Early Diagnosis Of Cardiac Involvement Research Articles

Anderson-Fabry Disease: Red Flags for Early Diagnosis of Cardiac Involvement.

Anderson-Fabry disease (AFD) is a lysosome storage disorder resulting from an X-linked inheritance of a mutation in the galactosidase A (GLA) gene encoding for the enzyme alpha-galactosidase A (α-GAL A). This mutation results in a deficiency or absence of α-GAL A activity, with a progressive intracellular deposition of glycosphingolipids leading to organ dysfunction and failure. Cardiac damage starts early in life, often occurring sub-clinically before overt cardiac symptoms. Left ventricular hypertrophy represents a common cardiac manifestation, albeit conduction system impairment, arrhythmias, and valvular abnormalities may also characterize AFD. Even in consideration of pleiotropic manifestation, diagnosis is often challenging. Thus, knowledge of cardiac and extracardiac diagnostic "red flags" is needed to guide a timely diagnosis. Indeed, considering its systemic involvement, a multidisciplinary approach may be helpful in discerning AFD-related cardiac disease. Beyond clinical pearls, a practical approach to assist clinicians in diagnosing AFD includes optimal management of biochemical tests, genetic tests, and cardiac biopsy. We extensively reviewed the current literature on AFD cardiomyopathy, focusing on cardiac "red flags" that may represent key diagnostic tools to establish a timely diagnosis. Furthermore, clinical findings to identify patients at higher risk of sudden death are also highlighted.

P393 MULTIMODALITY IMAGING IN ACROMEGALIC CARDIOMIOPATHY: A CASE REPORT

Abstract Background Acromegaly is a disease caused by an excessive production of GH. Cardiac involvement, called acromegalic cardiomyopathy, is present in 90% of cases. Clinical Presentation A 56–year–old woman with acromegaly from pituitary adenoma, hypertension and obesity undewent angioCT showing thoracic aortic aneurysm (Figure 1). She was referred to a cardiological visit to evaluate the possible presence of acromegalic heart disease. The EKG showed signs of left ventricular hypertrophy (LVH) and so transthoracic echocardiogram (TTE) was prescribed: it showed slightly dilated, very hypertrophic left ventricle with ejection fraction of about 50% and signs of diastolic dysfunction. The exam also highlighted moderate to severe aortic valve regurgitation (Figure 2). In order to better characterize the cardiomyopathy a Cardiac magnetic resonance with contrast media (CMR) was performed: cine images confirmed severe left ventricular hypertrophy associated with mild dilatation, global biventricular hypertrophy and hypokinesia. After contrast media administration, a diffuse mild–enhancement pattern was found, a possible expression of diffuse interstitial fibrosis (Figure 3). In consideration of the dilatation of the ascending aorta and the contextual valve insufficiency, the patient was referred for cardiac surgery outpatient visit. Discussion The most frequent manifestations of acromegalic cardiomyopathy are bi–ventricular hypertrophy, the presence of diastolic and systolic dysfunction and the presence of valve regurgitation. Concentric hypertrophy is due to the increase in cardiomyocytes secondary to the GH stimulus and it is often bi–ventricular. Another characteristic finding is diffuse interstitial fibrosis with consequent systo–diastolic dysfunction and heart rhythm abnormalities. Aortic valve anomalies are linked to matrix abnormalities and myxoid degeneration. Conclusions this case can be considered an example of acromegalic heart disease. Multimodality imaging plays a key role in the early diagnosis of cardiac involvement and therefore it allows an early start of therapeutic strategy. Additionally, it is useful for the risk stratification of acromegalic patient.

Diagnosis of Simultaneous Atrial and Ventricular Mechanical Performance in Patients with Systemic Sclerosis.

