MedWorm: Cardiomyopathy

Mitral Valve Surgery for Severe Mitral Regurgitation and Dilated Cardiomyopathy—A Bridge to Transplant: Case Report and a Review of Literature

Congenital Heart Disease — Tue, 07 Feb 2012 05:00:00 +0100

We report a child with myocardial necrosis, dilated cardiomyopathy, and severe mitral valve (MV) regurgitation following neonatal enteroviral myocarditis. He underwent MV annuloplasty at 18 months and MV replacement at 3 years of age. He remains asymptomatic on medical therapy at 8 years of age. Mitral valve surgery may stabilize the evolution of dilated cardiomyopathy and delay the ultimate need for heart transplant. (Source: Congenital Heart Disease)

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Cardiac involvement in muscular dystrophy: advances in diagnosis and therapy

Heart — Mon, 06 Feb 2012 05:00:00 +0100

The term muscular dystrophy (MD) comprises various neuromuscular disorders that are characterised by progressive muscle weakness affecting certain muscle groups, which are specific for the respective genetic disorder. Muscular dystrophy type Duchenne (DMD) and type Becker (BMD) represent the most common X-linked genetic diseases: DMD is believed to affect one in 3500 male births whereas BMD is less frequent (one in 18 450 male births).w1 w2 However, due to the longer life expectancy of BMD patients, the prevalence of DMD and BMD is rather similar and at least 2.4/100 000.w1 Apart from progressive proximal skeletal muscle weakness and wasting, DMD and BMD are characterised by cardiac muscle involvement. Indeed, progressive cardiomyopathy has become a major cause of morbidity and mortality i...

Tako-tsubo cardiomyopathy precipitated by alcohol withdrawal.

Cardiology Journal — Mon, 06 Feb 2012 00:36:02 +0100

We report a case of TCM in a post-menopausal woman that was precipitated by alcohol withdrawal. (Cardiol J 2012; 19, 1: 81-85). PMID: 22298173 [PubMed - in process] (Source: Cardiology Journal)

What Are the Major Diseases Involving Copper Metabolism?

PediatricEducation.org — Mon, 06 Feb 2012 00:08:49 +0100

Discussion Nutritional problems can occur in all parts of the world and in all socioeconomic strata. Caloric and/or protein inadequacy unfortunately plagues too many people because of inadequate supplies or availability. Supplemental food programs around the world attempt to provide appropriate nutrition, but can be stymied because of war, political instability, economic instability and many other social factors. The most common specific nutrient deficiencies are iron and Vitamin D deficiencies. Minerals important for essential nutrition include copper, iodine, selenium and zinc. A typical mixed diet usually provides enough minerals. In the United States, mineral deficiencies are usually uncommon unless there is an underlying disease process or abnormal food restrictions or diet. Vegan an...

Tool created to identify DCM children at risk for sudden cardiac death

MedWire News - Cardiology — Mon, 06 Feb 2012 00:00:00 +0100

Researchers have created a risk stratification tool to identify patients with pediatric dilated cardiomyopathy who are at risk for sudden cardiac death. (Source: MedWire News - Cardiology)

Amfetamine mixed salts overdose: Inverted Tako-Tsubo cardiomyopathy: case report

Reactions — Sun, 05 Feb 2012 18:29:54 +0100

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Immune globulin: Tako-Tsubo cardiomyopathy (first report) in an elderly patient: case report

Reactions — Sun, 05 Feb 2012 18:29:54 +0100

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Paracetamol/venlafaxine overdose: Cardiomyopathy: case report

Reactions — Sun, 05 Feb 2012 18:29:54 +0100

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Growth differentiation factor-15, a novel biomarker related with disease severity in patients with hypertrophic cardiomyopathy.

European Journal of Internal Medicine — Fri, 03 Feb 2012 00:26:31 +0100

CONCLUSIONS: The present results show that higher levels of GDF-15 are associated to conditions of severe disease in HCM. Hence, GDF-15 is suggested as a novel marker related to the severity and could represent a further useful tool in monitoring functional capacity of HCM patients. PMID: 22284249 [PubMed - in process] (Source: European Journal of Internal Medicine)

Incidence of and Risk Factors for Sudden Cardiac Death in Children With Dilated Cardiomyopathy: A Report From the Pediatric Cardiomyopathy Registry

Journal of the American College of Cardiology — Thu, 02 Feb 2012 13:55:34 +0100

Objectives: The purpose of this study was to establish the incidence of and risk factors for sudden cardiac death (SCD) in pediatric dilated cardiomyopathy (DCM). Background: The incidence of SCD in children with DCM is unknown. The ability to predict patients at high risk of SCD will help to define who may benefit most from implantable cardioverter-defibrillators. Methods: The cohort was 1,803 children in the PCMR (Pediatric Cardiomyopathy Registry) with a diagnosis of DCM from 1990 to 2009. Cumulative incidence competing-risks event rates were estimated. We achieved risk stratification using Classification and Regression Tree methodology. Results: The 5-year incidence rates were 29% for heart transplantation, 12.1% non-SCD, 4.0% death from unknown cause, and 2.4% for SCD. Of 280 d...

A Risk‐Prediction Model for In‐hospital Mortality After Heart Transplantation in US Children

American Journal of Transplantation — Thu, 02 Feb 2012 05:00:00 +0100

We sought to develop and validate a quantitative risk‐prediction model for predicting the risk of posttransplant in‐hospital mortality in pediatric heart transplantation (HT). Children <18 years of age who underwent primary HT in the United States during 1999–2008 (n = 2707) were identified using Organ Procurement and Transplant Network data. A risk‐prediction model was developed using two‐thirds of the cohort (random sample), internally validated in the remaining one‐third, and independently validated in a cohort of 338 children transplanted during 2009–2010. The best predictive model had four categorical variables: hemodynamic support (ECMO, ventilator support, VAD support vs. medical therapy), cardiac diagnosis (repaired congenital heart disease [CHD], unrepaired CHD vs...

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Stress Cardiomyopathy: A Syndrome of Catecholamine-Mediated Myocardial Stunning?

Cellular and Molecular Neurobiology — Thu, 02 Feb 2012 05:00:00 +0100

Authors: Wittstein IS Abstract During the past few years, a novel syndrome of heart failure and transient left ventricular systolic dysfunction precipitated by acute emotional or physical stress has been described. While patients with "stress cardiomyopathy" (SCM) typically present with signs and symptoms that resemble an acute coronary syndrome, it has become clear that this syndrome has unique clinical features that can readily be distinguished from acute infarction. In particular, in contrast to the irreversible myocardial injury seen with infarction, the myocardial dysfunction of SCM is completely reversible and occurs in the absence of plaque rupture and coronary thrombosis. There is increasing evidence that exaggerated sympathetic stimulation may play a pathogenic role in the...

Genetics of inherited cardiomyopathy

European Heart Journal — Wed, 01 Feb 2012 05:00:00 +0100

During the past two decades, numerous disease-causing genes for different cardiomyopathies have been identified. These discoveries have led to better understanding of disease pathogenesis and initial steps in the application of mutation analysis in the evaluation of affected individuals and their family members. As knowledge of the genetic abnormalities, and insight into cellular and organ biology has grown, so has appreciation of the level of complexity of interaction between genotype and phenotype across disease states. What were initially thought to be one-to-one gene-disease correlates have turned out to display important relational plasticity dependent in large part on the genetic and environmental backgrounds into which the genes of interest express. The current state of knowledge wi...

When Oral Anticoagulation Therapy is Needed in Patients With Cardiomyopathies: A Review of Literature

Heart, Lung and Circulation — Wed, 01 Feb 2012 05:00:00 +0100

The question whether to anticoagulate patients with cardiomyopathy or not is over 50years old. Multiple clinical trials have demonstrated the superior therapeutic effect of warfarin compared with placebo in the prevention of thromboembolic events amongst patients with nonvalvular atrial fibrillation. The purpose of our work is to review literature about the role of anticoagulation in the main cardiomyopathies. (Source: Heart, Lung and Circulation)

Dietary obesity-associated Hif1{alpha} activation in adipocytes restricts fatty acid oxidation and energy expenditure via suppression of the Sirt2-NAD+ system [Research Papers]

Genes and Development — Wed, 01 Feb 2012 05:00:00 +0100

Dietary obesity is a major factor in the development of type 2 diabetes and is associated with intra-adipose tissue hypoxia and activation of hypoxia-inducible factor 1α (HIF1α). Here we report that, in mice, Hif1α activation in visceral white adipocytes is critical to maintain dietary obesity and associated pathologies, including glucose intolerance, insulin resistance, and cardiomyopathy. This function of Hif1α is linked to its capacity to suppress β-oxidation, in part, through transcriptional repression of sirtuin 2 (Sirt2) NAD+-dependent deacetylase. Reduced Sirt2 function directly translates into diminished deacetylation of PPAR coactivator 1α (Pgc1α) and expression of β-oxidation and mitochondrial genes. Importantly, visceral adipose tiss...

Peripartum cardiomyopathy: Post-transplant outcomes from the united network for organ sharing database

The Journal of Heart and Lung Transplantation — Wed, 01 Feb 2012 05:00:00 +0100

Conclusions: This large report shows outcomes of graft failure and death are inferior for recipients with PPCM, which may be partly explained by younger age, higher allosensitization, higher pre-transplant acuity, and increased rejection. More research is needed to determine management strategies to improve outcomes in PPCM heart transplant recipients. (Source: The Journal of Heart and Lung Transplantation)

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Combined heart and liver transplantation in an adult with familial heterozygous hypercholesterolemia and severe ischemic cardiomyopathy

The Journal of Heart and Lung Transplantation — Wed, 01 Feb 2012 05:00:00 +0100

Familial hypercholesterolemia (FH) is an autosomal-dominant disorder caused by mutations in the low-density lipoprotein (LDL) receptor. The clinical manifestations of FH include severe atherosclerosis and premature heart disease. Liver transplantation restores defective LDL receptors and has been used for long-term treatment in homozygous FH. (Source: The Journal of Heart and Lung Transplantation)

Mannose-Binding Lectin and Toll-Like Receptor Polymorphisms and Chagas Disease in Chile.

The American Journal of Tropical Medicine and Hygiene — Wed, 01 Feb 2012 05:00:00 +0100

Authors: Weitzel T, Zulantay I, Danquah I, Hamann L, Schumann RR, Apt W, Mockenhaupt FP Abstract Abstract. Mannose-binding lectin (MBL) and Toll-like receptor (TLR) polymorphisms may influence susceptibility and manifestation of Trypanosoma cruzi infection. In northern Chile, we examined 61 asymptomatic patients with chronic Chagas disease (CD), 64 patients with chronic Chagas cardiomyopathy (CCC), and 45 healthy individuals. Low-producer MBL2*B genotypes were more common in CD patients (48%) than healthy individuals (31%; adjusted odds ratio = 2.3, 95% confidence interval = 1.01-5.4, P = 0.047) but did not differ with manifestation. In contrast, the heterozygous Toll-like receptor 4 (TLR4)-deficiency genotype D299G/T399I occurred more frequently in asymptomatic (14.8%) than CCC pa...

Prevalence and Spectrum Diseases Predisposing to Sudden Cardiac Death: Are They the Same for Both the Athlete and the Nonathlete?

Pediatric Cardiology — Tue, 31 Jan 2012 16:45:46 +0100

This report highlights the prevalence and spectrum of SCD-predisposing diseases and how they are affected by athletic participation. In addition, disease-specific guidelines for sports participation are addressed. Content Type Journal ArticleCategory Review ArticlePages 1-8DOI 10.1007/s00246-012-0159-1Authors Anjan S. Batra, Division of Pediatric Cardiology, Children’s Hospital of Orange County, University of California at Irvine, 455 S Main Street, Orange, CA 92868, USASeshadri Balaji, Division of Cardiology, Oregon Health and Science University, Portland, OR, USA Journal Pediatric CardiologyOnline ISSN 1432-1971Print ISSN 0172-0643 (Source: Pediatric Cardiology)

Histological and ultrastructural abnormalities in murine desmoglein 2-mutant hearts

Cell and Tissue Research — Tue, 31 Jan 2012 16:45:33 +0100

Abstract Mice carrying a deletion of the adhesive extracellular domain of the desmosomal cadherin desmoglein 2 develop an arrhythmogenic right ventricular cardiomyopathy-like phenotype with ventricular dilation, fibrosis and arrhythmia. To unravel the sequence of myocardial alterations and to identify potential pathomechanisms, histological analyses were performed on mutant hearts from the juvenile to the adult state, i.e., between 2 and 13 weeks. At an age of 2 weeks 30% of mutants presented lesions, which were visible as white plaques on the heart surface or in the septum. From 4 weeks onwards, all mutants displayed a cardiac phenotype. Dying cardiomyocytes with calcification were found in lesions of all ages. But lesions of young mutant animals contained ...

