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Pubblicazioni recenti - dilated cardiomyopathy
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Stress signaling and cellular proliferation reverse the effects of mitochondrial mistranslation.
EMBO J2019 Nov;():e102155. doi: 10.15252/embj.2019102155.
Ferreira Nicola, Perks Kara L, Rossetti Giulia, Rudler Danielle L, Hughes Laetitia A, Ermer Judith A, Scott Louis H, Kuznetsova Irina, Richman Tara R, Narayana Vinod K, Abudulai Laila N, Shearwood Anne-Marie J, Cserne Szappanos Henrietta, Tull Dedreia, Yeoh George C, Hool Livia C, Filipovska Aleksandra, Rackham Oliver,
Abstract
Translation fidelity is crucial for prokaryotes and eukaryotic nuclear-encoded proteins; however, little is known about the role of mistranslation in mitochondria and its potential effects on metabolism. We generated yeast and mouse models with error-prone and hyper-accurate mitochondrial translation, and found that translation rate is more important than translational accuracy for cell function in mammals. Specifically, we found that mitochondrial mistranslation causes reduced overall mitochondrial translation and respiratory complex assembly rates. In mammals, this effect is compensated for by increased mitochondrial protein stability and upregulation of the citric acid cycle. Moreover, this induced mitochondrial stress signaling, which enables the recovery of mitochondrial translation via mitochondrial biogenesis, telomerase expression, and cell proliferation, and thereby normalizes metabolism. Conversely, we show that increased fidelity of mitochondrial translation reduces the rate of protein synthesis without eliciting a mitochondrial stress response. Consequently, the rate of translation cannot be recovered and this leads to dilated cardiomyopathy in mice. In summary, our findings reveal mammalian-specific signaling pathways that respond to changes in the fidelity of mitochondrial protein synthesis and affect metabolism.
© 2019 The Authors.
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Myocardial Deformation Pattern Differs between Ischemic and Non-ischemic Dilated Cardiomyopathy: The Diagnostic Value of Longitudinal Strains.
Ultrasound Med Biol2019 Nov;():. doi: S0301-5629(19)31542-X.
Zuo Houjuan, Zhang Yan, Ma Fei, Li Rui, Wang Yan, Li Chenze, Wang Hong, Wang Dao Wen,
Abstract
Both ischemic cardiomyopathy (ICM) and non-ischemic cardiomyopathy (NICM) are characterized by left ventricular (LV) dysfunction and dilation. Differentiation between ICM and NICM using non-invasive image modalities is a clinical challenge. This study compared the myocardial deformation patterns of ICM and NICM using 2-D speckle tracking echocardiography (2-D STE) and sought to find parameters valuable in the diagnosis and management of dilated cardiomyopathy. The study population comprised 84 consecutive patients with LV end-diastolic dimension >55 mm and ejection fraction (EF) <45?%. Of these patients, 41 were diagnosed with ICM and 43 with NICM by coronary angiography. 2-D STE was performed in all patients. The LV dimension did not differ between ICM and NICM. Compared with patients with ICM, patients with NICM had lower EF (29.0% vs. 33.0%, p?=?0.024), lower global longitudinal strain (-5.4 ± 2.6% vs. -7.0 ± 2.5%, p?=?0.006) and lower global radial strain (7.5 ± 4.5% vs. 10.7 ± 4.7%, p?=?0.019). In contrast, global longitudinal strains did not differ significantly. However, NICM patients had higher apical and lower basal longitudinal strains compared with those with ICM. The ratio of basal to sum of mid- and apical longitudinal strains could predict NICM with a sensitivity of 63.4% and specificity of 88.4% by receiver operating characteristic curve analysis (cutoff value: 0.47, area under the curve: 0.792). Moreover, the concomitant presence of non-significant coronary artery stenosis (>50% and <70%) had no significant influence on global longitudinal strain in NICM. In conclusion, LV dilation and systolic dysfunction, relative apical sparing and a basal worsening pattern of LV longitudinal strain by 2-D STE were observed in patients with NICM but not ICM. The ratio of basal to sum of mid- and apical longitudinal strains could help differentiate NICM from ICM.
Copyright © 2019 The Authors. Published by Elsevier Inc. All rights reserved.
