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ESC Heart Fail 2021 Jun 5. Epub 2021 Jun 5.

Department of Cardiology, Emergency Institute for Cardiovascular Diseases 'Prof. Dr. C. C. Iliescu', 3rd Cardiology Department, 258 Fundeni Street, Bucharest, 022328, Romania.

Cardiac amyloidosis is a restrictive cardiomyopathy determined by the accumulation of amyloid, which is represented by misfolded protein fragments in the cardiac extracellular space. The main classification of systemic amyloidosis is determined by the amyloid precursor proteins causing a very heterogeneous disease spectrum, but the main types of amyloidosis involving the heart are light chain (AL) and transthyretin amyloidosis (ATTR). AL, in which the amyloid precursor is represented by misfolded immunoglobulin light chains, can involve almost any system carrying the worst prognosis among amyloidosis patients. Read More

Cureus 2021 Apr 18;13(4):e14546. Epub 2021 Apr 18.

Department of Orthopedic Surgery, Nova Southeastern University Dr. Kiran C. Patel College of Osteopathic Medicine, Fort Lauderdale, USA.

Amyloidosis is a group of disorders that occurs due to the aggregation of insoluble and misfolded proteins in the extracellular space, eventually resulting in organ dysfunction. Type II amyloidosis is caused by the deposition of transthyretin (TTR), which will be the main focus of this article. Deposition of TTR in the myocardium results in a restrictive form of cardiomyopathy. Read More

Arch Cardiol Mex 2021 ;91(2):196-201

División de Cardiología, Fundación CardioInfantil IC, Universidad El Bosque, Facultad de Medicina y Ciencias de la salud.

Objective: Endomyocardial fibrosis (EF) is an unusual restrictive cardiomyopathy. In Latin America there are few reports. Here, we made a description of patients diagnosed with EF in Colombia. Read More

Medicina (Kaunas) 2021 Mar 18;57(3). Epub 2021 Mar 18.

Department of Cardiology, College of Medicine, The Catholic University of Korea, Seoul 06591, Korea.

Restrictive cardiomyopathy (RCM) is one of the rarest cardiac disorders, with a very poor prognosis, and heart transplantation is the only long-term treatment of choice. We reported that a Korean family presented different cardiomyopathies, such as idiopathic RCM and hypertrophic cardiomyopathy (HCM), caused by the same mutation in different individuals. A 74-year-old male was admitted for the evaluation of exertional dyspnea, palpitations, and pitting edema in both legs for several months. Read More

Radiol Cardiothorac Imaging 2020 Aug 13;2(4):e190158. Epub 2020 Aug 13.

Department of Magnetic Resonance Imaging (S.L., B.W., G.Y., S.Z., M.L.), Key Laboratory of Cardiovascular Imaging (Cultivation), Chinese Academy of Medical Sciences, Beijing, China (M.L., G.Y.); Department of Cardiology (L.S.), Department of Echocardiography (Y.J.), and Heart-Lung Testing Center (J.H.), State Key Laboratory of Cardiovascular Disease, Fuwai Hospital, National Center for Cardiovascular Diseases, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100037, China; and Department of Radiology, The Second Hospital of Hebei Medical University, Shijiazhuang, Hebei, China (B.W.).

Purpose: To investigate the MRI characteristics, prevalence, and outcomes of hypertrophic cardiomyopathy (HCM) with restrictive phenotype.

Materials And Methods: A total of 2592 consecutive patients with HCM were evaluated to identify individuals who fulfilled the diagnostic criteria of restrictive phenotype. Thirty-four patients with HCM (mean age, 41 years ± 16 [standard deviation]; range, 21-62 years, 16 men) with restrictive phenotype were retrospectively identified. Read More

Indian J Radiol Imaging 2020 Oct-Dec;30(4):465-472. Epub 2021 Jan 13.

Barnard Institute of Radiology, Madras Medical College, Chennai, Tamil Nadu, India.

Aims: Newer cardiac magnetic resonance techniques like native T1 mapping are being used increasingly as an adjunct to diagnose myocardial diseases with fibrosis. However, its full clinical utility has not been tested extensively, especially in the Indian population. The purpose of this study was to find native T1 values in healthy individuals without cardiac disease in our 3-Tesla MRI system and examine whether native myocardial T1 values can be used to differentiate between normal and diffuse myocardial disease groups. Read More

J Clin Ultrasound 2021 Jun 14;49(5):520-524. Epub 2021 Mar 14.

Cardiology Department, University of Health Sciences Turkey Istanbul Mehmet Akif Ersoy Thoracic and Cardiovascular Surgery Training and Research Hospital, Istanbul, Turkey.