Simple SummarySystemic Sclerosis (SSc) is a chronic connective tissue disorder with an inflammatory and autoimmune nature. The disease presents with microvascular changes, endothelial cell dysfunction, and fibrosis in visceral organs and tissues including lung, skin and heart. Cardiac involvement is a predictor of poor prognosis in this disease; therefore, early and pre-clinical diagnosis of cardiac involvement can be helpful in management of SSc. Two-dimensional Speckle Tracking Echocardiography (2D-STE) is a new method for the evaluation of myocardial strain in longitudinal, circumferential and radial planes. This study aimed at evaluating the mechanical performance of all cardiac chambers by using 2D-STE in patients with scleroderma, and its comparison with normal individuals. This study demonstrated that using 2D-STE can result in the diagnosis of impaired chamber mechanics and function in subclinical stages. Based on our findings, the simultaneous evaluation of all cardiac chambers by 2D-STE provides valuable information regarding myocardial involvement in patients with SSc.Objective: Cardiac involvement is a predictor of poor prognosis in patients with systemic sclerosis (SSc); therefore, preclinical diagnosis of heart involvement is crucial. Two-dimensional speckle tracking echocardiography (2D-STE), a method for evaluating the myocardial strain, could be helpful for the early diagnosis of cardiac mechanical function abnormalities. In this study, the simultaneous evaluation of all cardiac chambers was studied in patients with SSc, compared with normal individuals. Methods: The results of transthoracic echocardiography (TTE) and 2D-STE of 37 patients with SSc and 37 healthy individuals and the longitudinal strain (LS) of all chambers was precisely evaluated. The collected data were analyzed using SPSS version 16, and independent-sample t test and Chi-square test were used for comparison between the groups. Results: The mean ± SD of the participants’ age was 45.7 ± 11.54 (range of 17 to 68) years; most of them were women (75.7%). TTE showed higher left atrial (LA) volume (p < 0.001), right atrial (RA) area (p = 0.007), the severity of ventricular dysfunction (p < 0.05) and inferior vena cava diameter (p = 0.005), compared with the control group. Spectral and tissue Doppler echocardiography showed higher systolic pulmonary arterial pressure (sPAP) and mitral A wave velocity, and lower E/A ratio, E’ velocity of left ventricular (LV) septal and lateral wall in the case group (p < 0.05). Color Doppler echocardiography showed a higher frequency of valvular regurgitation in the case group (p < 0.05). The results of 2D-STE showed lower LA roof LS (p < 0.001), LA average LS (p = 0.015), LA global LS (p = 0.028), and LA ejection fraction (LAEF) (p = 0.001), lower mean RA left wall (p = 0.048) and EF (p < 0.001), and higher RV global LS in the case group (p = 0.025). Conclusions: Simultaneous evaluation of all cardiac chambers by 2D-STE provides valuable information about the myocardial involvement in patients with SSc. Therefore, it is suggested to use this method for the early diagnosis of cardiac involvement in such patients.

Predictive value of cardiac markers in the prognosis of COVID-19 in children.

Background and aimOccasionally, children with COVID-19 may develop arrhythmia, myocarditis, and cardiogenic shock involving multisystemic inflammatory syndrome in children (MIS-C). This study aimed to identify the laboratory parameters that may predict early cardiovascular involvement in these patients.Materials and methodsData of 320 pediatric patients, aged 0–18 years (average age, 10.46 ± 5.77 years; 156 female), with positive COVID-19 reverse transcription–polymerase chain reaction test and with cardiac biomarkers at the time of admission to the pediatric emergency department were retrospectively scanned. The age, sex, COVID-19-associated symptoms, pro-brain natriuretic peptide (proBNP), CK-MB, and troponin I levels of the patients were recorded.ResultsFever was noted in 58.1% of the patients, cough in 29.7%, diarrhea in 7.8%, headache in 14.7%, sore throat in 17.8%, weakness in 17.8%, abdominal pain in 5%, loss of taste in 4.1%, loss of smell in 5.3%, nausea in 3.4%, vomiting in 3.8%, nasal discharge in 4.4%, muscle pain in 5%, and loss of appetite in 3.1%. The proBNP value ≥282 ng/L predicted the development of MIS-C with 100% sensitivity and 93% specificity [AUC: 0.985 (0.959–1), P < 0.001]; CK-MB value ≥2.95 with 80% sensitivity and 77.6% specificity [AUC: 0.792 (0.581–1), P = 0.026]; and troponin I value ≥0.03 with 60% sensitivity and 99.2% specificity [AUC: 0.794 (0.524–1)].ConclusionsCardiac markers (proBNP and troponin I), especially proBNP, could be used to detect early diagnosis of cardiac involvement and/or MIS-C in pediatric patients with COVID-19 and to predict related morbidity and mortality.

