athletes exceeded the cut-off values for RV volumes used as a major (n=10; 10%) or a minor (n=6; 6%) TFC with an inverse relationship between age and RV volumes (r=−41, p=0.001). None of the athletes fulfilled the TFC for RV ejection fraction. Applying the CMR TFC to the ARVC patient population showed a sensitivity of 33% for the major and 28% for the minor criteria. Applying the RV volume and systolic function TFC values in the entire study population showed a sensitivity of 53%, a specificity between 83% and an accuracy of 0.68. 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 Primary percutaneous coronary intervention (PCI) is the preferred reperfusion strategy in patients with ST-segment elevation myocardial infarction (STEMI). We evaluated whether direct transfer to a cardiac centre performing primary percutaneous coronary intervention (PPCI) leads to improved survival compared with transfer via a non-PPCI performing hospital in STEMI patients in a regional network. Methods This was an observational cohort study of 10 108 patients with STEMI treated with PPCI between 2004 and 2011 at eight tertiary cardiac centres across London, UK. Patient9s details were recorded at the time of the procedure into the British Cardiac Intervention Society (BCIS) database. Outcome was assessed by all-cause mortality. Anonymous datasets from the eight centres were merged for analysis. The primary end-point was all-cause mortality at a median follow-up of 3.0 years (IQR range 1.2–4.6 years). Results 6492 patients (64.2%) were transferred directly to a PCI performing centre (direct) and 3616, (35.8%) were transferred via a non-PCI performing centre (indirect). There were higher rates of previous MI and previous CABG in the indirect group, with higher rates of poor LV function in the direct group (table 1). Median time to reperfusion (symptom to balloon) in transferred patients was 58 min longer compared to patients admitted directly (p<0.001). However, symptom to first hospital door times were similar. Transferred patients had significantly lower rates of infarct-related artery (IRA) TIMI 0 flow (54.5% vs 62.9%, p<0.0001) and higher rates of IRA TIMI 3 flow (17% vs 10.7%, p>0.0001) at presentation compared to those transferred directly. Kaplan-Meier analysis demonstrated no significant difference in mortality rates between patients with and without transfer (12.3% direct vs 14.3% indirect, p=0.060). Age-adjusted Cox analysis revealed inter-hospital transfer for PPCI was associated with all cause mortality (HR 0.89 (95% CI 0.79 to 0.99)), however this was not maintained after multivariate adjustment (HR 0.84 (95% CI 0.62 to 1.14)). Conclusions In this large registry survival appear comparable in patients with STEMI admitted directly versus transferred for primary PCI. This is despite longer symptom to balloon times. This unexpected finding may reflect the earlier initiation of medical therapy (eg, anti-platelets and GpIIb/IIIa receptor inhibitors) and earlier pharmacological reperfusion, reflected by lower IRA TIMI 0 rates at angiography in the patients transferred from a non-PCI hospital.

Background Limited information exists regarding procedural success and clinical outcomes of ST-segment elevation myocardial infarction (STEMI) in patients with previous CABG undergoing primary PCI. We sought to compare outcomes in STEMI patients undergoing primary percutaneous coronary intervention (PCI) with or without previous coronary artery bypass grafts (CABG). Methods This was an observational cohort study of 10,920 patients with STEMI who were treated with PPCI between 2004 and 2011 at eight tertiary cardiac centres across London, UK. Patient's details were recorded at the time of the procedure into the British Cardiac Intervention Society (BCIS) database. Outcome was assessed by all-cause mortality. Anonymous datasets from the eight centres were merged for analysis. The primary end-point was all-cause mortality at a mean follow-up of 3.0 years. Results 347 (3.2%) patients had previous CABG. Patients with previous CABG were older and had more associated comorbidities than patients who have never had CABG. In patients with previous CABG, the infarct related artery (IRA) was split evenly between a bypass graft and a native vessel. Procedural success (defined as TIMI 3 flow at the end of procedure) was less likely in patients with previous CABG than in patients who had never undergone CABG (80.7 vs 88.2% respectively, p<0.001). Patients with previous CABG had higher all-cause mortality (30.1% vs 16.7%, p<=0.0001) during the follow-up period (figure 1). After multivariate adjustment this difference persisted (HR: 1.3, 95% CI 1.11 to 1.63, p=0.02). When stratifying prior CABG patients by the type of IRA (figure 2); long term MACE were significant more likely in patients who had bypass graft PCI than in patients that had native vessel PCI, 35.7% versus 20.4% (p=0.03). Figure 1 Figure 2 Conclusions Previous CABG patients with STEMI treated with primary PCI have higher long-term adverse events. The long-term outcome is also worse if the IRA is a bypass graft rather than a native coronary artery.