Endomyocardial fibrosis (EMF), a restrictive cardiomyopathy characterized by subendocardial fibrosis, is commonly seen in tropical and subtropical regions. EMF involving the left ventricle presents with severe pulmonary hypertension (PH) and is a rare cause of PH in non-tropical areas. Multimodality imaging is important for accurate diagnosis, especially cardiac magnetic resonance imaging which is the cornerstone. Read More

Caspian J Intern Med 2021 ;12(1):107-110

Department of Cardiology, Cheng Hsin General Hospital, Taipei, Taiwan.

Background: Loeffler's endocarditis is a rare disease, caused by endocardial involvement of esosinophils, which damages the heart and leads to endomyocardial fibrosis with consequent restrictive cardiomyopathy, mural thrombi or valvular dysfunction. The association between Loeffler's endocarditis and Churg-Strauss syndrome (CSS) was also reported. Abnormal elevation of peripheral eosinophil counts in a heart failure patient is a hint of disease. Read More

J Am Coll Radiol 2021 May 27;18(5S):S83-S105. Epub 2021 Feb 27.

Specialty Chair, UT Southwestern Medical Center, Dallas, Texas.

Nonischemic cardiomyopathies encompass a broad spectrum of myocardial disorders with mechanical or electrical dysfunction without evidence of ischemia. There are five broad variants of nonischemic cardiomyopathies; hypertrophic cardiomyopathy (Variant 1), restrictive or infiltrative cardiomyopathy (Variant 2), dilated or unclassified cardiomyopathy (Variant 3), arrhythmogenic cardiomyopathy (Variant 4), and inflammatory cardiomyopathy (Variant 5). For variants 1, 3, and 4, resting transthoracic echocardiography, MRI heart function and morphology without and with contrast, and MRI heart function and morphology without contrast are the usually appropriate imaging modalities. Read More

Heart Fail Rev 2021 Jul 15;26(4):861-879. Epub 2021 Jan 15.

Department of Clinical Therapeutics, National and Kapodistrian University of Athens, Athens, Greece.

Cardiac amyloidosis (CA) is an infiltrative restrictive cardiomyopathy caused by accumulation in the heart interstitium of amyloid fibrils formed by misfolded proteins. Most common CA types are light chain amyloidosis (AL) caused by monoclonal immunoglobulin light chains and transthyretin amyloidosis (ATTR) caused by either mutated or wild-type transthyretin aggregates. Previously considered a rare disease, CA is increasingly recognized among patients who may be misdiagnosed as undifferentiated heart failure with preserved ejection fraction (HFPEF), paradoxical low-flow/low-gradient aortic stenosis, or otherwise unexplained left ventricular hypertrophy. Read More

Curr Probl Cardiol 2021 Apr 26;46(4):100784. Epub 2020 Dec 26.

Department of Cardiology and Heart Failure Unit, Sanatorio Güemes, Buenos Aires, Argentina.

Endomyocardiofibrosis was described first time in Uganda as an infrequent restrictive cardiomyopathy with a poor prognosis, characterized by fibrosis of the ventricular subendocardium and severe restrictive physiology leading to difficult therapeutic management and frequently associated with hypereosinophilic syndrome. Its higher prevalence in the tropics and its relationship in some cases with hypereosinophilic endocarditis has led to the search for genetic, infectious, autoimmune and nutritional causes, but its etiology remains unclear. It is a rare cardiomyopathy, difficult to diagnose and with a nonexistent effective treatment. Read More

Eur Heart J Cardiovasc Imaging 2021 Jun;22(7):781-789

Members of the European Reference Network on Heart Diseases (ERN GUARD-HEART), Coordinating Centre: Academic Medical Center, Amsterdam, the Netherlands.

Aims : Cardiac magnetic resonance (CMR) is recommended in the diagnosis of cardiomyopathies, but it is time-consuming, expensive, and limited in availability in some European regions. The aim of this study was to determine the use of CMR in cardiomyopathy patients enrolled into the European Society of Cardiology (ESC) cardiomyopathy registry [part of the EURObservational Research Programme (EORP)].

Methods And Results : Three thousand, two hundred, and eight consecutive adult patients (34. Read More

Circ Cardiovasc Imaging 2021 May 7;14(5):e012093. Epub 2021 Jan 7.

Heart Institute of the University of São Paulo (INCOR/FMUSP) (C.B.B.V.C., V.T.H.).

J Intern Med 2021 Jun 7;289(6):895-905. Epub 2021 Jan 7.

Department of Immunology, Genetics and Pathology, Uppsala University, Uppsala, Sweden.

Background: Wild-type transthyretin (ATTRwt) amyloidosis is the most common systemic amyloidosis in Western countries and manifests mainly as progressive restrictive cardiomyopathy.

Objective: To study the prevalence of ATTR deposits in ligament tissue in patients undergoing surgery for lumbar spinal stenosis and to assess whether these deposits are associated with cardiac amyloidosis.

