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To identify biomarkers of cardiomyopathy in patients with type 2 diabetes mellitus (T2DM) using cardiovascular magnetic resonance (CMR) and to identify associations between functional status, metabolomic profile and myocardial fibrosis.In this prospective case control study, patients (n = 49) with T2DM without significant coronary artery disease, and matched controls (n = 18) underwent CMR, cardiopulmonary exercise testing, and plasma metabolomic analyses.Patients with T2DM (n = 49, median [interquartile range] age 61 [56-63] years, 61% male, diabetes duration 11 [7-20] years), historical HbA1c 7.6% (60 mmol/mol) (6.9-8.6) and matched controls (n = 18) were examined. Study patients had increased myocardial extracellular volume (ECV) (26.9 [23.8-30.0] vs 23.4 [22.4-25.5) %, p 0.001). Increased ECV was associated with male sex (p = 0.04), time with T2DM (p = 0.02), reduced peak VOPatients with well-controlled T2DM without significant coronary disease exhibit focal and diffuse myocardial fibrosis and diffuse myocardial fibrosis is associated with reduced exercise tolerance and metabolites. Plasma metabolites may provide mechanistic insights into diffuse myocardial fibrosis, and cardiopulmonary fitness.

Athletes sometimes experience transient arrhythmias during intense exercise, which may be difficult to capture with traditional Holter monitors. New and highly portable technology, such as smartphone electrocardiogram (ECG) devices, may be useful in documenting and contribute to diagnosis of exercise-induced arrhythmias. There are little data available regarding the new Kardia 6 lead device (6L) and no data regarding its use in athletic populations. In this short communication, we present pilot data from 30 healthy athletes who underwent a 12‑lead ECG and subsequent 6L reading. Our pilot data show relatively high levels of agreement for QTc and PR interval and QRS duration, with the 6L readings slightly but significantly shorter on average.

Sudden cardiac death (SCD) is a catastrophic complication of many cardiac conditions often occurring without warning. In these cases, a post-mortem examination is required to elucidate the cause of death and is regarded as the 'gold standard'. However, in circumstances of certain religious/cultural beliefs and advanced body decomposition an alternative non-invasive approach would be preferred. Although a developing field, post-mortem imaging using computed tomography (pmCT) or magnetic resonance imaging (pmMR) provides a non-invasive and accurate alternative to traditional post-mortem in specific circumstances. In particular, pmMR has an important role in younger decedents while pmCT is more suited to examination of adults with SCD. Despite encouraging results from several preliminary studies, more research is needed to determine the most appropriate role for post-mortem imaging in the clinical algorhythm for investigation of SCD.

Background Autopsy reports suggest that cardiac sarcoidosis occurs in 20 to 25% of patients with pulmonary sarcoidosis, yet the clinical ante-mortem diagnosis is made in only 5% of cases. Current diagnostic algorithms are complex and lack sensitivity. Cardiac Magnetic Resonance imaging (CMR) provides an opportunity to detect myocardial involvement in sarcoidosis. The aim of this study is to determine the prevalence and clinical significance of late gadolinium enhancement (LGE) on CMR in patients with sarcoidosis. Methods Consecutive patients with biopsy-proven sarcoidosis undergoing CMR were retrospectively evaluated for cardiac sarcoidosis. Medical records were correlated with CMR. Results Forty-six patients were evaluated. Late gadolinium enhancement was present in 22%, indicating myocardial involvement, and 70% had corresponding hyper-intense T2 signal indicating active inflammation. Late gadolinium enhancement was 18% +/− 9.7% of overall left ventricular (LV) mass and most commonly located in the basal to mid septum. There was no association between LGE and cardiovascular symptoms or pulmonary stage. Eighty per cent of patients with LGE did not fulfill conventional diagnostic criteria for cardiac sarcoidosis. However, LGE was associated with clinically significant arrhythmia (p Conclusions Using CMR, we identified a higher prevalence of cardiac sarcoidosis than previously reported clinical studies, a prevalence which is more consistent with autopsy data. The presence of LGE was highly correlated with clinically significant arrhythmias and lower LVEF.

