2,148 results match your criteria Cardiomyopathy Restrictive


Cardiac Amyloidosis: Evolving Diagnosis and Management: A Scientific Statement From the American Heart Association.

Circulation 2020 Jun 1:CIR0000000000000792. Epub 2020 Jun 1.

Transthyretin amyloid cardiomyopathy (ATTR-CM) results in a restrictive cardiomyopathy caused by extracellular deposition of transthyretin, normally involved in the transportation of the hormone thyroxine and retinol-binding protein, in the myocardium. Enthusiasm about ATTR-CM has grown as a result of 3 simultaneous areas of advancement: Imaging techniques allow accurate noninvasive diagnosis of ATTR-CM without the need for confirmatory endomyocardial biopsies; observational studies indicate that the diagnosis of ATTR-CM may be underrecognized in a significant proportion of patients with heart failure; and on the basis of elucidation of the mechanisms of amyloid formation, therapies are now approved for treatment of ATTR-CM. Because therapy for ATTR-CM may be most effective when administered before significant cardiac dysfunction, early identification of affected individuals with readily available noninvasive tests is essential. Read More

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http://dx.doi.org/10.1161/CIR.0000000000000792DOI Listing

Understanding the Relationships of Cardiac Diagnostics in Patients with Amyloid Transthyretin Cardiac Amyloidosis.

J Heart Lung Transplant 2020 Apr 30;39(4S):S36. Epub 2020 Mar 30.

Division of Cardiology, University Health Network, Toronto, ON, Canada.

Purpose: Amyloid transthyretin (ATTR) cardiac amyloidosis (CA) is an increasingly recognized cause of restrictive cardiomyopathy and associated heart failure with preserved ejection fraction (HFpEF). Despite improved diagnostic techniques to identify this condition, many patients experience delayed diagnosis. Interpretation of routine cardiac biomarkers (i. Read More

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http://dx.doi.org/10.1016/j.healun.2020.01.1192DOI Listing

Comparison of Six-Month Outcomes in Hypertrophic Cardiomyopathy Patients before and after UNOS Allocation System Change.

J Heart Lung Transplant 2020 Apr 30;39(4S):S263. Epub 2020 Mar 30.

Cardiology, Keck Medicine of USC, Los Angeles, CA.

Purpose: HCM patients may have increased waitlist (WL) mortality. Given the recent change in prioritizing patients with restrictive and hypertrophic cardiomyopathy requiring mechanical support, we sought to assess whether the policy change would affect the waitlist and post-transplant outcomes in HCM pts.

Methods: 59 pts were identified in UNOS registry that underwent heart transplant (HT) with a diagnosis of HCM during a 6-month period immediately before and after the UNOS policy change. Read More

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http://dx.doi.org/10.1016/j.healun.2020.01.572DOI Listing

Comparison of Six-Month Outcomes in Restrictive Cardiomyopathy Patients before and after UNOS Allocation System Change.

J Heart Lung Transplant 2020 Apr 30;39(4S):S262-S263. Epub 2020 Mar 30.

Cardiology, Keck Medicine of USC, Los Angeles, CA.

Purpose: Restrictive cardiomyopathy (RCM) patients represent a spectrum of disorders. Given the greater emphasis for status exceptions for RCM pts in the new allocation system, we sought to assess whether this change in the allocation policy would affect the wait list and post-transplant outcomes in RCM pts.

Methods: 89 RCM pts were identified in the UNOS registry that underwent heart transplant (HT) during a 6-mos period immediately before and after UNOS policy change. Read More

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http://dx.doi.org/10.1016/j.healun.2020.01.571DOI Listing

HeartMate 3 as Total Artificial Heart for the Treatment of Biventricular Heart Failure as Bridge to Transplantation.

J Heart Lung Transplant 2020 Apr 30;39(4S):S24. Epub 2020 Mar 30.

Cardiac, Thoracic, Transplantation and Vascular Surgery, Hannover Medical School, Hannover, Germany.

Purpose: Despite maximal medican and surgical therapy, the outcomes of severe biventricular failure remain poor with mortality rates of up to 100%. The only definite therapy option for this disease is the total replacement of the heart - either by cardiac transplantation or by total artificial heart (TAH) implantation. Due to the lack of donor organs, mechanical assist devices are the most relevant option for these patients. Read More

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http://dx.doi.org/10.1016/j.healun.2020.01.1160DOI Listing

Cardiac Cirrhosis is Largely Underdiagnosed by Non-Invasive Diagnostic Imaging Tests: A Retrospective Study.

J Heart Lung Transplant 2020 Apr 30;39(4S):S233-S234. Epub 2020 Mar 30.

Cardiology, IUCPQ, Québec, QC, Canada.

Purpose: Hepatic cirrhosis is a potential severe complication of chronic heart failure and has major implications on treatment options. In general, patients with cirrhosis are considered ineligible for isolated cardiac transplantation. Therefore, proper diagnosis of cirrhosis is paramount. Read More

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http://dx.doi.org/10.1016/j.healun.2020.01.895DOI Listing

Can Genetic Profile of Patients Undergoing Heart Transplantation Alter Clinical Decisions?

