Last Build Date: Tue, 22 Mar 2016 08:22:41 +0100
Mon, 21 Mar 2016 00:00:00 +0100Conclusion The aetiology of cardiomyopathy should be taken into consideration for the therapy of ICD patients. The protective role of CRT devices should be pointed out in ischaemic cardiomyopathy (ICM) and a more rigorous antiarrhythmic treatment should be considered for ICM patients with secondary prevention or for dilated cardiomyopathy patients with AF. (Source: Europace)
Mon, 21 Mar 2016 00:00:00 +0100Human populations exhibit significant allele frequencies of two non-synonymous single nucleotide polymorphisms within the coding block of the β1-adrenergic receptor (β1AR) gene, which cause alternative incorporation of amino acids at positions 49 (Gly/Ser) and 389 (Gly/Arg) of the receptor protein and give rise to functionally distinct β1AR-variants . Receptors encoded by the allele β1AR389Arg are more effective in promoting baseline and agonist-stimulated cAMP-production as compared to β1AR389Gly, while receptors encoded by the allele β1AR49Gly are more prone to agonist-induced internalisation and desensitisation as compared to β1AR49Ser . (Source: International Journal of Cardiology)
Mon, 21 Mar 2016 00:00:00 +0100Publication date: Available online 20 March 2016 Source:Pharmacology & Therapeutics Author(s): Lionel D. Jensen, David J. Marchant Myocarditis is a heterogeneous group of disorders defined by inflammation of the heart muscle. The primary clinical manifestations of myocarditis are heart failure and sudden death in children and young adults. Numerous interventions have been investigated for the treatment of myocarditis, including broad spectrum alteration of the immune response and antiviral treatments; however, success has been limited. Since the myocarditis treatment trials in the 1990s there has been an improved understanding of disease progression and new facets of the immune response have been discovered. This new information provides fresh opportunities to develop therapeut...
Fri, 18 Mar 2016 01:32:02 +0100In conclusion, PPARα elevated levels may be a direct cause of adverse remodeling, both metabolic and structural. Thus, there is limited time window for therapy modulating cardiac metabolism and protecting cardiomyocyte structure in failing heart. PMID: 26981112 [PubMed] (Source: PPAR Research)
Fri, 18 Mar 2016 00:00:00 +0100Publication date: Available online 17 March 2016 Source:Journal of Cardiology Cases Author(s): Masashi Kamioka, Minoru Nodera, Hitoshi Suzuki, Yasuchika Takeishi A high defibrillation threshold (DFT) infrequently occurs during device implantation. The major cause of high DFT is pneumothorax. However, there are few data about shock coil and left ventricular tip–right ventricular coil (LV tip–RV coil) impedance as a marker of a high DFT caused by pneumothorax. A 65-year-old man, diagnosed with dilated cardiomyopathy, was referred for cardiac resynchronization therapy (CRT) defibrillator implantation with single shock coil lead. The shock coil impedance was high and LV tip–RV coil impedance was normal just before DFT test. Then, high DFT was detected. Afterwards, pneumothorax was ...
Thu, 17 Mar 2016 01:39:01 +0100CONCLUSIONS: The presence of dilated cardiomyopathy, elevated CVP at 48 h and reduced CRP levels on day 7 predicted 5-year mortality in patients who developed postoperative LCOS after cardiac surgery. NT-proBNP levels in the first postoperative week were not predictors of long-term outcomes. PMID: 26977768 [PubMed - as supplied by publisher] (Source: Minerva Cardioangiologica)
Wed, 16 Mar 2016 00:00:00 +0100ConclusionsLA basic echocardiographic variables did not reflect the differences between ICM and DCM patients, but the systolic and late diastolic strain, as well as the strain rate, were lower in DCM patients. Two‐dimensional speckle‐tracking imaging is a promising method to differentiate these patients. © 2016 Wiley Periodicals, Inc. J Clin Ultrasound, 2016 (Source: Journal of Clinical Ultrasound)
Mon, 14 Mar 2016 00:00:00 +0100A 50-year-old man with dilated cardiomyopathy had persistent dyspnea despite receiving guideline-directed medical treatment, including cardiac resynchronization therapy. He underwent heart transplantation, which was successful without complications. (Source: The Journal of Heart and Lung Transplantation)
Mon, 14 Mar 2016 00:00:00 +0100CONCLUSIONS: Together, our findings suggest that cardiac myocyte GSK-3 is required to maintain normal cardiac homeostasis and its loss is incompatible with life due to cell cycle dysregulation that ultimately results in a severe fatal dilated cardiomyopathy. PMID: 26976650 [PubMed - as supplied by publisher] (Source: Circulation Research)
Fri, 11 Mar 2016 00:00:00 +0100Conclusions LVNC should be looked for at any dilated cardiomyopathy particularly in young patients. It requires a careful echocardiographic examination and sometimes CMR to confirm the diagnosis. It is characterized by severe systolic and diastolic dysfunction that would provide poor prognosis. (Source: Journal of the Saudi Heart Association)
Fri, 11 Mar 2016 00:00:00 +0100This study demonstrates that DCM mutant delta sarcoglycans can be stably expressed in adult rat cardiac myocytes and traffic similarly to wild-type delta sarcoglycan to the plasma membrane, without perturbing assembly of the dystrophin-glycoprotein complex. However, expression of DCM mutant delta sarcoglycan in adult rat cardiac myocytes is sufficient to alter cardiac myocyte plasma membrane stability in the presence of mechanical strain. Upon cyclical cell stretching cardiac myocytes expressing mutant delta sarcoglycan R97Q or R71T have increased cell-impermeant dye uptake and undergo contractures at greater frequencies than myocytes expressing normal delta sarcoglycan. Additionally, the R71T mutation creates an ectopic N-linked glycosylation site that results in aberrant glycosylation of...