Background Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited disorder of cardiac desmosomes associated with ventricular arrhythmias and sudden cardiac death. The clinical diagnosis is problematic and relies on a complex criteria composed of clinical and non-clinical parameters. In 2010, the original 1994 Task Force Criteria (TFC) was revised with particular attention given to the imaging parameters. Methods Five retrospective studies compared the diagnostic concordance between the 1994 and 2010 TFC. Three studies used cardiac magnetic resonance (CMR) and compared major and minor CMR criteria and three studies compared definite ARVC cases; one study assessed both. Results Three studies with 1435 patients compared the 1994 imaging and the 2010 CMR criteria. Using the 1994 criteria, 123 (8.6%) and 419 (29.2%) patients satisfied major and minor criteria compared to only 52 (3.6%) and 28 (1.9%) using the 2010 criteria; 57.7% and 94.3% reduction in major and minor criteria ( p value, 0.0001 and 0.0001). Three studies with 611 patients assessed for definite ARVC. Using the 1994 and 2010 criteria, 207 (33.9%) and 243 (39.8%) patients satisfied the parameters respectively. This resulted in a statistically significant 17.4% ( p value, 0.0379) increase in ARVC cases driven largely by two sub-groups. Conclusions The 2010 revised TFC have resulted in a significant reduction in the number of patients that satisfy CMR criteria particularly those that satisfied minor imaging abnormalities using the 1994 criteria. In addition, in certain groups the revised criteria have significantly increased the number of patients diagnosed with definite ARVC.

The autopsy (internal post mortem examination) is considered the gold standard for identifying the cause of sudden unexpected death. Despite its proven capabilities, the referral rate for conventional autopsy has declined over recent years. As a result, there is an emerging role for an alternative

Background The Freestyle stentless bioprosthesis (FSB) has been demonstrated to be a durable prosthesis in the aortic position. We present data following Freestyle implantation for up to 10 years post-operatively and compare this with previously published results. Methods A retrospective cohort analysis of 237 patients following FSB implantation occurred at five Australian hospitals. Follow-up data included clinical and echocardiographic outcomes. Results The cohort was 81.4% male with age 63.2±13.0 years and was followed for a mean of 2.4±2.3 years (range 0-10.9 years, total 569 patient-years). The FSB was implanted as a full aortic root replacement in 87.8% patients. The 30-day all cause mortality was 4.2% (2.0% for elective surgery). Cumulative survival at one, five and 10 years was 91.7±1.9%, 82.8±3.8% and 56.5±10.5%, respectively. Freedom from re-intervention at one, five and 10 years was 99.5±0.5%, 91.6±3.7% and 72.3±10.5%, respectively. At latest echocardiographic review (mean 2.3±2.1 years post-operatively), 92.6% had trivial or no aortic regurgitation. Predictors of post-operative mortality included active endocarditis, acute aortic dissection and peripheral vascular disease. Conclusions We report acceptable short and long term outcomes following FSB implantation in a cohort of comparatively younger patients with thoracic aortic disease. The durability of this bioprosthesis in the younger population remains to be confirmed.

Kearns-Sayre syndrome (KSS) is a mitochondrial disorder characterised by onset before the age of 20years, progressive external ophthalmoplegia, and pigmentary retinopathy, accompanied by either cardiac conduction defects, elevated cerebrospinal fluid protein or cerebellar ataxia. 50% of patients with KSS develop cardiac complications. The most common cardiac manifestation is conduction disease which may progress to complete atrioventricular block or bradycardia-related polymorphic ventricular tachycardia (PMVT). The management of cardiac electrical disease associated with KSS and mitochondrial cytopathy is systematically reviewed including the case of a 23year-old female patient with KSS who developed a constellation of cardiac arrhythmias including rapidly progressive conduction system disease and monomorphic ventricular tachycardia with myocardial scarring. The emerging role of cardiac magnetic resonance imaging (CMR) in detecting subclinical cardiac involvement is also highlighted. This review illustrates the need for cardiologists to be informed about this rare but emerging condition.

