Systolic Impairment Research Articles

Cardiac Decompression by Pericardiectomy for Constrictive Pericarditis: Multimodality Imaging to Identify Patients at Risk for Prolonged Inotropic Support.

BACKGROUNDPost-pericardiectomy right ventricular (RV) failure has been reported but it remains not well-studied. To investigate imaging parameters that could predict RV function and the outcome of patients post-pericardiectomy.METHODSWe analysed data from a total of 53 CP patients undergoing pericardiectomy. Preoperative, early and at 6 months postoperative echocardiographic (echo) imaging datasets were analysed and correlated with preoperative cardiac magnetic resonance (CMR), cardiac computed tomography scans and histology. The primary endpoint of the study was RV functional status early postoperatively and at 6 months. Secondary endpoint was the need for prolonged inotropic support.RESULTSA cause of CP was identified in 26 patients (49%). Inotropic support ≥ 48 hours was required in n = 28 (53%) of patients and was correlated with lower preoperative RV areas by echo or RV volumes by CMR (p < 0.05 for all). A pericardial score based on pericardial thickness/calcification and epicardial fat thickness had good diagnostic accuracy to identify patients requiring prolonged use of inotropes (area under the curve, 0.825; 95% confidence interval, 0.674–0.976). Pericardiectomy resulted in RV decompression and impaired RV function early postoperatively (fractional area change: 40.5% ± 8.8% preoperatively vs. 31.4% ± 10.4% early postoperatively vs. 42.5% ± 10.2% at 6 months, p < 0.001).CONCLUSIONSWe show that a smaller RV cavity size and a pericardial scoring system are associated with prolonged inotropic support in CP patients undergoing pericardiectomy. RV systolic impairment post decompression is present in most patients, but it is only transient.

Serum Copper Level in Patients with Heart Failure and Its Related Factors

Background: Congestive Heart Failure (CHF) is a debilitating disorder that affects 1-2% of the general population. Despite recent advances in the management of heart failure, symptomatic heart failure still has a poor prognosis, so the importance of evaluating these patients is emphasized. The micronutrients are not produced in the body, but they should be taken in the required amount of diet, and their required amount is generally less than 100 mg per day. Copper deficiency leads to hypertrophic cardiomyopathies in several experimental models, which ultimately leads to systolic and diastolic cardiac function impairment. Copper deficiency is the only nutritional factor that increases cholesterol, blood pressure, homocysteine, and uric acid, leads to glucose intolerance, and increases thrombotic and oxidative damage. Objectives: The aim of this assessment is to examine the serum copper level in patients with Heart failure hospitalized in Rajaie Cardiovascular, Medical and Research Center and its related factors in the period of 2017-18. Methods: This as a prospective descriptive study conducted on patients with heart failure hospitalized in Rajaee Cardiovascular, Medical and Research Center. Research variables included serum copper level, Pro-BNP serum level, heart failure etiology, Seattle Heart Failure Model (SHFM), age, gender, BMI, history of diabetes, history of airway disease, duration of heart failure diagnosis, smoking, presence of shortness of breath, the amount of ejection fraction (EF), initial blood pressure, serum hemoglobin levels, white blood cell count, creatinine, cholesterol, sodium and uric acid. Accordingly, 80 patients with heart failure have been evaluated and the results of the evaluation of patients have been put together based on the aims of the study. Results: Of the evaluated cases, there were 52 (65%) male and 35 (28%) female. The mean and standard deviation of the patients' age was 49.9 ± 16.2 years, also the mean and standard deviation of serum copper was 189.28 ± 58.3 (range:80-150). The evaluated indices in this study included severe NYHA (P = 0.90), ejection fraction (EF) in echocardiography (P = 0.40), serum Pro BNP (P = 0.45) and prognosis of disease according to Seattle Heart Failure Model (P = 0.63), because non of the patients copper serum level were under the baseline we could not found statistically significant relationship between serum copper level and EF, pro BNP, NYHA function class and prognosis of the disease according to Seattle Heart failure model, while there was a significant statistical relationship between serum copper levels and etiology of heart failure (P = 0.02). Conclusions: Copper does not have a significant effect on the status of patients with heart failure, and only some differences were observed in the etiology of heart failure.