Tue, 08 Mar 2016 00:00:00 +0100Authors: Lee E, Park KT, Kang J, Park H, Park JJ, Oh IY, Yoon YE PMID: 26951916 [PubMed - as supplied by publisher] (Source: The Korean Journal of Internal Medicine)
Mon, 07 Mar 2016 00:00:00 +0100Conclusion In patients with severe secondary MR, speckle-tracking GLS shows more deteriorated LV systolic function than LVEF. (Source: European Heart Journal)
Mon, 07 Mar 2016 00:00:00 +0100We describe a targeted metabolomics assay that quantifies a broad range of over 130 metabolites relevant to cardiac metabolism including the pathways of the citric acid cycle, fatty acid oxidation, glycolysis, the pentose phosphate pathway, amino acid metabolism, the urea cycle, nucleotides and reactive oxygen species using tandem mass spectrometry to produce quantitative, sensitive and robust data. This assay is illustrated by profiling cardiac metabolism in a lamin A/C (Lmna) mouse model of dilated cardiomyopathy (DCM). The model of DCM was characterised by increases in concentrations of proline and methyl-histidine suggestive of increased myofibrillar and collagen degradation, as well as decreases in a number of citric acid cycle intermediates and carnitine derivatives indicating reduce...
Thu, 03 Mar 2016 00:00:00 +0100Publication date: Available online 2 March 2016 Source:MethodsX Author(s): Petr G. Vikhorev, Michael A. Ferenczi, Steven B. Marston Many causes of heart muscle diseases and skeletal muscle diseases are inherited and caused by mutations in genes of sarcomere proteins which play either a structural or contractile role in the muscle cell. Tissue samples from human hearts with mutations can be obtained but often samples are only a few milligrams and it is necessary to freeze them for storage and transportation. Myofibrils are the fundamental contractile components of the muscle cell and retain all structural elements and contractile proteins performing in contractile event; moreover viable myofibrils can be obtained from frozen tissue. We are describing a versatile technique for measuring...
Thu, 03 Mar 2016 00:00:00 +0100This study describes a patient presenting with the cardinal clinical features, in whom a homozygous nonsense mutation (c.248_249insT; p.Arg84Profs*209) was identified in CHKB and who was treated by heart transplantation. Microscopic evaluation of skeletal and heart muscles typically showed enlarged mitochondria. Spectrophotometric evaluation in both tissues revealed a mild decrease of all OXPHOS complexes. Using BN-PAGE analysis followed by activity staining subcomplexes of complex V were detected in both tissues, indicating incomplete complex V assembly. Mitochondrial DNA content was not depleted in analysed tissues. This is the first report describing the microscopic and biochemical abnormalities in the heart from an affected patient. A likely hypothesis is that the biochemical findings ...
Thu, 03 Mar 2016 00:00:00 +0100“It's tough to make predictions, especially about the future” (Source: Journal of Cardiac Failure)
Thu, 03 Mar 2016 00:00:00 +0100“It's tough to make predictions, especially about the future” (Source: Journal of Cardiac Failure)
Wed, 02 Mar 2016 00:00:00 +0100Life-threatening arrhythmia events (LTEs) occur in approximately 5% of children with dilated cardiomyopathy (DCM). While prolonged QRS has been shown to be associated with LTEs, electrocardiographic (ECG) repolarization findings have not been examined. (Source: Heart Rhythm)
Tue, 01 Mar 2016 06:00:00 +0100We describe a case in which these conditions were successfully treated with no thrombotic complications and minimal need of vasopressors for hemodynamic stability. (Source: A&A Case Reports)
Tue, 01 Mar 2016 00:00:00 +0100Background and aim: Dilated Cardiomyopathy (DCM) is a common disease with high morbidity and mortality. While atherosclerotic coronary artery disease (CAD) is a common cause of a group of DCM; known as ischemic cardiomyopathy (CMP). There is another group of DCM which develops secondary to systemic diseases, drug exposure or even idiopathic; this group is known as non-ischemic CMP. Management and prognosis of DCM is variable between ischemic and ischemic CMP. Exclusion of significant atherosclerotic CAD therefore, is an important step in the management of DCM. (Source: Journal of Cardiovascular Computed Tomography)
Tue, 01 Mar 2016 00:00:00 +0100ConclusionsPhospholamban p.Arg14del cardiomyopathy is a biventricular cardiomyopathy characterized by large perinuclear PLN protein aggregates with a typical ultrastructural appearance of aggresomes. Immunohistochemistry for PLN appears to be a sensitive and specific marker for this disease.This article is protected by copyright. All rights reserved. (Source: Histopathology)
Fri, 26 Feb 2016 00:00:00 +0100Abstract Endomyocardial biopsy (EMB) and cardiac magnetic resonance (CMR) are useful modalities to study the characteristics of myocardial tissue. However, the prognostic impact of both diagnostic tools to predict subsequent left ventricular reverse remodeling (LVRR) has not been well elucidated. A total of 187 consecutive patients with idiopathic dilated cardiomyopathy (IDCM) who were treated by optimal pharmacotherapy (OPT) and underwent EMB of the LV wall were investigated. The myocardial specimens were semiquantitatively evaluated measuring cardiomyocyte degeneration (CD), interstitial fibrosis (IF), and hypertrophy. In addition, late gadolinium enhancement (LGE)-CMR was performed in 78 (48 %) patients. Seventy-eight (48 %) patients developed LVRR, defined as a ≥10 % incr...
Fri, 26 Feb 2016 00:00:00 +0100Authors: Rosa I, Marini C, Stella S, Ancona F, Spartera M, Margonato A, Agricola E Abstract Chronic functional mitral regurgitation (FMR) is a frequent finding of ischemic heart disease and dilated cardiomyopathy (DCM), associated with unfavourable prognosis. Several pathophysiologic mechanisms are involved in FMR, such as annular dilatation and dysfunction, left ventricle (LV) remodeling, dysfunction and dyssynchrony, papillary muscles displacement and dyssynchrony. The best therapeutic choice for FMR is still debated. When optimal medical treatment has already been set, a further option for cardiac resynchronization therapy (CRT) and/or surgical correction should be considered. CRT is able to contrast most of the pathophysiologic determinants of FMR by minimizing LV dyssynchrony ...