Prevalence of transthyretin amyloidosis in patients with heart failure and no left ventricular hypertrophy.

AimsAs evidenced by scintigraphy imaging, the prevalence of transthyretin (TTR) cardiac amyloidosis in heart failure patients with preserved ejection fraction (HFpEF) and left ventricular hypertrophy (LVH) ranges between 13% and 19%. The natural evolution of cardiac amyloidosis begins with the deposition of amyloid material in the myocardium, with LVH ensuing at later stages. With current imaging modalities, it is possible to detect TTR cardiac amyloidosis before the hypertrophic stage. The aim of this study was to determine the prevalence of TTR cardiac amyloidosis in HFpEF patients without LVH.Methods and resultsThe study prospectively enrolled patients admitted for HF with LV ejection fraction (LVEF) ≥ 50% and LV wall thickness <12 mm. TTR cardiac amyloidosis was diagnosed according to accepted criteria, which include positive cardiac 99‐Tc‐DPD scintigraphy in the absence of monoclonal protein expansion in blood. Transthyretin gene sequencing was performed in positive patients. From July 2017 to January 2020, 329 patients with HFpEF and LV thickness <12 mm were identified. After exclusions, 58 patients completed the study with cardiac scintigraphy (79 years, 54% men; median LVEF 60% and LV wall thickness 10.5 mm). Three patients (5.2%) were positive for TTR cardiac amyloidosis; genetic analysis excluded the presence of hereditary TTR amyloidosis. Positive patients baseline characteristics (84 years, 67% men, LVEF 60%, and LV wall thickness 11 mm) were similar to patients without TTR, except for troponin levels (0.05 vs. 0.02 ng/mL, P = 0.03) and glomerular filtration rate (82 vs. 60 mL/min, P = 0.032), which were higher in TTR patients.ConclusionsIn a cohort of patients with HFpEF without LVH, the prevalence of TTR cardiac amyloidosis was 5%. Early diagnosis of cardiac involvement in TTR amyloidosis (before manifest LVH) would seem recommendable because newly approved specific treatments can prevent additional deposition of amyloid material.

The assessment of high sensitivity cardiac troponin in patients with COVID-19: A multicenter study.

BackgroundRecent studies have shown that patients diagnosed with coronarivus disease 2019 (COVID-19) and also with previous cardiovascular diseases have a higher mortality due to worsening heart disease. At the same time, patients without previous cardiovascular disease may also have cardiac complications. The aim of this multicenter study was to assess high sensitivity cardiac troponin T (hs-cTnT) in patients with COVID-19 and to evaluate the incidence of myocardial injury.MethodsIn this multicenter study we enrolled 543 patients, 57.8% males, median age 63 years (range 18–99) from three selected hospitals: University Hospital Tor Vergata in Rome, Fondazione IRCCS Ca 'Granda Ospedale Maggiore Policlinico, in Milan, S Chiara Hospital in Trento. We measured hs-cTnT in all patients to assess myocardial injury and correlations with patient’s age, symptoms and disease course.ResultsThe data showed that, among the 543 patients studied, 257 patients (47.3%) had hs-cTnT values higher than the upper reference limit (URL) of 14 ng/L. Patients with high hs-cTnT had more frequently fever (p < 0.01) and respiratory symptoms (p < 0.01), compared to the group with hs-cTnT values below URL. The results showed also that patients with hs-cTnT above URL had a higher frequency of previous cardiovascular disease (p < 0.01) as well as of hypertension (p < 0.01). Instead, among 231 patients with no previous cardiovascular disease, 81 (31.5%) had hs-cTnT values above the URL. Finally. the majority of the patients with high hs-cTnT were admitted to the intensive care unit (p < 0.01).ConclusionOur data suggest the assessment of high sensitivity cardiac troponin in patients with COVID-19 for early diagnosis of cardiac involvement.

