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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.

Conventional autopsy is the gold standard for identifying unexplained death but due to declines in referrals, there is an emerging role for post-mortem imaging. We evaluated whether post-mortem magnetic resonance (PMMR) and computed tomography (PMCT) are inferior to conventional autopsy. Deceased individuals ≥ 2 years old with unexplained death referred for coronial investigation between October 2014 to December 2016 underwent PMCT and PMMR prior to conventional autopsy. Images were reported separately and then compared to the autopsy findings by independent and blinded investigators. Outcomes included the accuracy of imaging modalities to identify an organ system cause of death and other significant abnormalities. Sixty-nine individuals underwent post-mortem scanning and autopsy (50 males; 73%) with a median age of 61 years (IQR 50–73) and median time from death to imaging of 2 days (IQR 2–3). With autopsy, 48 (70%) had an organ system cause of death and were included in assessing primary outcome while the remaining 21 (30%) were only included in assessing secondary outcome; 12 (17%) had a non-structural cause and 9 (13%) had no identifiable cause. PMMR and PMCT identified the cause of death in 58% (28/48) of cases; 50% (24/48) for PMMR and 35% (17/48) for PMCT. The sensitivity and specificity were 57% and 57% for PMMR and 38% and 73% for PMCT. Both PMMR and PMCT identified 61% (57/94) of other significant abnormalities. Post-mortem imaging is inferior to autopsy but when reported by experienced clinicians, PMMR provides important information for cardiac and neurological deaths while PMCT is beneficial for neurological, traumatic and gastrointestinal deaths.

The Pfizer COVID-19 vaccine is associated with increased myocarditis incidence. Constantly evolving evidence regarding incidence and case fatality of COVID-19 and myocarditis related to infection or vaccination, creates challenge for risk-benefit analysis of vaccination programs. Challenges are complicated further by emerging evidence of waning vaccine effectiveness, and variable effectiveness against variants. Here, we build on previous work on the COVID-19 Risk Calculator (CoRiCal) by integrating Australian and international data to inform a Bayesian network that calculates probabilities of outcomes for the Delta variant under different scenarios of Pfizer COVID-19 vaccine coverage, age groups (≤12 years), sex, community transmission intensity and vaccine effectiveness. The model estimates that in a population where 5% were unvaccinated, 5% had one dose, 60% had two doses and 30% had three doses, the probabilities of developing and dying from COVID-19-related myocarditis were 239-5847 and 1430-384,684 times higher (depending on age and sex), respectively, than developing vaccine-associated myocarditis. For one million people with this vaccine coverage, where transmission intensity was equivalent to 10% chance of infection over two months, 68,813 symptomatic COVID-19 cases and 981 deaths would be prevented, with 42 and 16 expected cases of vaccine-associated myocarditis in males and females, respectively. The model may be updated to include emerging best evidence, data pertinent to different countries or vaccines, and other outcomes such as long COVID.

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.

The coronavirus pandemic has given everyone in society an education on the harms of spread of respiratory illness. Young healthy athletes are far less likely to suffer severe adverse consequences of viral illnesses than the elderly and frail, but they are not completely immune. Chronic fatigue (overtraining) is an uncommon outcome and myocarditis a rare one, but they both warrant due consideration. It is, therefore, a sensible individual strategy to ‘stay home when sick’ if only for these risks. Traditionally though, athletes have tended to push through (train and play when ill) because of competing concerns, such as key events/matches and ‘not wanting to let teammates down’. Data from both low COVID-19 and high COVID-19 countries show that the number of cardiovascular deaths in a society correlates with the number of respiratory deaths at the same time, further linking respiratory viruses to cardiovascular deaths. We are now more aware of public health obligations to prevent the spread of respiratory illnesses, in particular to protect the more vulnerable members the community. This hopefully will correspond with a change in the culture of sport to one where it is considered ‘the right thing to do’, to ‘stay home when sick’.

