Cardiomyopathies Research Papers - Academia.edu (original) (raw)

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- Menstruation, Electrocardiography, Sudden Death, Progesterone
- by Ali Yilmaz
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- Adolescent, Extracellular Matrix, Electrocardiography, Echocardiography

Apoptotic myocyte cell death, diastolic dysfunction and progressive deterioration in LV pump function, characterize the clinical course of diabetic cardiomyopathy. A key question concerns the mechanism(s) by which hyperglycemia (HG)-transmits danger signals in cardiac muscle cells (CMC). The growth factor adapter protein, p66ShcA is a genetic determinant of longevity, which controls mitochondrial metabolism and cellular responses to oxidative stress. Here, we demonstrate interventions that attenuate or prevent HG-induced phosphorylation at critical position 36 Ser residue (phospho-Ser-36), inhibit the redox function of p66ShcA and promote the survival phenotype. Adult rat ventricular myocytes (ARVM) obtained by enzymatic dissociation were transduced with mutant36 p66ShcA (mu-36) dominant negative expression vector and plated in serum free media (SFM) containing 5 mM or 25 mM glucose. At HG, ARVM exhibit marked increase in reactive oxygen species (ROS) production, upregulation of phospho-Ser-36, collapse of mitochondrial transmembrane potential (Δψm) and increased formation of p66ShcA/cytochrome c complexes. These indices of oxidative stress were accompanied by 40% increase in apoptosis and upregulation of cleaved caspase 3 and the apoptosis related proteins p53 and Bax. To test if p66ShcA functions as a redox sensitive molecular switch in vivo, we examined hearts of male Akita diabtic nonobese (C57BL/6J) mice. Immunoblot analysis detected upregulation of phospho-Ser-36, translocation of p66ShcA to mitochondria and formation of p66ShcA/cytochrome c complexes. Conversely, correction of HG by adenoassociated viral delivery of leptin

- by Himanshu Dube
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- Physiology, Oxidative Stress, Apoptosis, Hyperglycemia

Myocardial strain (⑀) is a dimensionless index of change in myocardial length in response to an applied force. ⑀ Rate (SR) is the rate of change of length and is usually obtained as the time derivative of the ⑀ signal. In echocardiography, SR is calculated as the difference between 2 velocities normalized to the distance between the 2 velocities. SR imaging (SRI) has a theoretic advantage over Doppler tissue imaging in that SRI is relatively immune to cardiac translational motion and tethering. Therefore, SRI may be superior to Doppler tissue imaging in quantitative assessment of regional myocardial function and may find clinical application in the interrogation of coronary artery disease. The high frame rates of SRI have also renewed interest in timings of global and regional mechanical events, and their potential clinical applications. The high temporal resolution allows SRI to depict regional systolic and diastolic asynchrony. Ongoing clinical trials will determine the sensitivity, specificity, and accuracy of SRI parameters for a variety of clinical conditions. Potential clinical applications include investigation of ischemia (at rest and with stress), myocardial viability, and altered global and regional systolic and diastolic function in cardiomyopathies. Suboptimal signal quality remains a major limitation of strain imaging, and advances in data acquisition and postprocessing capabilities will help determine its future incorporation into standard regional myocardial assessment. (J Am Soc Echocardiogr 2003;16:1334-42.) Mirsky and Parmley, 1 first introduced the concept of strain (⑀) and strain rate (SR) as measures of myocardial mechanical properties. Myocardial strain is a dimensionless index of change in myocardial length in response to an applied force and is expressed as fractional or percent change . SR is the time derivative of strain with unit of per second (s Ϫ1 ) and in ultrasound represents the differential velocity of 2 points normalized for the distance between them ). By convention, myocardial lengthening or thinning gives a positive strain value and shortening or thickening gives a negative value. For 1-dimensional strain ⑀ ϭ ⌬L L 0 and corresponding SR

- by Theodore Abraham and +1
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- Clinical Trial, Magnetic Resonance Imaging, Temporal Resolution, Echocardiography