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Early Treatment of Coxsackievirus B3-Infected Animals With Soluble Coxsackievirus-Adenovirus Receptor Inhibits Development of Chronic Coxsackievirus B3 Cardiomyopathy.
Circ Heart Fail2019 Nov;12(11):e005250. doi: 10.1161/CIRCHEARTFAILURE.119.005250.
Pinkert Sandra, Dieringer Babette, Klopfleisch Robert, Savvatis Konstantinos, Van Linthout Sophie, Pryshliak Markian, Tschöpe Carsten, Klingel Karin, Kurreck Jens, Beling Antje, Fechner Henry,
Abstract
BACKGROUND:
Coxsackie-B-viruses (CVB) are frequent causes of acute myocarditis and dilated cardiomyopathy, but an effective antiviral therapy is still not available. Previously, we and others have demonstrated that treatment with an engineered sCAR-Fc (soluble coxsackievirus-adenovirus receptor fused to the carboxyl-terminus of human IgG) efficiently neutralizes CVB3 and inhibits the development of cardiac dysfunction in mice with acute CVB3-induced myocarditis. In this study, we analyzed the potential of sCAR-Fc for treatment of chronic CVB3-induced myocarditis in an outbred NMRI mouse model.
METHODS:
NMRI mice were infected with the CVB3 strain 31-1-93 and treated with a sCAR-Fc expressing adeno-associated virus 9 vector 1, 3, and 7 days after CVB3 infection. Chronic myocarditis was analyzed on day 28 after infection.
RESULTS:
Initial investigations showed that NMRI mice develop pronounced chronic myocarditis between day 18 and day 28 after infection with the CVB3 strain 31-1-93. Chronic cardiac infection was characterized by inflammation and fibrosis as well as persistence of viral genomes in the heart tissue and by cardiac dysfunction. Treatment of NMRI mice resulted in a distinct reduction of cardiac inflammation and fibrosis and almost complete elimination of virus RNA from the heart by day 28 after infection. Moreover, hemodynamic measurement revealed improved cardiac contractility and diastolic relaxation in treated mice compared with mice treated with a control vector (mean±SD; maximal pressure, 81.9±9.2 versus 69.4±8.6 mm?Hg, =0.02; left ventricular ejection fraction, 68.9±8.5 versus 54.2±11.5%, =0.02; dP/dt, 7275.2±1674 versus 4432.6±1107 mm?Hg/s, =0.004; dP/dt, -4046.9±776 versus -3146.3±642 mm?Hg/s, =0.046). The therapeutic potential of sCAR-Fc is limited, however, since postponed start of sCAR-Fc treatment either 3 or 7 days after infection could not attenuate myocardial injury.
CONCLUSIONS:
Early therapeutic employment of sCAR-Fc, initiated at the beginning of the primary viremia, inhibits the development of chronic CVB3-induced myocarditis and improves the cardiac function to a level equivalent to that of uninfected animals.
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Left Ventricular Strain and Rotation in Patients with Dilated Cardiomyopathy and Severe Systolic Dysfunction.
Cardiology2019 Nov;():1-12. doi: 10.1159/000503682.
Kinova Elena, Somleva-Todorova Desislava, Goudev Assen,
Abstract
INTRODUCTION:
In dilated cardiomyopathy (DCM) left ventricular (LV) strain and twist are significantly decreased. However, the rate of attenuation has not been investigated well in patients with varying degrees of systolic dysfunction.
AIM:
The present study aimed to investigate the relationship between LV deformational and rotational mechanics and conventional and tissue Doppler imaging (TDI) parameters, and to search for a constellation of findings distinguishing patients with severe systolic dysfunction (SSD) in DCM.
METHODS:
Fifty-two patients with heart failure NYHA class III-IV and ejection fraction (EF) ?45% were prospectively enrolled (mean age 61.8 ± 13.4 years; 36 males, 69%). Severe systolic LV dysfunction was considered as EF <30%. Echocardiography with 2D-speckle tracking analysis was performed.