Objectives:Concerns exist over the long-term consequences of subclavian artery ligation in subclavian flap repair for coarctation of the aorta. We sought to analyse upper limb structural and functional performance in adults who have had surgery in childhood for coarctation of the aorta, using either subclavian flap repair or end to end aortic anastomosis.Methods:Two-group observational design using anatomical and upper limb functional performance measures. Purposive sampling from our specialist adult congenital heart disease database of patients who received subclavian flap repair or end to end anastomosis for coarctation of the aorta as children. Upper limb measurements were completed using MRI and blood flow velocity with ultrasound imaging. Bilateral standardised upper limb functional testing of assessment of strength, dexterity and a standardised self-report of upper limb disability was completed.Results:Eighteen right-handed patients, 9 with subclavian repair, (38 ± 12 years, 78% males) were studied. Age at repair was 4.7 ± 5.9 years; mean time from initial repair 32 ± 9 years. The subclavian group had a larger difference between right and left when compared the end to end anastomosis group in: lower arm muscle mass (94.5 ± 42.3 mls versus 37.8 ± 94.5 mls, p = 0.008), lower arm maximal cross-sectional area, (5.9 ± 2.8 cm2 versus 2.9 ± 2.6 cm2, p = 0.038) and grip strength (14.7 ± 8.3 lbs versus 5.9 ± 5.3 lbs, p = 0.016) There were no significant functional differences between groups.Conclusions:In adults with repaired coarctation of the aorta, those with subclavian flap repair had a greater right to left arm muscle mass and grip strength differential when compared to those with end to end anastomosis repair.

Aims To assess the reported prevalence of left ventricular non-compaction (LVNC) in different adult cohorts, taking in to consideration the role of diagnostic criteria and imaging modalities used. Methods and results A systematic review and meta-analysis of studies reporting LVNC prevalence in adults. Studies were sourced from Pre-Medline, Medline, and Embase and assessed for eligibility according to inclusion criteria. Eligible studies provided a prevalence of LVNC in adult populations (≥12 years). Studies were assessed, and data extracted by two independent reviewers. Fifty-nine eligible studies documenting LVNC in 67 unique cohorts were included. The majority of studies were assessed as moderate or high risk of bias. The pooled prevalence estimates for LVNC were consistently higher amongst cohorts diagnosed on cardiac magnetic resonance (CMR) imaging (14.79%, n = 26; I 2 = 99.45%) compared with echocardiogram (1.28%, n = 36; I 2 = 98.17%). This finding was unchanged when analysis was restricted to studies at low or moderate risk of bias. The prevalence of LVNC varied between disease and population representative cohorts. Athletic cohorts demonstrated high pooled prevalence estimates on echocardiogram (3.16%, n = 5; I 2 = 97.37%) and CMR imaging (27.29%, n = 2). Conclusion Left ventricular non-compaction in adult populations is a poorly defined entity which likely encompasses both physiological adaptation and pathological disease. There is a higher prevalence with the introduction of newer imaging technologies, specifically CMR imaging, which identify LVNC changes more readily. The clinical significance of these findings remains unclear; however, there is significant potential for overdiagnosis, overtreatment, and unnecessary follow-up.

BACKGROUND: Post-mortem cardiac magnetic resonance (CMR) is a non-invasive alternative to conventional autopsy. At present, diagnostic guidelines for cardiovascular conditions such as hypertrophic cardiomyopathy have not been established. We correlated post-mortem CMR images to definite conventional autopsy findings and hypothesed that elevated T2-weighted signal intensity and RV to LV area ratios can identify myocardial infarction and pulmonary emboli respectively. METHODS: For this unblinded pilot sub-study, we selected cases from the original blinded study that compared post-mortem imaging to conventional autopsy in patients referred for coronial investigation between October 2014 to November 2016. Three groups of scans were selected based on the cause of death identified by conventional autopsy: non-cardiovascular causes of death with no structural cardiac abnormality i.e., control cases, acute/subacute myocardial infarction and pulmonary emboli. Left ventricular (LV) wall thickness, LV myocardial signal intensity and ventricular cavity areas were measured. RESULTS: Fifty-six scans were selected [39 (69.6%) males]: 37 (66.1%) controls, eight (14.3%) acute/subacute myocardial infarction and eleven (19.6%) pulmonary emboli. The median age was 61 years [Interquartile range (IQR) 50–73] and the median time from death to imaging and autopsy was 2 days (IQR 2–3) and 3 days (IQR 3–4). The septal and lateral walls were thicker {15 mm [13–17] and 15 mm [14–18]} on post-mortem CMR than published ante-mortem measurements. Areas of acute/subacute myocardial infarction had significantly higher T2-weighted signal intensity (normalised to skeletal muscle) compared to normal myocardium in those who died from other causes {2.5 [2.3–3.0.] vs. 1.9 [1.8–2.3]; P