Thu, 25 Feb 2016 20:41:02 +0100CONCLUSION: The 3D tenting pattern, assessed with RT3DE, would be an important clinical parameter in predicting long-term prognosis in patients with DCM. PMID: 26897836 [PubMed - in process] (Source: Journal of Heart Valve Disease)
Thu, 25 Feb 2016 10:19:41 +0100(Can J Anesth. 2015;62:278–288) Cardiomyopathy is increasing as a cause of maternal death, up from 8% in 1991 to 1997 to over 12% in 1998 to 2005. A 2002 study in North Carolina showed cardiomyopathy to be the leading cause of maternal death, accounting for >20% of deaths. (Source: Obstetric Anesthesia Digest)
Thu, 25 Feb 2016 00:00:00 +0100Conclusion The rate of appropriate ICD therapies in secondary prevention is high. No useful predictors for them, especially not for life-threatening arrhythmias could be identified. (Source: Europace)
Thu, 25 Feb 2016 00:00:00 +0100Conclusion Epicardial only ablation seems feasible and effective and useful in a limited subset of patients with incessant VT. However, endpoints are more difficult to evaluate and long-term follow-up is needed. (Source: Europace)
Wed, 24 Feb 2016 00:00:00 +0100This article is part of a Special Issue entitled:Cardiomyocyte Biology: Integration of Develomental and Environmental Cues in the Heart edited by Marcus Schaub and Hughes Abriel. (Source: Biochimica et Biophysica Acta (BBA) Molecular Cell Research)
Wed, 24 Feb 2016 00:00:00 +0100This article discusses a case in which the diagnosis of typical atrioventricular nodal reciprocating tachycardia could be firmly established from the implantable cardioverter-defibrillator interrogation alone. (Source: Cardiac Electrophysiology Clinics)
Tue, 23 Feb 2016 00:00:00 +0100Authors: Sakamuri SS, Takawale A, Basu R, Fedak PW, Freed D, Sergi C, Oudit GY, Kassiri Z Abstract Adverse remodeling of the extracellular matrix (ECM) is a significant characteristic of heart failure. Reverse remodeling of the fibrillar ECM secondary to mechanical unloading of the left ventricle (LV) by left ventricular assist device (LVAD) has been subject of intense investigation; however, little is known about the impacts on nonfibrillar ECM and matricellular proteins that also contribute to disease progression. Explanted failing hearts were procured from patients with nonischemic dilated cardiomyopathy (DCM) with or without LVAD support, and compared to nonfailing control hearts. LV free wall specimens were formalin-fixed, flash-frozen or OCT-mount frozen. Histologic and bioch...
Mon, 22 Feb 2016 00:00:00 +0100Adverse remodeling of the extracellular matrix (ECM) is a significant characteristic of heart failure. Reverse remodeling of the fibrillar ECM secondary to mechanical unloading of the left ventricle (LV) by LV assist device (LVAD) has been subject of intense investigation, however little is known about the impacts on non-fibrillar ECM and matricellular proteins that also contribute to disease progression. Explanted failing hearts were procured from patients with non-ischemic dilated cardiomyopathy (DCM) with or without LVAD support, and compared to non-failing control hearts (NFC). (Source: Translational Research)
Mon, 22 Feb 2016 00:00:00 +0100Adverse remodeling of the extracellular matrix (ECM) is a significant characteristic of heart failure. Reverse remodeling of the fibrillar ECM secondary to mechanical unloading of the left ventricle (LV) by left ventricular assist device (LVAD) has been subject of intense investigation; however, little is known about the impacts on nonfibrillar ECM and matricellular proteins that also contribute to disease progression. Explanted failing hearts were procured from patients with nonischemic dilated cardiomyopathy (DCM) with or without LVAD support, and compared to nonfailing control hearts. (Source: Translational Research)
Sat, 20 Feb 2016 00:00:00 +0100Abstract Excess accumulation of iron in the heart is known to aggravate cardiac function in some cases of genetic and acquired iron overload. We investigated the possible association between cardiac function and iron content in the heart and liver, estimated non-invasively by T2 star (T2*)-weighted magnetic resonance (MR) imaging among patients with cardiomyopathy. MR images were acquired on a 3.0 T MR imaging system using an 8-channel phased-array cardiac coil. Average T2* values of the heart were estimated at regions of interest that were located on short axis mid-ventricular slices positioned at the cardiac septum. In total, 82 patients were enrolled: 48 patients with dilated cardiomyopathy (DCM), 16 patients with hypertrophic cardiomyopathy (HCM), and 18 patients without appa...
Fri, 19 Feb 2016 00:00:00 +0100Authors: Mittal A, Sharma R, Prasad R, Bahl A, Khullar M Abstract Dilated cardiomyopathy (DCM) is an important cause of heart failure and sudden cardiac death worldwide. Transcription factor TBX20 has been shown to play a crucial role in cardiac development and maintenance of adult mouse heart. Recent studies suggest that TBX20 may have a role in pathophysiology of DCM. In the present study, we examined TBX20 expression in idiopathic DCM patients and in an animal model of cardiomyopathy, and studied its correlation with echocardiographic indices of LV function. Endomyocardial biopsies (EMBs) from intraventricular septal from the right ventricle region were obtained from idiopathic DCM patients (IDCM, n = 30) and from patients with ventricular septal defect (VSD, n = 14) with no...
Thu, 18 Feb 2016 02:43:03 +0100DISCUSSION: miRNA level differences may provide the chance of using these miRNAs as new biomarkers. PMID: 26631154 [PubMed - as supplied by publisher] (Source: Biomarkers)
Thu, 18 Feb 2016 00:00:00 +0100We report the case of a 71-year-old woman with metastatic NSCLC treated with cisplatin/pemetrexed and then erlotinib maintenance therapy. After 26 months of TKI therapy, she developed dilated cardiomyopathy. Despite symptomatic treatment, left ventricular ejection fraction decreased to 25%. Ischemic heart disease was excluded by coronary angiography and cardiac magnetic resonance imaging, and no other cause was found. Erlotinib was stopped, and cardiac resynchronization therapy by pacemaker was initiated. This case report highlights the possible cardiotoxic effects of long-term erlotinib and suggests the need for close clinical and echocardiographic follow-up of patients receiving long-term TKI therapy. PMID: 26886479 [PubMed - as supplied by publisher] (Source: Oncology)
Thu, 18 Feb 2016 00:00:00 +0100In conclusion, autophagic vacuoles in cardiomyocytes are associated with a better HF prognosis in patients with DCM, suggesting autophagy may play a role in the prevention of myocardial degeneration. PMID: 26890610 [PubMed - as supplied by publisher] (Source: Autophagy)
Wed, 17 Feb 2016 17:12:45 +0100We report the case of a 71-year-old woman with metastatic NSCLC treated with cisplatin/pemetrexed and then erlotinib maintenance therapy. After 26 months of TKI therapy, she developed dilated cardiomyopathy. Despite symptomatic treatment, left ventricular ejection fraction decreased to 25%. Ischemic heart disease was excluded by coronary angiography and cardiac magnetic resonance imaging, and no other cause was found. Erlotinib was stopped, and cardiac resynchronization therapy by pacemaker was initiated. This case report highlights the possible cardiotoxic effects of long-term erlotinib and suggests the need for close clinical and echocardiographic follow-up of patients receiving long-term TKI therapy.Oncology (Source: Oncology)
Wed, 17 Feb 2016 15:49:03 +0100Authors: Merlo M, Cannatá A, Vitagliano A, Zambon E, Lardieri G, Sinagra G Abstract Dilated cardiomyopathy (DCM) is a primary heart muscle disease characterized by a progressive dilation and dysfunction of either the left or both ventricles. The management of DCM is currently challenging for clinicians. The persistent lack of knowledge about the etiology and pathophysiology of this disease continues to determine important fields of uncertainty in managing this condition. Molecular cardiology and genetics currently represent the most crucial horizon of increasing knowledge. Understanding the mechanisms underlying the disease allows clinicians to treat this disease more effectively and to further improve outcomes of DCM patients through advancements in etiologic characterization, pr...