RESULTS:
The relationships of global longitudinal strain (GLS) with EF, circumferential strain at mid-level (CSmid), and systolic medial mitral annulus velocity were strong (r = -0.53, 0.67, and -0.56, respectively, p < 0.0001 for all). A good correlation was found between CSmid and EF (r = -0.50, p < 0.0001). There were weak correlations between basal endocardial rotation (BRendo) and EF and CSmid. Multiple regression analysis found GLS (p < 0.0001) and BRendo (p = 0.04) to be predictors of the change of EF. In ROC curve analysis, the cut-off values of GLS -7.2% (AUC 0.81, p < 0.0001), CSmid -7.5% (AUC 0.76, p = 0.002), and BRendo -2.43° (AUC 0.68, p = 0.03) identified SSD.
CONCLUSIONS:
Parameters of LV mechanics were related to conventional and TDI systolic parameters in patients with DCM. The degree of alterations of LV longitudinal and circumferential deformation and basal rotation may identify patients with SSD and a higher risk, and may help in therapeutic decision making.
© 2019 S. Karger AG, Basel.
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Does Repeated Measurement of a 6-Min Walk Test Contribute to Risk Prediction in Children with Dilated Cardiomyopathy?
Pediatr Cardiol2019 Nov;():. doi: 10.1007/s00246-019-02244-7.
van der Meulen Marijke H, Boer Susanna den, du Marchie Sarvaas Gideon J, Blom Nico A, Ten Harkel Arend D J, Breur Hans M P J, Rammeloo Lukas A J, Tanke Ronald, Helbing Willem A, Boersma Eric, Dalinghaus Michiel,
Abstract
A single 6-min walk test (6MWT) can be used to identify children with dilated cardiomyopathy (DCM) with a high risk of death or heart transplantation. To determine if repeated 6MWT has added value in addition to a single 6MWT in predicting death or heart transplantation in children with DCM. Prospective multicenter cohort study including ambulatory DCM patients???6 years. A 6MWT was performed 1 to 4 times per year. The distance walked was expressed as percentage of predicted (6MWD%). We compared the temporal evolution of 6MWD% in patients with and without the study endpoint (SE: all-cause death or heart transplantation), using a linear mixed effects model. In 57 patients, we obtained a median of 4 (IQR 2-6) 6MWTs per patient during a median of 3.0 years of observation (IQR 1.5-5.1). Fourteen patients reached a SE (3 deaths, 11 heart transplantations). At any time during follow-up, the average estimate of 6MWD% was significantly lower in patients with a SE compared to patients without a SE. In both patients groups, 6MWD% remained constant over time. An absolute 1% lower 6MWD% was associated with an 11% higher risk (hazard) of the SE (HR 0.90, 95% CI 0.86-0.95 p?
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DNA Damage Prediction Tool in Dilated Cardiomyopathy: Don't Go Breaking My Heart.
JACC Basic Transl Sci2019 Oct;4(6):681-683. doi: 10.1016/j.jacbts.2019.08.005.
Jimenez Jesus, Rentschler Stacey L,
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Identification of Gene Mutations in Primary Pediatric Cardiomyopathy by Whole Exome Sequencing.
Pediatr Cardiol2019 Nov;():. doi: 10.1007/s00246-019-02240-x.
Rojnueangnit Kitiwan, Sirichongkolthong Boonchu, Wongwandee Ratthapon, Khetkham Thanitchet, Noojarern Saisuda, Khongkraparn Arthaporn, Wattanasirichaigoon Duangrurdee,
Abstract
Pediatric primary cardiomyopathy is rare but serious, having high mortality; hypertrophic and dilated types are the most common. Its etiology has been mainly considered idiopathic; however, next generation sequencing techniques have revealed nearly half of idiopathic pediatric cases arose from specific genetic mutations. Therefore, our study aimed to identify the genetic causes of primary idiopathic cardiomyopathy. Newborns to 15-year old patients with this condition were recruited between March 2016 and May 2017 at Thammasat University Hospital. Complete patient history and physical examination data were collected by a geneticist with cardiac examinations and echocardiograms by pediatric cardiologists. Whole exome sequencing was performed for all. Of the 12 patients enrolled, 5 cases were dilated type and 7 hypertrophic. Two with dilated type were excluded during follow-up as cause was determined (hypocalcemia and pacemaker induced). A list of 118 genes for cardiomyopathy was analyzed in the remaining 10 cases. Pathogenic and likely pathogenic mutations were identified in 5 patients: HRAS, PTPN11, SOS1, FLNC and TXNRD2; half our patients were not actually idiopathic. Despite its high cost, genetic testing is useful for determining familial risk as well as predicting patient cardiomyopathy progress.