J Heart Lung Transplant 2020 Apr 30;39(4S):S232-S233. Epub 2020 Mar 30.

Molecular Immunopathology and Histocombatibility, Onassis Cardiac Surgery Center, Athens, Greece.

Purpose: Although a significant proportion of cardiomyopathies are determined by genetic factors, the genetic testing of these patients has not been introduced routinely into clinical practice. The aim of this study is the genotyping of patients with advanced cardiomyopathy who have been bridged for heart transplantation or have been already transplanted, in order to evaluate the usefulness of genetic testing in the early diagnosis of the disease, and in the clinical management of these patients and their relatives.

Methods: Fifteen heart transplanted patients and 5 patients bridged for heart transplantation due to familial dilated, hypertrophic or restrictive cardiomyopathy were genotyped with next generation sequencing, using Illumina's Trusight Cardio sequencing panel, covering 174 clinically relevant genes to cardiac diseases. Read More

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http://dx.doi.org/10.1016/j.healun.2020.01.892DOI Listing

Heart Transplant Outcomes in Patients with Alcoholic Cardiomyopathy.

J Heart Lung Transplant 2020 Apr 30;39(4S):S228. Epub 2020 Mar 30.

Div of Cardiovascular Medicine, Keck School of Medicine of USC, Los Angeles, CA.

Purpose: We sought to better characterize differences in transplant outcomes between patients with alcoholic cardiomyopathy and those with other forms of cardiomyopathy in a national cohort.

Methods: 63,142 OHT recipients were identified from UNOS (1987-2019) of which 224 had alcoholic cardiomyopathy as indication for HT. Multivariate Cox proportional hazard regression analysis was adjusted for age, sex, DM2, ethnicity, ischemic time, dialysis, life support, waitlist time and HLA mismatch. Read More

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http://dx.doi.org/10.1016/j.healun.2020.01.879DOI Listing

First in Man Anatomical Fitting Study of the BiVACOR Total Artificial Heart.

J Heart Lung Transplant 2020 Apr 30;39(4S):S189. Epub 2020 Mar 30.

Cardiothoracic Surgery, St Vincent's Hospital (Sydney), Darlinghurst, Australia.

Purpose: BiVACOR is a new and novel total artificial heart utilising a single centrifugal magnetically levitated motor featuring a pulsatile flow. The device has been successfully tested in cattle with encouraging 90-day results. We sought to undertake the first-in-man fitting of the device. Read More

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http://dx.doi.org/10.1016/j.healun.2020.01.779DOI Listing

Impact of Bleeding Complications on Outcomes Following Total Artificial Heart Implantation.

J Heart Lung Transplant 2020 Apr 30;39(4S):S150. Epub 2020 Mar 30.

Smidt Heart Institute, Cedars-Sinai Medical Center, Los Angeles, CA.

Purpose: Bleeding complications are well recognized phenomena following left ventricular assist device (LVAD) implantation and have been associated with increased morbidity and mortality. This complication and its consequences, however, remain undefined for total artificial heart (TAH) implantation.

Methods: A prospectively maintained database was used to identify all patients receiving TAH from January 2012 to May 2019 at a single institution. Read More

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http://dx.doi.org/10.1016/j.healun.2020.01.1083DOI Listing

Heart Transplant Outcomes for Patients with Cardiac Sarcoidosis.

J Heart Lung Transplant 2020 Apr 30;39(4S):S143. Epub 2020 Mar 30.

Cardiology, USC Keck School of Medicine, Los Angeles, CA.

Purpose: Cardiac sarcoidosis (CS) is a known etiology of both end-stage dilated and restrictive cardiomyopathy requiring heart transplantation. We sought to evaluate the long-term post-transplant outcomes of patients with CS compared to patients without CS.

Methods: We evaluated the United Network of Organ Sharing (UNOS) registry for all heart transplant (HT) recipients from 1987 to 2019. Read More

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http://dx.doi.org/10.1016/j.healun.2020.01.1064DOI Listing

HCV Seropositive Donors Positively Impact Heart Transplantation Rates and Waitlist Mortality without Compromising Outcomes.

J Heart Lung Transplant 2020 Apr 30;39(4S):S118-S119. Epub 2020 Mar 30.

Surgery, Department of Cardiovascular Surgery, University of Pennsylvania, Philadelphia, PA.

Purpose: Transplant demand continues to exceed supply. With advances in hepatitis C virus (HCV) treatment, HCV+ donors have recently been considered suitable for select recipients; here we investigate the effects of transplantation of HCV+ donors on waitlist mortality and transplant survival.

Methods: Retrospective review of the Organ Procurement and Transplantation Network database was performed across two time periods - HCV+ donor era (6/'17-6/'19) and control (1/'13-1/'15). Read More

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http://dx.doi.org/10.1016/j.healun.2020.01.999DOI Listing

BAG3 Myopathy Presenting With Prominent Neuropathic Phenotype and No Cardiac or Respiratory Involvement: A Case Report and Literature Review.