Funding Acknowledgements Type of funding sources: Public grant(s) – National budget only. Main funding source(s): JO is supported by an Australian Government Research Training Program scholarship. CS is the recipient of a National Health and Medical Research Council (NHMRC) Practitioner Fellowship (#1154992). Background Athletes sometimes experience transient arrhythmias during intense exercise, which may be difficult to capture with traditional monitors. New highly portable technology, such as smartphone electrocardiogram (ECG) devices, may be useful in documenting and diagnosing exercise-induced arrhythmias. Accuracy of the Kardia single-lead ECG (1L) has been documented, but little data exists for the 6-lead device (6L). Purpose To examine the level of similarity between resting 6L and 12L readings to build evidence for the utility of the 6L as a practical diagnostic tool in athletes. Methods Participants (n = 30 healthy athletes, mean age 18.9 years, 57% male) had a resting supine 12-lead ECG (12L) as part of cardiac screening required by their sport. Within 1 hour, a 30 second 6L reading (leads I, II, III, aVR, aVL, aVF) was taken whilst seated. Data were analysed by 4 expert cardiologists. Manual measurements were taken for PR, QT and RR intervals and QRS duration using EPS digital calipers. To calculate mean 6L RR interval and QT prior to QTc, Continuous variables were expressed as the mean of 4 cardiologists’ values ± standard deviation. Two-tailed paired t-tests were used to compare continuous variables (p Results There were relatively high levels of agreement between the mean 6L and 12L measures for QTc and PR interval and QRS duration, with the 6L readings slightly but significantly shorter on average. The largest difference was seen in the QTc intervals (391ms vs 401ms, p = 0.003). The 6L QRS durations were shorter on average by 3ms (89ms vs 92ms, p = 0.025) and PR intervals were shorter on average by 6ms (163ms vs 169ms, p There was complete agreement for all cardiologists for sinus rhythm and the presence of ectopics for the 6L and 12L readings. Conclusions The 6L readings had relatively high agreement with the 12L. All 6L measures (except heart rate) were slightly shorter on average than 12L. These small differences are unlikely to have any clinical significance, and are similar to findings comparing the 1L to 12L. 6L heart rates were slightly higher, which is best explained by seated compared to lying position. The reading with the greatest variation was QT interval, some of which is explained by heart rate variation. These pilot data suggest the 6L is sufficiently accurate to be useful in an athletic population as an event monitor for exercise-induced arrhythmias. This may provide more useful diagnostic data than the 1L. Larger studies showing higher levels of agreement with 12L would be required to expand the role of 6L beyond an event monitor. Abstract Figure 1: 6L device, Bland-Altman plots

Background:Left ventricular non-compaction has been associated with heart failure, arrhythmia, thromboembolism and sudden death. The prevalence of non-compaction in patients with coarctation of the aorta and its clinical significance remains unknown, although obstructive left heart disease is common in patients with non-compaction. We sought to evaluate the prevalence of left ventricular non-compaction in patients with repaired aortic coarctation as well as its effect on left ventricular size and systolic function.Methods and results:In total, 268 patients (Mean age 26 (inter-quartile range 21–37) years, 63% male) undergoing cardiac magnetic resonance imaging for clinical follow-up were included from three tertiary centres for adult congenital heart disease. Clinical data was obtained from medical records and correlated with ventricular volumes and function. Left ventricular non-compaction was defined as a diastolic non-compacted:compacted dimension ratio >2.3 in the worst affected segment on a long-axis view. Left ventricular non-compaction was present in 8.2% of patients with repaired coarctation. Left ventricular end-diastolic volumes and stroke volumes were significantly higher in patients with non-compaction compared to those without. There were no significant differences in ventricular mass or ejection fraction in these two groups.Conclusions:Left ventricular non-compaction is relatively common in patients with repaired coarctation of the aorta and correlates with increased left ventricular end-diastolic volumes.