The Role of Cardiac MRI in the Diagnosis of Fabry Disease

파브리병(Fabry disease)은 매우 드문 X-연관 유전 대사 질환으로 알파 갈락토시다아제(alpha galactosidase A)의 결핍으로 인하여 다양한 세포 및 기관에 글리코스핑고지질(glycosphingolipid) 의 축적을 초래하는 질환이다. 심장 침범이 비교적 흔하며 비정상적인 지질침착으로 인한 심근 염증, 좌심실 비대 및 심근 섬유증을 일으킨다. 심장 침범은 환자 예후를 결정하는 중요한 요인이므로 이를 진단하는 것은 매우 중요하다. 심장 자기공명영상은 심실의 기능, 부피 측정을 위한 표준기법으로 알려져 있으며 심근의 조직 변화를 볼 수 있는 유용한 기법이다. 특히 최근 많이 쓰이는 T1 지도화 기법을 통한 심근 조영 전 T1 수치를 이용하여 파브리병의 심장 침범을 조기 진단할 수 있으며 자기공명영상을 이용한 심근 질량 측정으로 치료 모니터링을 할 수 있다. 심장 자기공명영상은 파브리병 환자에서 다양한 역할을 할 수 있을 것으로 생각되며 이에 대해 정리해보고자 한다.

Acute respiratory distress reveals a mitral endocarditis and complete a case of pneumococcal infection of Austrian syndrome

Introduction: Infectious endocarditis caused by Streptococcus pneumonia remains rare but serious. When it is associated with meningitis and pneumonia, it is called Austrian syndrome. Case Report: The authors describe a case of a 66-year-old female Moroccan patient who presented a congestive heart failure due to mitral valve disease after few days of hospitalization for pneumococcal meningitis associated with pneumonitis. The patient had a favorable course after combination of antibiotic therapy followed by surgical valve replacement. Conclusion: The recognition of this syndrome allows an early diagnosis of cardiac involvement often masked and which worsens the prognosis. Mortality can be lowered by appropriate medico-surgical management.

The Added Value of Cardiac Magnetic Resonance in Muscular Dystrophies.

Muscular dystrophies (MD) represent a heterogeneous group of rare genetic diseases that often lead to significant weakness due to progressive muscle degeneration. In many forms of MD, cardiac manifestations including heart failure, atrial and ventricular arrhythmias and conduction abnormalities can occur and may be a predominant feature of the disease. Cardiac magnetic resonance (CMR) can assess cardiac anatomy, global and regional ventricular function, volumes and mass as well as presence of myocardial inflammation, infiltration or fibrosis. The role for cardiac MRI has been well-established in a wide range of muscular dystrophies related cardiomyopathies. CMR is a more sensitive technique than echocardiography for early diagnosis of cardiac involvement. It has also great potential to improve the prediction of long-term outcome, particularly the development of heart failure and arrhythmic events; however it still has to be validated by longitudinal studies including large populations. This review will outline the utility of CMR in patients with muscular dystrophies for assessment of myocardial involvement, risk stratification, and in guiding therapeutic management.

