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Eur J Heart Fail 2019 Apr 16. 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

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 Mar;12(3):e002388

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

Eur Heart J 2019 Mar 25. Epub 2019 Mar 25.

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

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 2019 Jan 16:1-9. Epub 2019 Jan 16.

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

Background: 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.

Methods: We performed a retrospective analysis of consecutive EMBs performed in a single, non-academic, tertiary-care centre. Read More

Circ Cardiovasc Imaging 2018 Nov;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

Open Heart 2018 26;5(2):e000881. Epub 2018 Sep 26.

Cardiology Department, Division of Internal Medicine, University of Texas MD Anderson Cancer Center, Houston, Texas, USA.

Cardiac amyloidosis (CA) describes the pathological process of amyloid protein deposition in the extracellular space of the myocardium. Unfortunately, the diagnosis of CA is often made late and when the disease process is advanced. However, advances in cardiovascular imaging have allowed for better prognostication and establishing diagnostic pathways with high sensitivity and specificity. Read More

Am J Case Rep 2018 Oct 8;19:1197-1203. Epub 2018 Oct 8.

Department of Cardiology, Osaka Medical College, Takatsuki, Osaka, Japan.

BACKGROUND Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare systemic vasculitis of unknown cause accompanied by prominent eosinophilia. Intracardiac thrombosis is one of the major cardiac complications in EGPA that may cause thromboembolism. CASE REPORT A 46-year-old male presenting with intermittent chest pain and numbness of the lower extremities was admitted to our center. Read More

Echocardiography 2018 11 24;35(11):1755-1763. Epub 2018 Sep 24.

Department of Cardiology, Dupuytren University Hospital, Limoges, France.

Background: Fabry cardiomyopathy (FC) and light-chain amyloid cardiomyopathy (AL) present with concentric left ventricular (LV) hypertrophy/remodeling and diastolic rather than systolic dysfunction. Direct comparisons are difficult due to rarity and confounded by variability of LV thickness.

Aims: To compare LV diastolic and systolic properties between patients with FC and AL in a cohort matched for interventricular septal thickness (IVS). Read More

Eur Radiol 2019 Mar 20;29(3):1555-1564. Epub 2018 Aug 20.

Dipartimento di Medicina Clinica e Sperimentale, University of Messina, Messina, Italy.

Objectives: We sought to evaluate the role of cardiac magnetic resonance imaging (CMR) in the evaluation of diastolic function by a combined assessment of left ventricular (LV) and left atrial (LA) function in a cohort of subjects with various degrees of diastolic dysfunction (DD) detected by echocardiography.

Methods: Forty patients with different stages of DD and 18 healthy controls underwent CMR. Short-axis cine steady-state free precession images covering the entire LA and LV were acquired. Read More

Eur J Heart Fail 2018 10 2;20(10):1417-1425. Epub 2018 Aug 2.

Cardiology, Department of Experimental, Diagnostic and Specialty Medicine, Alma Mater Studiorum, University of Bologna, Italy.

Aims: Cardiac amyloidosis remains a great challenge for the cardiologist. One of the three main aetiological forms, transthyretin-related hereditary amyloidosis (ATTRm), can present with several phenotypes, depending mainly on the specific mutation. We aimed to characterize the phenotype of patients with ATTRm due to Ile68Leu mutation, comparing them to patients with wild-type transthyretin amyloidosis (ATTRwt). Read More

J Card Fail 2018 Aug 17;24(8):504-511. Epub 2018 Aug 17.

Department of Medicine, Columbia University Medical Center, New York, New York. Electronic address:

Background: Transthyretin amyloidosis (ATTR) is often associated with cardiac involvement manifesting as conduction disease as well as restrictive cardiomyopathy causing heart failure and death. Myocardial contraction fraction (MCF), the ratio of left ventricular stroke volume (SV) to myocardial volume (MV), is a volumetric measure of myocardial shortening that is superior to ejection fraction (EF) in predicting mortality in patients with primary amyloid light chain cardiac amyloidosis. We hypothesized that MCF would be an independent predictor of survival in TTR-CA. Read More

Echocardiography 2018 10 13;35(10):1512-1518. Epub 2018 Jul 13.

Department of Cardiology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA.