J Clin Neuromuscul Dis 2020 Jun;21(4):230-239

Division of Neurology, Department of Pediatrics, Arkansas Children's Hospital, University of Arkansas for Medical Sciences, Little Rock, AR.

Bcl-2-associated athanogene 3 (BAG3) myopathy is a rare myofibrillar myopathy characterized by toe walking and clumsiness in the first decade with rapid progression to cardiomyopathy and restrictive lung disease in the second decade. Most patients (18 patients) have the c.626C >T (p. Read More

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http://dx.doi.org/10.1097/CND.0000000000000300DOI Listing

Mutations in cause an autosomal-recessive form of hypertrophic cardiomyopathy.

Heart 2020 May 25. Epub 2020 May 25.

Cardiovascular Genetics, Health in Code, A Coruna, Spain.

Objective: Up to 50% of patients with hypertrophic cardiomyopathy (HCM) show no disease-causing variants in genetic studies. has been suggested as a candidate gene for the development of cardiomyopathies, although evidence for a causative role in HCM is limited. We sought to investigate the relationship between rare variants in and the development of HCM. Read More

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http://dx.doi.org/10.1136/heartjnl-2020-316913DOI Listing

[Value of left ventricular myocardial strain derived from cardiac magnetic resonance tissue tracking on differentiating constrictive pericarditis from restrictive cardiomyopathy].

Zhonghua Xin Xue Guan Bing Za Zhi 2020 May;48(5):386-392

Department of Cardiology, Beijing Anzhen Hospital, Capital Medical University, Beijing 100029, China.

To compare left ventricular myocardial mechanics detected by cardiac magnetic resonance tissue tracking(CMR-TT) between patients with constrictive pericarditis(CP) and restrictive cardiomyopathy(RCM),and see if those can be used to differentiate CP from RCM patients. A total of 23 patients with CP, 20 patients with RCM, who hospitalized in Beijing Anzhen Hospital from January 2014 to April 2019 were included in this study and 25 healthy subjects served as control group, all subjects underwent cardiac magnetic resonance examination. Myocardial mechanics were evaluated by 2-dimensional(2D) and 3-dimensional(3D) CMR-TT in terms of global longitudinal strain(GLS), circumferential strain(GCS), radial strain(GRS) and the lateral wall strain to septal wall strain ratio(lateral/septal ratio) of basal, mid-cavity and apical. Read More

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http://dx.doi.org/10.3760/cma.j.cn112148-20190906-00549DOI Listing

Genetics of inherited cardiomyopathies in Africa.

Cardiovasc Diagn Ther 2020 Apr;10(2):262-278

Cardiovascular Genetics Laboratory, Hatter Institute for Cardiovascular Research in Africa, Department of Medicine, Faculty of Health Sciences, University of Cape Town, Cape Town, South Africa.

In sub-Saharan Africa (SSA), the burden of noncommunicable diseases (NCDs) is rising disproportionately in comparison to the rest of the world, affecting urban, semi-urban and rural dwellers alike. NCDs are predicted to surpass infections like human immunodeficiency virus, tuberculosis and malaria as the leading cause of mortality in SSA over the next decade. Heart failure (HF) is the dominant form of cardiovascular disease (CVD), and a leading cause of NCD in SSA. Read More

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http://dx.doi.org/10.21037/cdt.2019.10.03DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7225421PMC

Restrictive mitral annuloplasty with or without coronary artery bypass grafting in ischemic mitral regurgitation.

ESC Heart Fail 2020 May 13. Epub 2020 May 13.

Department of Cardiovascular Surgery, Osaka University Graduate School of Medicine, 2-2-E1 Yamadaoka, Suita, Osaka, 565-0871, Japan.

Aims: In patients with ischaemic mitral regurgitation (MR), the impact of mitral valve surgery with concomitant coronary artery bypass grafting (CABG) on post-operative survival and left ventricular (LV) reverse remodelling remains unknown. Therefore, we investigated these outcomes following restrictive mitral annuloplasty (RMA) with and without CABG in those patients.

Methods And Results: This study included 309 patients with chronic MR and ischaemic cardiomyopathy for whom concomitant CABG was indicated (n = 225) or not indicated (n = 84) with RMA. Read More

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http://dx.doi.org/10.1002/ehf2.12705DOI Listing

Description of a large cohort of Caucasian patients with V122I ATTRv amyloidosis: Neurological and cardiological features.

J Peripher Nerv Syst 2020 May 12. Epub 2020 May 12.

Unit of Neurology and Neuromuscular Diseases, Department of Clinical and Experimental Medicine, University of Messina, Messina, Italy.

V122I is one of more than 130 mutations in transthyretin gene associated with hereditary TTR (ATTRv) amyloidosis. Main clinical expression is an infiltrative pseudohypertrophic cardiomyopathy with mild or no neurological symptoms. It is particularly common among African-Americans (prevalence: 3%-4%). Read More

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http://dx.doi.org/10.1111/jns.12385DOI Listing

Outcomes in Patients With Cardiac Amyloidosis Undergoing Heart Transplantation.