Prognostic significance of ventricular late potentials in patients with pulmonary sarcoidosis

Early detection of cardiac involvement in sarcoidosis is difficult but essential to achieve optimal treatment. Signal-averaged electrocardiography (SAECG) can detect subtle cardiac electrical abnormalities termed late potentials (LPs) and would be useful for the early diagnosis of cardiac involvement. This study aims to investigate the prognostic significance of LP in patients with pulmonary sarcoidosis. We prospectively studied 74 patients with pulmonary sarcoidosis without overt electrocardiographic abnormalities. All participants underwent SAECG, cardiac echocardiography, and 24-hour ambulatory Holter monitoring. Serum angiotensin-converting enzyme and B-type natriuretic peptide levels were also evaluated. We followed these patients for the evaluation of incidence of cardiac events including cardiac death, arrhythmias, and heart failure requiring hospital admission. Of the studied population, 29 patients (39.2%) had detectable LP. During a mean follow-up period of 9.8 years, 8 patients with LPs had cardiovascular events, including development of complete atrioventricular block (n = 4), ventricular tachycardia (n = 2), and heart failure (n = 2). Meanwhile, only 1 of 45 patients without LP developed cardiac event (heart failure). Multivariate analyses revealed that LPs were associated with an increased risk of developing cardiac events (hazard ratio 9.66; 95% confidence interval 1.20-78.01; P = .033) whereas age, sex, serum angiotensin-converting enzyme and B-type natriuretic peptide levels, number of premature ventricular contractions on 24-hour Holter monitoring, and echocardiographic parameters were not associated with subsequent cardiac events. SAECG might possibly be useful for the early detection of cardiac sarcoidosis and, if independently validated, could eventually be considered as a screening test for further risk stratification.

Early diagnosis of cardiac involvement in systemic lupus erythematosus via global longitudinal strain (GLS) by speckle tracking echocardiography.

Introduction: Systemic lupus erythematosus (SLE) myocarditis occurs in between 5% and 10% of patients with lupus. Global longitudinal strain (GLS) via speckle tracking echocardiography can detect cardiac involvement in patients suffering from SLE. We decided to determine the echocardiographic features and subsequent early diagnosis of cardiac involvement in patients with SLE utilizing the GLS index via speckle tracking echocardiography. Methods: In this cross-sectional study, we compared female patients with SLE of at least 2 years’ duration and healthy controls in terms of the left ventricular (LV) GLS via speckle tracking echocardiography. After data collection in both groups, the GLS index and the ejection fraction were evaluated. Results: We analyzed and compared the LV echocardiographic parameters of 33 patients with SLE (mean age=25.45±0.63 years) with those of 35 healthy controls (mean age=27±0.45 years). The apical 2-chamber view indicated a significant decrease in the LV GLS in the case group by comparison with the healthy controls (P=0.005). The LV GLS in the apical 3-chamber view was significantly lower in the case group than in the control group (P=0.006). The LV GLS in the apical 4-chamber view revealed no significant difference between the case and healthy control groups (P=0.2). While there was a significant difference between the case and control groups visà- vis the LV GLS (P=0.02), the LV ejection fraction measured with the Simpson method showed no significant difference between the 2 groups (P=0.96). Conclusion: GLS speckle tracking echocardiography is a noninvasive method with diagnostic and prognostic values; it may, therefore, be a sensitive marker for the diagnosis of myocarditis and other cardiac involvements in patients with SLE.

Autoimmune myocarditis in systemic sclerosis: an unusual form of scleroderma heart disease presentation

Primary cardiac involvement in systemic sclerosis is common, often subclinical, and is associated with significant mortality. We report the case of a patient who developed autoimmune myocarditis at an early stage of systemic sclerosis, who completely recovered from cardiac dysfunction under optimal medical therapy for heart failure and immunosuppression. This challenging case aims at increasing awareness around the fact that the heart is a target organ of scleroderma disease. It also highlights the importance of screening and early diagnosis of cardiac involvement, because a timely treatment may impact the quality of life of these patients and improve their prognosis.