Abstract 16119: Dexrazoxane Role in the Prevention of Anthracycline Cardiotoxicity in Sarcoma Patients - A Meta-analysis

Introduction: Cardiotoxic side effects limit anthracycline use. Dexrazoxane has been proved cardioprotective in the setting of low-dose exposure, but its value in young sarcoma patients, who are treated with higher doses, remains elusive. Purpose: To perform a meta-analysis in order to appraise the efficacy and safety of dexrazoxane in sarcoma patients managed with anthracyclines. Methods: We systematically searched Embase, MEDLINE, Web of Science, Cochrane CENTRAL and Google Scholar databases, using the terms “sarcoma”, “anthracycline” and “dexrazoxane”, from inception to June 1, 2020. Studies targeting cardiotoxicity, primary cancer outcome (suboptimal response to treatment, disease progression and oncologic death), secondary malignancy and mortality were selected. The primary endpoint was a composite of heart failure, ventricular arrhythmias and left ventricular systolic deterioration. Therapeutic arms were those of dexrazoxane and no drug or placebo. A random-effects model with Mantel-Haenszel method was used to calculate pooled odds ratios (OR) or mean differences (MD) and their 95% confidence interval (CI). Results: We encompassed 11 studies, of which 5 were prospective. 9 studies were conducted in children and doxorubicin was the most represented anthracycline. 1574 patients were included, of whom 679 were under dexrazoxane. There were 73 primary endpoint events, 21 secondary malignant neoplasms and 29 deaths. Cumulative doxorubicin dose was higher in the dexrazoxane arm (MD 70.66mg/m2, 95% CI 46.43-94.89, i 2 24%). Nevertheless, this agent reduced the odds of the primary endpoint (OR 0.37, 95% CI 0.20-0.71, i 2 11%), as well as numerically decreased acute heart failure events (OR 0.15, 95% CI 0.02-1.09, i 2 0%) and significantly halted left ventricular ejection fraction deterioration (MD 5.25%, 95% CI 1.98-8.53, i 2 26%). On the other hand, primary cancer outcome and risk of secondary malignancy did not differ between the two arms (OR 1, 95% CI 0.47-2.15, i 2 0% and OR 0.98, 95% CI 0.39-2.47, i 2 0%, respectively). Likewise, all-cause mortality was similar (OR 1.65, 95% CI 0.68-4.00, i 2 0%). Conclusion: Dexrazoxane seems to maintain its efficacy and safety in young sarcoma patients managed with high-dose anthracyclines.

Myocardial work analysis in screening of familial dilated cardiomyopathy

Abstract Introduction Early detection of affected family relatives of patients with dilated cardiomyopathy (DCM) is essential in order to guide follow up, outcomes and initiate early treatment. Myocardial work analysis is a novel method which integrated strain imaging and blood pressure and has the potential to identify patients with subclinical disease. Purpose We analysed myocardial work in family relatives of DCM patients with positive genotype but negative phenotype in order to identify whether myocardial work can identify early changes. Methods Seventy-four family relatives of DCM patients attending for screening were examined. All individuals were asymptomatic with either positive (45/74, G+) or negative (29/74, G-) genotype and no echocardiographic evidence of left ventricular dilatation or systolic impairment. Non-invasive myocardial work analysis using two-dimensional (2D) speckle tracking echocardiography was analysed. Global longitudinal strain (GLS) was measured by the same vendor specific software used for myocardial work analysis. Left ventricular (LV) ejection fraction (EF) was measured with the Simpson's biplane method. The peak systolic arm cuff blood pressure (BP) measurement at the time of echocardiography was used for the myocardial work study. Results In total we included 74 individuals (37±15 years old, 50.7% women) with mean systolic and diastolic BP of 121.3±14 and 73.2±10 mmHg respectively, mean EF was 58±5% and mean GLS at 18.4±2.5%. G+ individuals had pathogenic and very likely pathogenic mutations in 8 different genes (TTN, BAG3, DSP, FLNC, LMNA, DMD, RBM20, TPM1). There was no difference in age, systemic hypertension, diabetes or medical treatment between the 2 groups. No significant difference was found among G+ and G- individuals in mean systolic and diastolic BP (121.2±14.7 vs 121.2±15.2 mmHg), mean EF (57.3±5 vs 59.1±4%), GLS (−18.2±1.5 vs −18.6±2.9%), mean global work index (1818±403 vs 1928±295 mmHg%) and global constructive work (2192±464 vs 2260±318 mmHg%). However, we found significant reduction of the global work efficiency (GWE) with a GWE of 94.4±2.7% in the G+ versus 95.9±1.6% in the G- individuals (p 0.02). Moreover, the global wasted work (GWW) was increased in the G+ with a GWW of 111±58 mmHg% versus 82±41 mmHg% in the G- individuals (p 0.03). Conclusion DCM gene carriers show, early on, decreased myocardial work efficiency and increased wasted work compared to unaffected family members, which appears to be earlier than other parameters such as EF and GLS. Myocardial work analysis could potentially recognize individuals showing early cardiac involvement and guide closer follow up and early initiation of treatment. Funding Acknowledgement Type of funding source: None

