Search Our Scientific Publications & Authors

Curr Radiopharm 2020 Jun 10. Epub 2020 Jun 10.

Department of Nuclear Medicine, Kantonsspital Baden, Baden. Switzerland.

Background: Cardiac amyloidosis is a progressive infiltrative disease for which new treatments are now available. As therapy should be started as early as possible to avoid complications such restrictive cardiomyopathy, arrhythmias and heart failure, a prompt and reliable diagnosis by means of non-invasive tests would be highly warranted. Electrocardiography, echocardiography and cardiac magnetic resonance imaging are all used in the evaluation of cardiac amyloidosis with varying diagnostic and prognostic accuracy, but none of these modalities can effectively differentiate the cardiac amyloid subtypes. Read More

Circulation 2020 Jul 1;142(1):e7-e22. 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

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

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

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

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

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

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

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

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

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

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

Nat Rev Cardiol 2020 Jul 10;17(7):413-426. 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

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

Q J Nucl Med Mol Imaging 2020 Mar 24;64(1):51-73. Epub 2020 Jan 24.

Department of Cardiovascular Medicine, Mayo Clinic, Rochester, MN, USA -

Infiltrative heart disease is an encompassing term referring to different pathological entities that involve infiltration of the myocardium by either abnormal substances or inflammatory cells. These infiltrates can impair cellular function, induce necrosis and fibrosis, or otherwise disrupt myocardial architecture resulting in a wide spectrum of structural and functional impairment. Depending on the specific disorder and stage of disease, patients may present with minimal cardiac abnormalities, or may have findings of advanced restrictive and/or dilated cardiomyopathy. Read More

Catheter Cardiovasc Interv 2020 May 6;95(6):1240-1248. Epub 2020 Jan 6.

Veterans Administration Long Beach Health Care System, University of California, Irvine, California.

Constrictive pericarditis (CP) and restrictive cardiomyopathy (RCM) are indolent disabling diseases of diastolic function. The two conditions share common pathophysiologic features, resulting in similar and overlapping clinical presentations, echocardiographic findings, and hemodynamic characteristics. However, their clinical course differs, as CP is surgically curable whereas RCM is a chronic condition managed medically. Read More

Ann Clin Lab Sci 2019 Nov;49(6):838-840

The Second Xiangya Hospital of Central South University, Changsha, China

BMC Cardiovasc Disord 2019 12 19;19(1):302. Epub 2019 Dec 19.

Department of Internal Medicine 3, University Hospital St. Poelten, Karl Landsteiner University of Health Sciences, Dunantplatz 1, 3100, St. Poelten, Austria.

Background: Endomyocardial fibrosis (EMF) represents the most common cause of restrictive cardiomyopathy worldwide. Despite a high prevalence in tropical regions, it occasionally occurs in patients who have never visited these areas. While researches have proposed various possible triggers for EMF, etiology and pathogenesis remain largely unknown. Read More

J Nucl Cardiol 2020 Jun 16;27(3):915-920. Epub 2019 Dec 16.

Department of Advanced Biomedical Sciences, University Federico II, Via Pansini 5, 80131, Naples, Italy.

Cardiac amyloidosis is a restrictive infiltrative cardiomyopathy burdened by high mortality. The two more common forms are immunoglobulin light-chain amyloidosis and transthyretin-related amyloidosis with different prognoses and treatments. However, distinguishing between them is challenging. Read More

Cesk Patol 2019 ;55(4):209-217

Myocardial diseases are often encountered in cardiology and pose a significant diagnostic challenge. Myocarditis is an acute inflammatory disease of the heart muscle. Pathophysiology of myocarditis is a complex interplay of genetic background, innate immunity, viral or bacterial agents and formation of autoreactive antibodies and lymphocytes that maintain the inflammation after the infection was eliminated. Read More

J Cardiovasc Magn Reson 2019 12 12;21(1):76. Epub 2019 Dec 12.

Department of Cardiology, Royal Prince Alfred Hospital, Sydney, NSW, Australia.