Materials And Methods: A total of 250 patients, aged 50-89 (57% women), none with known cardiovascular disease, were included. Read More

Front Cardiovasc Med 2020 30;7:590557. Epub 2020 Oct 30.

Department of Cardiology, Blacktown Hospital, Sydney, NSW, Australia.

Cardiac amyloidosis (CA) is a unique disease entity involving an infiltrative process, typically resulting in a restrictive cardiomyopathy with diastolic heart failure that ultimately progresses to systolic heart failure. The two most common subtypes are light-chain and transthyretin amyloidosis. Early diagnosis of this disease entity, especially light-chain CA subtype, is crucial, as it portends a poorer prognosis. Read More

Am J Case Rep 2020 Nov 11;21:e926290. Epub 2020 Nov 11.

Department of Nephrology, General Hospital Zabok and Hospital of Croatian Veterans, Zabok, Croatia.

BACKGROUND Amyloidosis is a multisystem disease caused by deposition of dysfunctional protein-amyloid-in various organs. The heart is commonly involved, especially in primary (AL) and transthyretin (ATTR) amyloidosis. Most patients present with restrictive cardiomyopathy along with other systemic features of amyloid deposition. Read More

Int J Clin Pract 2021 Mar 9;75(3):e13756. Epub 2020 Nov 9.

Department of Dermatology, Venereology & Leprosy, Dr Rajendra Prasad Government Medical College, Himachal Pradesh, Kangra (Tanda), India.

Background: The relationship between psoriasis and cardiomyopathy is understudied in Indian patients.

Objective: We evaluated psoriasis patients for cardiomyopathy and other echocardiographic abnormalities.

Methods: About 98 (M:F = 67:31) patients with mild to moderate psoriasis aged 18-75 years (mean ± SD = 42. Read More

Open Heart 2020 10;7(2)

Cardiology, Rangueil University Hospital, Toulouse, France

Background: Cardiac amyloidosis (CA) is a life-threatening restrictive cardiomyopathy. Identifying patients with a poor prognosis is essential to ensure appropriate care. The aim of this study was to compare myocardial work (MW) indices with standard echocardiographic parameters in predicting mortality among patients with CA. Read More

J Am Coll Cardiol 2020 10;76(15):1723-1733

Centro Nacional de Investigaciones Cardiovasculares, Madrid, Spain; Icahn School of Medicine at Mount Sinai, New York, New York.

Background: Left ventricular (LV) hypertrabeculation fulfilling noncompaction cardiomyopathy criteria has been detected in athletes. However, the association between LV noncompaction (LVNC) phenotype and vigorous physical activity (VPA) in the general population is disputed.

Objectives: The aim of this study was to assess the relationship between LVNC phenotype on cardiac magnetic resonance (CMR) imaging and accelerometer-measured physical activity (PA) in a cohort of middle-aged nonathlete participants in the PESA (Progression of Early Subclinical Atherosclerosis) study. Read More

Cureus 2020 Aug 26;12(8):e10061. Epub 2020 Aug 26.

Cardiovascular Medicine, Cleveland Clinic Fairview Hospital, Cleveland, USA.

Loeffler's endocarditis is a rare form of restrictive cardiomyopathy associated with eosinophilia and endomyocardial fibrosis. It manifests most commonly as diastolic dysfunction or valvular abnormalities due to eosinophilic infiltration and degranulation. Herein, we chronicle a case of left ventricular involvement with Loeffler's endocarditis. Read More

Eur Heart J Case Rep 2020 Aug 28;4(4):1-6. Epub 2020 Jul 28.

Department of Cardiovascular Surgery, CHU Liege, Sart Tilman B35, 4000 Liege, Belgium.

Background: Constrictive pericarditis (CP) is a disease characterized by inflammation, progressive fibrosis, and thickening of the pericardium. Constrictive pericarditis after heart transplantation (HT) is a rare phenomenon, with a reported incidence of 1.4-3. Read More

Vnitr Lek 2020 ;66(3):44-49

Cardiac abnormalities associated with hypereosinophilia represent rare diseases and occurs most commonly due to hypersensitivity or allergic reactions, other possible etiologies cover infections, malignancy, vasculitis or hypereosinophilic syndromes. Three stages of cardiac involvement are usually described. Initially, myocardial inflammation occurs, that can continue with a thrombotic stage and eventually progress to the last irreversible stage called endomyocardial fibrosis, which represents one of the acquired forms of restrictive cardiomyopathy. Read More

Radiol Med 2020 Nov 24;125(11):1072-1086. Epub 2020 Sep 24.

Department of Surgical Sciences, Radiology Unit, University of Turin, Turin, Italy.