New onset left bundle branch block after transcatheter aortic valve implantation and the effect on long-term survival – a UK wide experience

Abstract Introduction New onset left bundle branch block (LBBB) is the most common conduction disturbance associated with transcatheter aortic valve implantation (TAVI). It has been shown to adversely affect cardiac function and increase re-hospitalisation, although its impact on mortality remains contentious. Methods We conducted an observational cohort analysis of all TAVI procedures performed by 13 heart teams in the United Kingdom from inception of their structural programmes until 31st July 2013. The primary outcome was 1-year all-cause mortality. Secondary outcomes included left ventricular ejection fraction (LVEF) at 30 days and need for a post-TAVI permanent pacemaker (PPM). Results 1785 patients were eligible for inclusion to the study. The primary analysis cohort was composed of 1409 patients with complete electrocardiographic (ECG) data pre- and post-TAVI. Pre-existing LBBB was present in 200 (14.2%) patients. New LBBB occurred in 323 (22.9%) patients post TAVI, which resolved in 99 (7%) patients prior to discharge. A balloon-expandable device was implanted in 968 (69%) patients, whilst 421 (30%) patients received a self-expandable valve. New LBBB was observed in 120 (12.4%) and 192 (45.6%) patients receiving a balloon- or self-expandable prosthesis respectively. Overall 1-year all-cause mortality post TAVI was 18.7%. New onset LBBB was not associated with an increase in 1-year all-cause mortality (p=0.416). Factors that were associated with mortality included an increasing logistic EuroScore (p=0.05), history of previous balloon aortic valvuloplasty (p=0.001), renal impairment (p=0.003), previous myocardial infarction with pre-existing LBBB (p=0.028) and atrial fibrillation (p=0.039). Lower baseline peak and mean AV gradients were also associated with greater mortality at 1 year (p=0.001), likely reflecting underlying left ventricular dysfunction. In the majority of patients, LVEF remained unchanged following TAVI. Interestingly, the presence or absence of new onset LBBB did not affect LVEF improvement at 30 days. 10% of patients required a PPM post TAVI. Predictors of PPM included new LBBB (OR 2.6, p&amp;lt;0.001), pre-TAVI left ventricular systolic impairment (OR 1.2, p=0.037), a self-expandable device (p&amp;lt;0.001), and pre-existing RBBB (OR 4.0, p&amp;lt;0.001). Conclusions These findings suggest that new onset LBBB post TAVI does not increase mortality at 1 year or adversely affect LVEF at 30 days. Funding Acknowledgement Type of funding source: None

Post-operative cardiac implantable electronic devices in patients undergoing cardiac surgery: a contemporary experience.

Optimum timing of pacemaker implantation following cardiac surgery is a clinical challenge. European and American guidelines recommend observation, to assess recovery of atrioventricular block (AVB) (up to 7 days) and sinus node (5 days to weeks) after cardiac surgery. This study aims to determine rates of cardiac implantable electronic devices (CIEDs) implants post-surgery at a high-volume tertiary centre over 3 years. Implant timing, patient characteristics and outcomes at 6 months including pacemaker utilization were assessed. All cardiac operations (n = 5950) were screened for CIED implantation following surgery, during the same admission, from 2015 to 2018. Data collection included patient, operative, and device characteristics; pacing utilization and complications at 6 months. A total of 250 (4.2%) implants occurred; 232 (3.9%) for bradycardia. Advanced age, infective endocarditis, left ventricle systolic impairment, and valve surgery were independent predictors for CIED implants (P < 0.0001). Relative risk (RR) of CIED implants and proportion of AVB increased with valve numbers operated (single-triple) vs. non-valve surgery: RR 5.4 (95% CI 3.9-7.6)-21.0 (11.4-38.9) CIEDs. Follow-up pacing utilization data were available in 91%. Significant utilization occurred in 82% and underutilization (<1% A and V paced) in 18%. There were no significant differences comparing utilization rates in early (≤day 5 post-operatively) vs. late implants (P = 0.55). Multi-valve surgery has a particularly high incidence of CIED implants (14.9% double, 25.6% triple valve). Age, left ventricle systolic impairment, endocarditis, and valve surgery were independent predictors of CIED implants. Device underutilization was infrequent and uninfluenced by implant timing. Early implantation (≤5 days) should be considered in AVB post-multi-valve surgery.

