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idiopathic cardiomyopathy

Christoph Niemietz, Christoph Röcken, Matthias Schilling, Jörg Stypman, Constantin E Uhlig, Hartmut H-J Schmidt
Transthyretin-related Familial Amyloid Polyneuropathy (ATTR Amyloidosis, former FAP, here called TTR-FAP) is a rare, progressive autosomal dominant inherited amyloid disease ending fatal within 5 - 15 years after final diagnosis. TTR-FAP is caused by mutations of transthyretin (TTR), which forms amyloid fibrils affecting peripheral and autonomic nerves, the heart and other organs. Due to the phenotypic heterogeneity and partly not specific enough clinical symptoms, diagnosis of TTR-FAP can be complicated...
March 2018: Deutsche Medizinische Wochenschrift
Antonio Esposito, Anna Palmisano, Sofia Antunes, Caterina Colantoni, Paola Maria Vittoria Rancoita, Davide Vignale, Francesca Baratto, Paolo Della Bella, Alessandro Del Maschio, Francesco De Cobelli
PURPOSE: Diffuse remodeling of myocardial extra-cellular matrix is largely responsible for left ventricle (LV) dysfunction and arrhythmias. Our hypothesis is that the texture analysis of late iodine enhancement (LIE) cardiac computed tomography (cCT) images may improve characterization of the diffuse extra-cellular matrix changes. Our aim was to extract volumetric extracellular volume (ECV) and LIE texture features of non-scarred (remote) myocardium from cCT of patients with recurrent ventricular tachycardia (rVT), and to compare these radiomic features with LV-function, LV-remodeling, and underlying cardiac disease...
March 13, 2018: Molecular Imaging and Biology: MIB: the Official Publication of the Academy of Molecular Imaging
David Binas, Hanna Daniel, Anette Richter, Volker Ruppert, Klaus-Dieter Schlüter, Bernhard Schieffer, Sabine Pankuweit
Objective: Several studies indicate a prognostic value of sST2 and galectin-3 in heart failure (HF). While previous studies focused on ischaemic cause of HF, we investigated the role of sST2 and galectin-3 in patients with non-ischaemic dilated cardiomyopathy (DCM). Methods: sST2 and galectin-3 serum concentrations were measured in 262 subjects with DCM. Survival rates were determined for all-cause mortality (ACM) and cardiac mortality (CM). Results: In a univariate model, sST2 as a continuous variable was a predictor of ACM (HR 1...
2018: Open Heart
Petra Bilić, Nicolas Guillemin, Alan Kovačević, Blanka Beer Ljubić, Ines Jović, Asier Galan, Peter David Eckersall, Richard Burchmore, Vladimir Mrljak
Idiopathic dilated cardiomyopathy (iDCM) is a primary myocardial disorder with an unknown aetiology, characterized by reduced contractility and ventricular dilation of the left or both ventricles. Naturally occurring canine iDCM was used herein to identify serum proteomic signature of the disease compared to the healthy state, providing an insight into underlying mechanisms and revealing proteins with biomarker potential. To achieve this, we used high-throughput label-based quantitative LC-MS/MS proteomics approach and bioinformatics analysis of the in silico inferred interactome protein network created from the initial list of differential proteins...
March 9, 2018: Journal of Proteomics
Karen L Posey, Francoise Coustry, Jacqueline T Hecht
Cartilage oligomeric matrix protein (COMP) is a large pentameric glycoprotein that interacts with multiple extracellular matrix proteins in cartilage and other tissues. While, COMP is known to play a role in collagen secretion and fibrillogenesis, chondrocyte proliferation and mechanical strength of tendons, the complete range of COMP functions remains to be defined. COMPopathies describe pseudoachondroplasia (PSACH) and multiple epiphyseal dysplasia (MED), two skeletal dysplasias caused by autosomal dominant COMP mutations...
March 9, 2018: Matrix Biology: Journal of the International Society for Matrix Biology
Heather N Anderson, Frank Cetta, David J Driscoll, Timothy M Olson, Michael J Ackerman, Jonathan N Johnson
Idiopathic restrictive cardiomyopathy (IRC) is a rare condition characterized by reduced ventricular compliance. Children with IRC have poor outcomes with most patients proceeding to cardiac transplantation. We sought to analyze our institutional experience and assess contemporary outcomes for children with IRC. We reviewed the medical record for patients (<21 years old) evaluated for a primary diagnosis of IRC between 1975 and 2013 at our institution. Demographic, clinical, echocardiographic, and catheterization data were abstracted...
February 12, 2018: American Journal of Cardiology
Huichun Xu, Gerald W Dorn Ii, Amol Shetty, Ankita Parihar, Tushar Dave, Shawn W Robinson, Stephen S Gottlieb, Mark P Donahue, Gordon F Tomaselli, William E Kraus, Braxton D Mitchell, Stephen B Liggett
Idiopathic dilated cardiomyopathy (IDC) is the most common form of non-ischemic chronic heart failure. Despite the higher prevalence of IDC in African Americans, the genetics of IDC have been relatively understudied in this ethnic group. We performed a genome-wide association study to identify susceptibility genes for IDC in African Americans recruited from five sites in the U.S. (662 unrelated cases and 1167 controls). The heritability of IDC was calculated to be 33% (95% confidence interval: 19-47%; p = 6...