Wed, 17 Feb 2016 15:42:02 +0100CONCLUSION: Neutrophil/Lymphocyte ratio is associated with the severity of chronic heart failure in patients with idiopathic DCM. PMID: 24912526 [PubMed - indexed for MEDLINE] (Source: Scandinavian Cardiovascular Journal)
Wed, 17 Feb 2016 14:24:02 +0100Authors: Dou Q, Peng Y, Zhou B, Lin J, Li Y, Yang H, Xie Q, Li C, Zhang L, Rao L Abstract The interferon-induced helicase C domain-containing protein 1 (IFIH1) is a cytosolic RNA sensor belonging to the pattern-recognition receptor (PPR) family. Activation of PPRs on innate immune cells is widely believed to control the development of virus-induced autoimmunity in myocarditis and subsequent dilated cardiomyopathy (DCM). We conducted a pilot study to test whether single nucleotide polymorphisms (SNPs) in IFIH1 were associated with the risk and prognosis of DCM. The TaqMan SNP Genotyping Assay was used to genotype rs1990760 and rs3747517 in 351 DCM patients and 359 controls. The frequency of T allele and CT/TT genotypes at rs1990760 were significantly increased in DCM patients compar...
Tue, 16 Feb 2016 00:00:00 +0100(Source: Circulation: Cardiovascular Genetics)
Mon, 15 Feb 2016 00:00:00 +0100A 31-year old heart transplant recipient presented for genetic counseling. He was born to nonconsanguineous healthy parents of European descent. A dilated cardiomyopathy was diagnosed at the age of 12years and heart transplantation was performed few months later. Pathologic examination of the explanted heart showed myocardial hypertrophy and interstitial fibrosis (Fig. 1). At the gross examination, the right ventricle, interventricular septum, and left ventricle of the explanted heart were 5mm, 11mm, and 13mm of thickness, respectively. (Source: International Journal of Cardiology)
Sat, 13 Feb 2016 00:00:00 +0100Neutral lipid storage disease with myopathy (NLSD-M; PNPLA2) is a rare, mostly autosomal recessive disorder, caused by mutations in the gene encoding patatin-like phospholipase domain-containing protein 2 (PNPLA2), also known as adipose triglyceride lipase (ATGL). ATGL catalyses the breakdown of triacylglycerol (TAG) to diacylglycerol. Dysfunction of ATGL leads to accumulation of TAG-containing cytoplasmic vacuoles in most tissues . Only about 40 patients have been described with NLSD-M worldwide. (Source: International Journal of Cardiology)
Fri, 12 Feb 2016 00:00:00 +0100Conclusion Data of metabolic scintigraphy may be useful for the integrated prediction of CRT efficacy. (Source: Annals of Nuclear Medicine)
Wed, 10 Feb 2016 00:00:00 +0100In this study, we identified Rnd3, a small Rho GTPase, as a proangiogenic factor participating in the regulation of the HIF1α–VEGF signaling cascade. Rnd3 physically interacted with and stabilized HIF1α, and consequently promoted VEGFA expression and endothelial cell tube formation. To demonstrate this proangiogenic role of Rnd3 in vivo, we generated Rnd3 knockout mice. Rnd3 haploinsufficient (Rnd3+/–) mice were viable, yet developed dilated cardiomyopathy with heart failure after transverse aortic constriction stress. The poststress Rnd3+/– hearts showed significantly impaired angiogenesis and decreased HIF1α and VEGFA expression. The angiogenesis defect and heart failure phenotype were partially rescued by cobalt chloride treatment, a HIF1α stabi...
Tue, 09 Feb 2016 00:00:00 +0100Authors: Román IS, Navarro M, Martínez F, Albert L, Polo L, Guardiola J, García-Molina E, Muñoz-Esparza C, López-Ayala JM, Molina MS, Gimeno JR Abstract Emery-Dreifuss muscular dystrophy (EDMD) is a heterogeneous genetic disorder characterized by peripheral muscular weakness often associated with dilated cardiomyopathy. We characterize clinically a large family with a mutation in FHL1 gene (p.Cys255Ser). Penetrance was 44%, 100% for males and 18% for females. The heart was the main organ involved. Affected adult males had mild hypertrophy, systolic dysfunction and restriction with non-dilated ventricles. Carriers had significant QTc prolongation. The proband presented with resuscitated cardiac arrest. There were 2 transplants. Pathological study of explanted heart demonstrated...
Mon, 08 Feb 2016 00:00:00 +0100We report a rare clinical presentation of incessant idiopathic fascicular ventricular tachycardia (FVT), presenting as multi-organ dysfunction (MOD) syndrome with cardiogenic shock. Our patient was a 19-year-old male who presented with slowly progressive dyspnea from New York Heart Association (NYHA) II to NYHA IV at the time of presentation, palpitations, and dilated cardiomyopathy due to drug-refractory FVT. The patient was in cardiogenic shock with raised central venous pressures and required inotropic support for maintaining systolic blood pressure above 90mmHg. The MOD was seen in the form of deranged liver and kidney parameters. Echocardiography showed a dilated left ventricle (LV, 58mm at end-diastole, 52mm at end-systole) and decreased ejection fraction (20%). Electrocardiography s...