Background The mechanisms whereby cardiac output is augmented with exercise in adult repaired tetralogy of Fallot (TOF) are poorly characterised. Methods 16 repaired TOF patients (25±7years of age) and 8 age and sex matched controls (25±4years of age) underwent cardiopulmonary exercise testing and then real-time cardiac MRI (1.5T) at rest and whilst exercising within the scanner, aiming for 30% heart rate reserve (Level 1) and 60% heart rate reserve (Level 2), using a custom-built MRI compatible foot pedal device. Results At rest, TOF patients had severely dilated RVs (indexed RV end-diastolic volume: 149±37mL/m 2 ), moderate-severe PR (regurgitant fraction 35±12%), normal RV fractional area change (FAC) (52±7%) and very mildly impaired exercise capacity (83±15% of predicted maximal work rate). Heart rate and RV FAC increased significantly in TOF patients (75±10 vs 123±17 beats per minute, p p =0.025), and similarly in control subjects (70±11 vs 127±12 beats per minute, p p =0.003), when rest was compared to Level 2. PR fraction decreased significantly but only modestly, from rest to Level 2 in TOF patients (37±15 to 31±15%, p =0.002). Pulmonary artery net forward flow was maintained and did not significantly increase from rest to Level 2 in TOF patients (70±19 vs 69±12mL/beat, p =0.854) or controls (93±9 vs 95±21mL/beat, p =0.648). Conclusions During exercise in repaired TOF subjects with dilated RV and free PR, increased total RV output per minute was facilitated by an increase in heart rate, an increase in RV FAC and a decrease in PR fraction.

article Background: The time course of progressive dilatation of the right ventricle (RV) in adults with pulmonary regurgitation (PR) late after repair of tetralogy of Fallot (TOF) is poorly characterized. Methods: We analysed cardiac MRI data (1.5 T) from 14 adult repaired TOF patients (26 ± 11 years of age) with dilated RVs and known significant PR, on 2 separate visits with a between MRI period of 2.1 ± 1.0 years. Results: Indexed RV end diastolic volume (RVEDVi) increased over 2 years (142 ± 19 to 151 ± 20 mL/m 2 , p = 0.005; change = 8.4 ± 9.3 mL/m 2 ,r ange =− 6t o 26 mL/m 2 ; annual mL/m 2 increase = 4.3 ± 4.6; annual percentage increase = 3.1 ± 3.3%), whilst RV ejection fraction decreased (53 ± 8 to 49 ± 7 %,p =0 .039). RV muscular corpus (RVMC) EDVi significantly increased (130 ± 19 to 138 ± 20 mL/m 2 , p =0 .014), whereas RV outfl ow tract (RVOT) EDVi did not (12 ±7v s 13 ±6m L/m 2 , p = 0.390). No other RV or LV measures significantly changed during the inter-MRI period. The change in RVEDVi correlated signif- icantly with LV end diastolic volume (r = −0.582, p = 0.029), RVEDVi:LVEDVi (r =0 .6,p = 0.023) and RVMC EDVi (r =0 .9,p b 0.001) but not RVOT EDVi (r =0 .225,p = 0.459). Conclusions: Adult repaired TOF patients with free PR experienced a mean 3.1%, or 4.3 mL/m 2 , annual in- crease in RVEDVi, unrelated to the initial RVEDVi or PR fraction. The increase in RVEDVi was due to RVMC rather than RVOT dilatation. This provides a guide to the frequency of MR surveillance and insights into the natural history of progressive RV dilatation in this setting. © 2014 The Authors. Published by Elsevier Ireland Ltd. This is an open access article under the CC BY-NC-ND license

Background Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited cardiomyopathy that can lead to sudden cardiac death. The diagnostic criterion has recently been revised and through the use of cardiac magnetic resonance (CMR) imaging this study aimed to assess the clinical impact of comparing the original 1994 task force (TF) criterion to the revised 2010 criterion. Methods We evaluated 173 consecutive CMR scans of patients referred with clinical suspicion of ARVC between 2008 and 2011. We then compared the prevalence of major and minor CMR criteria by applying the two criteria. Results Using the 1994 TF criterion, 13 (7.5%) patients had definite, 11 (6.4%) had borderline, and 39 (22.5%) had possible ARVC. Using the 2010 TF criterion, 10 (5.8%) patients had definite, 1 had borderline, and 7 had (0.04%) possible ARVC. With the 1994 criterion, 81 patients satisfied CMR criterion, of which 36 (44%) had major and 45 (56%) had minor criteria. Upon reclassification with the revised criterion, 61 of the 81 patients were not assigned any criteria, even though many patients had significant risk factors. The negative predictive values (NPV) for both CMR criteria were 100% but the positive predictive values (PPV) for combined CMR major or minor criteria improved from 23% to 55%. Conclusions Revision of the criterion has enhanced the diagnostic capabilities of CMR but has resulted in a large cohort of patients not classified. In these patients, there is presently no official consensus on imaging or clinical strategy for surveillance of the evolution of pathology over time.