Background: The Task Force Criteria (TFC) for arrhythmogenic right ventricular cardiomyopathy (ARVC) was updated in 2010 to improve specificity. There was concern however that the revised cardiovascular magnetic resonance (CMR) criteria was too restrictive and not sensitive enough to detect early forms of the condition. We previously described patients with clinically suspected ARVC who satisfied criteria from non-imaging TFC categories and fulfilled parameters from the original but not the revised CMR criteria; as a result, these patients were not confirmed as definite ARVC but may represent an early phenotype. Read More

Curr Cardiol Rep 2019 12 3;21(12):164. Epub 2019 Dec 3.

Heart Transplant Program, Cardiac Amyloidosis Program, 8536 Wilshire Blvd, 3rd floor, Beverly Hills, CA, 90211, USA.

Purpose Of Review: While morbidity and mortality remain high for amyloid cardiomyopathy (AC), increased awareness, earlier diagnosis, and advances in treatment have improved patient outcomes. This review will discuss the pathophysiology, contemporary diagnostic strategies, and novel and investigational therapeutic strategies for light-chain (AL) and transthyretin (ATTR) AC.

Recent Findings: Diagnostic strategies for AC now include cardiac magnetic resonance imaging and bone scintigraphy. Read More

Neurology 2020 01 2;94(1):e107-e113. Epub 2019 Dec 2.

From the Department of Neurology (A.C.F., D.P., P.N.A., T.P.e.M., J.M.F.), Hospital de Santa Maria, Institute of Molecular Medicine, University of Lisboa; Department of Neurology (J.P.M., M.V.-B.), Hospital Egas Moniz; Department of Cardiology (T.G., F.J.P., A.G.A.), Hospital de Santa Maria, University of Lisboa; and Department of Neurology (N.I.), Hospital Beatriz Ângelo, Lisbon, Portugal.

Objective: To determine whether cardiac magnetic resonance imaging (CMR) could be useful in identifying previously undiagnosed cardiomyopathies in a cohort of patients with ischemic stroke who underwent standard etiologic investigation and to describe the type and frequency of these cardiomyopathies.

Methods: We performed a subanalysis of a previously collected prospective cohort of patients with ischemic stroke. Patients with structural changes on echocardiography that are considered causal for stroke in the Trial of Org 10172 in Acute Stroke Treatment (TOAST) classification were excluded. Read More

Curr Cardiol Rep 2019 11 22;21(11):148. Epub 2019 Nov 22.

Imperial College London, London, UK.

Purpose Of Review: This review aims at highlighting the need to better understand the pathogenesis and natural history of endomyocardial fibrosis when set against its changing endemicity and disease burden, improvements in diagnosis, and new options for clinical management.

Recent Findings: Progress in imaging diagnostic techniques and availability of new targets for drug and surgical treatment of heart failure are contributing to earlier diagnosis and may lead to improvement in patient survival. Endomyocardial fibrosis was first described in Uganda by Davies more than 70 years ago (1948). Read More

Medicine (Baltimore) 2019 Sep;98(38):e17256

Department of Cardiology, "St. Spiridon" Emergency Clinical Hospital.

Rationale: Cardiac amyloidosis, considered for the last years to be a rare disease, is one of the determinants of HFpEF. The non-specific clinical presentation and the difficulties related to endomyocardial biopsy have made cardiac amyloidosis an underdiagnosed clinical entity. Improvement of non-invasive diagnostic techniques and the development of new therapies increased clinical awareness for this form of restrictive cardiomyopathy. Read More

Int Heart J 2019 Sep 4;60(5):1211-1218. Epub 2019 Sep 4.

Department of Cardiology and Nephrology, Mie University Graduate School of Medicine.

Extra- and/or intracorporeal renal replacement therapy can improve the cardiorenal hemodynamics in patients with advanced heart failure (HF) refractory to medical therapy and renal failure. Here, we report the case of a 51-year-old woman with inotrope-dependent end-stage HF and chronic renal failure due to anthracycline-induced cardiomyopathy, in whom the induction of hemodiafiltration and subsequent chronic peritoneal dialysis (PD) provided a dramatic improvement of her cardiac hemodynamics from restrictive to almost normal physiology assessed by echocardiography and cardiac catheterization. The patient returned to office work with New York Heart Association functional class I-II symptoms for at least 3 years with continuous ambulatory PD after hospital discharge. Read More

Expert Rev Cardiovasc Ther 2019 Sep 3;17(9):673-681. Epub 2019 Sep 3.