Left ventricular non-compaction (LVNC) is a complex clinical condition with no diagnostic gold standard. At present, there is trepidation about the accuracy of the diagnosis, the correlation to clinical outcomes and the long-term medical management. This article reviews the current imaging criteria, the limitations of echocardiography and cardiac magnetic resonance and the consequences of LV hypertrabeculation in athletes.

Conventional autopsy is the gold standard for identifying unexplained death but due to declines in referrals, there is an emerging role for post-mortem imaging. We evaluated whether post-mortem magnetic resonance (PMMR) and computed tomography (PMCT) are inferior to conventional autopsy. Deceased individuals ≥ 2 years old with unexplained death referred for coronial investigation between October 2014 to December 2016 underwent PMCT and PMMR prior to conventional autopsy. Images were reported separately and then compared to the autopsy findings by independent and blinded investigators. Outcomes included the accuracy of imaging modalities to identify an organ system cause of death and other significant abnormalities. Sixty-nine individuals underwent post-mortem scanning and autopsy (50 males; 73%) with a median age of 61 years (IQR 50-73) and median time from death to imaging of 2 days (IQR 2-3). With autopsy, 48 (70%) had an organ system cause of death and were included in assessing primary outcome while the remaining 21 (30%) were only included in assessing secondary outcome; 12 (17%) had a non-structural cause and 9 (13%) had no identifiable cause. PMMR and PMCT identified the cause of death in 58% (28/48) of cases; 50% (24/48) for PMMR and 35% (17/48) for PMCT. The sensitivity and specificity were 57% and 57% for PMMR and 38% and 73% for PMCT. Both PMMR and PMCT identified 61% (57/94) of other significant abnormalities. Post-mortem imaging is inferior to autopsy but when reported by experienced clinicians, PMMR provides important information for cardiac and neurological deaths while PMCT is beneficial for neurological, traumatic and gastrointestinal deaths.

The genetic etiology and heritability of left ventricular noncompaction (LVNC) in adults is unclear. This study sought to assess the value of genetic testing in adults with LVNC. Adults diagnosed with LVNC while undergoing screening in the context of a family history of cardiomyopathy were excluded. Clinical data for 35 unrelated patients diagnosed with LVNC at ≥18 years of age were retrospectively analyzed. Left ventricular (LV) dysfunction, electrocardiogram (ECG) abnormalities, cardiac malformations or syndromic features were identified in 25 patients; 10 patients had isolated LVNC in the absence of cardiac dysfunction or syndromic features. Exome sequencing was performed, and analysis using commercial panels targeted 193 nuclear and mitochondrial genes. Nucleotide variants in coding regions or in intron-exon boundaries with predicted impacts on splicing were assessed. Fifty-four rare variants were identified in 35 nuclear genes. Across all 35 LVNC patients, the clinically meaningful genetic diagnostic yield was 9% (3/35), with heterozygous likely pathogenic or pathogenic variants identified in the NKX2-5 and TBX5 genes encoding cardiac transcription factors. No pathogenic variants were identified in patients with isolated LVNC in the absence of cardiac dysfunction or syndromic features. In conclusion, the diagnostic yield of genetic testing in adult index patients with LVNC is low. Genetic testing is most beneficial in LVNC associated with other cardiac and syndromic features, in which it can facilitate correct diagnoses, and least useful in adults with only isolated LVNC without a family history. Cardiac transcription factors are important in the development of LVNC and should be included in genetic testing panels., Cardiomyopathy: Genetic testing largely unhelpful for rare heart disease Genetic testing is of limited use for diagnosing a rare heart muscle disorder called left ventricular non-compaction (LVNC), a structural abnormality of the lower left chamber of the heart in which the muscular wall appears soft and spongy rather than smooth and compacted. A team led by Christopher Semsarian from the University of Sydney, Australia, carried out a a retrospective analysis of genetic data from 35 patients diagnosed with LVNC but with no family history of the disease. They identified only three likely pathogenic gene variants, all in known heart regulatory genes and all in individuals with symptoms of cardiac dysfunction. The researchers conclude that genetic testing can facilitate a correct diagnosis of LVNC in patients showing signs of heart problems, but is largely unhelpful for those without symptoms and no family history.