Several imaging modalities are utilised in the assessment of disease progression in chronic aortic dissection. We present the case of a 66 year-old male who underwent ascending aorta repair for Stanford type A aortic dissection. On follow-up the persisting dissection of the descending thoracic aorta was observed to regress on magnetic resonance imaging (MRI). MRI has several advantages over computed tomography (CT) scanning and echocardiography in the follow-up phase of this disease.

Post-prandial hypertriglyceridaemia is a risk factor for cardiovascular disease, although the underlying mechanisms remain unclear. High density lipoproteins (HDL) have been shown to be atheroprotective, in part through attenuation of vascular inflammation. In this study, the influence of acute hypertriglyceridaemia on the composition and anti-inflammatory properties of HDL was investigated.Eight fasting healthy male subjects (34+/-2 years) received 20% Intralipid (15 mg/kg/h) or saline, on separate occasions in random order. At baseline and 60 min post-infusion, the total HDL fraction was isolated and its chemical composition determined. HDL were added to TNF-alpha stimulated human coronary artery endothelial cells and VCAM-1 and ICAM-1 expression was determined by flow cytometry.Serum triglyceride (97.4+/-8.5mg/dL baseline, 283.2+/-35.4 mg/dL post-infusion, p0.001) and HDL triglyceride content (3.8+/-0.5% HDL mass baseline, 5.3+/-0.9% HDL mass post-infusion, p0.05) increased significantly after Intralipid infusion. HDL post-Intralipid were significantly less anti-inflammatory compared with control (e.g. at 8 microM apoA-I, %VCAM-1 expression 54+/-5 post-saline, 73+/-4 post-Intralipid, p=0.01; %ICAM-1 expression 94+/-1 post-saline, 99.4+/-0.6 post-Intralipid, p0.01). There was also a significant correlation between HDL triglyceride content and VCAM-1 expression (R=0.70, p=0.005); as well as between plasma triglyceride levels and both VCAM-1 (R=0.71, p0.005) and ICAM-1 expression (R=0.80, p0.005).Acute hypertriglyceridaemia, simulating the post-prandial state, results in triglyceride-rich HDL with impaired anti-inflammatory capacity.

Although repair of Tetralogy of Fallot (TOF) and transposition of the great arteries (TGA) has facilitated survival into adulthood, many survivors have residual haemodynamic abnormalities, including exercise intolerance and late right ventricular (RV) failure.We studied 40 asymptomatic adult subjects (31.3+/-1.5 years) after congenital heart disease (CHD) surgery during childhood, using tissue Doppler echocardiography (TDE). We compared systolic (S') and diastolic (E' for early filling) RV and LV velocities, with 40 age matched controls (29.5+/-1.0 years).Both RV S' and E' velocities were significantly slower in the CHD group compared to controls (6.3+/-0.4 cm/s vs. 9.3+/-0.3 cm/s; 8.5+/-0.5 cm/s vs. 10.9+/-0.4 cm/s, respectively, p0.001 for both). By contrast, LV S' and E' velocities were similar in both groups. Interestingly, in 50% of CHD subjects where RV function was reported as 'normal', both RV S' and E' velocities were significantly slower compared with controls (6.5+/-0.6 cm/s vs. 9.3+/-0.3 cm/s, p0.0001 and 9.4+/-0.7 cm/s vs. 10.9+/-0.4 cm/s, p0.05 respectively).RV S' and E' velocities are frequently abnormal in asymptomatic survivors of TOF and TGA repair, even where RV function appears 'normal'. Hence TDE during follow up may be a sensitive means of detecting pre-clinical abnormalities in RV performance.

