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Left Ventricular Noncompaction Cardiomyopathy

2012; Lippincott Williams & Wilkins; Volume: 126; Issue: 16 Linguagem: Inglês

10.1161/circulationaha.112.095059

1524-4539

Markus Niemann, Stefan Störk, Frank Weidemann,

Williams Syndrome Research

HomeCirculationVol. 126, No. 16Left Ventricular Noncompaction Cardiomyopathy Free AccessBrief ReportPDF/EPUBAboutView PDFView EPUBSections ToolsAdd to favoritesDownload citationsTrack citationsPermissionsDownload Articles + Supplements ShareShare onFacebookTwitterLinked InMendeleyReddit Jump toSupplemental MaterialFree AccessBrief ReportPDF/EPUBLeft Ventricular Noncompaction CardiomyopathyAn Overdiagnosed Disease Markus Niemann, MD, Stefan Störk, MD, PhD and Frank Weidemann, MD Markus NiemannMarkus Niemann From the Department of Internal Medicine I and the Comprehensive Heart Failure Center, University of Würzburg, Würzburg, Germany. , Stefan StörkStefan Störk From the Department of Internal Medicine I and the Comprehensive Heart Failure Center, University of Würzburg, Würzburg, Germany. and Frank WeidemannFrank Weidemann From the Department of Internal Medicine I and the Comprehensive Heart Failure Center, University of Würzburg, Würzburg, Germany. Originally published16 Oct 2012https://doi.org/10.1161/CIRCULATIONAHA.112.095059Circulation. 2012;126:e240–e243Our echocardiography laboratory was consulted to determine whether a patient's echocardiogram would fulfill the criteria of left ventricular noncompaction cardiomyopathy (LVNC). Images of the left ventricle showed a 2-layer structure with a compacted, thin epicardial band and a much thicker noncompacted endocardial layer of trabecular meshwork with deep endomyocardial spaces. In addition, there was color Doppler evidence of deep perfusion of these intertrabecular recesses. The predominant localization of the pathology was lateral and apical, with a ratio of end-systolic noncompacted to compacted layers of 2.4. Thus, from an imaging point of view, everything seemed to fit the diagnosis of LVNC. However, comprehensive workup with medical information from the past revealed a different cause of cardiomyopathy.In 2010, the patient had already been referred to our hospital because of dyspnea. A transthoracic ECG revealed a markedly reduced ejection fraction of only 7%, with a dilated left ventricle. In addition, it showed prominent apical and anterior trabeculation, but none of the Jenni criteria1,2 for an LVNC were fulfilled (Figure 1 and Movie I in the online-only Data Supplement). ECG showed a left ventricular branch block with ST elevations in V1 through V5 (Figure 2). A coronary angiogram showed no coronary stenoses, but ascertained the markedly reduced ejection fraction. Cardiac magnetic resonance tomography confirmed the echocardiographic findings (Figure 3) and revealed a late enhancement pattern as usually seen in myocarditis, with late enhancement speckled and distributed over the left ventricle (Figure 4A and 4B). Last, left ventricular biopsies proved acute myocarditis (Figures 5 and 6). A subsequent ECG showed ST elevations only in V3 and V4, and they were not very prominent (Figure 7).Download figureDownload PowerPointFigure 1. Echocardiographic 4-chamber view of the patient's heart at baseline (taken from loop 1). Please note that no noncompaction can bee seen in the lateral wall.Download figureDownload PowerPointFigure 2. ECG at first presentation in 2010. Left bundle-branch block with subsequent ST elevations in V1 through V5 is seen, which fits a picture of myocarditis.Download figureDownload PowerPointFigure 3. MRI 4-chamber view of the patient's heart at baseline.Download figureDownload PowerPointFigure 4. Cardiac magnetic resonance images taken at baseline to demonstrate late enhancement, which was speckled and distributed over the left ventricle. (A) Late enhancement, anterior medial. (B) Late enhancement, inferior basal.Download figureDownload PowerPointFigure 5. Masson-Trichrome routine staining with small, speckled interstitial fibrosis and infiltration of mononuclear cells and inflammatory cells. Courtesy of Reinhard Kandolf, Institute of Pathology, University Tübingen, Germany.Download figureDownload PowerPointFigure 6. Immunohistochemistry: major histocompatability class II expression in activated macrophages, natural killer cells, and endothelium. Relatively fresh myocarditis with necrobiosis of myocytes is seen. Courtesy of Reinhard Kandolf, Institute of Pathology, University Tübingen, Germany.Download figureDownload PowerPointFigure 7. ECG at follow-up. ST elevations are seen only in V3 and V4, and they are not very prominent.In the recent (latest) echocardiogram, the ejection fraction had improved up to about 20%, but the echo pattern showed the previously described morphology with increased trabeculation, noncompaction-like layers, and