Is there heart disease in cases of neurodegeneration associated with mutations in C19orf12?

IntroductionIn mitochondrial membrane protein-associated neurodegeneration (MPAN), a subtype of neurodegeneration with brain iron accumulation (NBIA), patients suffer from optic nerve atrophy and dementia, which are also typical for another group of diseases, the mitochondrial diseases (MD). Around 30% of patients with MD have heart disease, commonly cardiomyopathy and arrhythmias, and 10% experience a major adverse cardiovascular event. The aim of this study was to assess cardiac involvement in MPAN. MethodsThirteen patients with MPAN were evaluated after written informed consent. All patients had echocardiography and 12 patients had 24-h Holter electrocardiogram (ECG) monitoring using 3-channel digital recorders. ResultsEchocardiography revealed normal values for the dimensions of all heart chambers. The systolic function of the left ventricle was normal in all cases. Right ventricle systolic impairment was found in three patients. 24-hour Holter ECG revealed predominant resting tachycardia during daytime with no physiological slowing of heart rate during sleep in seven cases. No significant arrhythmias were found. In nine patients, selected heart rate variability (HRV) parameters were lower than reference values. ConclusionCardiomyopathy, typical of MD, was not found in patients with MPAN. There were no significant arrhythmias, but disturbances in the circadian rhythm of the heart rate were observed in most cases. The decrease in HRV may reflect an early sign of autonomic dysfunction. A standard cardiac work-up is recommended for patients with MPAN to assess if additional treatment is needed.

Mitigating cardiac dysfunction by TOE-guided cardioplegia and mitral valve repair.

Based on current guidelines, 15% to 20% of patients undergoing mitral valve repair for regurgitation develop left ventricular dysfunction (ejection fraction < 50%-55%) despite a normal baseline. Two schools of thought have been debated: preexisting myocardial disease or suboptimal intraoperative myocardial protection. In our view, they could be reconciled. It is well recognized that left ventricular ejection fraction with a standard cut off at 50%-55% has limited sensitivity in detecting early systolic impairment in mitral regurgitation patients. Mitral regurgitation also leads to mitochondrial oxidative stress, thus rendering the myocardium more susceptible to ischemia-reperfusion injury and precipitating postoperative cardiac dysfunction. The fall in left ventricular ejection fraction early after mitral valve repair was shown to be caused by the reduction in both myocardial contractility and left ventricular stroke volume. To mitigate the risk to myocardial reperfusion injury, appropriate cardioplegia volume and distribution and well-defined surgical repair processes are equally important. We use transesophageal echocardiography-guided cardioplegia delivery, imaging the intramyocardial flow and ensuring adequate protection of the subendocardium during mitral valve repair. Mild aortic regurgitation on a beating heart often leads to left ventricular dilatation with diminished cardioplegia flow in the myocardium, thus requiring direct ostia cardioplegia. Systematic transesophageal echocardiography assessment before surgery is essential for establishing the mitral regurgitation mechanisms and translating them into precise surgical repair strategies. The benefits of transesophageal echocardiography-guided cardioplegia delivery warrant further clinical trials in order to evolve into part of a high surgical standard.

Assessing the left atrium of childhood cancer survivors.