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

: Cardiac amyloidosis is a disorder caused by the accumulation of abnormal protein products, amyloid, in the myocardium which subsequently impairs normal heart function. Heart failure with preserved ejection fraction has been increasingly attributed to amyloidosis and the resultant restrictive cardiomyopathy it creates. : Amyloid transthyretin (ATTR) is one of several identified amyloid products that have been pathologically implicated in cardiac amyloidosis through advanced diagnostics. Read More

Am J Cardiol 2019 09 25;124(6):932-938. Epub 2019 Jun 25.

Center for the Diagnosis and Treatment of Pericardial Diseases, Heart and Vascular Institute, Cardiovascular Section, Cleveland Clinic, Cleveland, Ohio. Electronic address:

Constrictive Pericarditis (CP) is a curable and reversible form of severe diastolic heart failure. We aimed to investigate the diagnostic accuracy of published echocardiographic Mayo Clinic Criteria in differentiating 107 patients with surgically proven CP from 30 patients with restrictive cardiomyopathy due to cardiac Amyloidosis. Five principal echocardiographic and Doppler variables were remeasured on preoperative transthoracic echocardiogram namely (1) respiration-related ventricular septal shift; (2) respiratory variation in mitral inflow E pulsed Doppler velocity; 3) tissue Doppler medial mitral annular e' velocity; (4) ratio of medial mitral annular e' to lateral mitral annular e' velocity; and 5) hepatic vein (HV) pulsed Doppler diastolic flow reversal ratio. Read More

J Am Heart Assoc 2019 08 23;8(15):e012531. Epub 2019 Jul 23.

DZHK (German Centre for Cardiovascular Research), partner site Berlin Berlin Germany.

Background Cardiomyopathies are heterogeneous diseases with clinical presentations varying from asymptomatic to life-threatening events, including severe heart failure and sudden cardiac death. The role of underlying genetic and disease-modulating factors in children and adolescents is relatively unknown. In this prospective study, in-depth phenotypic and genetic characterization of pediatric patients with primary cardiomyopathy and their first-degree family members (FMs) was performed. Read More

JACC Basic Transl Sci 2019 Jun 24;4(3):438-448. Epub 2019 Jun 24.

Division of Cardiology, Cardio-Oncology Center of Excellence, Washington University in St. Louis School of Medicine, Saint Louis, Missouri.

Cardiac amyloidosis is a restrictive cardiomyopathy that results from the deposition of misfolded light chain or transthyretin proteins, most commonly, in cardiac tissue. Traditionally, treatment options for light chain (AL) and transthyretin (ATTR) amyloidosis have been limited. However, there are now multiple novel therapeutics in development and several therapeutics recently approved that promise to revolutionize clinical management of AL and ATTR. Read More

BMC Cardiovasc Disord 2019 07 16;19(1):169. Epub 2019 Jul 16.

School of Biological Sciences, Georgia Institute of Technology, Atlanta, GA, USA.

Background: Anthracyclines are highly effective anticancer medication prescribed for the treatment of breast cancer. Nevertheless, the use of anthracyclines as chemotherapeutic agents involves a risk for development of cardiac toxicity which may cause restrictive and dilated cardiomyopathy. Currently, genetic predisposition is not considered as a risk factor for cardiotoxicity associated to the use of anthracyclines. Read More

Tex Heart Inst J 2019 Apr 1;46(2):136-138. Epub 2019 Apr 1.

Congenital complete heart block with concomitant biventricular noncompaction cardiomyopathy has been reported once previously. Although not universal, when restrictive physiology is present, impaired diastolic filling may pose a distinct challenge to pacing during the neonatal period. We present the case of a neonate with congenital complete heart block and biventricular noncompaction that resulted in severe diastolic dysfunction and atrioventricular dyssynchrony. Read More

Thromb J 2019 7;17. Epub 2019 Jun 7.

3Section of Cardiovascular Medicine, Yale New Haven Hospital, Yale School of Medicine, Dana Clinic Building, 3rd Floor, 789 Howard Avenue, New Haven, CT 06519 USA.

Background: Endocardial Fibroelastosis is diffuse, accentuated proliferation of ventricular endocardium causing a rare form of restrictive cardiomyopathy in both children and adults. It is an incompletely understood cause of heart failure predominantly in Sub-Saharan Africa associated with high morbidity and mortality. Atrial fibrillation and thrombus formation are common accompanying complications and portend a poor prognosis. Read More

Circ Cardiovasc Imaging 2019 06 30;12(6):e009012. Epub 2019 May 30.