The subclinical effect of celiac disease on the heart and the effect of gluten-free diet on cardiac functions.

This study aimed to investigate the effects of celiac disease (CD) on cardiac function in children by using conventional transthoracic echocardiography (TTE) and tissue Doppler echocardiography (TDE). Forty-nine patients diagnosed with CD were enrolled into the study. Group 1 consisted of 26 (53%) patients who had recently been diagnosed and had not been on gluten-free diet whereas Group 2 consisted of 23 (47%) patients who had been on regular gluten-free diet for at least 10 months. 20 healthy children were enrolled into the study as the control group. The deceleration time (DT) and the left ventricle (LV) isovolumetric relaxation time (IVRT) were significantly shorter in Group 1 compared to Group 2 and the control group (p=0.002, p=0.015). Mitral valve E/E' ratio was significantly lower in Group 1 and 2 when compared to the control group (p < 0.001). The study demonstrated that evaluation of these parameters by using TDE could be beneficial in the early diagnosis of cardiac involvement in children with CD.

An unusual reason for complete atrioventricular block ; Kearn Sayre Syndrome

Kearn sayre syndrome (KSS) is a rare disease with a typical onset before 20 years and charactarized by triad of chronic progressive external ophthalmoplegia, retinitis pigmentosa and progressive cardiac conduction abnormalities. The most important prognostic factor in KSS patients is the involvement of heart characterized by progressive degeneration of the conduction system. In patients with KSS, early diagnosis of cardiac involvement is very important because complete atrioventricular (AV) block may develop and it can be cause of sudden cardiac death. Herein we report a patients with KSS who presented with syncope due to complete AV block .

New tools to diagnose and follow FAC patients: biomarkers

In the last decade, the availability of the cardiac biomarkers N terminal pro-natriuretic peptide type-B (NT-proBNP) and troponins has radically changed the approach to diagnosis, staging, and response assessment of cardiac amyloidosis. Despite similar appearance at standard imaging, the main types of amyloidosis involving the heart are characterized by different rates of progression and different outcomes. In general, patients with ATTRm amyloidosis have lower concentrations of cardiac biomarkers and usually a better prognosis than subjects suffering from AL amyloidosis. Nevertheless, the clinical manifestations of ATTRm amyloidosis are heterogeneous. Different amyloidogenic TTR mutations give rise to different cardiac phenotypes, ranging from exclusively neuropathic diseases, through mixed phenotypes, to mutation characterized by severe cardiac dysfunction. For instance, one of the mutations associated with cardiac involvement in ATTRm amyloidosis, Ile68Leu, presents with high NT-proBNP concentrations, comparable to those observed in patients with cardiac AL amyloidosis. In our series, survival of patients with Ile68Leu ATTRm is not different from that of cardiac AL patients. Thus, it is possible that NT-proBNP represents a viable marker for early diagnosis of cardiac involvement in ATTR patients with “aggressive” mutations. This marker could be considered in the screening of carriers of these mutations. Despite the lack of systematic studies of cardiac biomarkers in ATTR amyloidosis, the results of several small published series and the analysis of the Pavia patient population, indicate that NT-proBNP and cardiac troponins have prognostic value in this disease, correlating to amyloid burden and adding to known prognostic factors. The availability of several novel therapeutic options for ATTRm, require objective means for the assessment of treatment efficacy in routine clinical practice and in clinical trials. Early results from the Pavia series indicate that NT-proBNP progression portends a poor prognosis, and cardiac biomarkers will probably find their place in the evaluation of response to therapy in ATTRm amyloidosis. Cardiac biomarkers will most likely become increasingly useful in the management of patients with ATTRm amyloidosis in the near future. However, studies of biomarkers in this disease are hindered by small numbers and disease heterogeneity. Prospective studies and international collaboration are warranted to define the role of cardiac biomarkers in the diagnosis and follow-up of cardiac ATTRm amyloidosis.