intertrabecular recesses (Figure 8 and Movie II in the online-only Data Supplement).Download figureDownload PowerPointFigure 8. Echocardiographic 4-chamber view of the patient's heart at follow-up (taken from loop 2). Please note the noncompaction of the lateral wall.This case highlights several problems related to the diagnosis of LVNC. First, there is no clear genetic or laboratory test for the diagnosis of LVNC. Second, over time, some well-defined cardiac diseases like myocarditis can remodel toward an echocardiographic pattern compatible with LVNC, as documented in our patient. Last, medical information from the past is of key importance; the documented myocarditis in the history excludes the diagnosis of LVNC, according to Jenni's1,2 criterion number 1 (ie, absence of other cardiac abnormalities). Thus, the pure documentation of the typical echocardiographic criteria is insufficient to establish the diagnosis of LVNC. In summary, these factors facilitate overdiagnosis of LVNC, which is, in fact, a very rare disease.DisclosuresNone.FootnotesThe online-only Data Supplement is available with this article at http://circ.ahajournals.org/lookup/suppl/10.1161/CIRCULATIONAHA.112.095059/-/DC1.Correspondence to Frank Weidemann, MD, Medizinische Klinik und Poliklinik I, Zentrum für Innere Medizin, Oberdürrbacher Str. 6, 97080 Würzburg, Germany. E-mail [email protected]uni-wuerzburg.deReferences1. Jenni R, Oechslin E, Schneider J, Attenhofer Jost C, Kaufmann PA. Echocardiographic and pathoanatomical characteristics of isolated left ventricular non-compaction: a step towards classification as a distinct cardiomyopathy. Heart. 2001; 86: 666– 671.CrossrefMedlineGoogle Scholar2. Jenni R, Oechslin EN, van der Loo B. Isolated ventricular non-compaction of the myocardium in adults. Heart. 2007; 93: 11– 15.CrossrefMedlineGoogle Scholar Previous Back to top Next FiguresReferencesRelatedDetailsCited By Macaione F, Meloni A, Positano V, Barison A, Todiere G, Pistoia L, Di Lisi D, Novo G, Novo S and Pepe A (2021) The prognostic role of CMR using global planimetric criteria in patients with excessive left ventricular trabeculation, European Radiology, 10.1007/s00330-021-07875-0, 31:10, (7553-7565), Online publication date: 1-Oct-2021. Lorca R, Martín M, Pascual I, Astudillo A, Díaz Molina B, Cigarrán H, Cuesta-Llavona E, Avanzas P, Rodríguez Reguero J, Coto E, Morís C and Gómez J (2020) Characterization of Left Ventricular Non-Compaction Cardiomyopathy, Journal of Clinical Medicine, 10.3390/jcm9082524, 9:8, (2524) Masso A, Uribe C, Willerson J, Cheong B and Davis B (2020) Left Ventricular Noncompaction Detected by Cardiac Magnetic Resonance Screening: A Reexamination of Diagnostic Criteria, Texas Heart Institute Journal, 10.14503/THIJ-19-7157, 47:3, (183-193), Online publication date: 1-Jun-2020. Ribeiro S, Coelho L, Puentes K, Miltenberger-Miltenyi G, Faria B, Calvo L, Primo J, Sanfins V and Lourenço A (2019) Postmortem genetic testing: Clinical diagnosis is not ended by the patient's death, Revista Portuguesa de Cardiologia (English Edition), 10.1016/j.repce.2019.09.001, 38:7, (503-509), Online publication date: 1-Jul-2019. 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Zuccarino F, Vollmer I, Sanchez G, Navallas M, Pugliese F and Gayete A (2015) Left Ventricular Noncompaction: Imaging Findings and Diagnostic Criteria, American Journal of Roentgenology, 10.2214/AJR.13.12326, 204:5, (W519-W530), Online publication date: 1-May-2015. Napp L and Bauersachs J (2015) Non-Compaction-Kardiomyopathie DGIM Innere Medizin, 10.1007/978-3-642-54676-1_173-1, (1-16), . KATAYAMA H, KISHI K and OZAKI N (2014) Diagnosis of left ventricular noncompaction and its underlying issues左室心筋緻密化障害の診断とその問題点, Choonpa Igaku, 10.3179/jjmu.JJMU.R.10, 41:1, (3-16), . Captur G, Muthurangu V, Cook C, Flett A, Wilson R, Barison A, Sado D, Anderson S, McKenna W, Mohun T, Elliott P and Moon J (2013) Quantification of left ventricular trabeculae using fractal analysis, Journal of Cardiovascular Magnetic Resonance, 10.1186/1532-429X-15-36, 15:1, Online publication date: 1-Dec-2013. Quaife R, Salcedo E and Wolfel E (2013) Non-Compaction Cardiomyopathy: Underdiagnosed or Over Diagnosed?, Current Cardiovascular Imaging Reports, 10.1007/s12410-013-9227-z, 6:6, (498-506), Online publication date: 1-Dec-2013. Peters F, Khandheria B, Govender S, Patel A, dos Santos C, Matioda H and Essop M (2013) Human immunodeficiency virus and left ventricular noncompaction, International Journal of Cardiology, 10.1016/j.ijcard.2013.07.254, 168:5, (5099-5100), Online publication date: 1-Oct-2013. October 16, 2012Vol 126, Issue 16 Advertisement Article InformationMetrics © 2012 American Heart Association, Inc.https://doi.org/10.1161/CIRCULATIONAHA.112.095059PMID: 23071178 Originally publishedOctober 16, 2012 PDF download Advertisement SubjectsCardiomyopathyEchocardiographyRemodeling