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Low mutation rate in the TTN gene in paediatric patients with dilated cardiomyopathy - a pilot study.
Sci Rep2019 Nov;9(1):16409. doi: 10.1038/s41598-019-52911-1.
Zaklyazminskaya Elena, Mikhailov Vadim, Bukaeva Anna, Kotlukova Natalia, Povolotskaya Inna, Kaimonov Vladimir, Dombrovskaya Anna, Dzemeshkevich Sergey,
Abstract
Idiopathic dilated cardiomyopathy (DCM) is a common cardiomyopathy with the prevalence of 1:250, and at least one-third of all the cases are inherited. Mutations in the TTN gene are considered as the most frequent cause of inherited DCM and cover 10-30% of the cases. The studies were mainly focused on the adult or mixed age group of patients with DCM. The mutation rate in the TTN gene, the characteristics of manifestations and their prognostic significance in childhood have not been studied. To determine TTN mutation rate in children with DCM and the relevance of including this gene in the DNA diagnostic protocol for paediatric DCM, complete clinical and instrumental examination of 36 DCM patients (up to 18 years) with the manifestation of the disease was conducted in specialised cardiology centres. Molecular genetic testing included sequencing of coding and adjacent regulatory regions of the major cardiac TTN isoform N2BA using IonTorrent ? semiconductor sequencing (for 25 isolated cases) and trio whole exome sequencing (trio WES)on the Illumina platform (for 11 family cases). Our pilot group included 36 probands with DCM diagnosis first established on the basis of the generally accepted criteria at the age of 5 days to 18 years(average age: 6.5 years). The sex ratio (M:F) was 23: 8. There were 25 sporadic DCM cases and 11 cases of familial DCM (at least one of the parents and/or siblings were also diagnosed with DCM). The only likely pathogenic truncating variant p.Arg33703*in the TTN gene (TTNtv) was found in a 16-year-oldmale proband out of 36 (3%). Apparently, TTN-dependent forms of DCMs manifest later at a young (but older than 18 years) or more mature age, and TTN gene cannot be considered as the first-line genetic testing for DCM in the paediatric group, despite several studies have reported a generally high mutation rate in this gene with DCM. Further research is needed to compare the representation of mutations in the TTN gene in different age groups of DCM patients.
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Prognostic significance of tissue Doppler imaging-derived myocardial performance index in pediatric patients with dilated cardiomyopathy.
Pediatr Transplant2019 Nov;():e13613. doi: 10.1111/petr.13613.
Wright Lydia K, McGaughy Falon, Kellerman Michael, Border William L, Sachdeva Ritu,
Abstract
TDI-MPI has been shown to predict cardiovascular mortality in adults; there are a paucity of data on its use in children. We sought to determine the prognostic significance of TDI-MPI at time of DCM diagnosis in children. Patients aged ?18 years diagnosed with DCM were included along with age- and sex-matched controls. Echo at diagnosis was analyzed to obtain standard measures of LV function, PW-MPI, and septal and LV free wall TDI-MPI. Survival analysis was used to assess the time to composite outcome of death, VAD, or transplant, stratified by TDI-MPI z-score. The study included 79 patients with DCM and 79 controls. During a median follow-up of 182 days (IQR 41-815 days), 16 underwent VAD placement, 21 underwent cardiac transplant, 6 died, and 36 had event-free survival. The median septal TDI-MPI for cases was 0.70 for patients with DCM vs 0.45 for controls (P < .001). Those with septal TDI-MPI z-scores ?2 develop events significantly earlier than those with z-score <2 (P = .014). In multivariable analysis, TDI-MPI z-score ?2 was significantly associated with poor outcomes (HR 2.12, 95% CI 1.06-4.23). TDI-MPI can be reliably performed in pediatric patients with DCM. A TDI-MPI z-score ?2 at diagnosis may be associated with earlier poor outcome. Further studies evaluating the use of TDI-MPI in longitudinal follow-up of patients with DCM may be helpful in refining its clinical use.