Pediatric and Congenital Cardiac Unit (P.-E.S., Z.J., J.-B.T.), Bordeaux University Hospital, France.

Skelet Muscle 2019 05 27;9(1):14. Epub 2019 May 27.

Dipartimento di Medicina di Precisione, Università della Campania "Luigi Vanvitelli", Naples, Italy.

Background: Myopalladin (MYPN) is a component of the sarcomere that tethers nebulin in skeletal muscle and nebulette in cardiac muscle to alpha-actinin at the Z lines. Autosomal dominant MYPN mutations cause hypertrophic, dilated, or restrictive cardiomyopathy. Autosomal recessive MYPN mutations have been reported in only six families showing a mildly progressive nemaline or cap myopathy with cardiomyopathy in some patients. Read More

Ter Arkh 2019 May;91(4):99-106

I.M. Sechenov First Moscow State Medical University of the Ministry of Health of the Russian Federation (Sechenov University), Moscow, Russia.

Loeffler's endocarditis remains is a very rare disease, develops due to eosinophilic inflammation predominantly of the endocardium with an outcome in fibrosis and massive thrombus formation and. He is generally characterized by an unfavorable prognosis. Clinical case of a 42-year-old patient with Loeffler endocarditis is presented. Read More

J Am Soc Echocardiogr 2019 07 8;32(7):845-853.e1. Epub 2019 May 8.

Department of Cardiac Imaging, University Hospital Puerta de Hierro-Majadahonda, Majadahonda, Spain. Electronic address:

Background: Cardiac amyloidosis (CA) causes restrictive cardiomyopathy usually associated with a poor prognosis. Two subtypes predominate: systemic light-chain CA (ALCA) and transthyretin-derived CA (either wild type transthyretin amyloidosis [TTRwt] or mutant transthyretin amyloidosis [TTRm]). Left ventricular (LV) apical sparing has been extensively studied using speckle-tracking echocardiography for diagnosis, but the right ventricular (RV) deformation pattern has not been described. Read More

Interv Neuroradiol 2019 Oct 9;25(5):516-520. Epub 2019 May 9.

Neurorradiology Unit, University Hospital Reina Sofía, Córdoba, Spain.

Introduction: Strokes in children are characterised by a high mortality rate while, at the same time, the low number of cases makes it difficult to gain practical experience. As heart disease is the most common risk factor, and as more and more cardiological interventions are being carried out, an increase in the incidence of paediatric stroke is expected. In some cases a transplant is required. Read More

BMJ Case Rep 2019 Apr 20;12(4). Epub 2019 Apr 20.

Department of Cardiovascular Radiology and Endovascular Interventions, All India Institute of Medical Sciences, New Delhi, Delhi, India.

Loeffler endocarditis is an uncommon restrictive cardiomyopathy associated with eosinophilia and endomyocardial fibrosis causing diastolic restriction, predominantly involving the right ventricle. Cardiac MRI plays a crucial role in early detection of disease. Early disease usually responds well to corticosteroids. Read More

Eur J Heart Fail 2019 May 16;21(5):553-576. Epub 2019 Apr 16.

Berlin-Brandenburg Center for Regenerative Therapies, Deutsches Zentrum für Herz-Kreislauf-Forschung (DZHK) Berlin, Department of Cardiology, Campus Virchow Klinikum, Charite - Universitaetsmedizin Berlin, Berlin, Germany.

Cardiomyopathies are a heterogeneous group of heart muscle diseases and an important cause of heart failure (HF). Current knowledge on incidence, pathophysiology and natural history of HF in cardiomyopathies is limited, and distinct features of their therapeutic responses have not been systematically addressed. Therefore, this position paper focuses on epidemiology, pathophysiology, natural history and latest developments in treatment of HF in patients with dilated (DCM), hypertrophic (HCM) and restrictive (RCM) cardiomyopathies. Read More

World J Clin Cases 2019 Mar;7(6):742-752

Department of Cardiovascular Sciences, East Carolina Heart Institute, East Carolina University, Greenville, NC 27834, United States.