Background: Previous studies have not evaluated the prevalence and specific risk factors for the development of left ventricular (LV) thrombus in patients with severely reduced left ventricular dysfunction due to chemotherapy-related cardiomyopathy. We sought to evaluate the prevalence and potential markers of LV thrombus in this patient population.

Methods: From January 2009 to December 2013, patients with chemotherapy-related severe LV dysfunction (LV ejection fraction [LVEF] ≤ 30%) identified from MD Anderson Cancer Center database were reviewed. Read More

Card Electrophysiol Clin 2018 06;10(2):413-429

Multimodality Cardiac Imaging Section, Policlinico San Donato, San Donato Milanese, Piazza Edmondo Malan, 2, 20097 San Donato Milanese MI, Italy.

Cardiovascular imaging has radically changed the management of patients with arrhythmogenic cardiomyopathies. This article focuses on the role of echocardiography and MRI in the diagnosis of these structural diseases. Cardiomyopathies with hypertrophic pattern (hypertrophic cardiomyopathy, restrictive cardiomyopathies, amyloidosis, Anderson-Fabry disease, and sarcoidosis), cardiomyopathies with dilated pattern, inflammatory cardiac diseases, and right ventricular arrhythmogenic cardiomyopathy are analyzed. Read More

Clin Res Cardiol 2018 Sep 16;107(9):845-857. Epub 2018 Apr 16.

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

Background: Left atrial decompression is considered in patients with symptomatic heart failure with preserved ejection fraction (HFpEF). We aimed to evaluate the feasibility and efficacy of transcatheter generation of a restrictive atrial septum communication to manage HFpEF from infancy to adulthood with cardiomyopathy and congenital heart defect.

Methods And Results: From June 2009 to December 2016, 24 patients (50% with an age less than 16 years) with HFpEF were palliated; NYHA-/Ross class IV (n = 10); median systemic ventricular ejection fraction 64 (range 35-78) %. Read More

J Ultrasound Med 2018 Nov 30;37(11):2637-2645. Epub 2018 Mar 30.

Department of Cardiovascular Ultrasound, First Hospital of China Medical University, Shenyang, China.

Objectives: The tissue motion of annular displacement provides an accurate and rapid assessment of left ventricular (LV) systolic function. However, it has rarely been used in patients with chronic constrictive pericarditis and restrictive cardiomyopathy. This study aimed to assess the differences in LV systolic function in patients with constrictive pericarditis and restrictive cardiomyopathy using tissue motion of annular displacement derived from speckle-tracking echocardiography. Read More

Medicine (Baltimore) 2018 Mar;97(11):e0079

Department of Cardiology, The First Hospital of Jilin University, Changchun, China.

Rationale: Hypereosinophilic syndrome (HES) is a rare disease characterized by hypereosinophilia and its ensuing organ damage. Cardiac involvement is divided into 3 chronological stages: an acute necrotic stage; a thrombus formation stage; and a fibrotic stage. Infiltration of the myocardium by eosinophilic cells followed by endomyocardial fibrosis is known as "Loeffler endocarditis. Read More

Eur Heart J Cardiovasc Imaging 2018 06;19(6):638

Department of Cardiology, CHU Saint-Pierre, Université Libre de Bruxelles (ULB), 322 rue Haute, Brussels B-1000, Belgium.

Radiol Med 2018 Jun 17;123(6):441-448. Epub 2018 Feb 17.

Department of Diagnostic and Therapeutic Services, IRCCS-ISMETT (Mediterranean Institute for Transplantation and Advanced Specialized Therapies), Palermo, Italy.

Purpose: Cerebral microbleeds (CMBs) are small rounded lesions representing cerebral hemosiderin deposits surrounded by macrophages that results from previous microhemorrhages. The aim of this study was to review the distribution of cerebral microbleeds in patients with end-stage organ failure and their association with specific end-stage organ failure risk factors.

Materials And Methods: Between August 2015 and June 2017, we evaluated 15 patients, 9 males, and 6 females, (mean age 65. Read More

Eur Heart J 2018 May;39(20):1784-1793

EURObservational Research Programme, European Society of Cardiology, 2035 Route des colles, CS 80179 Biot, 06903 Sophia-Antipolis Cedex, France.