The restrictive cardiomyopathies constitute a heterogeneous group of myocardial diseases with a different pathogenesis and overlapping clinical presentations. Diagnosing them frequently poses a challenge. Echocardiography, electrocardiograms and laboratory tests may show non-specific changes. Read More

ESC Heart Fail 2020 Sep 17. Epub 2020 Sep 17.

Department of Cardiology and Structural Heart Disease, School of Medicine in Katowice, Medical University of Silesia, Katowice, Poland.

Aims: Cardiomyopathies are a heterogeneous group of disorders that increase the risk for atrial fibrillation (AF). The aim of the study is to assess the prevalence of AF, anticoagulation management, and risk of stroke/transient ischaemic attack (TIA) in patients with cardiomyopathy.

Methods And Results: Three thousand two hundred eight consecutive adult patients with cardiomyopathy (34. Read More

Radiol Case Rep 2020 Oct 26;15(10):2036-2040. Epub 2020 Aug 26.

Department of Cardiology, Royal Lancaster Infirmary, NHS, Ashton Road, Lancaster, LA1 4RP United Kingdom.

Hypereosinophilic cardiomyopathy is a rare restrictive cardiomyopathy which often presents with left-sided heart failure. We present an interesting case of a 58-year-old male patient with known hypereosinophillic syndrome who had presented with congestive cardiac failure with predominant features of right-sided volume overload. Cardiac magnetic resonance imaging confirmed the diagnosis by demonstrating obliteration of the right ventricular apex and endocardial-delayed gadolinium enhancement at the mid-septal and inferior segment, at both the right ventricular and left ventricular apical region, consistent with fibrosis. Read More

Int J Cardiol 2021 01 1;323:251-258. Epub 2020 Sep 1.

Department of Genetics, University of Groningen, University Medical Center Groningen, Groningen, the Netherlands.

Background - Variants within the alpha-tropomyosin gene (TPM1) cause dominantly inherited cardiomyopathies, including dilated (DCM), hypertrophic (HCM) and restrictive (RCM) cardiomyopathy. Here we investigated whether TPM1 variants observed in DCM and HCM patients affect cardiomyocyte physiology differently. Methods - We identified a large family with DCM carrying a recently identified TPM1 gene variant (T201M) and a child with RCM with compound heterozygote TPM1 variants (E62Q and M281T) whose family members carrying single variants show diastolic dysfunction and HCM. Read More

Int J Cardiol 2021 01 25;323:140-147. Epub 2020 Aug 25.

Cardiovascular Department, Azienda Sanitaria Universitaria Integrata Giuliano Isontina (ASUGI), University of Trieste, Via P. Valdoni 7, 34100 Trieste, Italy.

Background: Little is known about the role of different types of atrial fibrillation (AF) in dilated cardiomyopathy (DCM). We investigated the epidemiological and prognostic impact of different types of AF in DCM during long-term follow-up.

Method: We evaluated consecutive DCM patients enrolled in the Trieste Muscle Heart Disease Registry. Read More

Rev Med Interne 2020 Oct 18;41(10):673-683. Epub 2020 Aug 18.

Centre de Référence National des Amyloses Cardiaques et Réseau Amylose Mondor, Service de Cardiologie, CHU Henri Mondor, 51 Avenue du Maréchal de Lattre de Tassigny, 94010 Créteil, France.

Transthyretin (TTR) cardiac amyloidosis results from the dissociation of the tetrameric, liver-synthetized transport protein, either because of a mutation (hereditary CA), or spontaneously due to ageing (wild type CA). Monomers self-associate into amyloid fibrils within the myocardium, causing heart failure, arrhythmias and conduction defects. This overlooked disease must be recognized in case of unexplained increased thickness of the myocardium, particularly in subjects of African descent, in patients with heart failure and preserved ejection fraction, and in those with aortic stenosis. Read More

Am J Cardiol 2020 10 28;133:126-133. Epub 2020 Jul 28.

Department of Cardiology, Rabin Medical Center, Petah Tikva, Israel; Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.

Since the diagnosis of cardiac amyloidosis (CA) is often delayed, echocardiographic findings are frequently indicative of advanced cardiomyopathy. We aimed to describe early echocardiographic features in patients subsequently diagnosed with CA. Preamyloid diagnosis echocardiographic studies were screened for structural and functional parameters and stratified according to the pathogenetic subtype (immunoglobulin light-chain [AL] or amyloid transthyretin [ATTR]). Read More

Rev Cardiovasc Med 2020 Jun;21(2):181-190

Infiltrative Cardiac Disease Program, Division of Cardiology, Rush University Medical Center, Chicago, IL 60612, USA.

Heart failure with preserved ejection fraction is a very common clinical problem. Its prevalence is increasing with aging of the population. A diverse group of risk factors and etiologies comprise the HFpEF syndrome. Read More