Background: Mitral annular calcification (MAC) is common, particularly in the elderly. While thought to occasionally produce significant mitral regurgitation, it is considered a rare cause of mitral stenosis. Methods: Echocardiogram reports from general cardiology outpatients were searched for phrases regarding severe MAC and, separately, for mitral stenosis. Rheumatic disease or other mitral valve (MV) pathology was excluded. Mean MV and aortic valve (AV) gradients were recorded. The presence or absence of anterior MAC was noted and a semi-quantitative assessment of anterior mitral leaflet (AML) mobility was made. Ten patients with annuloplasty rings served for comparison. Results: Group A (22 patients with moderately/severely reduced AML mobility) had a mean MV gradient of 7 mm Hg (range 3-14) vs. 3 mm Hg (range 1-5) in group B (21 patients with normal/mildly reduced AML mobility), p b 0.0001. Annuloplasty patients had a mean MV gradient of 3 mm Hg, p b 0.001 vs. group A but similar to group B. Mean AV gradient was 27 mm Hg (range 4-48) in group A vs. 14 mm Hg in group B (range 3-40), p = 0.013. No patient had more than mild mitral regurgitation. Conclusion: MAC producing a potentially important MV gradient is not rare in the general cardiology population. Reduced AML mobility appears to be necessary for a gradient N 5 mm Hg. Significant AV gradients are commonly associated, reflecting greater overall cardiac calcification. These patients can be easily identified by looking for reduced AML mobility as part of widespread cardiac calcification.

- by salman mac
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- Cardiology, Mitral Valve Repair, Aged, Calcinosis

Cirrhotic cardiomyopathy is a clinical syndrome in patients with liver cirrhosis characterized by an abnormal and blunted response to physiologic, pathologic, or pharmacologic stress but normal to increased cardiac output and contractility at rest. As many as 50% of cirrhotic patients undergoing liver transplantation show signs of cardiac dysfunction, and 7% to 21% of deaths after orthotopic liver transplantation result from overt heart failure. In this review, we critically evaluate the existing literature on the pathophysiology and clinical implications of cirrhotic cardiomyopathy. (J Am Coll Cardiol 2010;56:539-49)

- by Aldo Dobrina
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- Hepatology, Electrocardiography, Portal hypertension, Liver Cirrhosis

Background. Several studies have demonstrated that patients affected by heart failure have a compromised quality of life and, in the last few years, "health-related quality of life" has become an important outcome indicator for the evaluation of heart failure treatment and a basis for the improvement of its strategies. Methods. The translation into Italian of the Kansas City Cardiomyopathy Questionnaire (KCCQ), a new, 23 item, disease-specific health status instrument for patients with congestive heart failure, and its subsequent validation by asking 50 consecutive patients in our heart failure outpatient clinic to answer it. The KCCQ was compared to the "Minnesota Living with Heart Failure Questionnaire" (MLHF). Results. The Italian version of the KCCQ correlates well with the MLHF for all domains with the exclusion of symptom stability score and MLHF emotional domain. However, the KCCQ, due to its multiple domains, provided more detailed information about the patients' status, and identified the more compromised ones. Conclusions. The KCCQ appears to be a valid and reliable instrument for the assessment of a patient's quality of life and the degree of limitations imposed upon him/her by the disease. When compared to the MLHF, the KCCQ, however, is somewhat more sensitive in identifying more compromised patients. This capacity could be advantageously used for the identification of clinical changes in future trials and lead to a better planning of new therapeutic interventions.

- by Claudio Fresco
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- Quality of life, Italy, Heart Failure, Self Efficacy
- by Gerard Aurigemma and +1
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- Literature Review, Diabetes mellitus, Hemodynamics, Aortic stenosis

Class IC antiarrhythmic drugs (IC-AADs) can effectively suppress premature ventricular contractions (PVCs). However, IC-AADs increase mortality in patients with PVCs and left ventricular dysfunction after myocardial infarction. Whether IC-AADs can be safely used to treat premature ventricular contraction-induced cardiomyopathy (PVC-CM) remains to be established. The purpose of this study was to determine the safety and efficacy of IC-AADs in patients suspected of having PVC-CM. The electronic medical records at the Hospital of the University of Pennsylvania were screened to identify all patients suspected of having PVC-CM treated with flecainide or propafenone. Clinical, electrocardiographic, and imaging studies were reviewed. Twenty patients suspected of having PVC-CM were treated with IC-AADs. Patients had undergone an average of 1.3 ± 0.2 previous unsuccessful ablations. Six had an implantable or wearable defibrillator. With IC-AAD treatment, mean PVC burden decreased from 36.2% ...