Current perspectives on cardiac amyloidosis

AJP: Heart and Circulatory Physiology — Tue, 31 Jan 2012 05:00:00 +0100

Amyloidosis represents a group of diseases in which proteins undergo misfolding to form insoluble fibrils with subsequent tissue deposition. While almost all deposited amyloid fibers share a common nonbranched morphology, the affected end organs, clinical presentation, treatment strategies, and prognosis vary greatly among this group of diseases and are largely dependent on the specific amyloid precursor protein. To date, at least 27 precursor proteins have been identified to result in either local tissue or systemic amyloidosis, with nine of them manifesting in cardiac deposition and resulting in a syndrome termed "cardiac amyloidosis" or "amyloid cardiomyopathy." Although cardiac amyloidosis has been traditionally considered to be a rare disorder, as clinical appreciation and understandi...

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Cardiac dysfunction in cirrhosis – does adrenal function play a role? A hypothesis

Liver International — Tue, 31 Jan 2012 05:00:00 +0100

AbstractCirrhotic cardiomyopathy (CCM), a condition of unknown pathogenesis, is characterized by suboptimal ventricular contractile response to stress, diastolic dysfunction and QT interval prolongation. It is most often found in patients with advanced cirrhosis. It is clinically relevant during stressful conditions, such as sepsis, bleeding and surgery. CCM reverses after liver transplantation and potentially has a role in the pathogenesis of hepatorenal syndrome. In adrenal insufficiency (AI), cardiac dysfunction is a feature with low ejection fraction, decreased left ventricular chamber size and electrocardiographic abnormalities, including QT interval prolongation. With optimal diagnostic tests, AI is present in approximately 10% of patients with cirrhosis, particularly in those with a...

Decreased free sphingoid base concentration in the plasma of patients with chronic systolic heart failure.

Advances in Medical Sciences — Tue, 31 Jan 2012 05:00:00 +0100

Conclusions: We conclude that chronic heart failure is associated with decreased concentration of free sphingoid bases in the plasma. However, despite lower availability of substrates required for synthesis of cardioprotective sphingoid base-1 phosphates, their plasma level remains stable. PMID: 22296975 [PubMed - as supplied by publisher] (Source: Advances in Medical Sciences)

Type of Desmin Expression in Cardiomyocytes –a Good Marker of Heart Failure Development in Idiopathic Dilated Cardiomyopathy

Journal of Internal Medicine — Tue, 31 Jan 2012 05:00:00 +0100

Conclusion: The type of immunohistochemical DES expression correlated with the level of myocardial injury at the cellular and organ levels. This correlation was similar to that observed between DES expression and the well‐established biochemical, echocardiographic and clinical parameters of heart failure (HF). DES expression type could be used as an important diagnostic feature of HF development. (Source: Journal of Internal Medicine)

Genetic variation screening of TNNT2 gene in a cohort of patients with hypertrophic and dilated cardiomyopathy.

Physiological Research — Tue, 31 Jan 2012 05:00:00 +0100

Authors: Jáchymová M, Muravská A, Paleček T, Kuchynka P, Reháková H, Magage S, Král A, Zima T, Horký K, Linhart A Abstract Mutations in troponin T (TNNT2) gene represent the important part of currently identified disease-causing mutations in hypertrophic (HCM) and dilated (DCM) cardiomyopathy. The aim of this study was to analyze TNNT2 gene exons in patients with HCM and DCM diagnosis to improve diagnostic and genetic consultancy in affected families. All 15 exons and their flanking regions of the TNNT2 gene were analyzed by DNA sequence analysis in 174 patients with HCM and DCM diagnosis. We identified genetic variations in TNNT2 exon regions in 56 patients and genetic variations in TNNT2 intron regions in 164 patients. Two patients were found to carry unique mutations in ...

Cognitive decline in patients with metal-on-metal hips: think cobalt toxicity

The Poison Review — Tue, 31 Jan 2012 04:56:05 +0100

This weekend both the Daily Mail (U.K.) and the Sunday Telegraph (U.K.) had stories highlighting the dangers of metal-on-metal artificial hips, which have a high rate of failure, causing local tissue inflammation, pain, and leaching of chromium and cobalt into blood and other tissues. A recent letter to the BMJ (Arthroprosthetic cobaltism associated with metal on metal implants. BMJ 2012;344:e430) describes several patients who received the metal-on-metal hip implants who developed progressive hip pain, elevated cobalt levels, cognitive impairment, neuropathy, and cardiomyopathy. Associated symptoms included new onset depression, anxiety, tinnitus, and thyroid abnormalities. Dr. Stephen S. Tower, the author of the BMJ letter, suggests that patients with these implants should be suspe...

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Long-term survival of patients with ischemic cardiomyopathy treated by coronary artery bypass grafting versus medical therapy.

The Annals of Thoracic Surgery — Tue, 31 Jan 2012 04:20:40 +0100

CONCLUSIONS: Among a propensity-matched, risk-adjusted, observational cohort of patients with coronary artery disease, left ventricular ejection fraction less than 0.35, and no left main disease of greater than 50%, CABG is associated with a survival advantage over MED through 10 years of follow-up. PMID: 22269720 [PubMed - in process] (Source: The Annals of Thoracic Surgery)

Survival, Risks and Benefits of ICDs in Cardiomyopathy Survival, Risks and Benefits of ICDs in Cardiomyopathy

Medscape Today Headlines — Tue, 31 Jan 2012 04:00:00 +0100

Explore the risks and benefits of ICD therapy for hypertrophic cardiomyopathy treatment by reviewing determinants of shocks and implant-related complications. Heart (Source: Medscape Today Headlines)

Hsp90 and its co-chaperone, Sgt1, as autoantigens in dilated cardiomyopathy

Heart and Vessels — Mon, 30 Jan 2012 06:32:12 +0100

Abstract Recently, it has been suggested that some heat shock proteins such as Hsp70 and Hsp60 are involved in autoimmune diseases including cardiospecific ones. In this work we focused on the involvement of another well known heat shock protein, Hsp90, and its novel co-chaperone, Sgt1, in dilated cardiomyopathy (DCM). We found that the level of autoantibodies against these two proteins was significantly higher in patients with DCM and ischemic heart disease than in sera of healthy donors. We have also analyzed the expression level and subcellular localization of Hsp90 and Sgt1 in diseased myocardia. Using Western blot we found changes in subcellular localization of Hsp90 in the left ventricle of DCM hearts while the total level of this protein remained unchanged. Regardin...

Metabonomic study on the cumulative cardiotoxicity of a pirarubicin liposome powder.

Talanta — Mon, 30 Jan 2012 05:00:00 +0100

Authors: Cong W, Liang Q, Li L, Shi J, Liu Q, Feng Y, Wang Y, Luo G Abstract Pirarubicin (THP) is an anthracycline frequently used in the chemotherapy against acute leukemia, malignant lymphoma and several solid tumors. However, its clinical use is severely limited by the development of a progressive dose-dependent cardiomyopathy that results in irreversible congestive heart failure. To provide a strategy for constraining or minimizing the cumulative cardiotoxicity of THP, a pirarubicin liposome powder (L-THP) was appropriately prepared, and the cumulative cardiotoxicity of L-THP and free THP (F-THP) were investigated on Sprague-Dawley rats after 3 successive doses. Urinary samples for metabonomic study, serum samples for biochemical assay, and heart samples for histopathology test...

Effects of ivabradine on heart rate and left ventricular function in healthy cats and cats with hypertrophic cardiomyopathy.

American Journal of Veterinary Research — Sun, 29 Jan 2012 12:45:49 +0100

Conclusions and Clinical Relevance-Ivabradine had significant effects on several cardiovascular variables in anesthetized cats with HCM. Studies in awake cats with HCM are needed to clinically validate these findings. PMID: 22280379 [PubMed - in process] (Source: American Journal of Veterinary Research)

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Prevalence and Signal Characteristics of Late Gadolinium Enhancement on Contrast-Enhanced Magnetic Resonance Imaging in Patients With Takotsubo Cardiomyopathy.

Circulation Journal — Fri, 27 Jan 2012 05:00:00 +0100

Conclusions: rey myocardial signal on LGE MRI may be observed in patients with TC. However, the extent of LGE is substantially less than that of WMA and edema, and disappears within 12 months. PMID: 22293447 [PubMed - as supplied by publisher] (Source: Circulation Journal)

Complete Loss of Expression of the ANT1 GeneComplete Loss of Expression of the ANT1 Gene

Medscape Today Headlines — Fri, 27 Jan 2012 04:00:00 +0100

This case study describes a rare syndrome resulting from a complete loss of ANT1 protein, characterized by congenital cataracts, hypertrophic cardiomyopathy, and myopathy. Journal of Medical Genetics (Source: Medscape Today Headlines)

Predicting the Future in Hypertrophic Cardiomyopathy: From Histopathology To Flow To Function

Journal of the American Society of Echocardiography — Thu, 26 Jan 2012 14:17:37 +0100

Hypertrophic cardiomyopathy (HCM) is an autosomal dominant disease with both variable penetrance and a wide spectrum of phenotypic expression. Although there are several established risk factors for sudden cardiac death and progression to heart failure, these markers individually have low positive predictive value and only modestly high negative predictive value. Although the exact mechanisms are incompletely understood as yet, underlying abnormalities of myocardial architecture are thought to play a role in arrhythmogenesis and left ventricular (LV) remodeling. Alterations in myocardial structure at both macroscopic and microscopic levels predispose to myocardial ischemia, which may be associated with such complications as LV remodeling and progression to systolic dysfunction and heart fa...

Risk Factors for Malignant Ventricular Arrhythmias in Lamin A/C Mutation Carriers: A European Cohort Study

Journal of the American College of Cardiology — Thu, 26 Jan 2012 13:57:11 +0100

Objectives: The purpose of this study was to determine risk factors that predict malignant ventricular arrhythmias (MVA) in Lamin A/C (LMNA) mutation carriers. Background: LMNA mutations cause a variety of clinical phenotypes, including dilated cardiomyopathy and conduction disease. Many LMNA mutation carriers have a poor prognosis, because of a high frequency of MVA and progression to end-stage heart failure. However, it is unclear how to identify mutation carriers that are at risk for MVA. Methods: In this multicenter cohort of 269 LMNA mutation carriers, we evaluated risk factors for MVA, defined as sudden cardiac death, resuscitation, and appropriate implantable cardioverter-defibrillator (ICD) treatment. Results: In a median follow-up period of 43 months (interquartile range: 1...

Multimodality Imaging of Left Ventricular Apical Pouch With Midventricular Cavity Obliteration: Rare Variant of Hypertrophic Cardiomyopathy

Journal of the American College of Cardiology — Thu, 26 Jan 2012 13:57:11 +0100

Transthoracic echocardiography (TTE) performed on a 52-year-old man for an abnormal electrocardiogram (A) demonstrated severe midventricular hypertrophy (30 mm) (Online Videos 1 and 2), a left ventricular apical pouch (B, pink arrow, Online Videos 3 and 4), and a midventricular systolic gradient of 40 mm Hg (C, white arrows). Characteristic diastolic velocity profile of the pouch was noted (C, white arrowheads), with mitral inflow and apical pouch flow colliding “head-on” in the midventricle, producing a vivid color display (D and inset, white arrows, Online Video 5). Strain analysis revealed decreased peak systolic strain limited to the apicolateral wall (E). Cardiac magnetic resonance imaging confirmed TTE findings (F, pink arrow) but did not reveal delayed hyperenhancement (Online V...