Background: Cardiac amyloidosis, a disease caused by the precipitation of amyloid proteins in the myocardial extracellular matrix has been historically difficult to diagnose due to lack of specific clinical manifestations and necessity of biopsy to demonstrate amyloid deposition. However, advances in cardiovascular imaging techniques have facilitated earlier recognition of this disease. In addition, while once thought of as incurable, treatment strategies are emerging for cardiac amyloidosis, making early diagnosis essential. Read More

J Vet Intern Med 2019 May 28;33(3):1222-1231. Epub 2019 Mar 28.

U955-IMRB, INSERM, École Nationale Vétérinaire d'Alfort, UPEC, Maisons-Alfort Cedex, France.

Background: Restrictive cardiomyopathy (RCM) is a common primary cardiomyopathy of cats. However, little information is available regarding prognostic variables in large populations of cats with RCM.

Objectives: To characterize the epidemiological, clinical, and echocardiographic features of cats with RCM and to document their survival times and risk factors for cardiac death (CD). Read More

Kyobu Geka 2019 Mar;72(3):184-189

Department of Cardiovascular Surgery, Kagoshima University Hospital, Kagoshima, Japan.

Functional mitral regurgitation( FMR) is a risk factor that increases the mortality rate and incidence of heart failure. Surgical intervention is important. Mitral valve (MV) reconstruction can preserve the valvular apparatus;no anticoagulation therapy is required, left ventricular function is preserved. Read More

Circ Genom Precis Med 2019 03;12(3):e002388

Cardiology Department, Hereditary Cardiopathies Unit (A.R.-S., J.P.-D., R.S.-B., F.A.-Y.).

Eur Heart J 2019 06;40(21):1740-1741

Department of Ultrasound, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, 1277 Jiefang Avenue, Wuhan, China.

Cardiol Young 2019 Mar 26;29(3):355-362. Epub 2019 Feb 26.

1Hessen Pediatric Heart Center,Justus Liebig University Clinic Giessen,Giessen,Germany.

Background: Left atrial congestion results from backward failure in dilated cardiomyopathy. We aimed to evaluate feasibility and efficacy of percutaneous atrioseptostomy to create a restrictive atrial septum defect in management of dilated cardiomyopathy.Methods and resultsFrom June 2009 to December 2016, 27 interventions comprised left atria decompressions in 22 dilated cardiomyopathy patients; 9 females; age: 24 days to 36. Read More

Rev Assoc Med Bras (1992) 2018 Sep;64(9):787-790

. Department of Cardiology, Hospital Distrital de Santarém, Santarém, Portugal.

Cardiac amyloidosis is an infiltrative cardiomyopathy, resulting from amyloid deposition within the myocardium. In primary systemic (AL-type) amyloidosis, the amyloid protein is composed of light chains resulting from plasma-cell dyscrasia, and cardiac involvement occurs in up to 50% of the patients We present a case of a 43-year-old man, with complaints of periodical swollen tongue and xerostomia, bleeding gums and haematuria for two months. His blood results showed normocytic anaemia, thrombocytopenia and a high spontaneous INR, therefore he was referred to the Internal Medicine clinic. Read More

Acta Cardiol 2020 Apr 16;75(2):138-146. Epub 2019 Jan 16.

Department of Cardiology, Ziekenhuis Oost-Limburg, Genk, Belgium.

Endomyocardial biopsies (EMBs) remain the golden standard to diagnose underlying pathophysiologic process in heart failure (HF), when potential therapeutic decisions cannot be made by non-invasive techniques. However, changes in the field of non-invasive diagnostic testing might have an impact on the need for performing an EMB in certain scenarios. We performed a retrospective analysis of consecutive EMBs performed in a single, non-academic, tertiary-care centre. Read More

Circ Cardiovasc Imaging 2018 11;11(11):e007878

Center for the Diagnosis and Treatment of Pericardial Diseases, Heart and Vascular Institute (B.X., V.M., A.K., P.C.C., A.L.K.), Cleveland Clinic, OH.

There is a need to review the multimodality imaging techniques, as well as the emerging role of the newer noninvasive imaging modalities in the field of constrictive pericarditis (CP). Therefore, the aim of this review is to summarize the current available techniques that are useful for the diagnosis and differentiation of CP from restrictive cardiomyopathy. Also, we provide illustrative images and videos of typical CP noninvasive imaging findings, as well as a diagnostic and management algorithm. Read More