Aims: The Cardiomyopathy Registry of the EURObservational Research Programme is a prospective, observational, and multinational registry of consecutive patients with four cardiomyopathy subtypes: hypertrophic cardiomyopathy (HCM), dilated cardiomyopathy (DCM), arrhythmogenic right ventricular cardiomyopathy (ARVC), and restrictive cardiomyopathy (RCM). We report the baseline characteristics and management of adults enrolled in the registry.

Methods And Results: A total of 3208 patients were enrolled by 69 centres in 18 countries [HCM (n = 1739); DCM (n = 1260); ARVC (n = 143); and RCM (n = 66)]. Read More

Postgrad Med J 2018 03 20;94(1109):185-186. Epub 2018 Jan 20.

Department of Medicine, Division of Cardiology, Bronx-Lebanon Hospital Center, Bronx, New York, USA.

Mol Genet Metab 2018 03 6;123(3):388-399. Epub 2018 Jan 6.

Department of Child Neurology, Justus Liebig University Giessen, 35392 Giessen, Germany.

Myofibrillary myopathies (MFM) are hereditary myopathies histologically characterized by degeneration of myofibrils and aggregation of proteins in striated muscle. Cardiomyopathy is common in MFM but the pathophysiological mechanisms are not well understood. The BAG3-Pro209Leu mutation is associated with early onset MFM and severe restrictive cardiomyopathy (RCM), often necessitating heart transplantation during childhood. Read More

J Heart Valve Dis 2017 07;26(4):447-455

Department of Cardiovascular Surgery, Osaka University Graduate School of Medicine, Suita City, Osaka, Japan.

Background And Aim Of The Study: The impact of adding papillary muscle approximation (PMA) to restrictive mitral annuloplasty (RMA) on postoperative left ventricular (LV) function is unknown. Changes in LV function parameters and clinical outcome were evaluated following RMA with and without PMA in patients with clinically relevant functional mitral regurgitation (FMR).

Methods: A total of 176 patients with advanced cardiomyopathy underwent RMA either with (n = 59) or without (n = 117) PMA. Read More

Radiol Res Pract 2017 15;2017:2874902. Epub 2017 Nov 15.

Department of Cardiology, Chatzikosta General Hospital, Ioannina, Greece.

Restrictive cardiomyopathy (RCM) is the least common among cardiomyopathies. It can be idiopathic, familial, or secondary to systematic disorders. Marked increase in left and/or right ventricular filling pressures causes symptoms and signs of congestive heart failure. Read More

Clin Nucl Med 2018 Mar;43(3):e89-e92

Cardiac amyloidosis is an important cause of restrictive cardiomyopathy and congestive heart failure. Bone scintigraphy with [Tc]hydroxymethylene diphosphonate (Tc-HDP) and [F]sodium fluoride (F-NaF) have been investigated in the noninvasive diagnosis of transthyretin (ATTR)-related cardiac amyloidosis. We present a case of a 76-year-old man with metastatic prostate cancer who underwent Tc-HDP bone scintigraphy with an incidental finding of diffuse left ventricular abnormal uptake suggesting ATTR cardiac amyloidosis. Read More

World J Pediatr Congenit Heart Surg 2018 05 27;9(3):360-363. Epub 2017 Feb 27.

2 Department of Paediatric Cardiac Surgery, Cardiopulmonary Transplant Unit, Freeman Hospital, Newcastle Upon Tyne NE7 7DN, United Kingdom.

Cardiomyopathy may have a variety of causes and may lead to significant morbidity. Often, there is no "perfect" treatment. New investigative techniques may add insight but retain the possibility of uncertainty. Read More

J Cardiovasc Echogr 2017 Oct-Dec;27(4):141-142

Non Invasive Cardiology Unit, Emergency Department, University Hospital of Ferrara, Ferrara, Italy.

Amyloidosis is a disease characterized by the extracellular deposition of the protein amyloid. It is a multiorgan disease, and cardiac involvement is not uncommon, generally in the form of a restrictive cardiomyopathy. Typical aspects of cardiac amyloidosis have been described at echocardiography and magnetic resonance imaging (MRI). Read More

Int J Cardiovasc Imaging 2018 Apr 25;34(4):597-605. Epub 2017 Oct 25.