Mon, 08 Feb 2016 00:00:00 +0100We report a 6 year old boy who presented with severe hypoglycemia, lactic acidosis and severe dilated cardiomyopathy soon after birth. The MLCL:L4-CL ratio confirmed BTHS (3.90 on patient's fibroblast, normal 0-0.3). Subsequent sequencing of the TAZ gene revealed only the new synonymous variant NM_000116.3(TAZ):c.348C>T p.(Gly116Gly), which did not appear to affect the protein sequence. In silico prediction analysis suggested the new c.348C>T nucleotide change could alter the TAZ mRNA splicing processing. We analyzed TAZ mRNAs in the patient's fibroblasts and found an abnormal skipping of 24 bases (NM_000116.3:c.346_371), with the consequent ablation of 8 amino acid residues in the tafazzin protein (NP_000107.1:p.Lys117_Gly124del). Molecular analysis of at risk female family members ...
Thu, 04 Feb 2016 21:04:03 +0100Conclusions: High prevalence of a viral genome in the myocardium of patients with DCM did not have an influence on their long-term clinical outcome.Intervirology 2015;58:350-356 (Source: Intervirology)
Wed, 03 Feb 2016 00:00:00 +0100In conclusion, evaluating the presence and the extent of LGE is useful for predicting the clinical differences of LVRR time course and subsequent long-term outcomes. (Source: Heart and Vessels)
Wed, 03 Feb 2016 00:00:00 +0100Lamin proteins A and C are key structural and functional components of the nuclear lamina . Mutations in the lamin A/C gene (LMNA) are associated with a variety of clinical phenotypes, including premature aging, skeletal muscle disease, metabolic disorders and cardiac abnormalities . However, the majority of pathogenic LMNA mutations affect cardiac function, causing atrial fibrillation (AF), conduction disturbances, ventricular arrhythmias, and dilated cardiomyopathy (DCM) [3–7]. (Source: International Journal of Cardiology)
Tue, 02 Feb 2016 06:05:15 +0100No abstract available (Source: Journal of Cardiovascular Medicine)
Tue, 02 Feb 2016 06:04:14 +0100ConclusionsBiallelic truncating mutations in the newly identified gene ALPK3 give rise to severe, early-onset cardiomyopathy in humans. Our findings highlight the importance of transcription factor pathways in the molecular mechanisms underlying human cardiomyopathies. (Source: Journal of the American College of Cardiology)
Tue, 02 Feb 2016 00:00:00 +0100Enterovirus1 (EV) infections are common in all age groups. They are usually asymptomatic or cause only mild respiratory symptoms, but can also lead to more severe illness including hand, foot and mouth disease, myocarditis, meningitis, encephalitis, pancreatitis, systemic infection in newborns and paralysis. EV infections may also play a role in the pathogenesis of chronic diseases such as dilated cardiomyopathy , chronic fatigue syndrome  and type 1 diabetes2 (T1D) [3–5]. (Source: Journal of Clinical Virology)
Tue, 02 Feb 2016 00:00:00 +0100Clinical management of new onset dilated cardiomyopathy (NODC) has been an object of intensive research in recent years. Apart from established conventional heart failure, additional therapies remain still under investigation . Such ‘second line’ treatment options include immunomodulatory therapy (namely pathogen-driven therapies – PDT-, intravenous immunoglobulins and immunoadsorption), as well as immunosuppressive therapies namely steroids, azathioprine and cyclosporine A in various combinations . (Source: International Journal of Cardiology)
Mon, 01 Feb 2016 00:00:00 +0100Abstract We hypothesized that the direction of global left ventricular (LV) hemodynamic forces during diastolic filling are concordant with the main flow axes in normal LVs, but that this pattern would be altered in dilated and dysfunctional LVs. Therefore, we aimed to assess the LV hemodynamic filling forces in a group of healthy subjects and compare them to the results from a group of patients with dilated cardiomyopathy (DCM). Ten healthy subjects and 10 DCM patients were enrolled. Morphological short‐ (SAx) and long‐axis (LAx) images and 4D flow MRI data were acquired at 1.5T. The LV pressure gradients were computed from the 4D flow data using the Navier–Stokes equations. By integrating the pressure gradients over the LV volume at each time frame, the magnitude and direction of t...
Mon, 01 Feb 2016 00:00:00 +0100ConclusionsThe IRF pattern is associated with LV function. A large intraventricular vortex persisting during the IVR period should be indicative of increasingly depressed LV function. (Source: Echocardiography)
Mon, 01 Feb 2016 00:00:00 +0100(Source: The Journal of Dermatology)
Mon, 01 Feb 2016 00:00:00 +0100Conclusions: SCD and device therapies were relatively rare. Routine Holter screening rarely demonstrated significant findings or changed clinical care. Clinical history of ventricular arrhythmia was associated with poor clinical outcome.This article is protected by copyright. All rights reserved (Source: Pacing and Clinical Electrophysiology : PACE)
Sat, 30 Jan 2016 00:00:00 +0100We report a patient with non-ischemic dilated cardiomyopathy and low left ventricular systolic function (28%) presenting with an electrical storm originated in epicardial scar and ablated by radiofrequency. This case report suggests that a strategy of epicardial catheter ablation is reasonable for the patient presenting with electrical storm related to structural disease with a low left ventricular ejection fraction. (Source: Indian Heart Journal)
Fri, 29 Jan 2016 13:05:26 +0100Conclusions: The potential of SMCST to ameliorate both systolic and diastolic performance was proven. The SMCST may be an alternative therapy of conventional medical treatment in the dilated cardiomyopathy heart. (Source: Transplantation)
Wed, 27 Jan 2016 00:00:00 +0100Authors: Dan Lu, Dan Bao, Wei Dong, Ning Liu, Xu Zhang, Shan Gao, Wenping Ge, Xiang Gao & Lianfeng Zhang (Source: Laboratory Investigation)
Tue, 26 Jan 2016 00:00:00 +0100Narendra KumarIndian Journal of Anaesthesia 2016 60(1):73-74 (Source: Indian Journal of Anaesthesia)
Tue, 26 Jan 2016 00:00:00 +0100Authors: Gatzoulis KA, Tsiachris D, Arsenos P, Tousoulis D Abstract Sudden cardiac death threats ischaemic and dilated cardiomyopathy patients. Anti- arrhythmic protection may be provided to these patients with implanted cardiac defibrillators (ICD), after an efficient risk stratification approach. The proposed risk stratifier of an impaired left ventricular ejection fraction has limited sensitivity meaning that a significant number of victims will remain undetectable by this risk stratification approach because they have a preserved left ventricular systolic function. Current risk stratification strategies focus on combinations of non invasive methods like T wave alternans, late potentials, heart rate turbulence, deceleration capacity and others, with invasive methods like the ele...