JACC Heart Fail 2020 Jun 6;8(6):461-468. Epub 2020 May 6.

Stanford Amyloid Center, Stanford University School of Medicine, Stanford, California. Electronic address:

Objectives: The purpose of this study is to report outcomes after heart transplantation in patients with cardiac amyloidosis based on a large single-center experience.

Background: Cardiac amyloidosis causes significant morbidity and mortality, often leading to restrictive cardiomyopathy, progressive heart failure, and death. Historically, heart transplantation outcomes have been worse in patients with cardiac amyloidosis compared with other heart failure populations, in part due to the systemic nature of the disease. Read More

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http://dx.doi.org/10.1016/j.jchf.2019.12.013DOI Listing

COVID-19 in a Young Girl with Restrictive Cardiomyopathy and Chronic Lung Disease.

Indian Pediatr 2020 Apr 30. Epub 2020 Apr 30.

Department of Pediatry, University of Health Sciences Kartal Kosuyolu Research and Training Hospital, Kartal/Istanbul.

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Disease-Specific Biomarkers in Transthyretin Cardiac Amyloidosis.

Curr Heart Fail Rep 2020 Jun;17(3):77-83

Division of Cardiology, Department of Internal Medicine, University of Texas Southwestern Medical Center, Dallas, TX, USA.

Purpose Of Review: Transthyretin amyloidosis is an increasingly recognized cause of restrictive cardiomyopathy related to amyloid fibril deposition in cardiac tissues. As treatment therapies have emerged for transthyretin amyloidosis (ATTR), so has interest in using biomarkers to identify disease prior to advanced presentation.

Recent Findings: Lower levels of transthyretin and retinol binding protein-4 have been demonstrated in patients with pathogenic mutations of transthyretin either with or without clinical disease. Read More

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http://dx.doi.org/10.1007/s11897-020-00457-zDOI Listing

Accessory left ventricular chamber in a cat: multimodality imaging description by cardiac magnetic resonance imaging and echocardiography.

J Vet Cardiol 2020 Apr 3;28:55-61. Epub 2020 Apr 3.

Division of Clinical Veterinary Sciences, The Royal (Dick) School of Veterinary Studies, University of Edinburgh, Edinburgh, UK.

A left ventricular accessory chamber is a complex and uncommon phenotype consisting in a subdivision of the left ventricle by a thick-walled muscle bundle or septum into two cavities. Multiple aetiologies such as congenital double-chambered left ventricle and acquired endomyocardial form of restrictive cardiomyopathy have been reported. The endomyocardial form of restrictive cardiomyopathy, owing to its structural heterogeneity, can present a similar phenotype to the congenital abnormality with intraventricular lesions bridging the ventricular septum and left ventricular free wall. Read More

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http://dx.doi.org/10.1016/j.jvc.2020.02.003DOI Listing

Advances in the Treatment of Cardiac Amyloidosis.

Curr Treat Options Oncol 2020 Apr 23;21(5):36. Epub 2020 Apr 23.

Department of Internal Medicine, Discipline of Cardiology, Faculty of Medical Science, State University of Campinas, Campinas, Brazil.

Opinion Statement: Cardiac amyloidosis is associated with a high mortality rate, a long delay between the first signs and the diagnosis but a short interval between diagnosis and death. This scenario has changed recently due to improved disease awareness among doctors and significant progress in diagnosis thanks to multimodal imaging and a multidisciplinary approach. Therefore, during the last few years, we have had access to specific therapies for those patients. Read More

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http://dx.doi.org/10.1007/s11864-020-00738-8DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7181421PMC

Sudden Cardiac Death in Children Affected by Cardiomyopathies: An Update on Risk Factors and Indications at Transvenous or Subcutaneous Implantable Defibrillators.

Front Pediatr 2020 3;8:139. Epub 2020 Apr 3.

Istituto Auxologico Italiano, IRCCS, Department of Cardiovascular, Neural and Metabolic Sciences, San Luca Hospital, Milan, Italy.

In the present paper, we will discuss the main cardiomyopathies affecting children with a specific focus on risk stratification and prevention of sudden cardiac death (SCD). We will discuss the main clinical features of hypertrophic cardiomyopathy (HCM), dilated and restrictive cardiomyopathies, left ventricular non-compaction (LVNC) and arrhythmogenic cardiomyopathy (AC), always highlighting their peculiarities in the pediatric age. Since sudden cardiac death may be the first manifestation of the disease, even in children, the identification of the specific underlying condition and of risk factors are pivotal to carry out the appropriate preventing strategies. Read More

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http://dx.doi.org/10.3389/fped.2020.00139DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7146705PMC

A rare case of familial restrictive cardiomyopathy, with mutations in MYH7 and ABCC9 genes.

Discoveries (Craiova) 2019 Sep 30;7(3):e99. Epub 2019 Sep 30.

Department of Cardiology, University of Medicine and Pharmacy of Craiova, Romania.