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Cardiac MR Imaging of Nonischemic Cardiomyopathies: Imaging Protocols and Spectra of Appearances [Review]

Radiology — Thu, 26 Jan 2012 05:00:00 +0100

Recent technologic advances in cardiac magnetic resonance (MR) imaging have resulted in images with high spatial and temporal resolution and excellent myocardial tissue characterization. Cardiac MR is a valuable imaging technique for detection and assessment of the morphology and functional characteristics of the nonischemic cardiomyopathy. It has gained acceptance as a standalone imaging modality that can provide further information beyond the capabilities of traditional modalities such as echocardiography and angiography. Black-blood fast spin-echo MR images allow morphologic assessment of the heart with high spatial resolution, while T2-weighted MR images can depict acute myocardial edema. Contrast material–enhanced images can depict and be used to quantify myocardial edema, infil...

Influence of myocardial viability on responsiveness to cardiac resynchronization in ischemic dilated cardiomyopathy: a prospective observational cohort study.

Anadolu Kardiyol Der... — Thu, 26 Jan 2012 05:00:00 +0100

CONCLUSION: Patients with MV responded better than patients without MV with a significant improvement after the first week from CRT. PMID: 22281793 [PubMed - as supplied by publisher] (Source: Anadolu Kardiyol Der...)

First Pediatric Heart Transplantation From a Pediatric Donor Heart in Japan.

Circulation Journal — Wed, 25 Jan 2012 05:00:00 +0100

Conclusions: To increase pediatric heart transplantation in Japan, further enlightenment of the general population about pediatric organ donation is desirable. PMID: 22277316 [PubMed - as supplied by publisher] (Source: Circulation Journal)

CD8+ T-Cell Deficiency, Epstein-Barr Virus Infection, Vitamin D Deficiency, and Steps to Autoimmunity: A Unifying Hypothesis

International Journal of Photoenergy — Tue, 24 Jan 2012 18:18:57 +0100

CD8+ T-cell deficiency is a feature of many chronic autoimmune diseases, including multiple sclerosis, rheumatoid arthritis, systemic lupus erythematosus, Sjögren's syndrome, systemic sclerosis, dermatomyositis, primary biliary cirrhosis, primary sclerosing cholangitis, ulcerative colitis, Crohn's disease, psoriasis, vitiligo, bullous pemphigoid, alopecia areata, idiopathic dilated cardiomyopathy, type 1 diabetes mellitus, Graves' disease, Hashimoto's thyroiditis, myasthenia gravis, IgA nephropathy, membranous nephropathy, and pernicious anaemia. It also occurs in healthy blood relatives of patients with autoimmune diseases, suggesting it is genetically determined. Here it is proposed that this CD8+ T-cell deficiency underlies the development of chronic autoimmune dise...

The Janus face of OSM mediated cardiomyocyte dedifferentiation during cardiac repair and disease.

Cell Cycle — Tue, 24 Jan 2012 09:51:42 +0100

Authors: Pöling J, Gajawada P, Lörchner H, Polyakowa V, Szibor M, Böttger T, Warnecke H, Kubin T, Braun T Abstract Dedifferentiation is a common phenomenon among plants but has only been found rarely in vertebrates where it is mostly associated with regenerative responses such as formation of blastemae in amphibians to initiate replacement of lost body parts. Relatively little attention has been paid to dedifferentiation processes in mammals although a decline of differentiated functions and acquisition of immature, "embryonic" properties is seen in various disease processes. Dedifferentiation of parenchymal cells in mammals might serve multiple purposes including (1) facilitation of tissue regeneration by generation of progenitor-like cells and (2) protection of cells from hypo...

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Target of rapamcyin (TOR)-based therapeutics for cardiomyopathy: Insights from zebrafish genetics.

Cell Cycle — Tue, 24 Jan 2012 09:50:32 +0100

Authors: Ding Y, Sun X, Redfield M, Kushwaha S, Xu X Abstract Comment on: Ding Y, et al. Circ Res 2011; 109:658-69. PMID: 22262179 [PubMed - as supplied by publisher] (Source: Cell Cycle)

Long-Term Survival of Patients With Ischemic Cardiomyopathy Treated by Coronary Artery Bypass Grafting Versus Medical Therapy [ORIGINAL ARTICLES: ADULT CARDIAC]

The Annals of Thoracic Surgery — Tue, 24 Jan 2012 05:00:00 +0100

Conclusions Among a propensity-matched, risk-adjusted, observational cohort of patients with coronary artery disease, left ventricular ejection fraction less than 0.35, and no left main disease of greater than 50%, CABG is associated with a survival advantage over MED through 10 years of follow-up. (Source: The Annals of Thoracic Surgery)

Nox4-derived reactive oxygen species mediate cardiomyocyte injury in early type 1 diabetes

AJP: Cell Physiology — Tue, 24 Jan 2012 05:00:00 +0100

This study explored the role of the NADPH oxidase Nox4 as a source of reactive oxygen species (ROS) involved in the development of diabetic cardiomyopathy. Phosphorothioated antisense (AS) or sense (S) oligonucleotides for Nox4 were administered for 2 wk to rats made diabetic by streptozotocin. NADPH oxidase activity, ROS generation, and the expression of Nox4, but Nox1 or Nox2, were increased in left ventricular tissue of the diabetic rats. Expression of molecular markers of hypertrophy and myofibrosis including fibronectin, collagen, α-smooth muscle actin, and β-myosin heavy chain were also increased. These parameters were attenuated by the administration of AS but not S Nox4. Moreover, the impairment of contractility observed in diabetic rats was prevented in AS- but not S-tr...

Torsion analysis in the early detection of anthracycline-mediated cardiomyopathy

European Journal of Echocardiography — Tue, 24 Jan 2012 05:00:00 +0100

Conclusion LV torsion analysis could be a useful non-invasive approach for early detection of subclinical anthracycline cardiotoxicity. (Source: European Journal of Echocardiography)

Hyperamylinemia Contributes to Cardiac Dysfunction in Obesity and Diabetes: A Study in Humans and Rats.

Circulation Research — Tue, 24 Jan 2012 05:00:00 +0100

Conclusions:Hyperamylinemia promotes amylin deposition in the heart, causing alterations of cardiac myocyte structure and function. We propose that detection and disruption of cardiac amylin buildup may be both a predictor of heart dysfunction and a novel therapeutic strategy in diabetic cardiomyopathy. PMID: 22275486 [PubMed - as supplied by publisher] (Source: Circulation Research)

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Metoclopramide: Cardiomyopathy: case report

Reactions — Mon, 23 Jan 2012 18:35:21 +0100

(Source: Reactions)

Factors Associated With 30-Day Readmission Rates After Percutaneous Coronary Intervention [Original Investigation]

Archives of Internal Medicine — Mon, 23 Jan 2012 05:00:00 +0100

Conclusions Nearly 1 in 10 patients undergoing PCI were readmitted within 30 days. Thirty-day readmission after PCI was associated with a higher risk of 1-year mortality. (Source: Archives of Internal Medicine)

Prediction Is Very Hard, Especially About the Future: Comment on "Factors Associated With 30-Day Readmission Rates After Percutaneous Coronary Intervention" [Invited Commentary]

Archives of Internal Medicine — Mon, 23 Jan 2012 05:00:00 +0100

(Source: Archives of Internal Medicine)

MiR‐221 promotes cardiac hypertrophy in vitro through the modulation of p27 expression

Journal of Cellular Biochemistry — Mon, 23 Jan 2012 05:00:00 +0100

In conclusion, our results demonstrated that miR‐221 regulated cardiomyocyte hypertrophy probably through down‐regulation of p27, suggesting that miR‐221 may be a new intervention target for cardiac hypertrophy J. Cell. Biochem. © 2012 Wiley Periodicals, Inc. (Source: Journal of Cellular Biochemistry)

Cardiovascular Dysfunction in HIV-infected Children in a Sub-Saharan African Country: Comparative Cross-sectional Observational Study

Journal of Tropical Pediatrics — Mon, 23 Jan 2012 05:00:00 +0100

Conclusion: Structural and functional abnormalities are prevalent in HIV-infected African children and therefore justify inclusion of routine echocardiography in their standard care. (Source: Journal of Tropical Pediatrics)

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Blood Pressure Variability as Sign of Autonomic Imbalance in Patients with Idiopathic Dilated Cardiomyopathy

Pacing and Clinical Electrophysiology : PACE — Mon, 23 Jan 2012 05:00:00 +0100

Conclusion:Diastolic BPV indexes, especially those from symbolic dynamics, appear to be useful for risk stratification of sudden death in patients with IDC. (PACE 2011;1–9) (Source: Pacing and Clinical Electrophysiology : PACE)

Imaging in the Management of Ischemic Cardiomyopathy: Special Focus on Magnetic Resonance

Journal of the American College of Cardiology — Fri, 20 Jan 2012 13:57:13 +0100

Heart failure of ischemic origin has become increasingly common over the last decade because of the improved survival of patients with acute myocardial infarction. Revascularization with coronary bypass grafting or percutaneous coronary intervention plays a pivotal role in patients with ischemic cardiomyopathy, although these interventions are often associated with relatively high peri-procedural risk. The pathophysiological substrate of ischemic cardiomyopathy is heterogeneous, varying from predominantly hibernating myocardium to irreversible scarring. There is evidence to suggest that patients with hibernating myocardium benefit most from revascularization, whereas medical therapy is associated with an adverse prognosis. Therefore, noninvasive testing is recommended by relevant guideline...

Pre-hospital discharge testing after implantable cardioverter defibrillator implantation: A measure of safety or out of date? A retrospective analysis of 975 patients

Europace — Fri, 20 Jan 2012 05:00:00 +0100

Conclusions In this study predictors for PHD-failure are: (i) cardiomyopathy other than ischaemic or dilative, (ii) young age, and (iii) small or very large left ventricular end-diastolic diameter ( < 40 or > 65 mm). Particularly, (i) manufacture of device or leads, (ii) lead design, (iii) medical treatment, or (iv) gender have no significant influence on PHD failure. (Source: Europace)

Pacing-induced cardiomyopathy in patients with right ventricular stimulation for >15 years

Europace — Fri, 20 Jan 2012 05:00:00 +0100

Conclusion Considering the very long duration of RV stimulation in our study population (24.6 ± 6.6 years), the prevalence of PiCMP was remarkably low. Pacing-induced cardiomyopathy was associated with more pronounced intraventricular dyssynchrony. (Source: Europace)

Association of Heart Rate Variability With Arrhythmic Events in Patients With Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia.

Circulation Journal — Fri, 20 Jan 2012 05:00:00 +0100

Conclusions: Our data show that among ARVC/D patients HRV analysis might be helpful in identifying those with increased risk of major arrhythmic events. PMID: 22260941 [PubMed - as supplied by publisher] (Source: Circulation Journal)

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The paradox of left ventricular assist device unloading and myocardial recovery in end-stage dilated cardiomyopathy: implications for heart failure in the elderly

Heart Failure Reviews — Thu, 19 Jan 2012 06:54:52 +0100

Abstract Dilated cardiomyopathy (DCM) is a common debilitating condition with limited therapeutic options besides heart transplantation or palliation. It is characterized by maladaptive remodeling of cardiomyocytes, extracellular collagen matrix (ECCM) and left ventricular (LV) geometry which contributes to further dysfunction. LV assist devices (LVADs) can reverse adverse remodeling in end-stage DCM. However, there is a disconnect between the benefits of prolonged unloading with LVAD at molecular and cellular levels and the low rate of bridge to recovery (BTR). Potential explanations for this paradox include insufficient reverse ECCM remodeling and/or excessive reverse cardiomyocyte remodeling with atrophy. LVAD therapy is associated with decreased collagen turnover and c...