Department of Cardiac MR, Fuwai Hospital, National Center for Cardiovascular Diseases of China, Chinese Academy of Medical Sciences and Peking Union Medical College, No. 167 Beilishi Road, Beijing, 100037, People's Republic of China.

End-stage phase of hypertrophic cardiomyopathy (ES-HCM) is a recognized part of HCM disease spectrum. Information on cardiac magnetic resonance (CMR) studies for ES-HCM especially for those without ventricular remodeling has been limited. We aimed to evaluate the morpho-functional and tissue features of ES-HCM with or without ventricular remodeling and to explore CMR prognostic value in these patients. Read More

Heart 2018 06 14;104(11):950-958. Epub 2017 Oct 14.

Transplant Unit, Papworth Hospital, Cambridge, UK.

ESC Heart Fail 2018 04 11;5(2):233-240. Epub 2017 Oct 11.

Section of Emergency and Cardiovascular Medicine, Department of Molecular and Clinical Medicine, Institute of Medicine, Sahlgrenska Academy, University of Gothenburg, Sahlgrenska University Hospital/Östra, Gothenburg, Sweden.

Aim: Hospital discharges with a diagnosis of cardiomyopathy have more than doubled in Sweden since 1987. We validated the cardiomyopathy diagnoses over this time period to investigate that the increase was real and not a result of improved recognition of the diagnosis and better diagnostic methods.

Methods And Results: Every fifth year from 1989 to 2009, records for all patients with a cardiomyopathy diagnosis were identified by searching the local registers in three hospitals in Västra Götaland, Sweden. Read More

Eur Heart J Cardiovasc Imaging 2017 11;18(11):1297

CHU de Bordeaux, Hôpital Cardiologique du Haut-Lévêque, Avenue de Magellan, 33604, Bordeaux-Pessac, France.

Circ Res 2017 Sep;121(7):855-873

From the Department of Pediatrics, Columbia University Medical Center, New York, NY (T.M.L., W.K.C., L.J.A.); Department of Pediatrics, Albert Einstein College of Medicine, The Children's Hospital at Montefiore, Bronx, NY (D.T.H., J.M.L.); Department of Pediatrics, Stollery Children's Hospital, University of Alberta, Edmonton, Canada (P.K.); Department of Pediatrics, The Heart Institute, Le Bonheur Children's Hospital, Memphis, TN (J.A.T.); Indiana University School of Medicine, Indianapolis (S.M.W.); Department of Cardiology, Boston Children's Hospital, MA (S.D.C.); Department of Pediatrics, Cincinnati Children's Hospital Medical Center, OH (J.L.J., E.M.M.); Department of Pediatrics, Children's Hospital of Philadelphia, PA (J.W.R.); Department of Pediatrics, Washington University School of Medicine, St. Louis, MO (C.D.C.); Department of Pediatrics, Primary Children's Hospital, Salt Lake City, UT (A.K.L.); Department of Pediatrics, Ann and Robert H. Lurie Children's Hospital, Chicago, IL (P.T.T.); and Department of Pediatrics, Wayne State University School of Medicine and Children's Hospital of Michigan, Detroit (J.D.C., H.R., A.H., S.E.L.).

Pediatric cardiomyopathies are rare diseases with an annual incidence of 1.1 to 1.5 per 100 000. Read More

Circ Res 2017 Sep;121(7):819-837

From the Division of Hematology (E.M., M.A.G.) and Department of Cardiovascular Medicine (L.A.B.), Mayo Clinic, Rochester, MN.

Restrictive cardiomyopathy (RCM) is characterized by nondilated left or right ventricle with diastolic dysfunction. The restrictive cardiomyopathies are a heterogenous group of myocardial diseases that vary according to pathogenesis, clinical presentation, diagnostic evaluation and criteria, treatment, and prognosis. In this review, an overview of RCMs will be presented followed by a detailed discussion on 3 major causes of RCM, for which tailored interventions are available: cardiac amyloidosis, cardiac sarcoidosis, and cardiac hemochromatosis. Read More

Cardiovasc J Afr 2017 Jul/Aug;28(4):208-214

Academy Teaching Hospital, Khartoum, Sudan.