Cardiac involvement in Churg-Strauss syndrome

Churg-Strauss syndrome, recently renamed eosinophilic granulomatosis with polyangiitis (EGPA), is a rare form of systemic vasculitis, characterized by disseminated necrotizing vasculitis with extravascular granulomas occurring among patients with asthma and tissue eosinophilia. EGPA is classified as a small and medium-sized vessel vasculitis associated with antineutrophil cytoplasmic antibodies (ANCA) and the hypereosinophilic syndrome. Typical clinical features include asthma, sinusitis, transient pulmonary infiltrates and neuropathy. Blood eosinophils are often >1500/µl or more than 10% on the differential leukocyte count. Blood eosinophils should always be tested in unexplained cardiac disorders, and may normalize even after low doses of corticosteroids. ANCA are positive in 40-60% of cases, mainly anti-myeloperoxidase. Heart involvement occurs in approximately 15-60% of EGPA patients, especially those who are ANCA negative. Any cardiac structure can be involved, and patients present with myocarditis, heart failure, pericarditis, arrhythmia, coronary arteritis, valvulopathy, intracavitary cardiac thrombosis. Although cardiovascular involvement is usually an early manifestation, it can also occur later in the course of the disease. A significant proportion of patients with cardiac involvement is asymptomatic. In the absence of symptoms and major ECG abnormalities, cardiac involvement may be detected in nearly 40% of the patients. All patients with EGPA should be studied not only with a detailed history of cardiac symptoms and ECG, but also with echocardiography; if abnormalities are detected, a cardiac magnetic resonance study should be performed. Coronary angiography and endomyocardial biopsy should be reserved to selected cases. Heart involvement carries a poor prognosis and causes 50% of the deaths of these patients. It is often insidious and underestimated. Optimal therapy is therefore important and based on high-dose corticosteroids plus immunosuppressive agents, particularly cyclophosphamide in case of myocardial inflammation. Thus, early diagnosis of cardiac involvement and subsequent therapy may prevent progression of cardiac disease. At present, the role of troponin and brain natriuretic peptide in monitoring and therapy remains unclear. Orthotopic heart transplantation is feasible in case of severe disease, even if the experience is limited in -EGPA, and optimal post-transplantation immunosuppressive strategy has yet to be defined.

Early diagnosis of cardiac involvement in idiopathic inflammatory myopathy by cardiac magnetic resonance tomography.

The aim of this study was to investigate cardiac involvement in patients with idiopathic inflammatory myopathies excluding inclusion body myositis with cardiac magnetic resonance tomography (CMR). A case series of 53 patients with polymyositis, dermatomyositis, or non-specific myositis underwent CMR including functional imaging, T1-weighted, and late gadolinium enhancement (LGE) imaging. T1-weighted and LGE images were analyzed for myocardial enhancement. Reduced left ventricular function (LVF) was found in 9 (7%) patients. Patients with reduced LVF more often presented with early and late myocardial enhancement (p = 0.014 and p = 0.001). In 33 (62.3%) patients, LGE was observed by CMR. These patients had significantly lower left ventricular ejection fractions (p < 0.001) compared to patients without LGE. LGE was mainly present in the lateral (p < 0.01) and inferior (p < 0.02) segments. No correlations of LGE presence or reduced LVF to cardiovascular risk factors were found. Myocardial inflammation is very frequent in polymyositis, dermatomyositis, and non-specific myositis. In our patient, cohort CMR demonstrated signs of myocardial inflammation in 62.3%. CMR seems to offer a measurable and quantifiable diagnostic tool for cardiac involvement of idiopathic inflammatory myopathies and can thus be used to monitor disease progress and therapeutic success in these patients.