Antigens of persistent Chlamydia pneumoniae within coronary atheroma from patients undergoing heart transplantation

Journal of Clinical Pathology — Thu, 19 Jan 2012 05:00:00 +0100

Conclusions The protein antigens of persistent C pneumoniae infection found in the atheromatous lesions from patients in this study could potentially be used as markers to detect such infections and some may be virulence factors or immunogens specific to C pneumoniae, thus serving as target molecules for diagnostic use or therapeutic intervention. (Source: Journal of Clinical Pathology)

Republished article: Imaging assessment of ventricular mechanics

Postgraduate Medical Journal — Wed, 18 Jan 2012 05:00:00 +0100

This article describes the assessment of ventricular mechanics by echocardiographic techniques which are widely available to all cardiologists. The different deformation patterns and how the assessment of these can be used in the clinical setting are discussed. Left ventricular anatomy LV myocardial fibres... (Source: Postgraduate Medical Journal)

Myocardial transcriptome analysis of human arrhythmogenic right ventricular cardiomyopathy

Physiological Genomics — Wed, 18 Jan 2012 05:00:00 +0100

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited cardiomyopathy primarily of the right ventricle characterized through fibrofatty replacement of cardiomyocytes. The genetic etiology in ARVC patients is most commonly caused by dominant inheritance and high genetic heterogeneity. Though histological examinations of ARVC-affected human myocardium reveals fibrolipomatous replacement, the molecular mechanisms leading to loss of cardiomyocytes are largely unknown. We therefore analyzed the transcriptomes of six ARVC hearts and compared our findings to six nonfailing donor hearts (NF). To characterize the ARVC-specific transcriptome, we compared our findings to samples from seven patients with idiopathic dilated cardiomyopathy (DCM). The myocardial DCM and ARVC samples were ...

Proteomics Analysis of Cardiac Extracellular Matrix Remodeling in a Porcine Model of Ischemia-Reperfusion Injury.

Circulation — Wed, 18 Jan 2012 05:00:00 +0100

CONCLUSIONS: Our findings reveal a bio-signature of early and late stage ECM remodeling after myocardial ischemia-reperfusion injury, which may have clinical utility as prognostic markers and modifiable targets for drug discovery. PMID: 22261194 [PubMed - as supplied by publisher] (Source: Circulation)

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Immunoglobulin light chain amyloidosis: 2012 update on diagnosis, prognosis, and treatment

American Journal of Hematology — Tue, 17 Jan 2012 23:03:32 +0100

AbstractDisease Overview: Immunoglobulin (Ig) light chain amyloidosis is a clonal but nonproliferative plasma cell disorder in which fragments of an Ig light chain are deposited in tissues. The clinical features depend on the organs involved but can include restrictive cardiomyopathy, nephrotic syndrome, hepatic failure, and peripheral/autonomic neuropathy.Diagnosis: Tissue biopsy stained with Congo red demonstrating amyloid deposits with apple‐green birefringence is required for diagnosis. Invasive organ biopsy is not required because amyloid deposits can be found in bone marrow biopsy or subcutaneous fat aspirate in 85% of patients. Prognosis: N‐terminal pro–brain natriuretic peptide and serum troponin T values are used to classify patients into three groups of approximately equal ...

Clozapine: Dilated cardiomyopathy: 3 case reports

Reactions — Tue, 17 Jan 2012 19:08:18 +0100

(Source: Reactions)

Sorafenib/sunitinib: Cardiomyopathy: case report

Reactions — Tue, 17 Jan 2012 19:08:18 +0100

(Source: Reactions)

Echocardiography signs of early cardiac impairment in patients with breast cancer and trastuzumab therapy

Clinical Research in Cardiology — Tue, 17 Jan 2012 07:17:08 +0100

Conclusion The blockade of Her2/-neu receptors with trastuzumab in patients with breast cancer led to measurable alterations of left ventricular volume, left atrial volume, and systolic function as early as 3 months after start of treatment. Content Type Journal ArticleCategory Original PaperPages 1-12DOI 10.1007/s00392-011-0406-0Authors Stefan A. Lange, Medizinische Klinik I, Kardiologie und Angiologie, Klinikum Worms, Akademisches Lehrkrankenhaus der Johannes–Gutenberg–Universität, Mainz, GermanyBernd Ebner, Herzzentrum Dresden, Universitätsklinikum Carl-Gustav-Carus an der Technischen Universität, Dresden, GermanyAstrid Wess, Medizinische Klinik I, Kardiologie und Angiologie, Klinikum Worms, Akademisches Lehrkrankenhaus der Johannes–Gutenberg–Unive...

Mitochondrial electron transport chain deficiency, cardiomyopathy, and long‐term cardiac transplant outcome

Pediatric Transplantation — Tue, 17 Jan 2012 05:00:00 +0100

We report three patients with demonstrated oxidative phosphorylation defects and dilated cardiomyopathy who underwent cardiac transplant. All three patients tolerated immunosuppression medications and have had an excellent long‐term outcome. Our results suggest that with proper patient selection in this population, cardiac transplantation is feasible and can have good outcomes. (Source: Pediatric Transplantation)

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Current studies of diabetic cardiomyopathy and the advancement of our knowledge: time to learn from history, guidelines, ... and other disciplines?

European Journal of Heart Failure — Tue, 17 Jan 2012 05:00:00 +0100

(Source: European Journal of Heart Failure)

Detection, monitoring, and management of trastuzumab-induced left ventricular dysfunction: an actual challenge

European Journal of Heart Failure — Tue, 17 Jan 2012 05:00:00 +0100

The antibody trastuzumab, targeted to inhibit the signalling of ErbB2, a tyrosine kinase receptor overexpressed in 20–30% of breast cancers, improves the prognosis in women affected by this tumour, but produces cardiotoxicity, since ErbB2 is also involved in myocardial homeostasis. In this review, we discuss the pathophysiology of trastuzumab cardiomyopathy and the complex interplay between ErbB2 inhibition and anthracyclines, and we focus on the actual challenges of detecting, monitoring, and managing trastuzumab cardiotoxicity: the research of new, sensitive markers of early trastuzumab toxicity, before the ejection fraction is reduced, is an active field of research. (Source: European Journal of Heart Failure)

Echocardiographic quantification of regional deformation helps to distinguish isolated left ventricular non-compaction from dilated cardiomyopathy

European Journal of Heart Failure — Tue, 17 Jan 2012 05:00:00 +0100

Conclusion A special regional deformation pattern (preserved deformation in basal segments of LVNC) seems to be of major diagnostic help for the definite differential diagnosis of LVNC and DCM. (Source: European Journal of Heart Failure)

Long-term severe diabetes only leads to mild cardiac diastolic dysfunction in Zucker diabetic fatty rats

European Journal of Heart Failure — Tue, 17 Jan 2012 05:00:00 +0100

Conclusion The present study clearly shows that long term, severe DM in 45-week-old ZDF rats resulted in relatively mild impairment of diastolic LV function, whereas systolic function was well preserved. These data do not support the notion that diabetes per se is a critical factor in the induction of a clinically relevant degree of cardiac dysfunction. Co-morbidities such as hypertension and coronary artery disease probably have larger impacts on myocardial function in diabetic individuals. (Source: European Journal of Heart Failure)

Selenium- and zinc-deficient cardiomyopathy in human intestinal malabsorption: preliminary results of selenium/zinc infusion

European Journal of Heart Failure — Tue, 17 Jan 2012 05:00:00 +0100

Conclusion A reversible Se- and Zn-deficient cardiomyopathy may occur in patients with intestinal malabsorption. It is characterized by decline of myocardial antioxidant reserve, oxidative damage of cell membranes, and enhanced cell autophagy. (Source: European Journal of Heart Failure)

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Stroke aetiology and predictors of outcome in patients with heart failure and acute stroke: a 10-year follow-up study

European Journal of Heart Failure — Tue, 17 Jan 2012 05:00:00 +0100

Conclusions In patients with HF, stroke aetiology is influenced by the presence of AF and the underlying cause of HF. Early and late stroke outcome is associated with HF severity but not with the presence of AF. (Source: European Journal of Heart Failure)

Recurrent Stress Cardiomyopathy Induced by Sudafed PE.

Annals of Internal Medicine — Tue, 17 Jan 2012 05:00:00 +0100

Authors: Zlotnick DM, Helisch A PMID: 22250160 [PubMed - in process] (Source: Annals of Internal Medicine)

Enhanced cardiac PI3K{alpha} signalling mitigates arrhythmogenic electrical remodelling in pathological hypertrophy and heart failure

Cardiovascular Research — Mon, 16 Jan 2012 05:00:00 +0100

Conclusion In the setting of pathological hypertrophy or heart failure, enhanced PI3Kα signalling results in the up-regulation of K+ channel subunits, normalization of K+ current densities and preserved ventricular function. Augmentation of PI3Kα signalling, therefore, may be a useful and unique strategy to protect against the increased risk of ventricular arrhythmias and sudden death associated with cardiomyopathy. (Source: Cardiovascular Research)

Treatment with selumetinib preserves cardiac function and improves survival in cardiomyopathy caused by mutation in the lamin A/C gene

Cardiovascular Research — Mon, 16 Jan 2012 05:00:00 +0100

Conclusion Our results suggest that selumetinib or other related inhibitors that have been safely administered to humans in clinical trials could potentially be used to treat LMNA cardiomyopathy. (Source: Cardiovascular Research)

The cardioprotective effects of parathyroid hormone are independent of endogenous granulocyte-colony stimulating factor release

Cardiovascular Research — Mon, 16 Jan 2012 05:00:00 +0100

Conclusion The cardioprotective effects of PTH were shown to be independent of endogenous G-CSF release and therefore from stem cell mobilization. This puts more emphasis on the role of stem cell homing into ischaemic myocardium. (Source: Cardiovascular Research)

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Type 10 adenylyl cyclase mediates mitochondrial Bax translocation and apoptosis of adult rat cardiomyocytes under simulated ischaemia/reperfusion

Cardiovascular Research — Mon, 16 Jan 2012 05:00:00 +0100

Conclusion These results suggest a key role of sAC in SI-induced mitochondrial Bax translocation and activation of the mitochondrial pathway of apoptosis in adult cardiomyocytes. (Source: Cardiovascular Research)

A coupled biventricular finite element and lumped-parameter circulatory system model of heart failure.

Computer Methods in Biomechanics and Biomedical Engineering — Mon, 16 Jan 2012 05:00:00 +0100

Authors: Wenk JF, Ge L, Zhang Z, Soleimani M, Potter DD, Wallace AW, Tseng E, Ratcliffe MB, Guccione JM Abstract Numerical modelling of the cardiovascular system is becoming an important tool for assessing the influence of heart disease and treatment therapies. In the current study, we present an approach for modelling the interaction between the heart and the circulatory system. This was accomplished by creating animal-specific biventricular finite element (FE) models, which characterise the mechanical response of the heart, and by coupling them to a lumped-parameter model that represents the systemic and pulmonic circulatory system. In order to minimise computation time, the coupling was enforced in a weak (one-way) manner, where the ventricular pressure-volume relationships were...

TGFβ‐inducible early gene‐1 (TIEG1) mutations in hypertrophic cardiomyopathy

Journal of Cellular Biochemistry — Sun, 15 Jan 2012 07:29:34 +0100

AbstractHypertrophic cardiomyopathy (HCM) is the most common heritable cardiovascular disease. A recent study showed that male KLF10‐encoded TGFβ Inducible Early Gene‐1 knock‐out mice (TIEG‐/‐) develop HCM with 13‐fold up‐regulation of PTTG1‐encoded pituitary tumor‐transforming gene 1. We hypothesized TIEG1 could be a novel candidate gene in the pathogenesis of genotype negative HCM in humans, possibly through a loss of its repression on PTTG1 expression. A cohort of 923 unrelated patients from two independent HCM centers was analyzed for mutations in TIEG's 4 translated exons using DHPLC and direct DNA‐sequencing. Site directed mutagenesis was performed to clone novel variants. The effect of TIEG1 mutations on SMAD7 and PTTG1 promoters was studied using transient tran...