© 2019 Wiley Periodicals, Inc.
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Quantification of DNA Damage in Heart Tissue as a Novel Prediction Tool for Therapeutic Prognosis of Patients With Dilated Cardiomyopathy.
JACC Basic Transl Sci2019 Oct;4(6):670-680. doi: 10.1016/j.jacbts.2019.05.010.
Ko Toshiyuki, Fujita Kanna, Nomura Seitaro, Uemura Yukari, Yamada Shintaro, Tobita Takashige, Katoh Manami, Satoh Masahiro, Ito Masamichi, Domoto Yukako, Hosoya Yumiko, Amiya Eisuke, Hatano Masaru, Morita Hiroyuki, Fukayama Masashi, Aburatani Hiroyuki, Komuro Issei,
Abstract
This study evaluated myocardial nuclear staining for the DNA damage markers poly(ADP-ribose) (PAR) and ?-H2A.X in 58 patients with dilated cardiomyopathy. Patients with left ventricular reverse remodeling (LVRR) showed a significantly smaller proportion of PAR-positive nuclei and ?-H2A.X-positive nuclei in biopsy specimens compared with those without LVRR. Propensity analysis showed that the proportion of both PAR-positive and ?-H2A.X-positive nuclei were independent prognostic factors for LVRR. In conclusion, we showed the utility of DNA damage-marker staining to predict the probability of LVRR, thus revealing a novel prognostic predictor of medical therapy for dilated cardiomyopathy.
© 2019 The Authors.
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Heart failure etiologies and clinical factors precipitating for worsening heart failure: Findings from BIOSTAT-CHF.
Eur J Intern Med2019 Nov;():. doi: S0953-6205(19)30354-1.
Kobayashi Masatake, Voors Adriaan A, Girerd Nicolas, Billotte Maxime, Anker Stefan D, Cleland John G, Lang Chim C, Ng Leong L, van Veldhuisen Dirk J, Dickstein Kenneth, Metra Macro, Duarte Kevin, Rossignol Patrick, Zannad Faiez, Ferreira João Pedro,
Abstract
BACKGROUND:
Knowledge on the association between heart failure (HF) etiologies, precipitant causes and clinical outcomes may help in ascertaining patient's risk and in selecting tailored therapeutic strategies.
METHODS:
The prognostic value of both HF etiologies and precipitants for worsening HF were analyzed using the index cohort of BIOSTAT-CHF. The studied HF etiologies were: a) ischemic HF; b) dilated cardiomyopathy; c) hypertensive HF; d) valvular HF; and e) other/unknown. The precipitating factors for worsening HF were: a) atrial fibrillation; b) non-adherence; c) renal failure; d) acute coronary syndrome; e) hypertension; and f) Infection. The primary outcome was the composite of all-cause death or HF hospitalization.
RESULTS:
Among 2465 patients included in the study, 45% (N?=?=1102) had ischemic HF, 23% (N?=?=563) dilated cardiomyopathy, 15% (N?=?=379) other/unknown, 10% (N?=?=237) hypertensive and 7% (N?=?=184) valvular HF. Patients with ischemic HF had the worst prognosis, whereas patients with dilated cardiomyopathy had the best prognosis. From the precipitating factors for worsening HF, renal failure was the one independently associated with worse prognosis (adjusted HR (95%CI)?=?=1.48 (1.04-2.09), p??0.10 for all). Treatment up-titration benefited patients regardless of their underlying etiology or precipitating cause (p interaction?>?0.10 for all).
CONCLUSIONS:
In BIOSTAT-CHF, patients with HF of an ischemic etiology, and those with worsening HF precipitated by renal failure (irrespective of the underlying HF etiology), had the highest rates of death and HF hospitalization, but still benefited equally from treatment up-titration.
Crown Copyright © 2019. Published by Elsevier B.V. All rights reserved.
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Upregulation of Yy1 Suppresses Dilated Cardiomyopathy caused by Ttn insufficiency.
Sci Rep2019 Nov;9(1):16330. doi: 10.1038/s41598-019-52796-0.