Left ventricular non-compaction (LVNC) is a complex clinical condition with several diagnostic criteria but no diagnostic gold standard. We aimed to evaluate our thresholding technique in a group of patients with LVNC and assess the risk of major adverse cardiovascular and cerebrovascular events (MACCE).We retrospectively analyzed cardiac magnetic resonance (CMR) scans of patients with Petersen criteria LVNC and quantified noncompacted myocardial mass. We assessed the association of noncompacted myocardial mass, CMR derived LV volumetric parameters and late gadolinium enhancement (LGE) to MACCE including cardiac death, cardiac transplantation, sustained ventricular tachycardia/ventricular fibrillation (VT/VF) and ischemic stroke. Patients with known genetic mutations and cardiovascular disease were excluded.98 patients with LVNC were included (55 males,56.7%); 17(17.3%) patients had impaired LV function and five (5.1%) had LGE. Patients with impaired LV function had more end-systolic noncompacted mass (61.9 g±22.4 vs. 38.1 g±15.8, p 0.001) and larger end-systolic noncompacted to total myocardial mass (44%±9 vs. 36%±12, p = 0.003). At 78 months follow-up [interquartile range(IQR) 66-90], MACCE occurred in 11(11.3%) patients; nine(81.8%) had impaired LV function and two(18.2%) had LGE. Impaired LV function and LV LGE were predictors of MACCE (HR = 35.6, 95% CI = 7.65-165.21, p 0.001 and HR = 16.2, 95% CI = 4.54-57.84, p 0.001) whereas noncompacted mass were not.Noncompacted mass was not an independent predictor of major adverse events but in patients with impaired LV function and/or LV LGE, the risk of MACCE was high. These results highlight the importance of including LV volumetrics and scar in the assessment of patients with LV noncompaction.

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.

Objective:Left ventricular non-compaction is an architectural abnormality of the myocardium, associated with heart failure, systemic thromboembolism, and arrhythmia. We sought to assess the prevalence of left ventricular non-compaction in patients with single ventricle heart disease and its effects on ventricular function.Methods:Cardiac MRI of 93 patients with single ventricle heart disease (mean age 24 ± 8 years; 55% male) from three tertiary congenital centres was retrospectively reviewed; 65 of these had left ventricular morphology and are the subject of this report. The presence of left ventricular non-compaction was defined as having a non-compacted:compacted (NC:C) myocardial thickness ratio >2.3:1. The distribution of left ventricular non-compaction, ventricular volumes, and function was correlated with clinical data.Results:The prevalence of left ventricular non-compaction was 37% (24 of 65 patients) with a mean of 4 ± 2 affected segments. The distribution was apical in 100%, mid-ventricular in 29%, and basal in 17% of patients. Patients with left ventricular non-compaction had significantly higher end-diastolic (128 ± 44 versus 104 ± 46 mL/m2, p = 0.047) and end-systolic left ventricular volumes (74 ± 35 versus 56 ± 35 mL/m2, p = 0.039) with lower left ventricular ejection fraction (44 ± 11 versus 50 ± 9%, p = 0.039) compared to those with normal compaction. The number of segments involved did not correlate with ventricular function (p = 0.71).Conclusions:Left ventricular non-compaction is frequently observed in patients with left ventricle-type univentricular hearts, with predominantly apical and mid-ventricular involvement. The presence of non-compaction is associated with increased indexed end-diastolic volumes and impaired systolic function.