Gene testing worth it for hypertrophic cardiomyopathy

PharmacoEconomics and Outcomes News — Sun, 15 Jan 2012 04:11:42 +0100

(Source: PharmacoEconomics and Outcomes News)

Serum aldosterone and its relationship to left ventricular structure and geometry in patients with preserved left ventricular ejection fraction

European Heart Journal — Sat, 14 Jan 2012 05:00:00 +0100

Conclusion Circulating aldosterone but not ARR levels are independently related to echocardiographic parameters of LV structure, particularly in women. Higher SAC however was related to concentric LVH in either sex. Our findings in a large CV risk cohort with preserved LVEF indicate aldosterone-mediated pro-hypertrophic effects as a potential pathway for structural alterations of the left ventricular myocardium. (Source: European Heart Journal)

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Cardioprotective effect of curcumin against doxorubicin-induced myocardial toxicity in albino rats

Indian Journal of Pharmacology — Sat, 14 Jan 2012 05:00:00 +0100

Conclusion: The biochemical and histopathology reports support the cardioprotective effect of curcumin which could be attributed to antioxidant. (Source: Indian Journal of Pharmacology)

Dilated cardiomyopathy following trastuzumab chemotherapy

Indian Journal of Pharmacology — Sat, 14 Jan 2012 05:00:00 +0100

Saurabh Karmakar, Rakesh Dixit, Alok Nath, Santosh Kumar, Shilpi KarmakarIndian Journal of Pharmacology 2012 44(1):131-133The war against cancer has seen a proliferation in armamentarium over the last decades. A new antineoplastic agent, trastuzumab, was synthesized in 1991 and gained United States Food and Drug Administration approval in 1998 for treatment of metastatic breast cancer. Cardiotoxicity manifesting as dilated cardiomyopathy is a rarely reported adverse effect of trastuzumab. We hereby report a case of dilated cardiomyopathy, which occurred following trastuzumab chemotherapy in a 32-year-old female. The patient responded to discontinuation of trastuzumab and standard medical treatment. Extensive search of Indian literature revealed no reported case of dilated cardiomyopathy oc...

Cardiac Transplantation in Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy

Journal of the American College of Cardiology — Fri, 13 Jan 2012 13:52:06 +0100

Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is an inherited cardiomyopathy characterized by fibrofatty replacement of the ventricular myocardium, right ventricular (RV) dysfunction, and ventricular arrhythmias. The Johns Hopkins ARVD Program Registry, which was established in 1995 to provide clinical care for patients with ARVD/C and to study this disease, currently consists of over 1,000 patients, family members, and borderline phenotypes. From this registry, we sought to define the clinical characteristics, pre-transplant course, and indications for and outcomes of those who underwent cardiac transplantation. (Source: Journal of the American College of Cardiology)

A de novo interstitial deletion of 2p23.3–24.3 in a boy presenting with intellectual disability, overgrowth, dysmorphic features, skeletal myopathy, dilated cardiomyopathy

American Journal of Medical Genetics Part A — Fri, 13 Jan 2012 05:00:00 +0100

We describe a 14‐year‐old boy with an 8.97 Mb deletion of 2p23.3–24.3 detected by array comparative genomic hybridization (array CGH) who had intellectual disability (ID), unusual facial features, cryptorchidism, skeletal myopathy, dilated cardiomyopathy (DCM), and postnatal overgrowth (macrocephaly and tall stature). We compared the clinical features of the present case to previously described patients with an interstitial deletion within this chromosomal region and conclude that our patient exhibits a markedly different phenotype. Additional patients are needed to further delineate phenotype–genotype correlations © 2012 Wiley Periodicals, Inc. (Source: American Journal of Medical Genetics Part A)

Cardiac Hypertrophy in TnI-R21C Knock-in Mice [Protein Structure and Folding]

Journal of Biological Chemistry — Fri, 13 Jan 2012 05:00:00 +0100

The R21C substitution in cardiac troponin I (cTnI) is the only identified mutation within its unique N-terminal extension that is associated with hypertrophic cardiomyopathy (HCM) in man. Particularly, this mutation is located in the consensus sequence for β-adrenergic-activated protein kinase A (PKA)-mediated phosphorylation. The mechanisms by which this mutation leads to heart disease are still unclear. Therefore, we generated cTnI knock-in mouse models carrying an R21C mutation to evaluate the resultant functional consequences. Measuring the in vivo levels of incorporated mutant and WT cTnI, and their basal phosphorylation levels by top-down mass spectrometry demonstrated: 1) a dominant-negative effect such that, the R21C+/− hearts incorporated 24.9% of the mutant cTnI within the myo...

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Unusually High Association of Hypertrophic Cardiomyopathy and Complex Heart Defects in Children with Fasciculoventricular Pathways

Pacing and Clinical Electrophysiology : PACE — Fri, 13 Jan 2012 05:00:00 +0100

Conclusions:In this largest reported series of FVP in children, there is an unusually high association of FVP with complex CHDs, chromosomal anomalies, and hypertrophic cardiomyopathy. Any patient with such disorders and manifest preexcitation should be evaluated with a high index of suspicion for a FVP. (PACE 2012;1–6) (Source: Pacing and Clinical Electrophysiology : PACE)

Complete loss of expression of the ANT1 gene causing cardiomyopathy and myopathy

Journal of Medical Genetics — Thu, 12 Jan 2012 05:00:00 +0100

Conclusions Complete loss of expression of the ANT1 gene causes a clinical syndrome mainly characterised by cardiomyopathy and myopathy. This report expands the clinical spectrum of ANT1-related human diseases, and emphasises the crucial role of the mitochondrial ADP/ATP carriers in muscle function and pathophysiology of human myopathies. (Source: Journal of Medical Genetics)

[Renal disorders associated with monoclonal gammopathies: Diagnostic and therapeutic progress.]

Presse Medicale — Thu, 12 Jan 2012 05:00:00 +0100

Authors: Bridoux F, Delbes S, Sirac C, Pourreau F, Puyade M, Desport E, Jaccard A, Fermand JP, Touchard G Abstract Various renal disorders are associated with monoclonal gammopathies, secondary to tissue deposition or precipitation of a monoclonal immunoglobulin (Ig) or a fragment thereof (isolated Ig light chain or heavy chain). They are classified according to the localization of renal lesions, either glomerular or tubular and to the pattern of ultrastructural organization of Ig deposits. Renal disease in monoclonal gammopathies may be isolated, or associated with various systemic symptoms particularly in AL amyloidosis, Randall-type monoclonal Ig deposition disease and monoclonal cryoglobulinemias. Except for myeloma cast nephropathy, which occurs in the setting of high-mass mye...

A negative screen for mutations in calstabin 1 and 2 genes in patients with dilated cardiomyopathy.

Journal of Negative Results in BioMedicine — Wed, 11 Jan 2012 05:00:00 +0100

Conclusions: These data corroborate other studies suggesting that mutations in FKBP12 and FKBP12.6 genes are not commonly related to cardiac diseases. (Source: Journal of Negative Results in BioMedicine)

Cardiac effects of HDL and its components on diabetic cardiomyopathy.

Endocrine, Metabolic and Immune Disorders Drug Targets — Wed, 11 Jan 2012 05:00:00 +0100

Authors: Spillmann F, Van Linthout S, Tschöpe C Abstract Diabetic cardiopathy includes a specific cardiomyopathy, which occurs in the absence of coronary heart disease and hypertension under diabetes mellitus. Hyperglycemia, hyperinsulinemia, and hyperlipidemia, characteristic metabolic disturbances evident in diabetes mellitus, all three lead to a specific altered cardiac structure and function. Recently, it has been demonstrated that altered HDL, be it low HDL or dysfunctional HDL is not only a consequence of diabetes mellitus, but can also contribute to the development of diabetes mellitus, and therefore also of diabetic cardiomyopathy. This review summarizes how HDL can indirectly affect diabetic cardiomyopathy via their influence on the metabolic triggers hyperglycemia, hyper...

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Long-Term Mortality and Causes of Death in Isolated GHD, ISS, and SGA Patients Treated with Recombinant Growth Hormone during Childhood in Belgium, The Netherlands, and Sweden: Preliminary Report of 3 Countries Participating in the EU SAGhE Study.

The Journal of Clinical Endocrinology and Metabolism — Wed, 11 Jan 2012 05:00:00 +0100

Conclusions:In these cohorts, the majority of deaths (76%) were caused by accidents or suicides. Importantly, none of the patients died from cancer or from a cardiovascular disease. PMID: 22238393 [PubMed - as supplied by publisher] (Source: The Journal of Clinical Endocrinology and Metabolism)

HMGB1 is an independent predictor of death and heart transplantation in heart failure

Clinical Research in Cardiology — Tue, 10 Jan 2012 06:41:00 +0100

Conclusion Our findings demonstrate that HMGB1 plasma concentration is elevated in HF and correlates with disease severity and that is an independent predictor of the combined endpoint death and heart transplantation in HF patients. Content Type Journal ArticleCategory Original PaperPages 1-9DOI 10.1007/s00392-011-0409-xAuthors H. C. Volz, Department of Internal Medicine III, University of Heidelberg, Im Neuenheimer Feld 410, 69120 Heidelberg, GermanyD. Laohachewin, Department of Internal Medicine III, University of Heidelberg, Im Neuenheimer Feld 410, 69120 Heidelberg, GermanyD. Schellberg, Department of Internal Medicine II, University of Heidelberg, Heidelberg, GermanyA. R. Wienbrandt, Department of Internal Medicine III, University of Heidelberg, Im Neuenheimer F...

Role of cardiovascular magnetic resonance in the diagnosis of arrhythmogenic right ventricular cardiomyopathy/dysplasia with left ventricular involvement.

Anadolu Kardiyol Der... — Tue, 10 Jan 2012 05:00:00 +0100

Authors: Izgi C, Mohiaddin R PMID: 22231952 [PubMed - as supplied by publisher] (Source: Anadolu Kardiyol Der...)

Can quantitative regional myocardial dynamics contribute to the differential diagnosis of acute stress cardiomyopathy?

Anadolu Kardiyol Der... — Tue, 10 Jan 2012 05:00:00 +0100

Authors: Yalçın F, Küçükler N, Abraham TP, Garcia MJ Abstract Acute stress-induced cardiomyopathy has excessive sympathetic stimulation, microvascular dysfunction similar to hypertension. Regional prominence of left ventricular (LV) septal base and stress-induced LV hypercontractility are the particular features of both acute and chronic stress-related conditions. Novel imaging methods have shown that stress-induced cavity dilation and myocardial wall abnormalities can be a reflection of underlying previous exaggerated hypertensive episodes due to sympathetic overdrive, which results in microvascular dysfunction. Hypertension-mediated chronic stress due to increased after load episodes is possibly the main reason for blunted LV myocardial wall motion capability in patients wit...

An Unusual Cardiomyopathy after Physical Stress in a Child

Congenital Heart Disease — Tue, 10 Jan 2012 05:00:00 +0100

We described a celiac 4‐year‐old girl, following a gluten‐free diet, who developed features of cardiac failure few days after episodes of acute diarrhea with fever. The patient was treated with oral anticongestive therapy and intravenous immunoglobulins, and she had a dramatic and rapid improvement; echocardiographic features normalized in 48 hours. (Source: Congenital Heart Disease)

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Rasmussen's Aneurysm in Childhood: A Case Report

Congenital Heart Disease — Tue, 10 Jan 2012 05:00:00 +0100

ABSTRACTIn medical literature, few cases of Rasmussen's aneurysm have been reported until now, and to the best of our knowledge, there has been no description of a case of Rasmussen's aneurysm in childhood.The child described in the case report had a restrictive cardiomyopathy, complicated by pulmonary hypertension, in association with miliary tuberculosis. (Source: Congenital Heart Disease)

Association of Incident Dementia With Hospitalizations [Original Contribution]

JAMA — Tue, 10 Jan 2012 05:00:00 +0100

Conclusion Among our cohort aged 65 years or older, incident dementia was significantly associated with increased risk of hospitalization, including hospitalization for ACSCs. (Source: JAMA)

Neonatal onset of mitochondrial disorders in 129 patients: clinical and laboratory characteristics and a new approach to diagnosis

Journal of Inherited Metabolic Disease — Mon, 09 Jan 2012 19:34:51 +0100

Conclusion The most significant finding is the high incidence of neonatal cardiomyopathy and hyperammonemia. Based on our experience, we propose a diagnostic flowchart applicable to critically ill neonates suspicious for MD. This tool will allow for the use of direct molecular genetic analyses without the need for muscle biopsies in neonates with Alpers, Barth, MILS and Pearson syndromes, SCO1, SCO2, TMEM70, ATP5E, SUCLG1 gene mutations and PDH complex deficiency. Content Type Journal ArticleCategory Original ArticlePages 1-11DOI 10.1007/s10545-011-9440-3Authors Tomas Honzik, Department of Pediatrics and Adolescent Medicine, First Faculty of Medicine, Charles University in Prague and General University Hospital in Prague, Ke Karlovu 2, 120 00 Prague 2, Czech Repub...