Restrictive cardiomyopathy is the least common type of cardiomyopathy, being defined by diastolic dysfunction and often unimpaired systolic function. Restrictive cardiomyopathies can be classified as familial or non-familial. Patients with familial restrictive cardiomyopathy can develop signs and symptoms of this condition anytime from childhood to adulthood. Read More

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http://dx.doi.org/10.15190/d.2019.12DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7086075PMC
September 2019

Incidentally detected cardiac amyloidosis on Tc-MDP bone scintigraphy.

Authors:
Ahmed Fathala

Radiol Case Rep 2020 Jun 7;15(6):705-708. Epub 2020 Apr 7.

Department of Radiology, King Faisal Specialist Hospital & Research Center, MBC#28 P.O. Box 3354, Riyadh, Saudi Arabia.

Cardiac amyloidosis (CA) is an important cause of restrictive cardiomyopathy and heart failure with preserved ejection fraction (HFpEF). At present, 3 bone-seeking tracers, Tc-diphosphono-1,2-propanodicarboxylic acid (Tc-DPD), Tc-pyrophosphate (Tc-PYP), and Tc-hydroxymethylene diphosphonate (Tc-HMDP), have been evaluated for detecting CA, but they are not widely available. In contrast, methylene diphosphate (MDP) is widely available. Read More

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http://dx.doi.org/10.1016/j.radcr.2020.03.010DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7139139PMC

Meta-analysis of cardiomyopathy-associated variants in troponin genes identifies loci and intragenic hot spots that are associated with worse clinical outcomes.

J Mol Cell Cardiol 2020 Apr 9;142:118-125. Epub 2020 Apr 9.

Department of Pediatrics, Section of Cardiology, Baylor College of Medicine, Houston, TX, United States; Department of Pediatrics, Division of Cardiology, Duke University School of Medicine, Durham, NC, United States. Electronic address:

Introduction: Troponin (TNN)-encoded cardiac troponins (Tn) are critical for sensing calcium and triggering myofilament contraction. TNN variants are associated with development of cardiomyopathy; however, recent advances in genetic analysis have identified rare population variants. It is unclear how certain variants are associated with disease while others are tolerated. Read More

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http://dx.doi.org/10.1016/j.yjmcc.2020.04.005DOI Listing

A Novel Case of Idiopathic Restrictive Cardiomyopathy.

Cureus 2020 Mar 8;12(3):e7212. Epub 2020 Mar 8.

Cardiology, University of Maryland Medical Center, Baltimore, USA.

Restrictive cardiomyopathy (CM) usually develops and progresses slowly, over a course of years. The rapid development of idiopathic restrictive CM immediately following a liver transplant is unusual. We describe the case of a patient who developed idiopathic restrictive CM fairly rapidly following a liver transplant. Read More

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http://dx.doi.org/10.7759/cureus.7212DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7138457PMC

ACVIM consensus statement guidelines for the classification, diagnosis, and management of cardiomyopathies in cats.

J Vet Intern Med 2020 May 3;34(3):1062-1077. Epub 2020 Apr 3.

Department of Medicine and Epidemiology, School of Veterinary Medicine, University of California Davis, Davis, California, USA.

Cardiomyopathies are a heterogeneous group of myocardial disorders of mostly unknown etiology, and they occur commonly in cats. In some cats, they are well-tolerated and are associated with normal life expectancy, but in other cats they can result in congestive heart failure, arterial thromboembolism or sudden death. Cardiomyopathy classification in cats can be challenging, and in this consensus statement we outline a classification system based on cardiac structure and function (phenotype). Read More

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http://dx.doi.org/10.1111/jvim.15745DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7255676PMC

Alpha-Loop for Permanent Pacemaker Implantation in Restrictive Cardiomyopathy.

J Invasive Cardiol 2020 Apr;32(4):E98

Postgraduate Institute of Medical Education & Research (PGIMER), Chandigarh-160012, India.

High-grade and complete heart block commonly occurs in adult patients with restrictive cardiomyopathy, and requires aggressive monitoring and prophylactic pacemaker/defibrillator. There are limited data on the procedural details of pacemaker implantation in this group of patients, and as reported, special maneuvers may be required for ventricular lead placement. Read More

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April 2020
0.824 Impact Factor

The Public Health Burden of Cardiomyopathies: Insights from a Nationwide Inpatient Study.

J Clin Med 2020 Mar 27;9(4). Epub 2020 Mar 27.

APHP, Service de Cardiologie, Centre de référence des maladies cardiaques héréditaires ou rares, Hôpital Ambroise Paré, 92100 Boulogne Billancourt, France.

Cardiomyopathies are responsible for heart failure and sudden cardiac death, but epidemiological data are scarce and the public health burden may be underestimated. We studied aggregating data from all public or private hospitals in France. Patients were categorized from relevant ICD-10 codes into dilated, hypertrophic, restrictive, or other cardiomyopathies (DCM, HCM, RCM, or OCM, respectively). Read More

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http://dx.doi.org/10.3390/jcm9040920DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7230913PMC

Infantile restrictive cardiomyopathy: cTnI-R170G/W impair the interplay of sarcomeric proteins and the integrity of thin filaments.