February 26, 2018: Journal of Personalized Medicine
Roland Hetzer, Mariano Francisco Del Maria Javier, Eva Maria Delmo Walter
Background: While heart transplantation has gained recognition as the gold standard therapy for advanced heart failure, the scarcity of donor organs has become an important concern. The evolution of surgical alternatives such as ventricular assist devices (VADs), allow for recovery of the myocardium and ensure patient survival until heart transplantation becomes possible. This report elaborates the role of VADs as a bridge to heart transplantation in infants and children (≤18 years old) with end-stage heart failure...
January 2018: Annals of Cardiothoracic Surgery
Nadia Bouabdallaoui, Pierre Demondion, Sylvestre Maréchaux, Shaida Varnous, Guillaume Lebreton, Frédéric Mouquet, Pascal Leprince
BACKGROUND: Peripartum cardiomyopathy is an idiopathic disorder defined by the occurrence of acute heart failure during late pregnancy or post-partum period in the absence of any other definable cause. Its clinical course is variable and severe cases might require heart transplantation. OBJECTIVE: To investigate long-term outcomes after heart transplantation (HT) for peripartum cardiomyopathy (PPCM). METHODS: Out of a single-center series of 1938 HT, 14 HT were performed for PPCM...
February 19, 2018: Arquivos Brasileiros de Cardiologia
Paulo Sérgio Juliani, João-Carlos Das-Neves-Pereira, Rosangela Monteiro, Aristides Tadeu Correia, Luiz Felipe Pinho Moreira, Fabio Biscegli Jatene
AIMS: Some authors have hypothesized that left ventricular chamber dilatation in ischaemic and idiopathic cardiomyopathies results in spherical transformation. Aiming to characterize how this transformation occurs, a study was performed by comparing normal and dilated specimens regarding sphericity and proportionality in left heart chambers. It is important to provide data for the development of therapeutic strategies in these diseases. METHODS AND RESULTS: An anatomical study was performed by comparing normal (n = 10), ischaemic (n = 15), and idiopathic (n = 18) dilated human cardiomyopathic specimens regarding left ventricular chambers and their segmental proportionality to normal hearts...
February 21, 2018: ESC Heart Failure
Adnan Yamanoglu, Nalan Gokce Celebi Yamanoglu, Sumeyye Cakmak, Ozgur Sogut
Peripartum cardiomyopathy (PPCM) is an idiopathic cardiomyopathy presenting with heart failure (HF) secondary to left ventricular systolic dysfunction towards the end of pregnancy or in the months following delivery, where no other cause of HF is found. The symptoms and signs of this rare disorder mimic those of the physiological changes of pregnancy or other cardiovascular diseases. Consequently, its diagnosis is frequently delayed, which worsens an already poor prognosis. Here, we report a young adult who was diagnosed with PPCM, early, with the help of focused cardiac ultrasonography, performed after presenting to the emergency department with nonspecific respiratory complaints, including dyspnea and hemoptysis, which suggested at first pulmonary embolism...
December 2017: Turkish Journal of Emergency Medicine
Yu-Min Sun, Jun Wang, Ying-Jia Xu, Xin-Hua Wang, Fang Yuan, Hua Liu, Ruo-Gu Li, Min Zhang, Yan-Jie Li, Hong-Yu Shi, Liang Zhao, Xing-Biao Qiu, Xin-Kai Qu, Yi-Qing Yang
Dilated cardiomyopathy (DCM) is a common primary myocardial disease leading to congestive heart failure, arrhythmia and sudden cardiac death. Increasing studies demonstrate substantial genetic determinants for DCM. Nevertheless, DCM is of substantial genetic heterogeneity, and the genetic basis for DCM in most patients remains unclear. The present study was sought to investigate the association of a genetic variant in the ZBTB17 gene with DCM. A cohort of 158 unrelated patients with idiopathic DCM and a total of 230 unrelated, ethnically matched healthy individuals used as controls were recruited...
February 14, 2018: Heart and Vessels
Edin Begic, Zijo Begic, Nabil Naser
Objective: Demonstration of idiopathic dilated cardiomyopathy with unusual flow, unpredictable clinical picture and complex therapy. Case report: Patient A.P., female, 38 years old, had symptoms of dilated cardiomyopathy (with possible infectious myocarditis in the background) at age 17. After hospitalization for ten months and ten days, while waiting for heart transplantation (with threatening death outcome), without a clearly pronounced threatening arrhythmia, but with a low ejection fraction and a poor general condition, remission occurred...