Background Myocardial pathology is common in patients undergoing hemodialysis. To explore the effects of differing aspects of dialysis treatment on its evolution, we examined the impact of change in markers of volume status, hemodynamics and solute clearance on left ventricular (LV) parameters in a randomized trial of extended hours dialysis. Methods and Results A Clinical Trial of IntensiVE (ACTIVE) Dialysis randomized 200 patients undergoing hemodialysis to extended dialysis hours (≥ 24 hours/week) or standard hours (12–18 hours/week) for 12 months. In a prespecified substudy, 95 participants underwent cardiac magnetic resonance imaging (CMR) at baseline and at the study's end. Generalized linear regression was used to model the relationship between changes in LV parameters and markers of volume status (normalized ultrafiltration rate and total weekly interdialytic weight gain), hemodynamic changes (systolic and diastolic blood pressure) and solute control (urea clearance, dialysis hours and phosphate). Randomization to extended hours dialysis was not associated with change in any CMR parameter. Reduction in ultrafiltration rate was associated with reduction in LV mass index (P = 0.049) and improved ejection fraction (P = 0.024); reduction in systolic blood pressure was also associated with improvement in ejection fraction (P = 0.045); reduction in interdialytic weight gain was associated with reduced stroke volume (P = 0.038). There were no associations between change in urea clearance, phosphate or total hours per week and CMR parameters. Conclusions Reduction in ultrafiltration rate and blood pressure are associated with improved myocardial parameters in hemodialysis recipients independently of solute clearance or dialysis time. These findings underscore the importance of fluid status and related parameters as potential treatment targets in this population.

Background The Task Force Criteria (TFC) for arrhythmogenic right ventricular cardiomyopathy (ARVC) was updated in 2010 to improve specificity. There was concern however that the revised cardiovascular magnetic resonance (CMR) criteria was too restrictive and not sensitive enough to detect early forms of the condition. We previously described patients with clinically suspected ARVC who satisfied criteria from non-imaging TFC categories and fulfilled parameters from the original but not the revised CMR criteria; as a result, these patients were not confirmed as definite ARVC but may represent an early phenotype. Methods Patients scanned between 2008 and 2015 who had either right ventricular (RV) dilatation or regional dyskinesia satisfying at least minor imaging parameters from the original criteria and without contra-indication underwent serial CMR scanning using a 1.5 T scanner. The aims were to assess the risk of progressive RV abnormalities, evaluate the accuracy of the revised CMR criteria and the need for guideline directed CMR surveillance in at-risk individuals. Results Overall, 48 patients were re-scanned; 24 had a first-degree relative diagnosed with ARVC using the revised TFC or a first-degree relative with premature sudden death from suspected ARVC and 24 patients had either left bundle branch morphology ventricular tachycardia or > 500 ventricular extra-systoles in 24-h. Mean follow up was 69+/− 25 months. The indexed RV end-diastolic, end-systolic volumes and ejection fraction were calculated for both scans. There was significant reduction in RV volumes and improvement in RV ejection fraction (EF) irrespective of changes to body surface area; − 11.7+/− 15.2 mls/m2, − 6.4+/− 10.5 mls/m2 and + 3.3 +/− 7.9% (p = 0.01, 0.01 and 0.04). Applying the RV parameters to the revised CMR criteria, two patients from the family history group (one with confirmed ARVC and one with a premature death) had progressive RV abnormalities satisfying major criteria. The remaining patients (n = 46) did not satisfy the criteria and either had normal RV parameters with regression of structural abnormalities (27,56.3%) or stable abnormalities (19,43.7%). Conclusion The revised CMR criteria represents a robust tool in the evaluation of patients with clinical suspicion of ARVC, especially for those with ventricular arrhythmias without a family history for ARVC. For patients with RV abnormalities that do not fulfill the revised criteria but have a family history of ARVC or an ARVC associated gene mutation, a surveillance CMR scan should be considered as part of the clinical follow up protocol.