Introduction The use of primary percutaneous coronary intervention (PCI) in octogenarians to treat ST elevation myocardial infarction (STEMI) is less than in other age groups. This is due in part to underrepresentation in clinical trials and perceived increased risk. We present long-term survival of a large cohort of elderly patients following primary PCI in London. Table 1 Under 80 Over 80 p Value Gender (female) 1800 (19.6%) 474 (45.4%) <0.0001 Hypertension 3692 (42.3%) 501 (51.3%) 0.02 Hypercholesterolaemia 3708 (42.5%) 548 (56.1%) <0.0001 Previous MI 1442 (16.9%) 182 (18.7%) 0.150 Previous CABG 264 (3.0%) 46 (4.6%) 0.010 Multi vessel disease 3821 (41.8%) 562 (54.0%) <0.0001 GPIIb/IIIa 6515 (74.4%) 530 (53.8%) <0.0001 DES use 4058 (45.9%) 311 (30.9%) <0.0001 Access (radial) 2115 (23.4%) 194 (18.8%) 0.001 Procedural success 6932 (88.3%) 736 (84.7%) 0.003 Methods This was an observational study of 10 249 consecutive patients undergoing primary PCI for STEMI at eight London heart attack centres between January 2005 and November 2011. Patient's details were recorded at the time of procedure into the British Cardiac Intervention Society (BCIS) database. Outcome was assessed by all-cause mortality. Anonymous datasets from the eight centres were merged for analysis. The primary end-point was all-cause mortality at a median follow-up of 3.0 years (IQR range 1.2–4.6 years). Results A total of 1051 octogenarians (10.3% of the study population) with an average age of 84.2 years (IQR 80–101) were treated with primary PCI during the study period. Over time, the annual proportion of octogenarians gradually increased from 9.1% in 2005 to 10.5% in 2010. Unsurprisingly, when compared to patients under 80, octogenarian STEMI patients included a higher proportion of women, and had a higher prevalence of hypertension, hypercholesterolaemia, multi-vessel disease, previous infarction and previous CABG (table 1). They additionally were less likely to undergo radial access, receive GPIIb/IIIa inhibitors or a drug-eluting stent. When compared with younger patients, primary PCI in octogenarians was less likely to achieve TIMI flow grade 3. However between 2005 and 2011 the rates of post-procedural TIMI flow grade 3 increased significantly from 80.5% in 2005 to 90% in 2011 (p for trend 0.05). The cumulative incidence of all-cause mortality during follow-up was significantly higher in the octogenarian group compared to the younger subgroup (51.6% vs 12.8%, p<0.0001) (figure 1). As expected, the hazard of death during follow-up increased with age (unadjusted HR 1.069 per year increase (95% CI 1.064 to 1.074), p<0.0001), which persisted after adjustment for other predictors of mortality (HR of 1.059 (95% CI 1.048 to 1.071), p<0.0001). Figure 1 Conclusions Octogenarians constitute an important subgroup of STEMI patients. Data from London's experience would suggest that primary PCI rates are increasing in this group and that despite the high long term mortality, acute/year one rates survival rates are very encouraging.

Introduction In March 2010 NICE published guidelines for assessment of patients with chest pain of recent onset based on their pre-test likelihood (PL) for coronary artery disease (CAD). A novel aspect was the use of a CT calcium score (CaS) and CT coronary angiography (CTCA) to exclude CAD in low risk patients. Here we report our experience of integrating this approach to our Rapid Access Chest Pain Clinic (RACPC) over a 32 month period. Methods Data was retrospectively analysed from consecutive patients attending RACPC, referred for CT. Both CaS and CTCA were performed by default by our radiology department, regardless of CaS, using older 64-multislice and newer generation 128-multislice CT scanners (the latter capable of flash acquisition and prospective gating). The radiation dose (RD) in milli-Sieverts (mSv) received by each patient was calculated from the dose-length product. Patients with significant CAD on CTCA (at least 1 >50% lesion) were referred for invasive angiography (XA). Results 156 patients were included (Males n=68, females n=88; mean age 52.2±8.7). The median RD received was 4.45 mSv (0.48–20.59); patients scanned in the newer generation CT scanner received significantly less RD than those in the older scanner (median 2.54 (0.48–8.75) vs 11.55(8.07–20.59) mSv respectively, p<0.001). Ninety-six patients had a CaS=0 (male=32, female=64, mean age 50±9 years); of these, 5 (5.5%; mean age 47.7±14.7 years) had significant CAD of which 3 underwent intervention on prognostic grounds. Those with a CaS >1 were significantly older (mean age 55±7 years, p<0.001; male=36, female=24) with a higher proportion of significant CAD (36.7%, n=22;). Of those who NICE would have recommended had XA as their first line investigation (ie, PL 60–90%; n=26), 50% (n=13) had no pathology. Of those with PL of >90%, who NICE would have recommended to treat as stable angina without investigation (n=8), 3 had prognostic disease requiring either percutaneous or surgical revascularisation. Conclusions A CaS of 0 in symptomatic patients does not rule out significant and prognostic CAD, particularly in younger patients. Prospective gating with 128-multislice CT scanners in patients with optimum heart rate and rhythm significantly reduces the RD. Given that 50% of our patients recommended by the NICE guidelines to have XA as their first line investigation had no significant disease on CTCA, it is reasonable to consider CaS and CTCA as a first line investigation in selected higher risk patients. Our findings in the highest risk patients (PL CAD>90%), who NICE recommend treating medically as stable angina, suggest that this group may benefit from investigation with XA being the most appropriate choice.