Sun, 24 Jan 2016 04:05:04 +0100The objective of this study was to investigate functional effects of IA therapy using a tryptophan column in severe DCM patients. Of 49 patients enrolled, 44 were randomized from 10 sites in Japan. IA therapy was conducted in 40 patients with DCM (refractory to standard therapy for heart failure, New York Heart Association [NYHA] class III/IV, left ventricular ejection fraction [LVEF] <30%). Mean echocardiographic LVEF was significantly improved (23.8 ± 1.3% to 25.9 ± 1.3%, P = 0.0015). However, mean radionuclide LVEF over 3 months of IA therapy was not significantly improved (20.8 ± 1.1% to 21.9 ± 1%, P = 0.0605). The cardiothoracic ratio was also significantly decreased (P = 0.0010). NYHA functional class (P < 0.0001), subjective symptoms assesse...
Sat, 23 Jan 2016 09:24:03 +0100ConclusionHeart rate acceleration capacity and deceleration capacity are independent risk factors for DCM, and acceleration capacity is a predictive factor for heart failure exacerbation in DCM patients.This article is protected by copyright. All rights reserved. (Source: European Journal of Clinical Investigation)
Fri, 22 Jan 2016 00:00:00 +0100Conclusions In LCAP the systo-diastolic WHVC was significantly higher than controls, DCM, HCM and RVO patients and resulted an optimal quantitative criteria for the diagnosis of LCAP. (Source: European Journal of Radiology)
Fri, 22 Jan 2016 00:00:00 +0100CONCLUSION: Omega 3-PUFA supplementation was associated with improved left diastolic function and decreased BNP levels in patients with chronic heart failure. These findings suggest a beneficial role of omega 3-PUFAs on the hemodynamic course of patients with systolic heart failure. PMID: 26807502 [PubMed - as supplied by publisher] (Source: Vascular Pharmacology)
Fri, 22 Jan 2016 00:00:00 +0100Nature Reviews Cardiology 13, 121 (2016). doi:10.1038/nrcardio.2016.3 Author: Gregory B. Lim Women with peripartum cardiomyopathy (PPCM) and individuals who develop dilated cardiomyopathy (DCM) might share a genetic predisposition. This finding comes from a gene-sequencing study published in N. Engl. J. Med. in which PPCM was found to be associated with truncating variants in various genes (Source: Nature Reviews Cardiology)
Wed, 20 Jan 2016 13:34:12 +0100Authors: Szafron B, Smoczyński RH, Drobiński D, Pawlak A, Wojciechowski D, Liszka IM, Witkowska A, Kaliciński Z, Gil R, Suwalski P Abstract Cardiac surgeons have to face the problem of impaired left ventricle function in patients undergoing routine valve or coronary procedures. The intra-aortic balloon pump is not always effective in preventing cardiac failure. The idea of using a microaxial rotating pump as a short-term perioperative support seems to be a convenient solution. The case of a patient with dilated cardiomyopathy undergoing combined mitral and coronary surgery with elective use of the Impella LD pump is presented. Various options of applying the Impella device are discussed, especially as a bridge to transplant or bridge to recovery. PMID: 26336480 [PubMed] (So...
Wed, 20 Jan 2016 00:00:00 +0100Conclusions PVB19 can cause a devastating myocarditis in children. Children with fulminant myocarditis, ST segment changes or a short prodrome have the worst outcome. Transplantation may be considered, but is rarely required in the acute period if mechanical circulatory support is utilised. If the initial presentation is survived, recovery of the myocardium can occur even in those who had fulminant myocarditis. (Source: Archives of Disease in Childhood)
Wed, 20 Jan 2016 00:00:00 +0100We report the case of 70-year-old man with congestive dilated cardiomyopathy and bioprosthetic mitral valve who underwent surgical implantation of the Jarvik 2000® LVAD, using the miniaturized extracorporeal circulation (MECC) system.The LVAD was implanted through a left thoracotomy and the MECC system was used to avoid intraoperative spontaneous hemodynamic instability and/or malignant ventricular arrhythmia. The circulatory support with the MECC system was optimal and no complication in terms of hemodynamic instability and perioperative bleeding was recorded. The MECC system obliterated the adverse effects associated with conventional extracorporeal circulation, which are often fatal in critically-ill patients. PMID: 26791274 [PubMed - as supplied by publisher] (Source: Perfusion)
Wed, 20 Jan 2016 00:00:00 +0100James A. Spudich, Tural Aksel, Sadie R. Bartholomew, Suman Nag, Masataka Kawana, Elizabeth Choe Yu, Saswata S. Sarkar, Jongmin Sung, Ruth F. Sommese, Shirley Sutton, Carol Cho, Arjun S. Adhikari, Rebecca Taylor, Chao Liu, Darshan Trivedi, and Kathleen M. Ruppel Hypertrophic cardiomyopathy is the most frequently occurring inherited cardiovascular disease, with a prevalence of more than one in 500 individuals worldwide. Genetically acquired dilated cardiomyopathy is a related disease that is less prevalent. Both are caused by mutations in the genes encoding the fundamental force-generating protein machinery of the cardiac muscle sarcomere, including human β-cardiac myosin, the motor protein that powers ventricular contraction. Despite numerous studies, most performed with non-human or n...
Tue, 19 Jan 2016 00:00:00 +0100Authors: Wehbe M, Huguenin A, Leveque N, Semler BL, Hamze M, Andreoletti L, Bouin A Abstract Coxsackieviruses B (CV-B) (Picornaviridae) are a common infectious cause of acute myocarditis in children and young adults, a disease, which is a precursor to 10-20% of chronic myocarditis and dilated cardiomyopathy (DCM) cases. The mechanisms involved in the disease progression from acute to chronic myocarditis phase and toward the DCM clinical stage are not fully understood but are influenced by both viral and host factors. Subgenomic replicons of CV-B can be used to assess viral replication mechanisms in human cardiac cells and evaluate the effects of potential antiviral drugs on viral replication activities. Our objectives were to generate a reporter replicon from a cardiotropic prototy...