February 2018: Medical Archives
Alexander Fürnkranz, Hisaki Makimoto
Idiopathic ventricular tachycardia: Frequent monomorphic premature ventricular contractions or non-sustained ventricular tachycardia without underlying structural heart disease is not a rare condition and may cause significant symptoms. A subgroup of patients develops IVT-associated cardiomyopathy. Current ESC guidelines recommend primary catheter ablation in symptomatic patients with right ventricular outflow tract IVT. Catheter ablation is also recommended in symptomatic patients with a left ventricular outflow tract (or other rare) origin, if antiarrhythmic drugs are ineffective or not desired...
February 2018: Deutsche Medizinische Wochenschrift
A W G J Oomen, L R C Dekker, A Meijer
AIMS: This study was designed to gain insight into the patient characteristics, results and possible complications of ablation procedures for symptomatic idiopathic premature ventricular complexes (PVC) and idiopathic ventricular tachycardia (VT). METHODS: Data were collected from all patients who underwent radiofrequency catheter ablation for symptomatic PVCs and idiopathic VT in the Catharina Hospital between 1 January 2011 and 31 December 2015. The procedural endpoint was elimination or non-inducibility of the clinical arrhythmia...
January 30, 2018: Netherlands Heart Journal
Nandini Nair, Enrique Gongora
Growth and differentiation factor-15 (GDF-15) has been implicated in fibrosis, inflammation, and ventricular remodeling. The role of GDF-15 in the regulation of cardiac remodeling in idiopathic dilated cardiomyopathy (DCM) remains poorly defined. This study attempts to analyze the molecular interactions between GDF-15 and markers of fibrosis as well as its positive correlations with worsening functional capacity. The study population consisted of 24 DCM patients and 8 control subjects. All DCM patients had normal coronary angiographic studies...
January 2018: Journal of Circulating Biomarkers
Chin-Yu Lin, Fa-Po Chung, Yenn-Jiang Lin, Shih-Lin Chang, Li-Wei Lo, Yu-Feng Hu, Jo-Nan Liao, Ta-Chuan Tuan, Tze-Fan Chao, Yao-Ting Chang, Yun-Yu Chen, Abigail Louise D Te, Shinya Yamada, Shih-Ann Chen
Background: Epicardial catheter ablation has been shown to be an effective strategy for treating ventricular arrhythmias (VA). We investigated the efficacy and safety from a tertiary referral center in Taiwan. Methods: From 2010 to 2016, patients undergoing epicardial ablation for VAs were consecutively enrolled. The clinical characteristics, disease entity, electrophysiological studies, and ablation outcome were extracted for further analysis. Results: A total of 80 patients were eligible, including 34 patients for arrhythmogenic right ventricular cardiomyopathy (ARVC), 16 for Brugada syndrome (BrS), 13 for idiopathic VAs, 11 for idiopathic dilated cardiomyopathy (IDCM), 2 for ischemic cardiomyopathy, and 4 for other nonischemic cardiomyopathies (NICM)...
January 2018: Acta Cardiologica Sinica
Marco Luciani, Federica Del Monte
BACKGROUND: Dilated cardiomyopathy (DCM) is an independent nosographic entity characterized by left ventricular dilatation and contractile dysfunction leading to heart failure (HF). The idiopathic form of DCM (iDCM) occurs in the absence of coronaropathy or other known causes of DCM. Despite being different from other forms of HF for demographic, clinical, and prognostic features, its current pharmacological treatment does not significantly diverge. METHODS: In this study we performed a Pubmed library search for placebo-controlled clinical investigations and a post-hoc analysis recruiting iDCM from 1985 to 2016...
August 23, 2017: Journal of Cardiovascular Development and Disease
Kuo Zhang, Wenyao Wang, Shihua Zhao, Stuart D Katz, Giorgio Iervasi, A Martin Gerdes, Yi-Da Tang
BACKGROUND: Thyroid dysfunction and myocardial fibrosis are both associated with cardiovascular events in patients with dilated cardiomyopathy (DCM). HYPOTHESIS: The combination of thyroid hormone (TH) and myocardial fibrosis (detected by late gadolinium enhancement [LGE]) is an independent and incremental predictor of adverse events in DCM. METHODS: We consecutively enrolled 220 idiopathic DCM patients with thyroid function and LGE assessment at Fuwai Hospital (China) from January 2010 to October 2011 and followed up through December 2015...
January 23, 2018: Clinical Cardiology
Michael F Marmor
PURPOSE: To analyze an unusual case of widespread chorioretinopathy after cardiac transplantation for its potential etiology and clinical significance. METHODS: Clinical examinations included widefield and macular color and fundus autofluorescence photography, spectral domain optical coherence tomography, fluorescein angiography and indocyanine green angiography, full-field electroretinography, and Goldmann visual fields. PATIENT: A 44-year-old Hispanic woman was referred to rule out retinitis pigmentosa...
January 16, 2018: Retinal Cases & Brief Reports
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