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      Fetal Noncompaction Cardiomyopathy and Histologic Diagnosis of Spongy Myocardium: Case Report and Review of the Literature

      case-report

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          Abstract

          Noncompaction cardiomyopathy (NCCM) and left ventricular noncompaction (LVNC), in their isolated form, are rare cardiomyopathies. They are characterized by a thickened myocardium due to the presence of deep trabeculae recesses, and to thick trabeculae. This condition is associated with a variable clinical phenotype including heart failure, thromboembolism, and sudden death. We report a case of LVNC at 26 weeks and 4 days of gestation revised on the basis of what is currently reported in the literature. A review of the literature was performed to better describe this rare condition. Left ventricular noncompaction is a rare fetal condition and it should be suspected in case of cardiomyopathy.

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          Most cited references18

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          Classification of the cardiomyopathies: a position statement from the European Society Of Cardiology Working Group on Myocardial and Pericardial Diseases.

          In biology, classification systems are used to promote understanding and systematic discussion through the use of logical groups and hierarchies. In clinical medicine, similar principles are used to standardise the nomenclature of disease. For more than three decades, heart muscle diseases have been classified into primary or idiopathic myocardial diseases (cardiomyopathies) and secondary disorders that have similar morphological appearances, but which are caused by an identifiable pathology such as coronary artery disease or myocardial infiltration (specific heart muscle diseases). In this document, The European Society of Cardiology Working Group on Myocardial and Pericardial Diseases presents an update of the existing classification scheme. The aim is to help clinicians look beyond generic diagnostic labels in order to reach more specific diagnoses.
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            Contemporary definitions and classification of the cardiomyopathies: an American Heart Association Scientific Statement from the Council on Clinical Cardiology, Heart Failure and Transplantation Committee; Quality of Care and Outcomes Research and Functional Genomics and Translational Biology Interdisciplinary Working Groups; and Council on Epidemiology and Prevention.

            Classifications of heart muscle diseases have proved to be exceedingly complex and in many respects contradictory. Indeed, the precise language used to describe these diseases is profoundly important. A new contemporary and rigorous classification of cardiomyopathies (with definitions) is proposed here. This reference document affords an important framework and measure of clarity to this heterogeneous group of diseases. Of particular note, the present classification scheme recognizes the rapid evolution of molecular genetics in cardiology, as well as the introduction of several recently described diseases, and is unique in that it incorporates ion channelopathies as a primary cardiomyopathy.
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              Echocardiographic and pathoanatomical characteristics of isolated left ventricular non-compaction: a step towards classification as a distinct cardiomyopathy.

              To determine clear cut echocardiographic criteria for isolated ventricular non-compaction (IVNC), a cardiomyopathy as yet "unclassified" by the World Health Organization. The disease is not widely known and its diagnosis mostly missed. In seven out of a series of 34 patients with IVNC the in vivo echocardiographic characteristics were validated against the anatomical examination of the heart removed after death in four and due to heart transplantation in three patients. Four morphological criteria diagnostic for IVNC were found. (1) Coexisting cardiac abnormalities were absent (by definition). (2) A two layer structure was seen, with a compacted thin epicardial band and a much thicker non-compacted endocardial layer of trabecular meshwork with deep endomyocardial spaces. A maximal end systolic ratio of non-compacted to compacted layers of > 2 is diagnostic. (3) The predominant localisation of the pathology was to mid-lateral (seven of seven patients), apical (six), and mid-inferior (seven) areas. The pathological preparations confirmed the echocardiographic findings. Concomitant regional hypokinesia was not confined to the non-compacted segments. (4) There was colour Doppler evidence of deep perfused intertrabecular recesses. Four clear cut echocardiographic diagnostic criteria were established. It is suggested that the WHO classification of cardiomyopathies be reconsidered to include IVNC as a distinct cardiomyopathy.
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                Author and article information

                Journal
                Rev Bras Ginecol Obstet
                Rev Bras Ginecol Obstet
                10.1055/s-00030576
                RBGO Gynecology & Obstetrics
                Thieme Revinter Publicações Ltda (Rio de Janeiro, Brazil )
                0100-7203
                1806-9339
                11 October 2018
                November 2018
                1 October 2018
                : 40
                : 11
                : 722-725
                Affiliations
                [1 ]Department of Medical and Surgical Sciences, Institute of Obstetrics and Gynecology, Università di Foggia, Foggia, Italy
                [2 ]Section of Obstetrics and Gynecology, Department of Morphology, Surgery and Experimental Medicine, Università degli Studi di Ferrara, Ferrara, Italy
                Author notes
                Address for correspondence Piergiorgio Iannone, MD Instituto di Ginecologia ed Obstetricia, Dipartimento Di Morfologia Chirurgia e Medicina Sperimentale, Università degli Studi di Ferrara, Azienda Ospedaliero-Universitaria S. Anna, Via Aldo Moro, 8. 44121, Cona, FerraraItaly pg.iannone88@ 123456gmail.com
                Article
                0199
                10.1055/s-0038-1673677
                10309442
                30308683
                b01fe563-d1c1-4290-a9b4-9a88d624dd03

                This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                : 21 June 2018
                : 09 August 2018
                Categories
                Case Report

                noncompaction cardiomyopathy,spongy myocardium,left ventricular noncompaction cardiomyopathy

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