Objective: Endomyocardial fibrosis (EMF) is a rare disease and is often an underdiagnosed and forgotten cardiomyopathy. The objective of this study was to document the current frequency of EMF in Sudan by defining and selecting cases from patients attending the echocardiography laboratory. Additionally we aimed to create an EMF registry for Sudan. Read More

Amyloid 2017 Dec 14;24(4):219-225. Epub 2017 Sep 14.

c Department of Cardiology , Indiana University School of Medicine , Indianapolis , IN , USA.

Objectives: Cardiomyopathy is a major cause of death in both the hereditary form of transthyretin (TTR) amyloidosis and the sporadic late-age-onset transthyretin amyloidosis (ATTR wild-type (ATTR)). Clinically disease progression from time of diagnosis to death is usually quoted as 5- to 15-years. In prior studies, significant progression of cardiac parameters in patients with moderate to severe cardiomyopathy has been noted within a 12-month time span. Read More

J Nucl Cardiol 2019 Feb 8;26(1):174-187. Epub 2017 Sep 8.

Department of Nuclear Medicine and Molecular Imaging (EB50), Medical Imaging Center, University Medical Center Groningen, University of Groningen, Hanzeplein 1, P.O. Box 30.001, 9700 RB, Groningen, The Netherlands.

Cardiac amyloidosis is a form of restrictive cardiomyopathy resulting in heart failure and potential risk on arrhythmia, due to amyloid infiltration of the nerve conduction system and the myocardial tissue. The prognosis in this progressive disease is poor, probably due the development of cardiac arrhythmias. Early detection of cardiac sympathetic innervation disturbances has become of major clinical interest, because its occurrence and severity limits the choice of treatment. Read More

Medicine (Baltimore) 2017 Sep;96(36):e7886

aDepartment of Cardiology, Asan Medical Center, University of Ulsan College of Medicine bDepartment of Internal Medicine, Seoul National University Hospital cDepartment of Internal Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul dDepartment of Cardiology, Translational Research Center on Aging, Chonnam National University Hospital, Gwangju eDepartment of Internal Medicine, Wonju College of Medicine,Yonsei University, Wonju fDepartment of Internal Medicine, Kangbuk Samsung Hospital, Seoul gDepartment of Internal Medicine, CHA Bundang Medical Center, CHA University school of Medicine, Bundang hDepartment of Cardiology, Sejong General Hospital iDepartment of Cardiovascular Medicine, Gachon University Gil Medical Center, Incheon jDepartment of Internal Medicine, Chungbuk National University School of Medicine, Cheongju, Korea.

Idiopathic restrictive cardiomyopathy (RCMP) has not been fully understood because this disease is difficult to diagnose. The present study aimed to assess the clinical profile and outcome of idiopathic RCMP from a multicenter cohort.This investigation is a retrospective study of consecutive patients with idiopathic RCMP at 10 centers in Korea between 1990 and 2010. Read More

Trends Cardiovasc Med 2018 01 13;28(1):10-21. Epub 2017 Jul 13.

Section of Cardiovascular Medicine, Department of Medicine, Boston University School of Medicine, Boston Medical Center, 88 East Newton Street, Boston, MA; Amyloidosis Center, Boston University School of Medicine, Boston Medical Center, Boston, MA; Department of Radiology, Boston University School of Medicine, Boston Medical Center, Boston, MA. Electronic address:

The amyloidoses are a group of systemic diseases characterized by organ deposition of misfolded protein fragments of diverse origins. The natural history of the disease, involvement of other organs, and treatment options vary significantly based on the protein of origin. In AL amyloidosis, amyloid protein is derived from immunoglobulin light chains, and most often involves the kidneys and the heart. Read More

Kardiol Pol 2017 ;75(7):723

Department of Cardiomyopathy, Institute of Cardiology, Warsaw, Poland, Poland.

Pacing Clin Electrophysiol 2017 Sep;40(9):986-994

Department of Cardiology, The First Affiliated Hospital of Harbin Medical University, Harbin, Heilongjiang, P. R. China.

Background: 20-30% of patients do not benefit from cardiac resynchronization therapy (CRT) when the established selection criteria were applied. We hypothesized that a combined assessment of mechanical dyssynchrony, myocardial deformation, and diastolic function would identify patients who would benefit most from CRT.