Objectives To report the compliance and results of an electrocardiogram (ECG) cardiac screening program in male and female elite Australian cricketers. Design cross-sectional study. Methods Elite cricketers were offered screening in accordance with Cricket Australia policy. Players who consented provided a personal and family history, physical examination and resting 12-lead ECG. An audit (1 February 2019) examined all cardiac screening records for male and female players in all Australian Cricket state squads from 16 years upwards. Data extracted from the Cricket Australia database included the number of players who underwent screening; signed waivers opting out; and had follow-up tests. ECGs were re-reviewed according to the International Criteria. Results 710 players were included in the cohort (mean age 20.4 ± 4.9 years, 62% male). 692 (97.5%) players underwent recommended cardiac screening or signed a waiver opting out (1.1%). 173 (24.4%) players were screened (or signed a waiver) more than once. Follow-up testing was conducted for 59 (6.9%) cases. No players were excluded from sport due to a cardiac problem and no major cardiac incidents occurred to any player in the audit cohort. Review of 830 ECGs showed benign athlete heart changes, including sinus bradycardia (33.5%), left ventricular hypertrophy (16.3%), and incomplete/partial right bundle branch block (8.4%), were common but abnormal screening ECGs were uncommon (2.0%). Conclusions An audit of a cardiac screening program in elite Australian cricketers found excellent compliance. A small proportion required follow-up testing and no player was excluded from sport due to a cardiac problem. ECG analysis suggested cricket is a sport of moderate cardiac demands, with benign athlete heart changes common.

Imaging modalities are central to diagnosis and prognostication of confirmed or suspected inherited cardiomyopathies. The availability and use of cardiovascular magnetic resonance imaging (CMR) to supplement traditional modalities has increased substantially and has several advantages over traditional imaging techniques. CMR is unique in its ability to easily acquire images in any plane. Moreover, advances in CMR sequences have begun to enable characterisation of the myocardium without the need for invasive biopsy and has provided a major step forward in the understanding of inherited heart disease pathology and genotype-phenotype interactions. This review summarises the current role of CMR in inherited cardiomyopathies depending on their genotype and phenotype status, using arrhythmogenic right ventricular dysplasia/cardiomyopathy and hypertrophic cardiomyopathy as prototypical examples.

Background X-linked dilated cardiomyopathy (XLDCM) is a rare but rapidly progressive cardiomyopathy caused by dystrophin gene mutation. Mutations are more often associated with Duchenne and Becker Muscular Dystrophy, which are characterized by skeletal muscle weakness or limb girdle dystrophy. However, patients with isolated XLDCM have normal skeletal muscle but complete dystrophin loss in cardiac muscle resulting in isolated myocardial involvement without overt signs of skeletal myopathy. Case summary A previously well 16-year-old boy developed sudden onset dense left-sided weakness and facial droop. Computed tomography (CT) angiography and CT brain showed an occluded right internal carotid artery extending to the right middle cerebral artery. He underwent successful endovascular clot retrieval but developed frank pulmonary oedema and cardiogenic shock requiring inotropic support and intubation. Transthoracic echocardiography demonstrated severe left ventricular (LV) cardiomyopathy and an apical thrombus. Subsequent cardiac magnetic resonance (CMR) imaging confirmed the LV parameters and diffuse late gadolinium enhancement. Despite absence of skeletal manifestations, subsequent genetic testing revealed an X-linked dystrophin gene mutation [c.31+G>T (IVS1G>T)]. He was commenced on empirical heart failure therapy and underwent successful cardiac transplantation. Discussion X-linked dilated cardiomyopathy is a rare, rapidly progressing cardiomyopathy. Patients show normal skeletal muscle dystrophin but absent expression in cardiac muscle, resulting fibrosis, and atrophy. About 20% of affected young males have significantly reduced survival and thus the diagnosis must be considered in cases of idiopathic cardiomyopathy with CMR and genetic testing key to the diagnosis. Whilst evidence exists for empirical heart failure medications, cardiac transplantation remains the definitive treatment.