Survivors of childhood cancer are at risk of cancer therapy-related cardiac dysfunction (CTRCD) characterized by systolic impairment, with little known about diastolic function. Left atrial strain (LAS) is a surrogate measure of left ventricular filling. We hypothesized that survivors (1) have lower LAS versus controls, and (2) survivors exposed to higher anthracycline dosage have even lower LAS. Cross-sectional study of 45 survivors exposed to anthracyclines ≥ 1year after chemotherapy and 45 healthy controls. Echo variables included mitral spectral and tissue Doppler, left ventricular ejection fraction (LV EF), LV dimension, LA volume, LV global longitudinal strain (GLS), and LAS. Peak strain (Ɛ) and strain rate (SR) at three phases were obtained: atrial contraction (ac), reservoir (res), and conduit (con). Two sub-analyses of cancer survivors were performed: (1) those with anthracycline dosage ≥ 250mg/m2, and (2) those with Ɛres in the lowest quartile. On the whole, survivors had lower Ɛres and Ɛcon values. The majority of survivors had relatively normal LAS, while a subset had very low LAS values and were more likely to be older. Survivors exposed to ≥ 250mg/m2 anthracycline also had lower Ɛres than those < 250mg/m2. There were no differences in mitral spectral/tissue Doppler, LV dimension, left atrial volume, or GLS. A subset of childhood cancer survivors have lower LAS than their healthy counterparts, while most are essentially normal. Those exposed to higher anthracycline dosage have even lower Ɛres. Longitudinal study of LAS may prove useful in monitoring for CTRCD.

Dilated Cardiomyopathy Concomitant with Acute Rheumatic Fever and Sporadic Hereditary Spherocytosis

Dilated cardiomyopathy (DCM) is a heart muscle disorder defined by the presence of a dilated and poorly functioning left ventricle in the absence of abnormal loading conditions (hypertension, valve disease) or ischaemic heart disease sufficient to cause global systolic impairment. A 5 years old and 6 months old girl with unremarkable cardiac history hospitalized for congestive heart failure due to dilated cardiomyopathy concomitant with acute rheumatic feverandsporadic hereditary spherocytosis.Acute rheumatic fever diagnosed based on the presentation of carditis, fever, high erythrocyte sedimentation rate, C-Reactive Protein (+), and antistreptolysin titer O (+).DCM was diagnosed after echocardiography. Sporadic hereditary spherocytosis was diagnosed based on anamnesis of pale and jaundice, splenomegaly on physical examination, hemolytic anemia, reticulocytosis, spherosit (+). Both parents reveals normal hematologic finding and the osmotic resistance test showed increasing of osmotic fragility. Prognosis of this patient remains worse because of inadequateleft ventricle-right ventricle (LV-RV) function and highly increasing The N-terminal prohormone of brain natriuretic peptide (NT-proBNP) level.

Functional and morphological cardiovascular alterations associated with neurofibromatosis 1

Subjects with Neurofibromatosis 1 (NF1) develop vascular complications. The protein product of the gene affected in NF1, neurofibromin, physiologically modulates endothelial function and preserves vascular and myocardial structure. Our study aimed to verify whether subjects with NF1 have early, preclinical abnormalities of carotid artery structure, brachial artery function, and cardiac function. We recruited 22 NF1 subjects without previous cardiovascular events and 22 healthy control subjects. All subjects underwent measurement of carotid artery intima-media thickness (IMT), evaluation of brachial artery endothelial function after ischemia and exercise, and cardiac function. Mean IMT was 543 ± 115 μ in NF1 subjects and 487 ± 70 μ in Controls (p < 0.01). Endothelial function was significantly dumped in NF1 subjects. The dilation after ischemia and exercise was respectively 7.5(± 4.8)% and 6.7(± 3.0)% in NF1 versus 10.5(± 1.2)% and 10.5(± 2.1)% in control subjects (p < 0.02; p < 0.002). Left ventricular systolic function assessed by Global Longitudinal Strain was significantly different between NF1 subjects and Controls: − 19.3(± 1.7)% versus − 21.5(± 2.7)% (p < 0.008). These findings demonstrate that NF1 patients have early morphological and functional abnormalities of peripheral arteries and systolic cardiac impairment and suggest the need for a tight cardiovascular risk evaluation and primary prevention in subjects with NF1.

“Missing” acute coronary syndrome hospitalizations during the COVID‐19 era in Greece: Medical care avoidance combined with a true reduction in incidence?