Comprehensive myocardial tissue characterization with cardiac magnetic resonance in patients with Churg Srauss Syndrome

Background Churg-Strauss syndrome (CSS) is a rare systemic necrotizing vasculitis characterized by asthma, hypereosinophilia, cardiac failure, renal damage and peripheral neuropathy. CSS typically develops in three clinical phases, beginning with asthma, followed by tissue eosinophilia and finally in a systemic small-vessel vasculitis. During the late phase, coronary vessels and myocardium are interested leading to a variety of clinical manifestations such as myocarditis, heart failure, pericarditis and coronary vasculitis. Heart involvement is found in over 50% of autopsied CSS patients with evidence of an extremely high mortality in those with cardiac disease left untreated. Cardiac MR has emerged as a promising technique not only for the early diagnosis of cardiac involvement in the setting of this insidious and complex condition, but also for a better risk stratification and tailoring of immunosuppressive therapy. Methods

Cardiac troponin‐I on diagnosis predicts early death and refractoriness in acquired thrombotic thrombocytopenic purpura. Experience of the French Thrombotic Microangiopathies Reference Center

Cardiac involvement is a major cause of mortality in patients with thrombotic thrombocytopenic purpura (TTP). However, diagnosis remains underestimated and delayed, owing to subclinical injuries. Cardiac troponin-I measurement (cTnI) on admission could improve the early diagnosis of cardiac involvement and have prognostic value. To assess the predictive value of cTnI in patients with TTP for death or refractoriness. The study involved a prospective cohort of adult TTP patients with acquired severe ADAMTS-13 deficiency (< 10%) and included in the registry of the French Reference Center for Thrombotic Microangiopathies. Centralized cTnI measurements were performed on frozen serum on admission. Between January 2003 and December 2011, 133 patients with TTP (mean age, 48 ± 17 years) had available cTnI measurements on admission. Thirty-two patients (24%) had clinical and/or electrocardiogram features. Nineteen (14.3%) had cardiac symptoms, mainly congestive heart failure and myocardial infarction. Electrocardiogram changes, mainly repolarization disorders, were present in 13 cases. An increased cTnI level (> 0.1 μg L(-1) ) was present in 78 patients (59%), of whom 46 (59%) had no clinical cardiac involvement. The main outcomes were death (25%) and refractoriness (17%). Age (P = 0.02) and cTnI level (P = 0.002) showed the greatest impact on survival. A cTnI level of > 0.25 μg L(-1) was the only independent factor in predicting death (odds ratio [OR] 2.87; 95% confidence interval [CI] 1.13-7.22; P = 0.024) and/or refractoriness (OR 3.03; 95% CI 1.27-7.3; P = 0.01). A CTnI level of > 0.25 μg L(-1) at presentation in patients with TTP appears to be an independent factor associated with a three-fold increase in the risk of death or refractoriness. Therefore, cTnI level should be considered as a prognostic indicator in patients diagnosed with TTP.

Multimodality Imaging in Cardiooncology.

Cardiotoxicity represents a rising problem influencing prognosis and quality of life of chemotherapy-treated patients. Anthracyclines and trastuzumab are the drugs most commonly associated with development of a cardiotoxic effect. Heart failure, myocardial ischemia, hypertension, myocarditis, and thrombosis are typical manifestation of cardiotoxicity by chemotherapeutic agents. Diagnosis and monitoring of cardiac side-effects of cancer treatment is of paramount importance. Echocardiography and nuclear medicine methods are widely used in clinical practice and left ventricular ejection fraction is the most important parameter to asses myocardial damage secondary to chemotherapy. However, left ventricular ejection decrease is a delayed phenomenon, occurring after a long stage of silent myocardial damage that classic imaging methods are not able to detect. New imaging techniques including three-dimensional echocardiography, speckle tracking echocardiography, and cardiac magnetic resonance have demonstrated high sensitivity in detecting the earliest alteration of left ventricular function associated with future development of chemotherapy-induced cardiomyopathy. Early diagnosis of cardiac involvement in cancer patients can allow for timely and adequate treatment management and the introduction of cardioprotective strategies.