Liao Dan, Chen Weiming, Tan Chia Yee, Wong Jing Xuan, Chan Pui Shi, Tan Lek Wen, Foo Roger, Jiang Jianming,
Abstract
Truncating variants in TTN (TTNtv), coding for the largest structural protein in the sarcomere, contribute to the largest portion of familial and ambulatory dilated cardiomyopathy (DCM). TTN haploinsufficiency caused by TTNtv is suggested as the disease mechanism. However, it is unclear whether TTN insufficiency causes DCM. Moreover, it is unknown whether modulation of downstream pathways serves as a therapeutic strategy for DCM caused by TTN insufficiency. Here, we show that reduction of cardiac Ttn expression by adeno-associated virus mediated shRNA (Ttn shRNA) generated DCM in mouse, demonstrating impaired cardiac performance, enlarged left ventricle (LV) and reduced LV wall thickness. A screen of 10 dysregulated and selected genes identified that Yin Yang 1 (Yy1) significantly suppressed DCM caused by Ttn shRNA. Gene profiling by RNAseq showed Yy1 modulated cell growth related genes. Ttn insufficiency activated cardiomyocyte cell cycle reentry by upregulating of Ccnd1 and Ccnd2. Cardiomyocytes activated by Ttn insufficiency did not advance to S phase by EdU incorporation assay. Yy1 promoted cardiomyocyte cell cycle by further enhancing Ccnd1 and Ccnd2 and increasing DNA replication without undergoing cell division. Importantly, upregulation of Ccnd1 and Ccnd2 suppressed DCM caused by Ttn insufficiency. Our findings demonstrate that DCM caused by Ttn insufficiency can be treated by therapeutically promoting cardiac cell cycle.
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Fenofibrate Reverses Dysfunction of EPCs Caused by Chronic Heart Failure.
J Cardiovasc Transl Res2019 Nov;():. doi: 10.1007/s12265-019-09889-y.
Huang Wen-Pin, Yin Wei-Hsian, Chen Jia-Shiong, Huang Po-Hsun, Chen Jaw-Wen, Lin Shing-Jong,
Abstract
The enhanced activity of endothelial progenitor cells (EPCs) by AMP-activated protein kinase (AMPK) agonists might explain the reversal of chronic heart failure (CHF)-mediated endothelial dysfunction. We studied baseline circulating EPC numbers in patients with heart failure and clarified the effect of fenofibrate on both circulating angiogenic cell (CAC) and late EPC activity. The numbers of circulating EPCs in CHF patients were quantified by flow cytometry. Blood-derived mononuclear cells were cultured, and CAC and late EPC functions, including fibronectin adhesion, tube formation, and migration, were evaluated. We focused on the effect of fenofibrate, an AMPK agonist, on EPC function and Akt/eNOS cascade activation in vitro. The number of circulating EPCs (CD34/KDR) was significantly lower in CHF patients (ischemic cardiomyopathy (ICMP): 0.07%, dilated cardiomyopathy (DCMP): 0.068%; p?
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Relationship between left ventricular isovolumic relaxation flow patterns and mitral inflow patterns studied by using vector flow mapping.
Sci Rep2019 Nov;9(1):16264. doi: 10.1038/s41598-019-52680-x.
Han Yu, Huang Liang, Li Zhiguo, Ma Na, Li Qiaozhen, Li Yiwei, Wu Ling, Zhang Xiaoxia, Wu Xiaoyi, Che Xinyi, Zhang Haibin,
Abstract
The purpose of this study was to investigate the relationship between isovolumic relaxation flow (IRF) patterns in left ventricle (LV) and mitral inflow patterns. Color Doppler loops were acquired for vector flow mapping in apical long-axis view in 57 patients with coronary artery disease, 31 patients with dilated cardiomyopathy, and 58 healthy controls. IRF patterns were classified into three categories: pattern A, apically directed flow; pattern B, bidirectional flow with small scattered vortices; and pattern C, a large vortex. All normals and patients with normal LV filling (n?=?10) showed pattern A. Patients with impaired relaxation consisted of 31 (66%) patients having pattern A, 11 (23%) having pattern B, and 5 (11%) having pattern C. Patients with pseudonormal filling included 4 (31%) patients having pattern A, 7 (54%) having pattern B, and 2 (15%) having pattern C. In patients with restrictive filling, 14 (78%) showed pattern C, 4 (22%) showed pattern B, and no patient showed pattern A. IRF patterns were associated with LV filling patterns (??=?52.026, p?