- by David Callans
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- Biomedical Engineering, Electrocardiography, Echocardiography, Pennsylvania

In 2008, The ESC Working Group on Myocardial and Pericardial Diseases proposed an updated classification of cardiomyopathies based on morphological and functional phenotypes and subcategories of familial/genetic and non-familial/non-genetic disease. In this position statement, we propose a framework for the clinical approach to diagnosis in cardiomyopathies based on the recognition of diagnostic 'red flags' that can be used to guide rational selection of specialized tests including genetic analysis. The basic premise is that the adoption of a cardiomyopathy-specific mindset which combines conventional cardiological assessment with non-cardiac and molecular parameters increases diagnostic accuracy and thus improves advice and treatment for patients and families.

- by H. Seggewiss
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- Electrocardiography, Autoimmune diseases, Genetic Testing, Pedigree

Background: Congestive cardiac failure (CCF) has emerged as a major public health problem worldwide and imposes an escalating burden on the health care system. Objective: To determine the causes and mortality rate of CCF in the University of Port Harcourt Teaching Hospital (UPTH), south Nigeria, over a five-year period from January 2001 to December 2005. Methods: A retrospective study of CCF cases were identified from the admission and discharge register of the medical wards of UPTH and the case notes were retrieved from the medical records department and analyzed. Results: There were 423 patients: 242 males and 181 females. Their ages ranged from 18 to 100 years with a mean of 54.4 ± 17.3. The commonest causes of CCF were hypertension (56.3%) and cardiomyopathy (12.3%). Chronic renal failure, rheumatic heart disease, and ischemic heart disease accounted for 7.8%, 4.3%, and 0.2% of CCF, respectively. Peripartum heart disease was rare despite being commonly reported in northern Nigerian females. Eighteen patients died from various complications with a mortality rate of 4.3%. Conclusion: The burden of CCF in the Niger Delta is mainly attributed to hypertension. Efforts should be geared towards hypertension awareness, detection, treatment, and prevention in the region.

- by Arthur Onwuchekwa
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- Nigeria, Adolescent, Heart Failure, Pregnancy

The effect of inosine on adriamycin-induced cardiomyopathy was studied. Total adriamycin (ADR) dose of 25 mg/kg i.p. injected in 15 equal partial doses 3 times a week for five following weeks evoked fully developed cardiomyopathy in rats. Inosine 200 mg/kg i.p. injected 5 times a week parallel to ADR diminished ADR cardiotoxicity evaluated by electrocardiographic recordings and histopathological examination. Moreover lower cytostatic toxicity was observed as judged by less-expressed leucopenia and lower SGOT activities in inosine treated animals.

- by Aleksander Hinek
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- Electrocardiography, Doxorubicin, Rats, Myocardium

Tako-tsubo cardiomyopathy is a cardiac syndrome precipitated by profound emotional stress and anxiety, particularly in middle-aged women. It presents as a mimic of acute myocardial infarction, but coronary angiography shows normal coronary arteries and a characteristic left ventriculogram resembling an "octopus pot". The condition seems to have a favourable prognosis. Initially described in Japan, and with many names in the literature, it is being increasingly recognised in the West owing to early coronary angiography and primary coronary intervention, accounting for up to 1 in 30 primary cases of angioplasty in some institutions. A typical case is described, and the clinical features, pathophysiology and management reviewed.

The cardiovascular system undergoes significant changes during pregnancy to adapt to and accommodate the increased metabolic demands of the fetus and the mother. 1-5 These adaptations produce an important hemodynamic burden on patients with underlying heart disease, and confer an increase in morbidity and mortality. Furthermore, pregnancy may cause specific cardiovascular disorders, which can impose a risk to the pregnant woman and to her fetus. It is estimated that in the western world 0.2-4% of all pregnancies are complicated by cardiovascular diseases (CVD). 4 This risk is in the ascending order as the age of first pregnancy is increasing and as the number of cardiovascular risk factors is rising (e.g. smoking, hypercholesterolemia, diabetes, hypertension, obesity). During pregnancy, the most frequent cardiovascular events relate to hypertension (6-8%). On the other hand, in the western world, the most frequent CVD present during pregnancy is congenital heart disease-CHD (circa 75%), 4,6,7 while rheumatic heart disease predominates in the other countries (circa 70%) and CHD is seen in ~15%. In pregnant women with heart disease, maternal death is estimated around 1% but it varies depending on the underlying CVD; neonatal complications occur in 20-28% and neonatal mortality ranges between 1% and 4%. In general, CVDs are the most common cause of maternal death during pregnancy in the Western industrialized world. 4 Thus, women of child-bearing age with CVD or cardiovascular risk factors should be counseled and managed early by an interdisciplinary team of gynecologists, cardiologists, and, when necessary, cardiothoracic surgeons. To meet the metabolic demands of mother and fetus, cardiac output increases 1 L/min at 8 weeks' gestation, representing >50% of the total change seen, which culminates in an increase of cardiac output of 30-50% during normal pregnancy, maintained until term. 9,10 Cardiac output increases primarily because of stroke volume rather than heart rate, at least in early pregnancy, while later the heart rate also increases. By 8 weeks' gestation, systemic vascular resistance has fallen to 70% of its preconceptional value. The majority of the pregnancy-induced changes in these parameters occur during the embryonic period. Plasma volume has increased by ~40% at 24 weeks of gestation. Blood pressure falls mainly due to vasodilation, primarily conferred by nitric oxide and relaxin, affecting both systolic and diastolic components. In the third trimester, diastolic pressure gradually rises and may normalize to baseline values at term. Another important aspect of the cardiovascular status in pregnancy relates to hemostatic changes, which lead to hypercoagulability via an increase in concentration of coagulation factors, fibrinogen, and