BackgroundReports from countries severely hit by the COVID‐19 pandemic suggest a decline in acute coronary syndrome (ACS)‐related hospitalizations. The generalizability of this observation on ACS admissions and possible related causes in countries with low COVID‐19 incidence are not known.HypothesisACS admissions were reduced in a country spared by COVID‐19.MethodsWe conducted a nationwide study on the incidence rates of ACS‐related admissions during a 6‐week period of the COVID‐19 outbreak and the corresponding control period in 2019 in Greece, a country with strict social measures, low COVID‐19 incidence, and no excess in mortality.ResultsACS admissions in the COVID‐19 (n = 771) compared with the control (n = 1077) period were reduced overall (incidence rate ratio [IRR]: 0.72, P < .001) and for each ACS type (ST‐segment elevation myocardial infarction [STEMI]: IRR: 0.76, P = .001; non‐STEMI: IRR: 0.74, P < .001; and unstable angina [UA]: IRR: 0.63, P = .002). The decrease in STEMI admissions was stable throughout the COVID‐19 period (temporal correlation; R2 = 0.11, P = .53), whereas there was a gradual decline in non‐STEMI/UA admissions (R2 = 0.75, P = .026) following the progressively stricter social measures. During the COVID‐19 period, patients admitted with ACS presented more frequently with left ventricular systolic impairment (22.2 vs 15.5% control period; P < .001).ConclusionsWe observed a reduction in ACS hospitalizations during the COVID‐19 outbreak in a country with strict social measures, low community transmission, and no excess in mortality. Medical care avoidance behavior is an important factor for these observations, while a true reduction of the ACS incidence due to self‐isolation/quarantining may have also played a role.

Dilated Cardiomyopathy Concomitant with Acute Rheumatic Fever and Sporadic Hereditary Spherocytocis

Dilated cardiomyopathy (DCM) is a heart muscle disorder defined by the presence of a dilated and poorly functioning left ventricle in the absence of abnormal loading conditions (hypertension, valve disease) or ischaemic heart disease sufficient to cause global systolic impairment. A 5 years old and 6 months old girl with unremarkable cardiac history hospitalized for congestive heart failure due to dilated cardiomyopathy concomitant with acute rheumatic fever and sporadic hereditary spherocytosis. Acute rheumatic fever diagnosed based on presentation of carditis, fever, high erythrocyte sedimentation rate, C-Reactive Protein (+) and anti-streptolysin titer O (+). DCM was diagnosed after echocardiography. Sporadic hereditary spherocytosis was diagnosed based on anamnesis of pale and jaundice, splenomegaly on physical examination, hemolytic anemia, reticulocytosis, sferosit (+). Both parents reveals normal hematologic finding and the osmotic resistance test showed increasing of osmotic fragility. Prognosis of this patient remain worse because of poor left ventricle-right ventricle (LV-RV) function and highly incerasing The N-terminal prohormone of brain natriuretic peptide (NT-proBNP) level.

Aetiological and morphological spectrum of cardiomyopathies in French Guiana: a retrospective study

IntroductionCardiomyopathies are a heterogeneous heart diseases group in terms of morphology and aetiology. Hypothesising a tropical specificity and given an absence of data in French Guiana, the primary objective of...

Body Composition in Young Adults Living With a Fontan Circulation: The Myopenic Profile.

BackgroundWe sought to characterize body composition abnormalities in young patients living with a Fontan circulation and explore potential pathophysiologic associations.Methods and ResultsTwenty‐eight patients with a Fontan circulation were prospectively recruited in this cross‐sectional study. Participants underwent cardiopulmonary exercise testing, dual‐energy X‐ray absorptiometry, echocardiography, and biochemical assessment. Mean age was 26±7 years. Skeletal muscle mass, estimated by appendicular lean mass index Z score, was reduced compared with reference data (−1.49±1.10, P<0.001). Percentage body fat Z score overall was within normal range (0.23±1.26, P=0.35), although 46% had elevated adiposity. Those with reduced skeletal muscle mass (appendicular lean mass index Z score of −1 or lower) had lower percent predicted oxygen pulse (55±15 versus 76±16%, P=0.002). Overall agreement between body mass index and dual‐energy X‐ray absorptiometry to assess adiposity was fair only (weighted [linear] κ coefficient: 0.53; 95% CI, 0.34–0.73) and slight in the setting of muscle mass deficiency (weighted κ coefficient: 0.32; 95% CI, 0.13–0.50). Appendicular lean mass was independently associated with absolute peak VO 2 (β=70.6 mL/min, P=0.001). Appendicular lean mass index Z score was inversely associated with hemoglobin (r=−0.4, P=0.04), and the degree of muscle deficit was associated with ventricular systolic impairment.ConclusionsYoung patients with a Fontan circulation have a body composition characterized by reduced skeletal muscle mass, which is associated with peak exercise capacity. Increased adiposity is common despite a normal body mass index. Low skeletal muscle mass is associated with systolic dysfunction and compensatory erythrocytosis.