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[Prevention of sudden cardiac death and implantable cardioverter defibrillators: Understanding the competitive risk!]
Presse Med2019 Nov;():. doi: S0755-4982(19)30386-0.
Rischard Julien, Narayanan Kumar, Garcia Rodrigue, Marijon Eloi,
Abstract
Sudden cardiac death represents a major public health issue, with up to 50% of the cardiovascular mortality. Coronary artery disease and dilated cardiomyopathy both represent almost 90% of sudden cardiac death burden. Primary prevention using implantable cardioverter defibrillator relies, in this population, on the left ventricle ejection fraction simple measurement. In this paper, we aim to discuss in which extent a better understanding of competing risk situation may help for a better patient selection and eventually for optimizing primary prevention using implantable cardioverter defibrillator.
Copyright © 2019 Elsevier Masson SAS. All rights reserved.
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Comparison of Clinical Characteristics and Outcomes of Patients With Reversible Versus Persistent Methamphetamine-Associated Cardiomyopathy.
Am J Cardiol2019 Oct;():. doi: S0002-9149(19)31099-9.
Zhao Susan X, Seng Sakara, Deluna Andres, Yu Elizabeth C, Crawford Michael H,
Abstract
Anecdotal cases of reversible methamphetamine-associated cardiomyopathy (rMAC) have been reported, but not well understood. This study sought to determine the clinical characteristics, outcomes and predictors of reversibility among patients with rMAC as compared with patients with persistent MAC (pMAC). We retrospectively studied adult MAC patients with left ventricular ejection fraction (LVEF) ?40% at a single center between 2004 and 2018. rMAC was defined as increase in LVEF by ?20 points or to ?50%. Those with persistent LVEF ?40% constituted the pMAC group. 357 MAC cases were identified: 250 patients had pMAC and 107 had rMAC. After a median follow-up of 45 months (interquartile range 27 to 70), LVEF increased by 28.3 ± 6.9% in rMAC (p <0.001), whereas it was unchanged in pMAC (?: -0.5 ± 8.7%, p?=?0.350). Heart failure hospitalizations and New York Heart Association Class III/IV heart failure were both significantly reduced for rMAC than the pMAC group. All-cause mortality was 21.6% overall, 28% in pMAC and 6.5% in the rMAC group (p <0.001). Kaplan-Meier survival curves demonstrated significantly higher cumulative survival for rMAC (Log Rank p <0.001). Multivariable logistic regression identified MA cessation (odds ratio/OR: 4.23, 95% confidence interval/CI: 2.47 to 7.38, p <0.001) and baseline right ventricular end systolic area (OR: 0.92, 95% CI: 0.87 to 0.97, p?=?0.001) as strongly predictive of MAC reversal. In conclusion, MAC reversal is not uncommon and is associated with significant clinical improvement including reduced mortality. It can be facilitated by MA cessation when the cardiac chambers, especially the right ventricle, are not severely dilated.
Copyright © 2019 Elsevier Inc. All rights reserved.
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Improving the accuracy of heart failure diagnosis in low-resource settings through task sharing and decentralization.
Glob Health Action2019 ;12(1):1684070. doi: 10.1080/16549716.2019.1684070.