- by Antonis Manolis
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- Diet, Pulmonary Hypertension, Anxiety, Pregnancy

Advances in cancer therapy have resulted in significant improvement in long-term survival for many types of cancer but have also resulted in untoward side effects associated with treatment. One such complication that has become increasingly recognized is the development of cardiomyopathy and heart failure. Whether a previously healthy person from a cardiovascular perspective develops cancer therapy–related cardiac dysfunction or a high-risk cardiovascular patient requires cancer therapy, the team of oncologists and cardiologists must be better equipped with an evidence-based approach to care for these patients across the spectrum. Although the toxicities associated with various cancer therapies are well recognized, limitations to our understanding of the appropriate course of action remain. In this first of a 2-part review, we discuss the epidemiologic, pathophysiologic, risk factors, and imaging aspects of cancer therapy–related cardiac dysfunction and heart failure. In a subsequen...

- by Carine Hamo
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- Biomarkers, Treatment Outcome, Risk assessment, Radiotherapy

Magnetocardiography (MCG) is a non-invasive and risk-free technique allowing body surface recording of the magnetic fields generated by the electrical activity of the heart. The MCG recording system allows spatially and temporally accurate measurements of the very weak magnetic fields produced by currents flowing within myocardial fibers during cardiac activity. MCG has now been around for over 30 years, but only recently has progress in instrumentation put the technique on the verge of clinical applicability. This review summarizes the physical principles, instrumentation, main clinical applications and perspectives for the clinical use of MCG. This first part is devoted to the description of the physical principles and instrumentation.

- by Silvia Comani
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- Pregnancy, Heart, Myocarditis, Electromagnetic phenomena

ARIAS, MA, PUCHOL, A., COLCHERO, T., PACHÓN, M., CASTELLANOS, E. and RODRÍGUEZ-PADIAL, L.(2010), An Appropriate Implantable Cardioverter-Defibrillator Shock Triggering Unusual Phenomenons. Pacing and Clinical Electrophysiology, 33: ...

In laboratory animals, histology is most commonly used to study doxorubicin-induced cardiotoxicity. However, for monitoring during treatment, large numbers of animals are needed. Recently we developed a new method to measure ECG values in freely moving mice by telemetry. With this model we investigated the effect of chronic doxorubicin administration on the ECG of freely moving BALB/c mice and the efficacy of ICRF-187 as a protective agent. The ST interval significantly widened from 15.0$1.5 to 56.8$11.8 ms in week 10 (7 weekly doses of 4 mg/kg doxorubicin given i.v. plus 3 weeks of observation). The ECG of the control animals did not change during the entire study. After sacrifice the hearts of doxorubicin-treated animals were enlarged and the atria were hypertrophic. As this schedule exerted more toxicity than needed to investigate protective agents, the protection of ICRF-187 was determined using a dose schedule with lower general toxicity (6 weekly doses of 4 mg/kg doxorubicin given i.v. plus 2 weeks of observation). On this schedule, the animals' hearts appeared normal after sacrifice and ICRF-187 (50 mg/kg given i.p. 1 h before doxorubicin) provided almost full protection. These data were confirmed by histology. The results indicate that this new model is very sensitive and enables monitoring of the development of cardiotoxicity with time. These findings result in a model that allows the testing of protectors against doxorubicin-induced cardiotoxicity as demonstrated by the protection provided by ICRF-187.