Objectives. (1) To document, using tissue Doppler echocardiography (TDE), the regional variations in myocardial velocities (MV) in the left and right ventricles (LV and RV) in healthy young adults. (2) To determine the factors predicting MV. Background. The pattern of tissue velocities within the right ventricle have yet to be determined and their patterns compared to the left ventricular velocities have not yet been described. Methods. Forty healthy subjects, mean age 29±6 years, were studied using TDE. Left ventricular long-axis velocities ( V LV-LX ) were obtained by sampling from anteroseptal, anterior, lateral, posterior, inferior and inferoseptal LV walls, and long-axis RV velocities ( V RV-LX ) from the free wall of the RV, in standard apical views. LV radial velocities ( V LV-RAD ) and RV radial velocities ( V RV-RAD ) were assessed from the parasternal long and short-axis views. Regression analyses were performed to assess for correlations of MV with the variables: age, sex, QRS duration, heart rate, systolic and diastolic blood pressure, LV mass, width, LV or RV lengths, LA or RA areas. Results. There were marked but consistent regional variations in systolic and diastolic tissue velocities in the LV and RV. Systolic ( S ′) and early diastolic ( E ′) velocities differed significantly around the left ventricular base, the highest velocities being located within the free wall at 6.4±2.2cm/s and 11.3±3.1cm/s, respectively. The E ′/ S ′ratio remained constant and independent of position. V LV-LX were significantly higher than V LV-RAD ( p V LV-LX S ′ velocities were consistently lower than V RV-LX S ′ velocities ( p V LV-LX S ′ and V LV-LX E ′ values ( p Conclusions. In healthy young adults, there is a consistent pattern of non-uniform MV throughout the heart, including differences in longitudinal and radial axis velocities both within the LV and between the LV and RV. Age, heart rate and LV structure are important determinants of MV. Condensed Abstract. The patterns of left and right ventricular myocardial velocities and their relationships to each other are not well characterized. Furthermore, the determinants of myocardial velocities are not known. This study evaluated the myocardial longitudinal and radial axis tissue velocities in both the left (LV) and right (RV) ventricles and found that a consistent but non-uniform relationship exists between the LV and RV in both longitudinal and radial axes. Furthermore, age, heart rate and LV dimensions account for between 20% and 70% of the variability seen in LV systolic and diastolic velocities.

The arterial endothelium is a vital homeostatic cell layer responsible for a variety of functions such as thromboresistance, control of vessel tone, and vessel growth. Recent evidence has indicated that both active and passive cigarette smoking are associated with dysfunction of normal endothelial physiology, in a dose dependent and potentially reversible way. Endothelial abnormalities may predispose to the atherogenic and thrombotic problems associated with cigarette smoking.

Although inflammation is a key process in atherogenesis, little is known about the inflammatory characteristics of culprit plaque in premature coronary artery disease (CAD). We investigated inflammation in coronary atheroma from subjects who died of premature CAD. From 2001–2005, we collected coronary plaque samples from consecutive cases of CAD ( n =23) reported to the Department of Forensic Medicine which led to unexpected death in men aged n =12) had lower levels of T cells (CD3+) ( p =0.03), higher macrophage (CD 68+) ( p =0.01) and T regulator cells (FOXP3+) ( p =0.03) infiltration when compared to older CAD decedents (>35years, n =11). Interestingly, there was no significant age-related difference between groups in the smooth muscle cell, apoA-I, myeloperoxidase and MMP-2 content within plaque. Hence, we demonstrate that higher expression of FOXP3 is associated with younger age at the time of fatal outcomes from CAD. These findings may have implications for plaque pathophysiology and thus warrant further investigation.

The acute event of thoracic aortic dissection carries with it high mortality and morbidity. Despite optimal initial surgical or medical management strategies, the risk of further complications in the long-term, including aneurysmal dilatation and false lumen (FL) expansion, are not insignificant. Adequate follow-up of such conditions requires dedicated imaging where relevant prognostic indicators are accurately assessed. We perform a systematic review of the literature and report the current evidence for the use of magnetic resonance imaging (MRI) in assessment of chronic aortic dissection. We then make a comparison with traditional imaging modalities including computed tomography and echocardiography. We discuss new ways in which MRI may extend existing aortic assessment, including identification of blood-flow dynamics within the TL and FL using phase-contrast imaging.