Mood Disorders and Outcome in Patients Receiving Cardiac Resynchronization Therapy

Pacing and Clinical Electrophysiology : PACE — Mon, 09 Jan 2012 05:00:00 +0100

Conclusions:MD in patients with advanced but stable HF receiving CRT‐D therapy was a predictor of HFH alone or when combined with death but not mortality alone. (PACE 2011;1–9) (Source: Pacing and Clinical Electrophysiology : PACE)

CMR With Late Gadolinium Enhancement in Genotype Positive-Phenotype Negative Hypertrophic Cardiomyopathy

Journal of the American College of Cardiology: Cardiovascular Imaging — Mon, 09 Jan 2012 05:00:00 +0100

(Source: Journal of the American College of Cardiology: Cardiovascular Imaging)

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The Transitionalist: Optimizing Inpatient-to-Outpatient Transitions of Care [Editor's Correspondence]

Archives of Internal Medicine — Mon, 09 Jan 2012 05:00:00 +0100

(Source: Archives of Internal Medicine)

The Transitionalist: Optimizing Inpatient-to-Outpatient Transitions of Care--Reply [Editor's Correspondence]

Archives of Internal Medicine — Mon, 09 Jan 2012 05:00:00 +0100

(Source: Archives of Internal Medicine)

Takotsubo cardiomyopathy with concurrent multivessel obstructive coronary artery disease: proposition for a new clinical entity and first case surgical experience

Interactive CardioVascular and Thoracic Surgery — Mon, 09 Jan 2012 05:00:00 +0100

We present a seldom seen case of Takotsubo cardiomyopathy (TCM) with concurrent obstructive coronary artery disease (OCAD) and its first case surgical experience. We propose that TCM and OCAD can coexist and that the presence of OCAD should not be an exclusion criterion for the diagnosis of TCM. (Source: Interactive CardioVascular and Thoracic Surgery)

Cardiac involvement in patients with lamin A/C gene mutations: A cohort observation

Muscle and Nerve — Fri, 06 Jan 2012 05:00:00 +0100

DISCUSSION:Cardiac involvement represents a very common phenotypic expression of LMNA gene mutation. Subjects sharing common genetic background seem to suffer from analogue pattern of cardiac manifestation. © 2012 Wiley‐Liss, Inc. (Source: Muscle and Nerve)

Dose‐Dependent Functional Benefit of Human Cardiosphere Transplantation in Mice with Acute Myocardial Infarction

Journal of Cellular and Molecular Medicine — Fri, 06 Jan 2012 05:00:00 +0100

AbstractDespite mounting preclinical and clinical evidence of the beneficial effects of cell‐based therapy, optimal cell dosing and delivery approaches have not been identified. Cardiospheres are self‐assembling 3D microtissues formed by cardiac stem cells and supporting cell types. The ability of cardiospheres to augment cardiac function has been demonstrated in animal models of ischemic cardiomyopathy. In the present study, we studied the dose‐dependence of the benefits of human cardiospheres, delivered via intramyocardial injection, upon cardiac function and ventricular remodeling in SCID mice with acute myocardial infarction. Four doses of cardiospheres were used: 1x104, 5x104, 1x105, and 5x105 (expressed as number of plated cardiosphere‐forming cells). Acute (24 hour) cell ret...

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Natural history of transthyretin Val30Met familial amyloid polyneuropathy: analysis of late-onset cases from non-endemic areas

Journal of Neurology, Neurosurgery and Psychiatry — Fri, 06 Jan 2012 05:00:00 +0100

Conclusions The ages of onset of each clinical landmark were extremely variable between patients. However, once an initial symptom appeared, the chronological sequence of other clinical landmarks tended to be uniform, occurring within a relatively short time span. (Source: Journal of Neurology, Neurosurgery and Psychiatry)

Takotsubo Cardiomyopathy and Fluorouracil: Case Report and Review of the Literature [DIAGNOSIS IN ONCOLOGY]

Journal of Clinical Oncology — Fri, 06 Jan 2012 05:00:00 +0100

(Source: Journal of Clinical Oncology)

Hypertrophic Cardiomyopathy: Infants, Children, and Adolescents

Congenital Heart Disease — Fri, 06 Jan 2012 05:00:00 +0100

ABSTRACTHypertrophic cardiomyopathy (HCM) is characterized by inappropriate left ventricular hypertrophy (LVH) in the setting of a nondilated left ventricle. HCM is often associated with asymmetric LVH, a family history of HCM, sarcomeric genetic mutations, and an increased risk of sudden cardiac death. There is a wide clinical variability in HCM presenting during childhood and a relative lack of data on the pediatric population. This review will cover HCM presenting in infancy, childhood, and adolescence. (Source: Congenital Heart Disease)

Dose-Dependent Functional Benefit of Human Cardiosphere Transplantation in Mice with Acute Myocardial Infarction.

J Cell Mol Med — Fri, 06 Jan 2012 05:00:00 +0100

Authors: Shen D, Cheng K, Marbán E Abstract Despite mounting preclinical and clinical evidence of the beneficial effects of cell-based therapy, optimal cell dosing and delivery approaches have not been identified. Cardiospheres are self-assembling 3D microtissues formed by cardiac stem cells and supporting cell types. The ability of cardiospheres to augment cardiac function has been demonstrated in animal models of ischemic cardiomyopathy. In the present study, we studied the dose-dependence of the benefits of human cardiospheres, delivered via intramyocardial injection, upon cardiac function and ventricular remodeling in SCID mice with acute myocardial infarction. Four doses of cardiospheres were used: 1x10(4) , 5x10(4) , 1x10(5) , and 5x10(5) (expressed as number of plated cardi...

Effect of stress testing on left ventricular mechanical synchrony by phase analysis of gated positron emission tomography in patients with normal myocardial perfusion

European Journal of Nuclear Medicine and Molecular Imaging — Thu, 05 Jan 2012 06:52:13 +0100

Conclusion LV mechanical dyssynchrony indices by phase analysis are smaller when derived from peak stress versus rest gated PET imaging in patients with normal MPI, irrespective of the resting LVEF. Content Type Journal ArticleCategory Original ArticlePages 1-8DOI 10.1007/s00259-011-2025-4Authors Wael AlJaroudi, Heart and Vascular Imaging Institutes, Cleveland Clinic, Cleveland, OH, USAM. Chadi Alraies, Heart and Vascular Imaging Institutes, Cleveland Clinic, Cleveland, OH, USAFrank DiFilippo, Heart and Vascular Imaging Institutes, Cleveland Clinic, Cleveland, OH, USARichard C. Brunken, Heart and Vascular Imaging Institutes, Cleveland Clinic, Cleveland, OH, USAManuel D. Cerqueira, Heart and Vascular Imaging Institutes, Cleveland Clinic, Cleveland, OH, USAWael A. Jabe...

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Drug‐like actions of autoantibodies against receptors of the autonomous nervous system and their impact on human heart function

British Journal of Pharmacology — Thu, 05 Jan 2012 05:00:00 +0100

In conclusion, such autoantibodies seem to cause or promote chronic human left ventricular dysfunction by acting on their receptor targets in a drug‐like fashion. However, the pharmacology of this interaction is poorly understood. It is unclear how the autoantibodies trigger changes in receptor activity and second messenger coupling and how that is related to the pathogenesis and severity of the associated diseases. Here, we summarise the available evidence regarding these issues and discuss these findings in the light of recent knowledge about the conformational activation of the human β2‐adrenergic receptor and the properties of bona fide cardio‐pathogenic autoantibodies derived from immune‐adsorption therapy of DCM patients. These considerations might contribute to the concepti...

Gene Analysis Helps Identify Basis Of Histiocytoid Cardiomyopathy - A Rare Infant Heart Disorder

Health News from Medical News Today — Wed, 04 Jan 2012 09:00:00 +0100

Researchers are closing in on a rare genetic disorder causing a heart condition in infants. Histiocytoid cardiomyopathy (HC) often causes sudden death before a child reaches 2 years of age. Gene analysis is helping to narrow the many theories surrounding the genetic basis of HC. A study reported in the current issue of the journal Pediatric and Developmental Pathology compares cardiac tissue from 12 patients with HC and 12 age-matched controls. Researchers found differences in gene expression that could indicate a predisposition for HC. HC typically occurs in the first 2 years of life... (Source: Health News from Medical News Today)

Extracardiac findings detected by cardiac magnetic resonance imaging

European Radiology — Wed, 04 Jan 2012 07:00:56 +0100

Conclusions About one fifth of patients undergoing CMR were found to have potentially significant ECF requiring additional work-up. The second dedicated reading detected significantly more ECF compared with the first clinical reading emphasising the importance of active search for extracardiac abnormalities when evaluating CMR studies. Key Points • Many patients undergoing cardiac MR have significant extracardiac findings (ECF) • These impact on management and require additional work-up. • Wide review of scout and cine sequences will detect most ECFs. • Education of radiologists is important to identify ECFs on CMR studies. Content Type Journal ArticleCategory CardiacPages 1-8DOI 10.1007/s00330-011-2369-yAuthors...

Phidippides Cardiomyopathy: A Review and Case Illustration

Clinical Cardiology — Wed, 04 Jan 2012 05:00:00 +0100

AbstractPhidippides was a Greek messenger who experienced sudden death after running more than 175 miles in two days. In today's world, marathon running and other endurance sports are becoming more popular and raising concern about sudden deaths at these events. Once etiologies such has hypertrophic cardiomyopathy, anomalous coronary arteries, and coronary atherosclerosis have been excluded, there is now an additional consideration termed Phidippides cardiomyopathy. Because endurance sports call for a sustained increase in cardiac output for several hours, the heart is put into a state of volume overload. It has been shown that approximately one‐third of marathon runners experience dilation of the right atrium and ventricle, have elevations of cardiac troponin and natriuretic peptides, a...

Recurrent and founder mutations in the Netherlands: the cardiac phenotype of DES founder mutations p.S13F and p.N342D.

Netherlands Heart Journal — Wed, 04 Jan 2012 05:00:00 +0100

CONCLUSION: DRM may occur as an apparently isolated cardiological disorder. The cardiac phenotypes of the DES founder mutations p.S13F and p.N342D are characterised by cardiac conduction disease and cardiomyopathy, often with right ventricular involvement. PMID: 22215463 [PubMed - as supplied by publisher] (Source: Netherlands Heart Journal)

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Miracle toddler Kai Clark survives rare Hypertrophic Cardiomyopathy illness

the Mail online | Health — Tue, 03 Jan 2012 09:48:27 +0100

Kai Clark, from Clacton-on-Sea, Essex,miraculously beat the odds to survive a heart attack aged just two after medics declared him dead for six minutes. (Source: the Mail online | Health)

Genetic polymorphisms in TNFA/TNFR2 genes and Chagas disease in a Colombian endemic population.

Cytokine — Tue, 03 Jan 2012 05:00:00 +0100

This study included 313 patients from Colombia serologically positive for Trypanosoma cruzi antigens (cardiomyopathic, N=159; asymptomatic, N=154). Genotypes were determined by polymerase chain reaction (PCR)-restriction fragment length polymorphism method. We found a significant difference in the distribution of the TNFA -1031C (p=0.0153, OR=1.69, CI=1.10-2.58) and -308A (p=0.0002, OR=2.60, CI=1.53-4.39) alleles between cardiomyopathic and asymptomatic subjects. In addition, we observed that the TNFR2 +676T allele was monomorphic in our population. Our results suggest that TNFA -1031C and -308A gene polymorphisms may influence the susceptibility to develop chagasic cardiomyopathy in the population under study. PMID: 22221522 [PubMed - as supplied by publisher] (Source: Cytokine)

Home monitoring may help manage and reduce costs for heart failure

UCLA Newsroom: Health Sciences — Mon, 02 Jan 2012 21:00:00 +0100

UCLA RESEARCH ALERT FINDINGS: Heart failure affects 5.8 million people in the U.S. alone and is responsible for nearly 1 million hospitalizations each year, most resulting from a build-up of body fluid in the lungs and other organs due to the heart's inability to pump effectively. The disease needs to be closely tracked in order to avoid such hospitalizations, and home-monitoring interventions may be especially useful, UCLA researchers say. In their new paper, the UCLA authors discuss the importance of heart failure disease-management and early identification, as well as the treatment of body-fluid congestion, using a number of home-monitoring strategies, including self-care, such as daily weighing and medication management; phone calls from a nurse or a...