- by Jun Zhang
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- Animal Behavior, Drug interactions, Electrocardiography, Mice
- by Jesus Arellano Vallejo
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- Cytokines, Risk assessment, Stress response, Prognosis

Introduction to the SeriesGenetic disorders have characteristic cardiovascular manifestations. These cardiovascular abnormalities often are a major determinant of the morbidity and mortality in this patient population. Some characteristics are unique and can be detected with echocardiography. Drs Alizad and Seward have compiled in this series a review of genetic disorders that have recognizable morphologic and/or functional cardiovascular abnormalities. The following

- by Azra Alizad
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- Genetics, Adolescent, Williams Syndrome, Down Syndrome
- by Patrice Delafontaine
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- Risk assessment, Multivariate Analysis, Heart Failure, Prospective studies
- by Emmanuel Dupont
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- Gap Junctions, Fluorescence Microscopy, Confocal Microscopy, Gene expression
- by David Orlikowski
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- Duchenne Muscular Dystrophy, Cardiomyopathies

Cardiac arrest is classified as an 'in-hospital' if it occurs in a hospitalised patient who had a pulse at the time of admission. A probability of patient's survival until hospital discharge is very low. The reasons for this are old age, multiple comorbidity of patients, late recognition of cardiac arrest, poor knowledge about basic life support algorithm, insufficient equipment, absence of qualified resuscitation teams and poor organization.

- by Tomislav Ružman and +2
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- Survival Analysis, Treatment Outcome, Risk assessment, Comorbidity

Classifications of heart muscle diseases have proved to be exceedingly complex and in many respects contradictory. Indeed, the precise language used to describe these diseases is profoundly important. A new contemporary and rigorous classification of cardiomyopathies (with definitions) is proposed here. This reference document affords an important framework and measure of clarity to this heterogeneous group of diseases. Of particular note, the present classification scheme recognizes the rapid evolution of molecular genetics in cardiology, as well as the introduction of several recently described diseases, and is unique in that it incorporates ion channelopathies as a primary cardiomyopathy. (Circulation. 2006;113:1807-1816.) Key Words: AHA Scientific Statements Ⅲ cardiomyopathies Ⅲ arrhythmias Ⅲ terminology Ⅲ genetics C ardiomyopathies are an important and heterogeneous The American Heart Association makes every effort to avoid any actual or potential conflicts of interest that may arise as a result of an outside relationship or a personal, professional, or business interest of a member of the writing panel. Specifically, all members of the writing group are required to complete and submit a Disclosure Questionnaire showing all such relationships that might be perceived as real or potential conflicts of interest. This statement was approved by the American Heart Association Science Advisory and Coordinating Committee on February 1, 2006. A single reprint is available by calling 800-242-8721

- by Charles Antzelevitch
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- Cardiology, Genomics, Treatment Outcome, Functional Genomics
- by Ajay Singh
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- Facial Paralysis, Magnetic Resonance Imaging, Atherosclerosis, Rheumatoid Arthritis

Magnetocardiography (MCG) is a non-invasive and risk-free technique allowing body surface recording of the magnetic fields generated by the electrical activity of the heart. The MCG recording system allows spatially and temporally accurate measurements of the very weak magnetic fields produced by currents flowing within myocardial fibers during cardiac activity. MCG has now been around for over 30 years, but only recently has progress in instrumentation put the technique on the verge of clinical applicability. This review summarizes the physical principles, instrumentation, main clinical applications and perspectives for the clinical use of MCG. This second part is devoted to the description of the main current clinical applications and perspectives.

- by Silvia Comani
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- Pregnancy, Heart, Myocarditis, Electromagnetic phenomena

The small circle of mitochondrial DNA (mtDNA) present in all human cells has proven to be a veritable Pandora's box of pathogenic mutations and rearrangements. In this review, we summarize the distinctive rules of mitochondrial genetics (maternal inheritance, mitotic segregation, heteroplasmy and threshold effect), stress the relatively high prevalence of mtDNA-related diseases, and consider recent additions to the already long list of pathogenic mutations (especially mutations affecting protein-coding genes). We then discuss more controversial issues, including the functional or pathological role of mtDNA haplotypes, the pathogenicity of homoplasmic mutations and the still largely obscure pathophysiology of mtDNA mutations.