Coronary heart disease, often associated with fatal outcomes, is increasingly common. Immune dysregulation is a key process in atherogenesis, yet age and other immune related factors are poorly characterised, especially in premature disease. The current study is to investigate the differential immunological mediators in the plaque specific to older and younger decedents.Coronary artery plaques were collected from male decedents as follows: Younger (50 years; n=30), older (60 years; n=30) and control (n=10). Demographic and forensic pathological data were collected. Plaques were investigated, using detailed immunohistochemistry.Older subjects had 20% elevated heart to body weight ratio compared to younger subjects (p0.05), as well as 40% higher levels of plaque necrosis (p0.05) and 90% more calcification (p0.05). There was a three-fold higher amount of infiltrating CD3(+) T cells (p0.05) and myeloperoxidase production (p0.05) within plaque from older subjects than younger. Interestingly, older subjects had 50% and 70% lower numbers of infiltrating macrophage (p0.05) and Foxp3(+) Treg cells (p0.05), respectively, in the plaque compared to younger subjects. There were no significant age-dependent differences in IL-17 or IL-10 secretion.Our data suggests that there are indeed age-dependent characteristics of culprit coronary plaque inflammation. In particular, older subjects demonstrate a plaque phenotype with higher amounts of inflammation and lower levels of Treg cells. Despite lower levels of inflammation, younger subjects also had fatal outcomes, indicating that alternative factors may be more important in plaque stability. These observations may have therapeutic consequences and hence warrant further investigation.

I. Rashid , H. Klimis , J. Duflou , D. Sullivan , R. Puranik a,e,⁎ a Department of Cardiology, Royal Prince Alfred Hospital, Sydney, NSW, Australia b Department of Forensic Medicine, Central Sydney Laboratory Service, Australia c Sydney Medical School, University of Sydney, NSW, Australia d Department of Biochemistry, Royal Prince Alfred Hospital, Sydney, NSW, Australia e Department of Medicine, University of Sydney, NSW, Australia

Isolated ventricular noncompaction, also known as left ventricular noncompaction (LVNC), isolated noncompaction of the ventricular myocardium (INVM), noncompaction of the left ventricular myocardium (INLVM), left ventricular hypertrabeculation, and spongy myocardium, is a rare and recently described cardiomyo-pathy with characteristic gross morphological abnormalities. It can present in paediatric and adult patients. Currently, the diagnosis can be made by echocardiography or magnetic resonance imaging (MRI). To the best of our knowledge, so far, there is no histo-pathology description of this entity in the English literature. Although aetiology, pathogenesis and histopathological features of IVNC are not clear, there is evidence for genetic basis in at least some cases, with subsequent implication of genetic counselling for family members of the patients. Here, we report a case of isolated ventricular noncompaction cardiomyopathy, and discuss its clinical, imaging and histopathology features.

The diagnosis of aortic dissection is difficult because of often non-specific clinical features. It is often confused with a range of both benign and potentially lethal conditions. Experience demonstrates that in those cases referred for coronial investigation, it is relatively uncommon that aortic dissection be considered antemortem. A retrospective review of cases of autopsy verified death due to aortic dissection performed at the Department of Forensic Medicine, Glebe, is presented. This 16 year review includes data on demographics, pre-existing, familial and syndromic conditions, potential risk factors, the characteristics of disease as well as the circumstances surrounding presentation and death. Results include a strong correlation with pre-existing cardiovascular disease and the relative rarity of the various connective tissue diseases typically cited as being significant causes of aortic dissection. The clinical course appears to be rapid with almost 1:4 deaths occurring in the emergency department and around 1:5 deaths occurring during or after surgery. This study has the additional significance of providing detailed baseline information in a large number of cases on the nature of aortic dissection for the purposes of a subsequent study where autopsy and post-mortem MRI features are compared in a smaller prospective series of cases.

Of the number of interpretable segments in the older cohort, detection of a significant stenosis (>50%) was similar between the two reviewers (3.4% vs. 4.9%), p= 0.3). Conclusions:Seventy-ninepercentofpatients≥70years of age have interpretable coronary segments using 320slice CT scanning despite higher incidence of calcification compared to younger cohort. Of the interpretable segments in older patients, assessment of stenosis severity demonstrated good agreement between two reviewers. doi:10.1016/j.hlc.2009.05.077

Early operative repair of coarctation of the aorta (CoA) is associated with improved patient prognosis; however the rates of re-coarctation in follow-up are variable across institutions. We investigated the impact of early CoA repair (