Mon, 18 Jan 2016 01:56:03 +0100CONCLUSIONS: We may be able to improve the clinical outcome of HF by examining the differences in the clinical characteristics and medications at admission and discharge in hospitalized patients with HF. PMID: 26767077 [PubMed] (Source: Clin Med Res)
Mon, 18 Jan 2016 00:00:00 +0100Truncating mutations in the TTN gene are the most common genetic cause of dilated cardiomyopathy in adults but their role in young patients is unknown. We studied 82 young dilated cardiomyopathy subjects and found that the prevalence of truncating TTN mutations in adolescents was similar to adults, but surprisingly few truncating TTN mutations were identified in affected children, including one confirmed de novo variant. In several cases, truncating TTN mutations in children with dilated cardiomyopathy had evidence of additional clinical or genetic risk factors. (Source: Progress in Pediatric Cardiology)
Mon, 18 Jan 2016 00:00:00 +0100Authors: Akinrinade O, Alastalo TP, Koskenvuo JW Abstract Dilated cardiomyopathy (DCM), a genetically heterogeneous cardiac disease characterized by left ventricular dilatation and systolic dysfunction, is caused majorly by truncations of titin (TTN), especially in A-band region. Clinical interpretation of TTN truncating variants (TTNtv) has been challenged by the existing inaccurate variant assessment strategies and uncertainty in the true frequency of TTNtv across the general population. We aggregated TTNtv identified in 1788 DCM patients and compared the variants with those reported in the over 60,000 Exome Aggregation Consortium (ExAC) reference population. We implemented our current variant assessment strategy that prioritizes TTNtv affecting all transcripts of the gene, and o...
Sat, 16 Jan 2016 00:00:00 +0100This report will give the reader a summary of the current status in the field of feline diastology with focus on the noninvasive diagnostic methods and interpretation of echocardiographic surrogate measures of LV diastolic function. Lastly, a grading system using a composite of left atrial size and various DE variables potentially useful in the functional classification of LV diastole in cats is introduced. (Source: Journal of Veterinary Cardiology)
Sat, 16 Jan 2016 00:00:00 +0100Cardiomyopathy is the leading diagnosis for pediatric heart transplantation in children over 1year of age. The majority of these patients usually have dilated cardiomyopathy. Children with hypertrophic and restrictive types of cardiomyopathy comprise less than 20% of the total children transplanted. Hypertrophic cardiomyopathy is the smallest subgroup to receive a cardiac transplant (6%). The mean age at listing in the Pediatric Heart Transplant Study (PHTS) was 7.6years, however, 39% were infants listed at a mean of 3.2+2.8months of age. (Source: Progress in Pediatric Cardiology)
Sat, 16 Jan 2016 00:00:00 +0100Non-ischemic, dilated, cardiomyopathy (NIDCM) is defined as left ventricular dysfunction in the absence of causative coronary artery disease. It typically presents with impaired effort tolerance which is important prognostic factor.1 (Source: The Journal of Heart and Lung Transplantation)
Sat, 16 Jan 2016 00:00:00 +0100Non-ischemic dilated cardiomyopathy (NIDCM) is defined as left ventricular dysfunction in the absence of causative coronary artery disease. It typically presents with impaired effort tolerance, which is a major prognostic factor.1 (Source: The Journal of Heart and Lung Transplantation)
Fri, 15 Jan 2016 00:00:00 +0100CONCLUSION: In idiopathic dilated cardiomyopathy, RV dysfunction with TAPSE≤15mm offers additional prognostic information, independent of the extent of LV dysfunction. PMID: 26782624 [PubMed - as supplied by publisher] (Source: Archives of Cardiovascular Diseases)
Thu, 14 Jan 2016 00:00:00 +0100Sarcoidosis is a systemic granulomatous disease of unknown aetiology. The frequency of cardiac involvement (cardiac sarcoidosis (CS)) varies in the different geographical regions, but it has been reported that it is an absolutely important prognostic factor in this disease. Complete atrioventricular block is the most common, and ventricular tachycardia/ventricular fibrillation the second most common arrhythmia in this disease, both of which are associated with cardiac sudden death. Diagnosing CS is sometimes difficult because of the non-specific ECG and echocardiographic findings, and CS is sometimes misdiagnosed as dilated cardiomyopathy, arrhythmogenic right ventricular cardiomyopathy or an idiopathic ventricular aneurysm, and therefore, endomyocardial biopsy is important, but has a low ...
Thu, 14 Jan 2016 00:00:00 +0100Publication date: Available online 14 January 2016 Source:Indian Heart Journal Author(s): Mangalachulli Pottammal Ranjith, Kayakkal Shajudeen, Sankaran Prasanth A 52-year-old male with history of dilated cardiomyopathy was admitted with progressive dizziness and worsening dyspnea. His blood pressure was 100/60 with a regular pulse of 70 per minute. Jugular vein wave showed a prominent pulse like regular cannon waves. Cannon wave occurs in conditions with atrioventricular dissociation and right atrial contraction against a closed tricuspid valve. Large A waves are associated with reduced right ventricular compliance or elevated right ventricular end-diastolic pressure. (Source: Indian Heart Journal)
Thu, 14 Jan 2016 00:00:00 +0100Prior to the highly active antiretroviral therapy (HAART)I era, and currently in countries with limited access to care, up to 38% of adult asymptomatic human immunodeficiency virus (HIV)-infected persons had clinical and echocardiographic evidence of dilated cardiomyopathy associated with lower CD4 counts.1,2 In HIV-infected children, poor nutritional status was also associated with echocardiographic abnormalities that demonstrate decreased left ventricular (LV) systolic dysfunction.3 The pre-HAART era Pediatric Pulmonary and Cardiac Complications of Vertically Transmitted HIV Infection Study (P2C2) looked at longitudinal changes in LV structure and function and mortality in 193 children perinatally infected with HIV enrolled between May 1990 and April 1993. (Source: The American Journal o...