- by Laura Greaves
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- Aging, DNA replication, Oxidative Stress, Neurodegenerative Diseases

Anthracycline-based antineoplastic therapy is the standard of care for various cancers today and represents a breakthrough in this area. The cardiac toxicity of anthracyclines is well established. The acute form is often reversible and has no predictive value for the future. This early form does not prevent continuation of chemotherapy. Late cardiac toxicity due to anthracycline is the leading limiting factor in its use. In adults, this resembles dilated cardiomyopathy, while in children it may be expressed as restrictive cardiomyopathy. The discovery of modifiable risk factors has made it possible to identify patients at high risk of developing late cardiac toxicity and heart failure. Because left ventricular dysfunction and heart failure may develop long after anthracycline treatment ends, prolonged close follow-up is mandatory in asymptomatic subjects. Follow-up of asymptomatic patients requires serial echocardiography (M-mode, 2D echo, Doppler, tissue Doppler, speckle tracking, etc.). Anthracycline-induced cardiomyopathy must be treated according to the standard guidelines for chronic heart failure with left ventricular dysfunction, by angiotensin-converting enzyme (ACE) inhibitors and beta-blockers. Lifestyle changes may reduce the long-term risk. Close collaboration between cardiologists and oncologists is highly desirable for optimizing management of these patients.

- by Gerald Roul and +1
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- Cardiology, Life Style, Risk assessment, Biopsy

The content of these European Society of Cardiology (ESC) Guidelines has been published for personal and educational use only. No commercial use is authorized. No part of the ESC Guidelines may be translated or reproduced in any form without written permission from the ESC. Permission can be obtained upon submission of a written request to Oxford University Press, the publisher of the European Heart Journal and the party authorized to handle such permissions on behalf of the ESC. Disclaimer: The ESC Guidelines represent the views of the ESC and were produced after careful consideration of the scientific and medical knowledge and the evidence available at the time of their publication. The ESC is not responsible in the event of any contradiction, discrepancy and/or ambiguity between the ESC Guidelines and any other official recommendations or guidelines issued by the relevant public health authorities, in particular in relation to good use of healthcare or therapeutic strategies. Health professionals are encouraged to take the ESC Guidelines fully into account when exercising their clinical judgment, as well as in the determination and the implementation of preventive, diagnostic or therapeutic medical strategies; however, the ESC Guidelines do not override, in any way whatsoever, the individual responsibility of health professionals to make appropriate and accurate decisions in consideration of each patient's health condition and in consultation with that patient and, where appropriate and/or necessary, the patient's caregiver. Nor do the ESC Guidelines exempt health professionals from taking into full and careful consideration the relevant official updated recommendations or guidelines issued by the competent public health authorities, in order to manage each patient's case in light of the scientifically accepted data pursuant to their respective ethical and professional obligations. It is also the health professional's responsibility to verify the applicable rules and regulations relating to drugs and medical devices at the time of prescription.

- by John Camm
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- Pregnancy, Child, Mental Disorders, Myocarditis
- by Jean-marie Cuisset and +3
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- Research Design, Clinical Trial, Adolescent, France
- by Alessandra Giavarini
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- Treatment Outcome, Child, Paris, Heart

Purpose: The identification of subjects with systemic sarcoidosis at higher risk for sudden death is an unresolved issue. An influence of the autonomic activity on the genesis of ventricular arrhythmias was postulated. Heart rate variability (HRV) analysis provides a useful method to measure autonomic activity, and is a predictor of increased risk of death in various conditions. Therefore, the aim of the study was to evaluate HRV in patients with systemic sarcoidosis.Methods: The study included 35 patients with biopsy proven systemic sarcoidosis who were not taking antiarrhythmic medications. Thallium scintigraphy was performed to all patients with systemic sarcoidosis. The cardiac sarcoidosis was accepted in 16 patients as abnormal thallium scintigraphy and normal coronary arteriography. The time-domain analysis of HRV was expressed as the standard deviation of all normal to normal NN intervals (SDNN) detected during 24-hour Holter monitoring. Twenty-four healthy subjects represented a control group for HRV analysis.Results: There were no differences in age (44 ± 13 years for cardiac sarcoidosis, 42 ± 15 years for noncardiac sarcoidosis, and 40 ± 10 years for control group; P = NS), sex (the ratio of female; 63%, 68%, and 55%, respectively; P = NS), and echocardiographic ejection fraction (63 ± 10%, 67 ± 8%, and 69 ± 6%, respectively; P = NS) among study groups. The mean SDNN value of the group with cardiac sarcoidosis was significantly lower than both the group with noncardiac sarcoidosis and the control group (72 ± 32 ms vs 110 ± 46 ms and 152 ± 36 ms; P < 0.05, respectively).Conclusion: HRV is decreased in patients with systemic sarcoidosis compared to the control group. This decreasing is more obvious in patients with cardiac sarcoidosis.