The electrocardiogram in the diagnosis and management of patients with dilated cardiomyopathy.

The term dilated cardiomyopathy (DCM) defines a heterogeneous group of cardiac disorders, which are characterized by left ventricular or biventricular dilatation and systolic dysfunction in the absence of abnormal loading conditions or coronary artery disease sufficient to cause global systolic impairment. In approximately one third of cases, DCM is familial with a genetic pathogenesis and various patterns of inheritance. Although the electrocardiogram (ECG) has been considered traditionally non-specific in DCM, the recently acquired knowledge of the genotype-phenotype correlations provides novel opportunities to identify patterns and abnormalities that may point toward specific DCM subtypes. A learned ECG interpretation in combination with an appropriate use of other ECG-based techniques including ambulatory ECG monitoring, exercise tolerance test and imaging modalities, such as echocardiography and cardiovascular magnetic resonance, may allow the early identification of specific genetic or acquired forms of DCM. Furthermore, ECG abnormalities may reflect the severity of the disease and provide a useful tool in risk stratification and management. In the present review, we discuss the current role of the ECG in the diagnosis and management of DCM. We describe various clinical settings where the appropriate use and interpretation of the ECG can provide invaluable clues, contributing to the important role of this basic tool as cardiovascular medicine evolves.

Exercise Training Attenuates Cirrhotic Cardiomyopathy.

Cirrhotic cardiomyopathy is a condition where liver cirrhosis is associated with cardiac dysfunction. Triggers and blockers of cirrhotic cardiomyopathy are poorly understood, which might compromise the prognosis of chronic liver disease patients. We tested whether exercise training would reduce liver damage induced by thioacetamide and prevent liver cirrhosis-associated cardiomyopathy. Wistar rats were divided into three groups: control, thioacetamide (TAA), or TAA plus exercise. Thioacetamide increased liver weight and serum alanine aminotransferase and aspartate aminotransferase levels. Also, TAA treatment was involved with hepatic nodule formation, fibrotic septa, inflammatory infiltration, and hepatocyte necrosis. The exercise group presented with a reduction in liver injury status. We found that liver injury was associated with disordered cardiac hypertrophy as well as diastolic and systolic dysfunction. Exercise training attenuated cirrhosis-associated cardiac remodeling and diastolic dysfunction and prevented systolic impairment. These results provided insights that exercise training can mitigate cirrhotic cardiomyopathy phenotype. Graphical Abstract Exercise training attenuated liver injury as well as cirrhosis-associated cardiac remodeling and diastolic dysfunction and prevented systolic impairment.

P52 Two pathologies one heart: life-threatening lupus myocarditis in a patient with pre-existing dilated cardiomyopathy