Cardiac disease accounts for a large burden of premature mortality and morbidity in patients with type 1 myotonic dystrophy (MD). However, little is known about structural cardiac abnormalities particularly in asymptomatic patients with MD. We sought to describe the prevalence and extent of structural cardiac abnormalities in patients with MD and to assess their association with functional, electrical, biochemical and genetic disturbances.In this case-control study, 40 adults with MD who had no contraindications to cardiac MRI (CMR) were identified from the Grampian region genetic database. Forty-one age-and-gender-matched healthy volunteers were also recruited. All subjects underwent detailed assessment including CMR, echocardiography, electrocardiography, signal-averaged electrocardiography, Holter monitoring and quantification of serum B-type natriuretic peptide (BNP). Genetic testing of patients with MD was performed with quantification of CTG trinucleotide repeat sequences. Results of clinical, electrical, genetic and biochemical investigations were correlated with cardiac structural and functional abnormalities detected on CMR.Electrical disturbances including prolongation of PR (187±29 vs 156±23 ms, p0.001) and QRS intervals (99±11 vs 89±9 ms, p0.001) were the most prevalent abnormality. Patients with MD had a significantly lower left ventricular (LV) mass (142±44 vs 172±73 g, p=0.03) and lower right ventricular (RV) ejection fraction (46±9 vs 50±7%, p=0.02) compared with controls, although LV ejection fraction was similar between the groups (58±8 vs 59±6%, p=0.34). LV non-compaction was also significantly more prevalent in the MD cohort (35% vs 12%, p=0.019). Late gadolinium enhancement was present in 13% of patients with MD. Muscular disability scores correlated with electrical changes (r=0.529, p0.001); however, the number of CTG repeat sequences did not correlate with either electrical or structural abnormalities.Patients with MD have a high prevalence of both electrical and structural abnormalities. These include reduced LV mass, impaired RV contractility, a high prevalence of LV non-compaction and myocardial fibrosis. These findings illustrate the potential utility of CMR detecting subclinical disease in otherwise asymptomatic patients with MD.

Background: Bicuspid aortic valve (BAV) is a common congenital heart disease which is associated with aortic dilatation. There is controversy in the literature regarding the various measures of the biomechanical properties of the aorta in these patients and their relationship to aortic dilatation. The present study aimed to assess both conventional 2D Cardiac Magnetic Resonance Imaging (CMRI) measurements of aortic biomechanics (compliance and distensibility) and a computational fluid dynamics (CFD) approach in patients with BAV and either normal or dilated ascending aorta. Methods: 2D CMRI was performed in 18 patients (6 controls, 6 BAV with dilated ascending aorta and 6 BAV with normal ascending aorta i.e.

Aims The optimal timing for pulmonary valve replacement in asymptomatic patients with repaired Tetralogy of Fallot (rTOF) and pulmonary regurgitation remains uncertain but is often guided by increases in right ventricular (RV) end-diastolic volume. As cardiopulmonary exercise testing (CPET) performance is a strong prognostic indicator, we assessed which cardiovascular magnetic resonance (CMR) parameters correlate with reductions in exercise capacity to potentially improve identification of high-risk patients. Methods and results In all, 163 patients with rTOF (mean age 24.5 ± 10.2 years) who had previously undergone CMR and standardized CPET protocols were included. The indexed right and left ventricular end-diastolic volumes (RVEDVi, LVEDVi), right and left ventricular ejection fractions (RVEF, LVEF), indexed RV stroke volume (RVSVi), and pulmonary regurgitant fraction (PRF) were quantified by CMR and correlated with CPET-determined peak oxygen consumption (VO2) or peak work. On univariable analysis, there was no significant correlation between RVEDVi and PRF with peak VO2 or peak work (% Jones-predicted). In contrast, RVEF and RVSVi had significant correlations with both peak VO2 and peak work that remained significant on multivariable analysis. For a previously established prognostic peak VO2 threshold of Conclusion In rTOF, CMR indices of RV systolic function are better predictors of CPET performance than RV size. An RVEF

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

Aims Pulmonary regurgitation (PR) and right ventricular (RV) dilatation are common in repaired tetralogy of Fallot (rTOF). Left ventricular (LV) dysfunction is an important risk factor in rTOF. The effect of PR/RV dilatation on LV performance and RV-LV interactions in rTOF are incompletely understood. We examined LV responses and exercise capacity in rTOF, both before and after pulmonary valve replacement (PVR). Methods and results Cardiac magnetic resonance imaging scans in 126 rTOF patients (age 17.3 ± 7.6 years) were analysed, comparing subjects with indexed RV end-diastolic volume (RVEDVi) Conclusions Chronic PR with severe RV dilatation is associated with increased NPFFi, LVEDVi, and LVSVi. This may potentially explain preserved exercise capacity in rTOF with severe PR and RV dilatation.

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.