- by Ahmet Akyol
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- Sarcoidosis, Echocardiography, Heart rate variability, Heart rate

Left ventricular noncompaction (LVNC) is a form of cardiomyopathy resulting from a disorder of endomyocardial morphogenesis. It has been associated with significant morbidity and mortality. The aim of this study was to characterize associated cardiac findings in children with LVNC and to identify risk factors associated with increased mortality. From our echocardiography database, we identified 46 patients diagnosed with LVNC between December 1999 and February 2005. The mean age at presentation was 3.6 ± 5.6 years, and the mean duration of follow-up was 1.9 ± 2.1 years. Left ventricular ejection fraction was decreased in 24 patients (52%; mean 39.5% ± 13.1%). Thirty-six patients (78%) had associated cardiac lesions, including atrial septal defect (n = 16 [35%]), ventricular septal defect (n = 17 [37%]), patent ductus arteriosus (n = 14 [30%]), and Ebstein's anomaly (n = 5 [11%]). Electrocardiogram abnormalities were found in 80% of patients; most commonly they included left (n = 15 [43%]) and right ventricular hypertrophy (n = 19 [54%]). Documented arrhythmias included ectopic atrial rhythm (n = 2), junctional rhythm (n = 2), supraventricular tachycardia (n = 2), and ventricular tachycardia (n = 1). Overall mortality was 20%, and there was no association with ejection fraction, morphologic defect, or arrhythmia. Mean age at diagnosis in survivors (4.5 ± 6.1 years) was higher than nonsurvivors (0.4 ± 0.7 years) (p \ 0.0001). LVNC is a rarely isolated form of cardiomyopathy, and it is associated with significant additional cardiac abnormalities. Although it does not have an invariably fatal course, early presentation in infancy does carry an increased risk of mortality.

- by Eric Ebenroth and +2
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- Adolescent, Pediatric Cardiology, Child, Left Ventricular Assist Device

Background: Non-compaction of ventricular myocardium is a rare congenital cardiomyopathy characterized by the presence of an extremely thickened endocardial layer with prominent trabeculations and deep recesses in communication with ventricular chamber and determining the typical spongeous aspect. The diagnosis of non-compaction of ventricular myocardium is possible through the identification of morphological alterations by echocardiographic evaluation. Ebstein's anomaly is a rare congenital cardiac disease, defined as the significant apical displacement of the part of the tricuspid valve causing significant tricuspid regurgitation and reduction of the functional right ventricle, right atrial and right ventricular dilatation and atrial and ventricular arrhythmias. Case report: We present a case of biventricular non-compaction and Ebstein's anomaly in a 29-year-old Italian man that was referred for chest pain. Diagnosis of Ebstein's anomaly was made during a medical control for military service through an echocardiographic evaluation which left the suspicion of myocardium noncompaction. We present the cardiac image of the 2D and 3D eco, RMN, scintigraphy and ventriculaography.

- by Emanuele Grassedonio and +2
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- Cardiology, Magnetic Resonance, Cardiomyopathies, Right Ventricle
- by Ramzan Zakir
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- Heart Failure, Prospective studies, Congestive Heart Failure, Hospitalization

Several studies have demonstrated that patients affected by heart failure have a compromised quality of life and, in the last few years, "health-related quality of life" has become an important outcome indicator for the evaluation of heart failure treatment and a basis for the improvement of its strategies. The translation into Italian of the Kansas City Cardiomyopathy Questionnaire (KCCQ), a new, 23 item, disease-specific health status instrument for patients with congestive heart failure, and its subsequent validation by asking 50 consecutive patients in our heart failure outpatient clinic to answer it. The KCCQ was compared to the "Minnesota Living with Heart Failure Questionnaire" (MLHF). The Italian version of the KCCQ correlates well with the MLHF for all domains with the exclusion of symptom stability score and MLHF emotional domain. However, the KCCQ, due to its multiple domains, provided more detailed information about the patients' status, and ident...