Serum tenascin-C levels as a prognostic biomarker of heart failure events in patients with hypertrophic cardiomyopathy.

Journal of Cardiology — Mon, 02 Jan 2012 05:00:00 +0100

CONCLUSIONS: Heart failure events were more frequently observed in patients with high serum TN-C levels than in those with low TN-C levels. Serum TN-C levels may be a new prognostic biomarker for heart failure in patients with HCM. PMID: 22218323 [PubMed - as supplied by publisher] (Source: Journal of Cardiology)

Sudden death due to commotio cordis associated to non compacted myocardium. - Aquieri A, Rodríguez Granillo G, Vázquez Blanco M, Lerman J.

SafetyLit: All (Unduplicated) — Sun, 01 Jan 2012 12:07:07 +0100

Non compact of the left ventricular myocardium is a rare congenital cardiomyopathy characterized by the presence of multiple and prominent deep trabeculations in the ventricular wall, that define recesses communicated with the main ventricular chamber. Thi... (Source: SafetyLit: All (Unduplicated))

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APACHE II Score, Rather Than Cardiac Function, May Predict Poor Prognosis in Patients With Stress-Induced Cardiomyopathy.

J Korean Med Sci — Sun, 01 Jan 2012 05:00:00 +0100

Authors: Joe BH, Jo U, Kim HS, Park CB, Hwang HJ, Sohn IS, Jin ES, Cho JM, Park JH, Kim CJ Abstract While the disease course of stress-induced cardiomyopathy (SIC) is usually benign, it can be fatal. The prognostic factors to predict poorer outcome are not well established, however. We analyzed the Acute Physiology And Chronic Health Evaluation (APACHE) II score to assess its value for predicting poor prognosis in patients with SIC. Thirty-seven consecutive patients with SIC were followed prospectively during their hospitalization. Clinical factors, including APACHE II score, coronary angiogram, echocardiography and cardiac enzymes at presentation were analyzed. Of the 37 patients, 27 patients (73%) were women. The mean age was 66.1 ± 15.6 yr, and the most common presentation was ...

Real Time Three‐Dimensional Echocardiography for the Evaluation of Cardiomyopathy

Echocardiography — Sun, 01 Jan 2012 05:00:00 +0100

Cardiomyopathy refers to a set of diseases that are characterized by myocardial dysfunction. Classically, two‐dimensional echocardiography has been used in the diagnosis of these disorders and to help guide their management. Three‐dimensional transthoracic echocardiography is now increasingly being used in the diagnosis, management, and prognostication of intrinsic cardiomyopathies. In this article, we summarize the available data on the use of three‐dimensional transthoracic echocardiography in various forms of intrinsic cardiomyopathy as well as some of its advantages over traditional two‐dimensional transthoracic echocardiography. (Echocardiography 2012;29:76‐87) (Source: Echocardiography)

Cardiac Magnetic Resonance Imaging Predicts Recovery of Left Ventricular Function in Acute Onset Cardiomyopathy

Heart, Lung and Circulation — Sun, 01 Jan 2012 05:00:00 +0100

Background: In acute onset cardiomyopathy, acute myocarditis is an important cause, as it is associated with a greater likelihood of recovery of cardiac function and its presence may direct specific therapies. Myocarditis can be detected by cardiac magnetic resonance imaging (CMR); however its diagnostic utility and relation to prognosis in acute onset cardiomyopathy are unknown.Methods: We performed CMR on 61 patients with acute onset cardiomyopathy and a left ventricular ejection fraction (LVEF) (Source: Heart, Lung and Circulation)

Anaesthetic management of peripartum cardiomyopathy using "epidural volume extension" technique: A case series.

Annals of Cardiac Anaesthesia — Sun, 01 Jan 2012 05:00:00 +0100

We present a case series of five parturients that were posted for elective cesarean section and managed successfully by the epidural volume extension technique. PMID: 22234021 [PubMed - in process] (Source: Annals of Cardiac Anaesthesia)

Effect of 40 mg Versus 10 mg of Atorvastatin on Oxidized Low‐Density Lipoprotein, High‐Sensitivity C‐Reactive Protein, Circulating Endothelial‐Derived Microparticles, and Endothelial Progenitor Cells in Patients With Ischemic Cardiomyopathy

Clinical Cardiology — Sun, 01 Jan 2012 05:00:00 +0100

Conclusions:Use of 40 mg of atorvastatin might decrease the levels of circulating EMPs and increase the number of circulating EPCs in patients with ICM in comparison with 10 mg of atorvastatin, and the effect might be independent of the decrease of lipids, oxLDL, and hsCRP. © 2012 Wiley Periodicals, Inc.This study was supported by the Bureau of Health of Guangzhou city (2009‐YB‐186). The authors have no other funding, financial relationships, or conflicts of interest to disclose. (Source: Clinical Cardiology)

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Beneficial effects of edaravone, a novel antioxidant, in rats with dilated cardiomyopathy

Journal of Cellular and Molecular Medicine — Sun, 01 Jan 2012 05:00:00 +0100

AbstractEdaravone, a novel antioxidant acts by trapping hydroxyl radicals, quenching active oxygen and so on. Its cardioprotective activity against experimental autoimmune myocarditis (EAM) was reported. Nevertheless, it remains to be determined whether edaravone protects against cardiac remodeling in dilated cardiomyopathy (DCM). The present study was undertaken to assess whether edaravone attenuates myocardial fibrosis and examine the effect of edaravone on cardiac function in rats with DCM after EAM. Rat model of EAM was prepared by injection with porcine cardiac myosin. 28 days after immunization, we administered edaravone intraperitoneally at 3 and 10 mg/kg/day to rats for 28 days. The results were compared with vehicle treated rats with DCM. Cardiac function, by hemodynamic and echoc...

Effectiveness of Cardiac Resynchronization Therapy in Diabetic Patients with Ischemic and Nonischemic Cardiomyopathy

Annals of Noninvasive Electrocardiology — Sun, 01 Jan 2012 05:00:00 +0100

Conclusions: The present study shows that treatment with CRT‐D in at‐risk cardiac patients with DM is associated with substantial reductions in the risk of HF or death and improvement in cardiac remodeling in those with ischemic and nonischemic cardiomyopathy, with a more pronounced benefit in patients with nonischemic disease.Ann Noninvasive Electrocardiol 2012;17(1):14–21 (Source: Annals of Noninvasive Electrocardiology)

Late Gadolinium Enhancement CMR in Patients with Tachycardia‐Induced Cardiomyopathy Caused by Idiopathic Ventricular Arrhythmias

Pacing and Clinical Electrophysiology : PACE — Sun, 01 Jan 2012 05:00:00 +0100

Conclusions:LGE is a rare finding in patients with TICMP caused by idiopathic ventricular arrhythmias. LGE‐CMR can be used in the diagnostic work‐up of patients with TICMP. Further prospective studies are required to determine the role of LGE‐CMR in predicting the recovery of left ventricular systolic dysfunction in patients with presumptive TICMP.(PACE 2012;1–6) (Source: Pacing and Clinical Electrophysiology : PACE)

Hemodynamic manifestation of hypertrophic obstructive cardiomyopathy.

Texas Heart Institute Journal — Sat, 31 Dec 2011 08:54:03 +0100

Authors: Ren X PMID: 22199449 [PubMed - in process] (Source: Texas Heart Institute Journal)

Hypertension, tachycardia, and reversible cardiomyopathy temporally associated with milnacipran use.

Texas Heart Institute Journal — Sat, 31 Dec 2011 08:54:03 +0100

Authors: Forman MB, Sutej PG, Jackson EK Abstract Elevated catecholamine levels are a well-recognized cause of various types of cardiomyopathy. Causes of catecholamine elevation include tumors, toxins, drugs, emotional stress, and sepsis. Milnacipran is a dual and equipotent inhibitor of norepinephrine and serotonin uptake. It is frequently prescribed as therapy for fibromyalgia, and the drug has a good safety profile. Herein, we report the case of a 42-year-old woman with undefined connective-tissue disease and fibromyalgia who developed a severe and reversible cardiomyopathy while taking recommended doses of milnacipran. The cardiomyopathy was associated with a hyperadrenergic state manifested by tachycardia, hypertension, and elevated plasma catecholamine levels. The discontinua...

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Encapsulation of cardiac stem cells in superoxide dismutase‐loaded alginate prevents doxorubicin‐mediated toxicity

Journal of Tissue Engineering and Regenerative Medicine — Fri, 30 Dec 2011 05:00:00 +0100

In conclusion, this study demonstrates that encapsulation of CSCs in SOD‐loaded alginate hydrogel enhances CSC survival in the presence of DOX, raising the possibility of its application as a novel therapy for the treatment of acute and early onset DOX‐induced cardiotoxicity. Copyright © 2011 John Wiley & Sons, Ltd. (Source: Journal of Tissue Engineering and Regenerative Medicine)

Proteome changes in the myocardium of experimental chronic diabetes and hypertension Role of PPARα in the associated hypertrophy.

Journal of Proteomics — Fri, 30 Dec 2011 05:00:00 +0100

Authors: Ares-Carrasco S, Picatoste B, Camafeita E, Carrasco-Navarro S, Zubiri I, Ortiz A, Egido J, López JA, Tuñón J, Lorenzo O Abstract Diabetes with or without the presence of hypertension damages the heart. However, there is currently a lack of information about these associated pathologies and the alteration of linked proteins. For these reasons, we were interested in the potential synergistic interaction of diabetes and hypertension in the heart, focusing on the proteome characterization of the pathological phenotypes and the associated hypertrophic response. We treated normotensive and spontaneously hypertensive (SHR) rats with either streptozotocin or vehicle. After 22weeks, type-I diabetic (DM1), SHR, SHR/DM1 and control left-ventricles were studied using proteomic appr...

Cardiac and skeletal muscle expression of mutant β‐myosin heavy chains, degree of functional impairment and phenotypic heterogeneity in hypertrophic cardiomyopathy

Journal of Cellular Physiology — Thu, 29 Dec 2011 05:00:00 +0100

In this study, the expression of the mutant β‐myosin protein isoform, isolated from slow‐twitch fibres of skeletal muscle, was demonstrated by Northern and Western blot analysis; this myosin showed a decreased in vitro motility activity and produced a lower actin‐activated ATPase activity. Isometric tension, measured in single slow‐twitch fibres isolated from the affected individuals, also showed a significant decrease. The degree of impairment of β‐myosin function, as well as the loss in isometric tension development, were strictly dependent on the amount of the isoform transcribed from the mutated allele. Interestingly, a strong correlation was also demonstrated between mutant β‐myosin content and clinical features of FHC. On the other hand, we were unable to detect any co...

Autosomal Recessive Dilated Cardiomyopathy due to DOLK Mutations Results from Abnormal Dystroglycan O-Mannosylation

PLoS Genetics — Thu, 29 Dec 2011 05:00:00 +0100

by Dirk J. Lefeber, Arjan P. M. de Brouwer, Eva Morava, Moniek Riemersma, Janneke H. M. Schuurs-Hoeijmakers, Birgit Absmanner, Kiek Verrijp, Willem M. R. van den Akker, Karin Huijben, Gerry Steenbergen, Jeroen van Reeuwijk, Adam Jozwiak, Nili Zucker, Avraham Lorber, Martin Lammens, Carlos Knopf, Hans van Bokhoven, Stephanie Grünewald, Ludwig Lehle, Livia Kapusta, Hanna Mandel, Ron A. Wevers Genetic causes for autosomal recessive forms of dilated cardiomyopathy (DCM) are only rarely identified, although they are thought to contribute considerably to sudden cardiac death and heart failure, especially in young children. Here, we describe 11 young patients (5–13 years) with a predominant presentation of dilated cardiomyopathy (DCM). Metabolic investigations showed deficient protein N-g...