PLoS One 2020 17;15(3):e0229227. Epub 2020 Mar 17.

Department of Clinical Pharmacology and Molecular Cardiology, Ruhr-University of Bochum, Bochum, Germany.

TNNI3 encoding cTnI, the inhibitory subunit of the troponin complex, is the main target for mutations leading to restrictive cardiomyopathy (RCM). Here we investigate two cTnI-R170G/W amino acid replacements, identified in infantile RCM patients, which are located in the regulatory C-terminus of cTnI. The C-terminus is thought to modulate the function of the inhibitory region of cTnI. Read More

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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0229227PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7077804PMC

Mitral and tricuspid stenosis caused by light chain cardiac amyloid deposition.

ESC Heart Fail 2020 Jun 16;7(3):1130-1135. Epub 2020 Mar 16.

Department of Cardiovascular Medicine, Cleveland Clinic, Cleveland, OH, 44195, USA.

Cardiac amyloidosis results in an infiltrative restrictive cardiomyopathy, with a number of characteristic features: biventricular hypertrophy, abnormal myocardial global longitudinal strain with relative apical sparing, biatrial dilation, and small pericardial effusion along with conduction abnormalities. Amyloid deposits leading to hemodynamically significant valvular heart disease are very rare. We describe a rare case of concomitant moderately severe tricuspid and mitral valve stenosis because of ongoing amyloid deposition in a patient with progressive multiple myeloma and fat pad biopsy-proven light chain amyloidosis. Read More

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http://dx.doi.org/10.1002/ehf2.12668DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7261535PMC

Clinical exome sequencing revealed that FLNC variants contribute to the early diagnosis of cardiomyopathies in infant patients.

Transl Pediatr 2020 Feb;9(1):21-33

Center for Molecular Medicine, Children's Hospital of Fudan University, Shanghai 201102, China.

Background: FLNC encodes actin-binding protein and is mainly concentrated in skeletal and cardiac muscle. Mutations in were found in cardiomyopathies. To date, studies on FLNC-cardiomyopathies have mainly been reported in adults. Read More

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http://dx.doi.org/10.21037/tp.2019.12.02DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7036646PMC
February 2020

Amyloidosis as a Systemic Disease in Context.

Can J Cardiol 2020 Mar 17;36(3):396-407. Epub 2020 Jan 17.

Brigham and Women's Hospital Amyloidosis Program, Division of Cardiovascular Medicine Department of Medicine, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts, USA.

The systemic amyloidoses are a group of diseases characterized by the deposition of amyloid, a material formed from misfolding of proteins, in one or more organs. The 2 commonest forms of amyloidosis are transthyretin amyloidosis (ATTR), derived from wild-type or mutant transthyretin, and light-chain (AL) amyloidosis, derived from abnormal circulating light chains produced by plasma cell dyscrasia. Both frequently involve the heart, producing an infiltrative cardiomyopathy with restrictive pathophysiology. Read More

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http://dx.doi.org/10.1016/j.cjca.2019.12.033DOI Listing

Hiding in Plain Sight: Cardiac Amyloidosis, an Emerging Epidemic.

Can J Cardiol 2020 Mar 17;36(3):373-383. Epub 2019 Dec 17.

Division of Cardiology, Peter Munk Cardiac Centre, Toronto General Hospital, University Health Network, University of Toronto, Toronto, Ontario, Canada.

Amyloidosis is a term used to describe a group of rare heterogeneous diseases that ultimately result in the deposition and accumulation of misfolded proteins. These misfolded proteins, known as amyloids, are associated with a variety of precursor proteins that have amyloidogenic potential. Ultimately, the specific type of amyloidosis is dependent on multiple factors including genetic variability of precursor proteins and the tissue or organ in which the amyloid accumulates. Read More

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http://dx.doi.org/10.1016/j.cjca.2019.12.015DOI Listing

New insights provided by myofibril mechanics in inherited cardiomyopathies.

Cond Med 2019 Oct;2(5):213-224

National Heart Research Institute Singapore, National Heart Centre Singapore, Singapore.

Cardiomyopathies represent a heterogeneous group of cardiac disorders that perturb cardiac contraction and/or relaxation, and can result in arrhythmias, heart failure, and sudden cardiac death. Based on morphological and functional differences, cardiomyopathies have been classified into hypertrophic cardiomyopathy (HCM), dilated cardiomyopathy (DCM), and restrictive cardiomyopathy (RCM). It has been well documented that mutations in genes encoding sarcomeric proteins are associated with the onset of inherited cardiomyopathies. Read More

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7055865PMC
October 2019

[Clinical practice guidelines for hereditary cardiomyopathy].

Zhonghua Yi Xue Yi Chuan Xue Za Zhi 2020 Mar;37(3):300-307

The First Affiliated Hospital, SRRS Hospital, Zhejiang University School of Medicine, Hangzhou, Zhejiang 310058, China; Zhejiang DIAN Diagnostics Co., Ltd., Hangzhou, Zhejiang 310013, China; University of Rochester Medical Center, New York, NY 14642, USA.