- by claudio fresco
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- Quality of life, Italy, Heart Failure, Self Efficacy
- by M. Bahloul
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- Treatment Outcome, Heart Failure, Pregnancy, Tunisia

Background-Iron cardiomyopathy is a leading cause of death in transfusion dependent thalassemia major (TM) patients and MRI (T2*) can recognize preclinical cardiac iron overload, but, is unavailable to many centers.

- by Yaniv Bar-cohen and +1
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- Algorithms, Risk, Adolescent, Electrocardiography

Technological advances continue to expand the clinical role of echocardiography in the intensive care unit, particularly in patients with heart failure. It has many advantages over tomographic techniques such as echo cardiac magnetic resonance imaging and cardiac computed tomography, can provide rapid bedside cardiac assessment, and facilitate emergent decisionmaking for critically ill patients. Image quality problems in the intensive care setting have largely been overcome by the use of harmonic imaging, contrast opacification, and when indicated, transesophageal echocardiography. Newer techniques promise to advance the scope and prognostic power of echocardiography, and to expand the portability and availability of this tool. (Crit Care Med 2008; 36[Suppl.]:S28-S39) S28

- by Victor Ferrari
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- Nursing, Critical Care, Critical Care Medicine, Echocardiography
- by Amos Korczyn
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- Microbiology, Cognitive Science, Neurology, Africa

diography and the poor inter-and intraobserver variability limit these techniques. 2,3 The development of real time three-dimensional echocardiography (RT3DE) with matrix transducer technology and analyzing software made a more reliable analysis of LV function feasible. The increasing accuracy and reproducibility of RT3DE for LV quantification has been shown in many studies. There are several online and off-line software programs for LV volume quantification by RT3DE. The endocardial border tracking algorithms which can be used for generation of a 3D LV volume represent a continuum spectrum

- by Osama Soliman and +1
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- Algorithms, Magnetic Resonance Imaging, Linear models, Echocardiography

Stimulation of the local renin-angiotensin system and apoptosis characterize the diabetic heart. Because IGF-1 reduces angiotensin (Ang) II and apoptosis, we tested whether streptozotocin-induced diabetic cardiomyopathy was attenuated in IGF-1 transgenic mice (TGM). Diabetes progressively depressed ventricular performance in wild-type mice (WTM) but had no hemodynamic effect on TGM. Myocyte apoptosis measured at 7 and 30 days after the onset of diabetes was twofold higher in WTM than in TGM. Myocyte necrosis was apparent only at 30 days and was more severe in WTM. Diabetic nontransgenic mice lost 24% of their ventricular myocytes and showed a 28% myocyte hypertrophy; both phenomena were prevented by IGF-1. In diabetic WTM, p53 was increased in myocytes, and this activation of p53 was characterized by upregulation of Bax, angiotensinogen, Ang type 1 (AT 1 ) receptors, and Ang II. IGF-1 overexpression decreased these biochemical responses. In vivo accumulation of the reactive O 2 product nitrotyrosine and the in vitro formation of H 2 O 2 -˙OH in myocytes were higher in diabetic WTM than TGM. Apoptosis in vitro was detected in myocytes exhibiting high H 2 O 2 -˙OH fluorescence, and apoptosis in vivo was linked to the presence of nitrotyrosine. H 2 O 2 -˙OH generation and myocyte apoptosis in vitro were inhibited by the AT 1 blocker losartan and the O 2 scavenger Tiron. In conclusion, IGF-1 interferes with the development of diabetic myopathy by attenuating p53 function and Ang II production and thus AT 1 activation. This latter event might be responsible for the decrease in oxidative stress and myocyte death by IGF-1.

- by Federica Limana
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- Diabetes, Oxidative Stress, Apoptosis, DNA

JG Howlett, RS McKelvie, JMO Arnold, et al. Canadian Cardiovascular Society Cconsensus Conference guidelines on heart failure, update 2009: Diagnosis and management of right-sided heart failure, myocarditis, device therapy and recent... more

- by Michel White
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- Cardiology, Evidence Based Medicine, Exercise therapy, Heart Failure

Background-DMD is characterized by progressive cardiac dysfunction and myocardial fibrosis late in the disease process. We hypothesized that left ventricular myocardial peak circumferential strain (ε cc ) would decrease in DMD prior to... more

- by Linda Cripe and +1
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- Cardiology, Magnetic Resonance Imaging, MRI, Adolescent

- by Haissam Haddad and +1
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- Evidence Based Medicine, Clinical Trial, Pulmonary Hypertension, Heart Failure