Thu, 14 Jan 2016 00:00:00 +0100Nature Reviews Cardiology 13, 72 (2016). doi:10.1038/nrcardio.2015.202 Author: Donna K. Arnett Cardiovascular genomics has evolved substantially in the past 2 decades. Numerous papers published in 2015 demonstrate that new genomic approaches, often used synergistically, can yield noteworthy findings. A range of laboratory, analytical, and bioinformatic techniques to uncover genetic contributors to coronary artery disease, myocardial infarction, and dilated cardiomyopathy are described. (Source: Nature Reviews Cardiology)
Thu, 14 Jan 2016 00:00:00 +0100Familial dilated cardiomyopathy (DCM) is genetically heterogeneous. Mutations in more than 40 genes have been identified in familial cases, mostly inherited in an autosomal dominant pattern. DCM due to recessi... (Source: BMC Medical Genetics)
Thu, 14 Jan 2016 00:00:00 +0100Before the highly active antiretroviral therapy (HAART; it is defined as the concomitant use of ≥3 antiretroviral drugs from at least 2 classes) era and currently in countries with limited access to care, up to 38% of adult asymptomatic human immunodeficiency virus (HIV)–infected people had clinical and echocardiographic evidence of dilated cardiomyopathy associated with lower CD4 counts.1,2 In HIV-infected children, poor nutritional status was also associated with echocardiographic abnormalities that demonstrate decreased left ventricular (LV) systolic dysfunction. (Source: The American Journal of Cardiology)
Wed, 13 Jan 2016 00:00:00 +0100Dilated cardiomyopathy is a major cause of heart failure (HF) that affects millions. Corin cleaves and biologically activates pro-atrial natriuretic peptide (pro-ANP) and pro–B-type natriuretic peptide (pro-BNP). High corin levels reduce the development of systolic dysfunction and HF in experimental dilated cardiomyopathy. Yet, patients with significant HF unexpectedly show low corin levels with high plasma ANP/BNP levels. Therefore, we examined the relationship between cardiac corin expression, ANP/BNP levels, and the stages of HF. We used a well-established, dilated cardiomyopathy model to evaluate gene and protein expression as mice longitudinally developed Stages A–D HF. Cardiac systolic function (ejection fraction) continuously declined over time (P<0.001). Cardiac cori...
Tue, 12 Jan 2016 00:00:00 +0100Conclusions Current prediction scores are useful in predicting mortality risk of patients considered for ICD generator replacement and can potentially help identify patients who may not benefit from continuous ICD treatment due to high mortality rates regardless of the ICD. (Source: Journal of Interventional Cardiac Electrophysiology)
Tue, 12 Jan 2016 00:00:00 +0100Publication date: Available online 12 January 2016 Source:Revista Española de Cardiología (English Edition) Author(s): Fernando Dominguez, Uwe Kühl, Burkert Pieske, Pablo Garcia-Pavia, Carsten Tschöpe Myocarditis is defined as an inflammatory disease of the heart muscle and is an important cause of acute heart failure, sudden death, and dilated cardiomyopathy. Viruses account for most cases of myocarditis or inflammatory cardiomyopathy, which could induce an immune response causing inflammation even when the pathogen has been cleared. Other etiologic agents responsible for myocarditis include drugs, toxic substances, or autoimmune conditions. In the last few years, advances in noninvasive techniques such as cardiac magnetic resonance have been very useful in supporting diagnosis...
Mon, 11 Jan 2016 17:13:50 +0100A novel therapy tested scientists for treating a fatal heart disorder in dogs might ultimately help in diagnosing and treating heart disease in humans. The team also identified potential causes of inherited dilated cardiomyopathy (DCM) or "weak heart." (Source: ScienceDaily Headlines)
Mon, 11 Jan 2016 00:00:00 +0100(Source: Genome Biology)
Mon, 11 Jan 2016 00:00:00 +0100Stem cell transplantation is an emerging method of treatment for patients with cardiovascular disease. There are few studies completed or ongoing on stem cell therapy in patients with idiopathic dilated cardiomyopathy (IDCM). Information on stem cell homing and distribution in the myocardium after transplantation might provide important insight into effectiveness of transplantation procedure.Aimto assess early engraftment, retention and migration of intracoronarily transplanted stem cells in the myocardium of patients with advanced dilated cardiomyopathy of non-ischaemic origin using stem cell labeling with 99mTc-exametazime (HMPAO). (Source: Nuclear Medicine and Biology)
Mon, 11 Jan 2016 00:00:00 +0100A 43-year-old male patient was admitted to hospital with dyspnoea, edema and atypical chest pain. His weight was 160 kg and he smoked 40 cigarettes per day. (Source: International Journal of Cardiology)
Mon, 11 Jan 2016 00:00:00 +0100Conclusions In patients with ICDs and electrical storm refractory to optimal medical treatment, renal sympathetic denervation significantly reduced arrhythmia load and, consequently, antitachycardia pacing and shocks. Randomized clinical trials in the context of renal sympathetic denervation to control refractory cardiac arrhythmias are needed to further support these findings. (Source: Revista Brasileira de Cardiologia Invasiva)
Fri, 08 Jan 2016 00:00:00 +0100This study features the first hiPSC model of RBM20 familial DCM. By monitoring human cardiac disease according to stage-specific cardiogenesis, this study demonstrates RBM20 familial DCM is a developmental disorder initiated by molecular defects that pattern maladaptive cellular mechanisms of pathological cardiac remodeling. Indeed, hiPSC-CMs recapitulate RBM20 familial DCM phenotype in a dish and establish a tool to dissect disease-relevant defects in RBM20 splicing as a global regulator of heart function. (Source: Human Molecular Genetics)
Fri, 08 Jan 2016 00:00:00 +0100A recently published review of the association of pheochromocytoma (PHEO) or paraganglioma (PGO) and Takotsubo syndrome (TTS) provides evidence that the above 3 entities and in addition the catecholamine-mediated dilated cardiomyopathy (CC) may be etiologically linked . The authors refer to some patients with catecholamine secreting tumors (PHEO and PGO) who are being asymptomatic, or “may present with multiple and nonspecific symptoms”, and that the “chronic or acute catecholamine intoxication may lead to structural myocardial alterations”, and “result to different types of PHEO-, and PGO-induced cardiomyopathies”, “both chronic and acute, among which is TTS” . (Source: International Journal of Cardiology)