DeWyer Alyssa, Scheel Amy, Otim Isaac Omara, Longenecker Christopher T, Okello Emmy, Ssinabulya Isaac, Morris Stephen, Okwir Mark, Oyang William, Joyce Erine, Nabongo Betty, Sable Craig, Alencherry Ben, Tompsett Alison, Aliku Twalib, Beaton Andrea,
Abstract
: Task sharing of TTE may improve capacity for heart failure diagnosis and management in patients in remote, low-resource settings but the impact on diagnostic accuracy and patient outcomes has not been studied.: Determine feasibility and impact of non-expert training in transthoracic echocardiography (TTE) to improve the diagnosis and outcomes of patients with suspected heart failure in Uganda.: This two-part study examined an innovative training program to develop TTE competency among non-experts and used a pre-post design to determine the impact of decentralized TTE. Four of 8 non-experts (50%) passed a three-part training course. The training comprised of distance learning through a web-based curriculum, a 2-day hands-on workshop with cardiologists, and independent practice with remote mentorship. Continuous measures were compared (pre- vs. post-TTE) using t-tests or Wilcoxon rank-sum tests as distributionally appropriate and categorical variables assessed through chi-square testing. Sensitivity and specificity were calculated according to standard methodology comparing diagnosis pre- and post-TTE during phase 2.: Performance in the post-training phase showed good agreement with expert categorization (? = 0.80) with diagnostic concordance in 421 of 454 studies (92.7%). TTE changed the preliminary diagnosis in 81% of patients, showing low specificity of clinical decision-making alone (14.2%; 95% CI 10.1-19.2%). Dilated cardiomyopathy, hypertensive heart disease with preserved systolic function, and right heart failure were the most underdiagnosed conditions prior to TTE while hypertensive heart disease with decreased systolic function was the most over-diagnosed condition.: In conclusion, non-expert providers can achieve a high level of proficiency for the categorization of heart failure using handheld TTE in low-resource settings and use of telemedicine and remote mentorship may improve performance and feasibility. The addition of TTE resulted in substantial improvement in etiological specificity. Further study is needed to understand implications of this strategy on healthcare utilization, long-term patient outcomes, and cost.
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[Structured Reporting in Cross-Sectional Imaging of the Heart: Reporting Templates for CMR Imaging of Cardiomyopathies (Myocarditis, Dilated Cardiomyopathy, Hypertrophic Cardiomyopathy, Arrhythmogenic Right Ventricular Cardiomyopathy and Siderosis)].
Rofo2019 Nov;():. doi: 10.1055/a-1034-2379.
Bunck Alexander Christian, Baeßler Bettina, Ritter Christian, Kröger Jan Robert, Persigehl Thorsten, Pinto Dos Santos Daniel, Steinmetz Michael, Niehaus Adelheid, Bamberg Fabian, Beer Meinrad, Ley Sebastian, Tiemann Klaus, Beerbaum Philipp, Maintz David, Lotz Joachim,
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Peripartum cardiomyopathy: disease or syndrome?
Heart2019 Mar;105(5):357-362. doi: 10.1136/heartjnl-2018-314252.
Baris Lucia, Cornette Jérôme, Johnson Mark R, Sliwa Karen, Roos-Hesselink Jolien W,
Abstract
Peripartum cardiomyopathy (PPCM) is a rare form of pregnancy-associated heart failure and is considered to be a diagnosis of exclusion. There are many hypotheses on the aetiology of PPCM; however, the exact pathophysiological mechanism remains unknown. It shows many resemblances to other conditions, such as familial dilated cardiomyopathy or myocarditis, and therefore it can be hard to make a definite diagnosis. We describe four cases of peripartum-onset heart failure in women who were suspected of having PPCM. We discuss the differential diagnosis, pathophysiological mechanisms and various diagnostic modalities.
© Author(s) (or their employer(s)) 2019. Re-use permitted under CC BY. Published by BMJ.
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Inflammation in myocardial disease: From myocarditis to dilated cardiomyopathy.
Pathol Int2019 Nov;():. doi: 10.1111/pin.12868.
Imanaka-Yoshida Kyoko,
Abstract
Dilated cardiomyopathy (DCM) is a heterogeneous group of myocardial diseases clinically defined by the presence of left ventricular dilatation and contractile dysfunction. Among various causes of DCM, a progression from viral myocarditis to DCM has long been hypothesized. Supporting this possibility, studies by endomyocardial biopsy, the only method to obtain a definite diagnosis of myocarditis at present, have provided evidence of inflammation in the myocardium in DCM patients. A number of experimental studies have elucidated a cell-mediated autoimmune mechanism triggered by viral infection in the progression of myocarditis to DCM. In addition, the important role of inflammation in the pathogenesis of heart failure has been recognized, and many terms including myocarditis, inflammatory cardiomyopathy, and inflammatory DCM have been used for myocardial diseases associated with inflammation. This review discusses the pathophysiology of inflammation in the myocardium, and refers to diagnosis and treatment based on these concepts.
© 2019 Japanese Society of Pathology and John Wiley & Sons Australia, Ltd.
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