Cardiomyopathies are a group of heterogeneous diseases which can be caused by various factors (often genetic) and can lead to heart failure, arrhythmia and sudden death. Primary cardiomyopathies includes hereditary hypertrophic cardiomyopathy, arrhythmogenic right ventricular cardiomyopathy, mitochondrial cardiomyopathy, mixed (hereditary and acquired) dilated cardiomyopathy and restrictive cardiomyopathy, left ventricular densification insufficiency, and other unclassified cardiomyopathies. With the help of genomic technology, common mutations in the population have been identified. Read More

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http://dx.doi.org/10.3760/cma.j.issn.1003-9406.2020.03.013DOI Listing

Variant R94C in -Encoded Troponin T Predisposes to Pediatric Restrictive Cardiomyopathy and Sudden Death Through Impaired Thin Filament Relaxation Resulting in Myocardial Diastolic Dysfunction.

J Am Heart Assoc 2020 Mar 26;9(5):e015111. Epub 2020 Feb 26.

Division of Paediatric Cardiology Department of Pediatrics Duke University School of Medicine Durham NC.

Background Pediatric-onset restrictive cardiomyopathy (RCM) is associated with high mortality, but underlying mechanisms of disease are under investigated. RCM-associated diastolic dysfunction secondary to variants in -encoded cardiac troponin T (TNNT2) is poorly described. Methods and Results Genetic analysis of a proband and kindred with RCM identified TNNT2-R94C, which cosegregated in a family with 2 generations of RCM, ventricular arrhythmias, and sudden death. Read More

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http://dx.doi.org/10.1161/JAHA.119.015111DOI Listing

A case report on transient global ventricular wall thickening secondary to acute myocarditis: Focus on the unique role of cardiac MRI.

Medicine (Baltimore) 2020 Feb;99(8):e19223

Department of Radiology, The Sir Run Run Shaw Hospital, Zhejiang University School of Medicine, Hangzhou, Zhejiang Province, China.

Introduction: Transient left ventricular wall thickening is known to develop in the acute phase of myocarditis, with several reports documenting this unusual mode of myocarditis. Diagnosing myocarditis can be challenging because symptoms, clinical exam findings, electrocardiogram results, biomarkers, and echocardiogram results are often non-specific. Therefore, cardiac magnetic resonance imaging has become the primary non-invasive imaging tool in patients with suspected myocarditis. Read More

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http://dx.doi.org/10.1097/MD.0000000000019223DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7034667PMC
February 2020

Hot hearts on bone scintigraphy are not all amyloidosis: hydroxychloroquine-induced restrictive cardiomyopathy.

Eur Heart J 2020 Feb 20. Epub 2020 Feb 20.

The Knight Cardiovascular Institute, Oregon Health and Science University, 3181 SW Sam Jackson Park Rd, Portland, OR 97239, USA.

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http://dx.doi.org/10.1093/eurheartj/ehaa091DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7197609PMC
February 2020

Paediatric cardiomyopathies: echocardiographic diagnosis, clinical profile, and demographic characteristics: the experience of a tertiary referral centre for Latin American paediatric cardiology.

Cardiol Young 2020 Apr 17;30(4):462-467. Epub 2020 Feb 17.

Division of Pediatric Cardiology, Fundación Cardioinfantil, Cardiology Institute, Bogotá, Colombia.

Background: Although multiple studies have been conducted in the adult population, there is a vast knowledge gap regarding the epidemiologic characteristics of cardiomyopathies in the paediatric population. This issue is even more crucial when the precarious situation of medical research in Latin America is considered. Given the potential impact that these disorders could have on Latin American health systems, a comprehensive epidemiologic study regarding the clinical profile and sociodemographic characteristics of these patients will influence the way we approach paediatric cardiomyopathies. Read More

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http://dx.doi.org/10.1017/S1047951120000281DOI Listing

Comprehensive review of pericardial diseases using different imaging modalities.

Int J Cardiovasc Imaging 2020 May 11;36(5):947-969. Epub 2020 Feb 11.

Department of Radiology, University of Washington Medical Center, Seattle, WA, USA.

Pericardial abnormalities are common cardiovascular disease entity, which are encountered in various clinical settings. Imaging plays an integral role in evaluation of pericardial abnormalities. The appropriate use of multiple imaging modalities is crucial to initiate the diagnosis and guide the referring providers to establish a management plan. Read More

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http://dx.doi.org/10.1007/s10554-020-01784-xDOI Listing

Diagnostic imaging of cardiac amyloidosis.

Nat Rev Cardiol 2020 Feb 10. Epub 2020 Feb 10.

National Amyloidosis Centre, Division of Medicine, University College London, Royal Free Hospital, London, UK.