Abstract Background Acute myocarditis is a life-threatening complication of systemic lupus erythematosus (SLE) often indicative of severe multisystem disease. Treatment of lupus myocarditis (LM) is well described in the literature with high-dose corticosteroids and cyclophosphamide providing the backbone of induction therapy. Less commonly reported are outcomes of patients with SLE and pre-existing structural heart disease. Methods We present a case of acute LM occurring in the context of established non-ischaemic dilated cardiomyopathy (DCM) presenting a significant diagnostic challenge. Results A 45-year-old woman from Afghanistan with known SLE presented with acute chest pain and dyspnoea. She had a previous mild SLE disease course limited to her skin and joints and was historically non-compliant with immunosuppressive treatment. She also had non-ischaemic DCM diagnosed six years prior to presentation with non-progressive imaging over this time. Upon acute assessment at the referring centre a widespread livedoid rash and cool peripheries were noted. Initial investigations revealed an elevated Troponin-I of 111ng/L and Brain Natriuretic Peptide (BNP) of 4497ng/L. An electrocardiogram revealed no acute ischaemic changes whilst her chest radiograph revealed features in keeping with pulmonary oedema. Transthoracic echocardiogram (TTE) demonstrated a dilated left ventricle with severe global systolic impairment. Her left ventricular ejection fraction (LVEF) was visually estimated at 10-15%, significantly reduced from her last recorded LVEF of 52% on a cardiovascular magnetic resonance (CMR) scan 18-months previously. Intravenous (iv) methylprednisolone, iv diuresis and B-cell depletion therapy were commenced prior to transfer to our department. Once transferred, a CMR scan confirmed an ejection fraction of only 12%. Septal mid-wall delayed enhancement was seen consistent with a non-ischaemic DCM, however, subepicardial delayed enhancement was visualised inferolaterally on native T1-mapping in keeping with perimyocarditis. Additional results on admission were supportive of a multisystem SLE flare. She was lymphopaenic (1.0x109/L), thrombocytopaenic (66x109/L), hypocomplimentaemic (C3 0.33g/L, C4 &amp;lt;0.03g/L), and had elevated dsDNA antibodies (83unit/mL). Serum creatinine had risen from a baseline of 47µmol/L to 110µmol/L and her urine protein: creatinine ratio (414mg/mmol) raised the suspicion of lupus nephritis (LN). Renal biopsy was not performed due to thrombocytopaenia. An MDT discussion with the cardiology, CMR imaging and renal teams took place with CMR imaging proving pivotal in establishing new LM on a background of established DCM. The decision to treat with intravenous (iv) cyclophosphamide was made and repeat TTE at 14 days revealed a significant improvement in LVEF to 25-30%. Conclusion CMR imaging was crucial in delineating acute and potentially reversible myocarditis on a background of chronic cardiomyopathy. This finding supported the escalation in immunosuppressive treatment to a cyclophosphamide-based regimen and led to the rapid improvement in ejection fraction in this young patient. She received 6 fortnightly doses of iv cyclophosphamide and is now maintained on hydroxychloroquine, mycophenolate mofetil and rituximab. Disclosures H. Wilson-Morkeh: None. T. Youngstein: None. S. Shabbir: None. J. Bakshi: None. T. Pihlajavaara: None. M. Bellamy: None. T. Cairns: None.

Difference in prevalence of exertional oscillatory ventilation between healthy subjects and patients with cardiovascular disease.

Exercise oscillatory ventilation (EOV) is an ominous sign in heart failure due to reduced left ventricular ejection fraction (HFrEF) whatever it is represented. But EOV is detected also in normal healthy individuals and in other cardiovascular disease (CVD) patients, however, its prevalence in these is not completed clear. The aim was to describe the occurrence of EOV in healthy subjects and the overall population all CVD patients who performing symptom limited cardiopulmonary exercise testing (CPET). Healthy subjects were divided in athletes and normal subjects, while, CVD patients were subdivided into: i) t hose with preserved left ventricular ejection fraction (LVEF); ii) those with mild to moderate impairment of LVEF (41-49%); iii) those with severe impairment of LVEF (≤40%); iv) HFrEF or with preserved LVEF (HFpEF); and iv) patients after heart transplantation (HXT). EOV was observed only in CVD patients and in those with depressed LVEF; the prevalence of EOV was observed 1.9% (3/55) those with mild to moderate impairment of LVEF (41-49%), 3.4% (56/1613) those with severe impairment of LVEF (≤40%), and 7.3% (214/2903) in HFrEF); no EOV was observed in CVD with preserved LVEF. Kremser's EOV was observed in patients, and, particularly, in those with systolic function impairment. Moreover, as EOV impacts prognosis in HFrEF, its occurrence can modify prognostic-decision models. Even though, EOV prevalence was derived from largest single center population, more studies are needed to tackle the EOV prevalence in different CVD conditions and in normal subjects.

Idiopathic Eosinophilic Myocarditis Presenting with Features of an Acute Coronary Syndrome

SummaryA 62-year-old female was admitted with severe left-sided chest pain, nausea and pre-syncope. She had widespread T wave inversion on ECG and elevated troponins and was suspected to have an acute coronary syndrome event. Invasive coronary angiogram revealed normal coronary anatomy with no flow-limiting lesions. Echocardiography and cardiac MRI revealed impaired left ventricular (LV) systolic impairment, a mobile LV apical thrombus and a moderate global pericardial effusion with no significant compromise. Full blood count analysis indicated the patient to have significant eosinophilia, and the patient was diagnosed with idiopathic eosinophilic myocarditis. She was commenced on Prednisolone and Apixaban, and eosinophil levels returned to normal after 10 days of steroids. Over the course of 3 months, the patient had a complete recovery of her LV function and resolution of the LV thrombus. This case highlights a rare, reversible case of idiopathic eosinophilic myocarditis which may present similar to acute coronary syndrome.