Introduction During Primary Percutaneous Coronary Intervention (PPCI) post ST-Segment Myocardial Infarction (STEMI), distal embolisation of thrombus may lead to failure to re-establish normal flow in the infarct-related artery. Manual thrombus aspiration has been shown to improve coronary perfusion as assessed by time to ST-segment resolution and myocardial blush grade. Evidence supporting the benefit of thrombus aspiration on clinical outcomes, however, is limited and inconsistent. We aimed to assess the impact of manual thrombectomy on mortality in patients presenting with STEMI across all PPCI centres in London over a 5 year period from 2007 until 2012. Methods This was an observational cohort study of 9935 consecutive patients with STEMI treated with PPCI between 2007 and 2012 at eight tertiary cardiac centres across London, UK. Patient9s details were recorded at the time of the procedure into the British Cardiac Intervention Society (BCIS) database. Outcome was assessed by all-cause mortality. Anonymous datasets from the eight centres were merged for analysis. The primary end-point was all-cause mortality at a median follow-up of 2.0 years (IQR range 1.1–3.1 years). Results Of the 9935 consecutive STEMI patients presenting for PPCI, 2859 had mechanical thrombectomy. Patients who had manual thrombectomy were significantly younger (average age 60.6 vs 62.9) and were less likely to have had a previous myocardial infarction (11.9% of thrombectomy patients vs 14.7% of non-thrombectomy patients). Patients receiving manual thromectomy were found to be significantly more likely to have had PPCI via a radial approach (33.1% in thrombectomy patients vs 19.9% in non-thromectomy patients). Procedural success (defined as TIMI 3 flow at the end of procedure) was found to be significantly more likely in patients receiving manual thrombectomy (89.5% vs 86.7%) (table 1). Patients with thrombectomy use had similar unadjusted all-cause mortality rates to those without thrombectomy use (12.7% vs 16.5%, p=NS) during the 5-year follow-up period (figure 1). After multivariable adjustment thrombectomy use was associated with significantly decreased mortality rates (HR: 0.82, 95% CI 0.68 to 0.9, p=0.04). Conclusion Mechanical thrombectomy use appears to be associated with improved outcome, in the form of decreased mortality, in this large observational trial.

Patent foramen ovale and atrial septal defects are common cardiac defects that can be routinely closed with transcatheter devices, rather than more invasive open surgical techniques. There is good evidence supporting both surgical and transcatheter closure of atrial septal defects, but there remains controversy regarding some of the currently applied indications for patent foramen ovale closure. Both procedures require appropriate diagnostic preprocedural and intraprocedural imaging for optimal closure results, and while contrast enhanced transcranial doppler, intracardiac echocardiography and cardiac MRI are increasingly utilized, most would still consider transesophageal echocardiography to be the gold standard of imaging for these patients. There are many septal closure devices available and careful consideration should be given to the patients septal and defect anatomy before selecting the most appropriate device for closure. With a few anatomical exceptions, the Amplatzer® closure devices remain the ...

Cardiac resynchronisation therapy (CRT) is a recognised treatment for symptomatic left ventricular (LV) failure associated with a broad QRS.It has been suggested that >15% myocardial scarring predicted failure to respond to CRT. To determine if the scar burden is a major determinant in response to CRT, we prospectively studied 50 pts (66± 1 years, ischaemic aetiology 57%) undergoing CRT for standard indications at baseline (NYHA: 3±0.4, EF: 23±0.8%, QRS: 150±7 ms, BNP: 495±461 pmol/l) and 6 months post-CRT. Clinical response was defined by a reduction in NYHA class >1 and in BNP level >30%. Reverse LV remodelling was defined as a reduction in end-systolic volume ≥10%. All patients had an echocardiographic examination, including an assessment of dyssynchrony. Additionally, a Cardiac Magnetic Resonance (CMR) study, with delayed enhancement, was performed in patients without contraindications. Of the 30 patients that had CMR, 23 pts (77%) responded clinically to CRT (reduction in NYHA: 1.6±0.6, reduction in BNP: 34±0.4%) and 19/30 (63%) additionally displayed reverse LV remodelling. Among the clinical responders 8 pts (35% of responders) were found to have extensive full thickness myocardial scarring on CMR (in 7 ±1 segments), predominantly in the anteroseptum and apex (86%). Three of these patients also showed significant echocardiographic reverse remodelling. The mechanism of response to CRT in these patients with extensive infarction was more frequently interventricular resynchronisation (43%) or atrio-ventricular resynchronisation (43%) rather than intra-ventricular resynchronisation (14%).The previously suggested 12 segment dyssynchrony index (DI) would have identified none of these responders with extensive myocardial infarction. The DI identified only 7/23 responders (30%). Conclusion : Even patients with extensive myocardial scar are potential responders to CRT and should not be excluded if they fulfil the standard AHA/ESC criteria for biventricular pacing. Intraventricular resynchronisation is not the only mechanism by which patients respond to CRT and measures of intraventricular dyssynchrony alone are inadequate for identifying potential response