Compound and Digenic Heterozygosity in Desmosome Genes as a Cause of Arrhythmogenic Right Ventricular Cardiomyopathy in Japanese Patients.

Circulation Journal — Wed, 28 Dec 2011 05:00:00 +0100

Conclusions: Our comprehensive genetic analysis of desmosome genes identified 3 cases of compound heterozygosities in trans and 1 of digenic heterozygosity among 7 definite Japanese ARVC patients, providing novel insights into the genetic basis of Japanese ARVC. PMID: 22214898 [PubMed - as supplied by publisher] (Source: Circulation Journal)

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Serum copeptin/NT-proBNP ratio: A more reliable index of absolute endogenous stress and prognosis during the course of Tako-tsubo cardiomyopathy?

International Journal of Cardiology — Wed, 28 Dec 2011 05:00:00 +0100

Diagnostic approaches for the non-invasive differentiation of Tako-tsubo cardiomyopathy (TTC) and acute coronary syndromes (ACS) have drawn particular attention in the recent years.These approaches are generally based on a variety of suggested initial electrocardiographic (ECG) findings and non-invasive imaging modalities. However, serum levels of a variety of biomarkers including copeptin (C-terminal provasopressin) (CP) and the ratios of serum N-terminal pro-brain natriuretic peptide (NT-proBNP)/myocardial enzymes (myoglobin and troponin T) have also been recently suggested for the differentiation of these two entities. Notwithstanding the recent progress in the non-invasive diagnosis of TTC, clinical tools for the risk stratification (serum markers, etc.) that may potentially serve as p...

Marked lipomatous hypertrophy of the right ventricular wall: A distinct clinicopathological entity from arrhythmogenic right ventricular cardiomyopathy?

International Journal of Cardiology — Wed, 28 Dec 2011 05:00:00 +0100

A 21-year-old male was referred to our hospital because of palpitations due to sustained ventricular tachycardia (VT) (A) after hitting his chest hard while snow-boarding. Since the VT was resistant to antiarrhythmic drugs, it was converted to sinus rhythm by cardioversion. His 12-lead electrocardiogram (ECG) during sinus rhythm showed conduction abnormalities, with a complete right bundle branch block morphology, right axis deviation, and first degree atrioventricular block, accompanied by inverted T waves in leads V1-3 (B). Although he had remained asymptomatic by that time, he was diagnosed with a cardiac tumor of unknown pathogenesis in his right ventricle (RV) when he was three years old. Annual checkups with non-invasive imagings in a pediatrics department had demonstrated no remarka...

Takotsubo-pericarditis association

The American Journal of Emergency Medicine — Wed, 28 Dec 2011 05:00:00 +0100

I have read the interesting article by Jimmy and Foo in a recent issue of the journal and congratulate them for their observation. The article clearly exemplified that Takotsubo cardiomyopathy (TC) can present with electrocardiographic (ECG) features of pericarditis. In the presented ECG, the widespread concave upwards ST-segment elevation and PR-segment depression (except in aVR and V1) and the absence of reciprocal ST-segment depression is a clear ECG evidence of acute pericarditis. TC with ECG criteria of acute pericarditis is known in the literature and has been previously reported. demonstrates all previously published cases taking into account the sequence of events, outcome, and possible explanations provided by the authors for this association. Regarding the sequence of both pathol...

[Three recurrent episodes of Tako-Tsubo cardiomyopathy precipitated by an emotional stressful event: A case report.]

Annales de Cardiologie et d'Angeiologie — Tue, 27 Dec 2011 05:00:00 +0100

Authors: Montassier E, Segard J, Martinage A, Goffinet N, Piriou N, Le Conte P, Potel G, Gueffet JP Abstract Tako-Tsubo cardiomyopathy, first described in 1990 by Sato in Japan, has recently gained increasing consideration when reported in non-Japanese patients, including the United States and Europe. Typical presentation mimics acute coronary syndrome, with acute chest pain and/or dyspnoea, associated to electrocardiographic changes and moderate cardiac biomarkers release, but in which coronary angiography reveals no coronary arteries lesions and echocardiography or left ventriculography shows a reversible left ventricle systolic dysfunction. Prognosis is good, in contrast to acute coronary syndrome, provided that the patients survive the possible life-threatening acute presentati...

[A cause of dilated cardiomyopathy in child: Primary carnitine deficiency.]

Annales de Cardiologie et d'Angeiologie — Tue, 27 Dec 2011 05:00:00 +0100

CONCLUSION: Primary carnitine deficiency is a cause of dilated cardiomyopathy in child. It must systematically be suspected when a child presents a primitive cardiomyopathy. The treatment with oral carnitine for live is simple, with excellent prognosis. PMID: 22260907 [PubMed - as supplied by publisher] (Source: Annales de Cardiologie et d'Angeiologie)

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Mid-septal hypertrophy and apical ballooning; potential mechanism of ventricular tachycardia storm in patients with hypertrophic cardiomyopathy.

Yonsei Medical Journal — Tue, 27 Dec 2011 03:55:01 +0100

Authors: Hwang ES, Pak HN Abstract Medically refractory ventricular tachycardia (VT) storm can be controlled with radiofrequency catheter ablation (RFCA), however, it may be difficult to control in some patients with hemodynamic overload. We experienced a patient with intractable VT storm controlled by hemodynamic unloading. The patient had mid-septal hypertrophic cardiomyopathy with an implantable cardioverter defibrillator (ICD) back-up. Because of the severe mid-septal hypertrophy, his left ventricle (LV) had an hourglass-like morphology and showed apical ballooning; the focus of VT was at the border of apical ballooning. Although we performed VT ablation because of electrical storm with multiple ICD shocks, VT recurred 1 hour after procedure. As the post-RFCA monomorphic VT was...

Inverted Takotsubo-Like Left Ventricular Dysfunction with Pulmonary Oedema Developed after Caesarean Delivery Complicated by Massive Haemorrhage in a Severe Preeclamptic Parturient with a Prolonged Painful Labour

Computational Intelligence and Neuroscience — Tue, 27 Dec 2011 00:39:43 +0100

Inverted takotsubo cardiomyopathy (TTC), a variant of stress-induced cardiomyopathy, features transient myocardial dysfunction characterized by a hyperdynamic left ventricular apex and akinesia of the base. Herein, we describe a 38-year-old primigravida with severe preeclampsia who had active labour for 4 h followed by an emergency caesarean delivery. She developed postpartum haemorrhage due to uterine atony complicated by pulmonary oedema, which was managed with large-volume infusion and hysterectomy. Her haemodynamic instability was associated with cardiac biomarkers indicative of diffuse myocardial injury and echocardiographic findings of an “inverted” TTC. The patient was almost fully recovered one month later. Our case shows that a reversible inverted TTC may result...

Tissue engineered myoblast sheets improved cardiac function sufficiently to discontinue LVAS in a patient with DCM: report of a case

Surgery Today — Mon, 26 Dec 2011 16:45:45 +0100

Abstract Dilated cardiomyopathy (DCM) is a heart muscle disease characterized by progressive heart failure, and is a leading cause of mortality and morbidity. Recently, cellular therapy for end-stage heart failure has been emerging. We herein report a 56-year-old male who received a transplant of autologous myoblast sheets manufactured in temperature-responsive culture dishes. His clinical condition improved markedly, leaving him without any arrhythmia and able to discontinue using a left ventricular assist system and avoid cardiac transplantation. These findings suggest that cellular therapy using myoblast sheets is a promising new strategy for treating patients with end-stage DCM. This method might be an effective alternative to heart transplantation in the near future. ...

Prevalence and progression of recipient‐twin cardiomyopathy in early‐stage twin–twin transfusion syndrome

Ultrasound in Obstetrics and Gynecology — Sun, 25 Dec 2011 20:40:53 +0100

ConclusionEchocardiography demonstrates a high incidence of recipient‐twin cardiomyopathy in early‐stage TTTS. The more advanced the recipient‐twin cardiomyopathy is, the more likely is progression to occur during observation or following amnioreduction. Copyright © 2011 ISUOG. Published by John Wiley & Sons, Ltd. (Source: Ultrasound in Obstetrics and Gynecology)

Nerve growth factor gene therapy using adeno-associated viral vectors prevents cardiomyopathy in type 1 diabetic mice.

Diabetes — Sun, 25 Dec 2011 06:07:36 +0100

This study investigated whether NGF gene transfer can prevent diabetic cardiomyopathy in mice. We worked with mice with streptozotocin-induced type 1 diabetes and with nondiabetic control mice. After having established that diabetes reduces cardiac NGF mRNA expression, we tested NGF gene therapies with adeno-associated viral vectors (AAVs) for the capacity to protect the diabetic mouse heart. To this aim, after 2 weeks of diabetes, cardiac expression of human NGF or β-Gal (control) genes was induced by either intramyocardial injection of AAV serotype 2 (AAV2) or systemic delivery of AAV serotype 9 (AAV9). Nondiabetic mice were given AAV2-β-Gal or AAV9-β-Gal. We found that the diabetic mice receiving NGF gene transfer via either AAV2 or AAV9 were spared the progressive deterioration of c...

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Multiple coronary-left ventricular fistulae associated with apical hypertrophic cardiomyopathy: Coronary angiogram compared to coronary scan and cardiac magnetic resonance scan.

Cardiology Journal — Sat, 24 Dec 2011 18:12:03 +0100

Authors: PMID: 22113763 [PubMed - in process] (Source: Cardiology Journal)

ICD implantation in left ventricular noncompaction: A case report and review of the literature.

Cardiology Journal — Sat, 24 Dec 2011 18:12:03 +0100

We report the case of a 43 year-old patient with LVNC, nonsustained ventricular tachycardia and family history of sudden cardiac death (SCD). An implantable cardioverter-defibrillator (ICD) was prophylactically implanted because of the individual's high SCD risk. Although ICD is an effective option for preventing SCD, data on the long-term follow-up of patients with LVNC is limited. (Cardiol J 2011; 18, 6: 691-694). PMID: 22113759 [PubMed - in process] (Source: Cardiology Journal)

Cardioprotection by Hepc1 in cTnT(R141W) transgenic mice.

Transgenic Research — Sat, 24 Dec 2011 05:00:00 +0100

Authors: Zhang L, Lu D, Zhang W, Quan X, Dong W, Xu Y, Zhang L Abstract Hepcidin 1 (Hepc1) is a peptide hormone secreted by the liver in response to iron loading. It is expressed in the heart and is thought to play a role in the regulation of iron homeostasis in an autocrine and paracrine fashion. We have shown that expression of Hepc1 is strongly down-regulated in the heart of the cTnT(R141W) transgenic mouse model of dilated cardiomyopathy (DCM) at 3 months of age. Transgenic mice with heart tissue-specific Hepc1 expression alone or in combination with the cTnT(R141W) mutation were produced to study the effects of Hepc1 on DCM. Transgenic expression of Hepc1 was found to be nonlethal and resulted in decreased mortality in cTnT(R141W) transgenic mice, from 29.6 to 7.4%(n = 27;...

TOR-autophagy signaling in adult zebrafish models of cardiomyopathy.

Autophagy — Fri, 23 Dec 2011 23:42:12 +0100

Authors: Ding Y, Sun X, Xu X Abstract The target of rapamycin (TOR) kinase is part of an evolutionarily conserved signaling pathway that coordinates cell growth, survival, and autophagy. Previously, pharmacological studies using rapamycin have suggested a cardioprotective effect of TOR signaling inhibition on cardiomyopathy. We found that rapamycin exerts a conserved cardioprotective effect in two adult zebrafish models of cardiomyopathy of different etiology, and provided the first genetic evidence to support a long-term cardioprotective effect of TOR signaling inhibition. Moreover, we detected dynamic TOR-autophagy activities along different stages of cardiomyopathy. This needs to be considered when developing TOR-autophagy-based therapeutics for cardiomyopathy. PMID: 2218622...