Method: In 36 CRT patients, clinical evaluation and echocardiography were performed before and after CRT. Read More

J Cardiovasc Med (Hagerstown) 2017 10;18(10):835-836

Cardiovascular Department, Azienda Sanitaria Universitaria Integrata of Trieste 'ASUITS', Trieste, Italy.

J Stroke Cerebrovasc Dis 2017 Aug 21;26(8):e172-e175. Epub 2017 Jun 21.

Neurovascular Imaging Research Core, University of California, Los Angeles, California.

Late-onset Pompe disease (LOPD) is an adult type of classical Pompe disease and presents without cardiomyopathy. Neuroimaging in LOPD is typically limited to posterior circulation and involves dilative arteriopathy, especially dolichoectasia and intracranial aneurysms. We report an interesting case of an established diagnosis of asymptomatic LOPD in a young man with a restrictive-variant pattern in posterior vasculature. Read More

Acta Cardiol 2017 Feb;72(1):9-18

a Centre Hospitalier Universitaire de Nancy, Service de Cardiologie, Institut Lorrain du Cœur et des Vaisseaux , Vandœuvre-lès-Nancy , France.

Hypereosinophilia may be due to several aetiologies. Cardiac complications are not uncommon, whatever the causes. Clinical presentations of hypereosinophilic cardiac diseases may vary widely from asymptomatic form to fatal necrotic myocarditis or irreversible restrictive cardiomyopathy. Read More

Turk Kardiyol Dern Ars 2017 Jun;45(4):358-361

Department of Cardiology, Ege University Faculty of Medicine, İzmir, Turkey.

Eosinophilic myocarditis (EM) is a rare condition that may result from several heterogeneous eosinophilic diseases, including parasite infection, hypersensitivity reaction, vasculitis, and hypereosinophilic syndrome. Regardless of etiology, the disease may present with various cardiac conditions, such as acute coronary syndrome, heart failure, or arrhythmia. Irreversible endomyocardial fibrosis, which causes restrictive cardiomyopathy, occurs in the late phase of the disease. Read More

Eur Heart J Cardiovasc Imaging 2017 Sep;18(9):1071

First Department of Internal Medicine, Nara Medical University, 840 Shijo, Kashihara, Nara 634-8522, Japan.

Rom J Morphol Embryol 2017 ;58(1):225-230

Department of Cardiology, "Prof. Dr. C. C. Iliescu" Institute of Emergency for Cardiovascular Diseases, "Carol Davila" University of Medicine and Pharmacy, Bucharest, Romania;

A 25-year-old woman with a five years history of syncope, mild left ventricular hypertrophy and moderately enlarged atria, was diagnosed with third degree atrioventricular heart block alternating with atrioventricular heart block 2:1, and received a dual chamber pacemaker. After three years of evolution, she developed atrial fibrillation, marked biatrial enlargement, severely depressed longitudinal myocardial velocities, associated with mild girdle weakness and slight increase in creatine kinase level. The diagnosis of restrictive cardiomyopathy with mild skeletal myopathy imposed the screening for a common etiology. Read More

Rom J Morphol Embryol 2017 ;58(1):201-206

Department of Ophthalmology, "Grigore T. Popa" University of Medicine and Pharmacy, Iasi, Romania;

Cardiac amyloidosis may occur in any type of systemic amyloidosis. The clinical picture is often characterized by restrictive cardiomyopathy. We report the case of a 41-year-old female patient admitted to the Department of Cardiology with clinical signs of right heart failure: congested jugular veins, hepatomegaly, peripheral edema, ascites associated with atrial fibrillation, low values of arterial blood pressure and oliguria. Read More

Eur Heart J Cardiovasc Imaging 2017 Oct;18(10):1090-1121

University Hospital Ramon y Cajal Carretera de Colmenar Km 9,100, 28034 Madrid, Spain.

Restrictive cardiomyopathies (RCMs) are a diverse group of myocardial diseases with a wide range of aetiologies, including familial, genetic and acquired diseases and ranging from very rare to relatively frequent cardiac disorders. In all these diseases, imaging techniques play a central role. Advanced imaging techniques provide important novel data on the diagnostic and prognostic assessment of RCMs. Read More