Systemic amyloidosis encompasses a debilitating, under-diagnosed but increasingly recognized group of disorders characterized by the extracellular deposition of misfolded proteins in one or more organs. Cardiac amyloid deposition leads to an infiltrative or restrictive cardiomyopathy and is the major contributor to poor prognosis in patients with systemic amyloidosis. In total, >30 proteins can form amyloid fibrils, but the two main types of amyloid that can infiltrate the heart are monoclonal immunoglobulin light-chain amyloid and transthyretin amyloid. Read More

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http://dx.doi.org/10.1038/s41569-020-0334-7DOI Listing
February 2020

Iliocaval Venous Obstruction, Cardiac Preload Reserve and Exercise Limitation.

J Cardiovasc Transl Res 2020 Feb 10. Epub 2020 Feb 10.

School of Cardiovascular Medicine and Sciences, King's College London, London, UK.

Cardiac output during exercise increases by as much as fivefold in the untrained man, and by as much as eightfold in the elite athlete. Increasing venous return is a critical but much overlooked component of the physiological response to exercise. Cardiac disorders such as constrictive pericarditis, restrictive cardiomyopathy and pulmonary hypertension are recognised to impair preload and cause exercise limitation; however, the effects of peripheral venous obstruction on cardiac function have not been well described. Read More

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http://dx.doi.org/10.1007/s12265-020-09963-wDOI Listing
February 2020

Restrictive cardiac phenotype as primary cause of impaired aerobic capacity in Afro-Caribbean patients with val122ile variant transthyretin amyloid cardiomyopathy.

Amyloid 2020 Feb 5:1-8. Epub 2020 Feb 5.

Department of Cardiology CHU Martinique, University Hospital of Martinique, Fort de France, France.

Impaired aerobic capacity in cardiac amyloidosis patients may be related to limited inotropic myocardial reserve and heart rate (HR) response limiting cardiac output rise. This study sought to investigate whether chronotropic incompetence (CI) and blunted HR recovery would be prevalent in patients with mutant transthyretin (ATTRv) cardiomyopathy. Eighteen ATTRv (Val122Ile) patients (72 ± 8-year) and 15 age-matched controls (73 ± 3-year) were prospectively enrolled. Read More

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http://dx.doi.org/10.1080/13506129.2020.1722098DOI Listing
February 2020

Pathology of radiation induced heart disease.

Authors:
Ivo Šteiner

Rep Pract Oncol Radiother 2020 Mar-Apr;25(2):178-181. Epub 2019 Dec 17.

The Fingerland Department of Pathology, Charles University Faculty of Medicine and Faculty Hospital, Sokolska 581, 500 05 Hradec Kralove, Czech Republic.

The aim of this paper is dual - to review the relevant literature on pathology of radiation induced heart disease (RIHD), and to present an illustrative case of our own. Therapeutic ionizing radiation, such as that used in the treatment of Hodgkin´s lymphoma and cancers of left breast, lungs, esophagus, and thymus, can cause cardiac damage that may take several years to manifest. The spectrum of RIHD is broad and includes [1] pericarditis and pericardial effusion; [2] endocardial fibrosis and valvular dysfunction; [3] nonischemic myocardial fibrosis; [4] obstructive coronary artery disease with resultant myocardial ischemia; [5] damage to the great vessels; and [6] conduction system dysfunction. Read More

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http://dx.doi.org/10.1016/j.rpor.2019.12.015DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6994278PMC
December 2019

Novel Missense Variant in Gene Associated With Hypertrophic Cardiomyopathy Showing High Incidence of Restrictive Physiology.

Circ Genom Precis Med 2020 Apr 31;13(2):e002824. Epub 2020 Jan 31.

Department of Cardiac, Thoracic, Vascular Sciences and Public Health (R.V., C.B., A.A., P.M., G.T., D.C., S.I., C.C.), University of Padua, Italy.

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http://dx.doi.org/10.1161/CIRCGEN.119.002824DOI Listing

Comprehensive Assessment of Endomyocardial Fibrosis with Cardiac MRI: Morphology, Function, and Tissue Characterization.

Radiographics 2020 Mar-Apr;40(2):336-353. Epub 2020 Jan 31.

From Diagnósticos da America (DASA), Rio de Janeiro, Brazil (F.P.d.C., C.F.A.); Americas Serviços Médicos, Rio de Janeiro, Brazil (F.P.d.C.); and Division of Cardiology, Duke University Medical Center, Duke Medical Pavilion, 10 Medicine Circle, Room 1E63, DUMC 3934, Durham, NC 27710 (C.F.A.).

Endomyocardial fibrosis (EMF) affects approximately 12 million persons worldwide and is an important cause of restrictive cardiomyopathy in the developing world, with the highest prevalence reported in sub-Saharan Africa, South Asia, and South America. EMF is characterized by apical filling with fibrotic tissue of one or both ventricles, often associated with thrombus, calcification, and atrioventricular valve regurgitation, leading to typical symptoms of restrictive heart failure. Transthoracic echocardiography (TTE) is the first-line modality for assessment of EMF, basically owing to its widespread availability. Read More

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http://dx.doi.org/10.1148/rg.2020190148DOI Listing
January 2020