Ebstein Anomaly Associated With Left Ventricular Noncompaction
2008; Lippincott Williams & Wilkins; Volume: 118; Issue: 16 Linguagem: Inglês
10.1161/circulationaha.108.767822
ISSN1524-4539
AutoresRodrigo Bagur, Mathieu Léderlin, Michel Montaudon, V. Latrabe, O. Corneloup, Xavier Iriart, François Laurent,
Tópico(s)Muscle Physiology and Disorders
ResumoHomeCirculationVol. 118, No. 16Ebstein Anomaly Associated With Left Ventricular Noncompaction Free AccessReview ArticlePDF/EPUBAboutView PDFView EPUBSections ToolsAdd to favoritesDownload citationsTrack citationsPermissionsDownload Articles + Supplements ShareShare onFacebookTwitterLinked InMendeleyReddit Jump toSupplemental MaterialFree AccessReview ArticlePDF/EPUBEbstein Anomaly Associated With Left Ventricular Noncompaction Rodrigo H. Bagur, Mathieu Lederlin, Michel Montaudon, Valérie Latrabe, Olivier Corneloup, Xavier Iriart and François Laurent Rodrigo H. BagurRodrigo H. Bagur From the Hospital Privado de Córdoba (R.H.B.), Córdoba, Argentina; Hôpital cardiologique du Haut-Lévèque (M.L., M.M., V.L., O.C., X.I., F.L.), Pessac, France; and Laboratoire de Physiologie Cellulaire Respiratoire, INSERM, E356 (F.L.), Bordeaux, France. , Mathieu LederlinMathieu Lederlin From the Hospital Privado de Córdoba (R.H.B.), Córdoba, Argentina; Hôpital cardiologique du Haut-Lévèque (M.L., M.M., V.L., O.C., X.I., F.L.), Pessac, France; and Laboratoire de Physiologie Cellulaire Respiratoire, INSERM, E356 (F.L.), Bordeaux, France. , Michel MontaudonMichel Montaudon From the Hospital Privado de Córdoba (R.H.B.), Córdoba, Argentina; Hôpital cardiologique du Haut-Lévèque (M.L., M.M., V.L., O.C., X.I., F.L.), Pessac, France; and Laboratoire de Physiologie Cellulaire Respiratoire, INSERM, E356 (F.L.), Bordeaux, France. , Valérie LatrabeValérie Latrabe From the Hospital Privado de Córdoba (R.H.B.), Córdoba, Argentina; Hôpital cardiologique du Haut-Lévèque (M.L., M.M., V.L., O.C., X.I., F.L.), Pessac, France; and Laboratoire de Physiologie Cellulaire Respiratoire, INSERM, E356 (F.L.), Bordeaux, France. , Olivier CorneloupOlivier Corneloup From the Hospital Privado de Córdoba (R.H.B.), Córdoba, Argentina; Hôpital cardiologique du Haut-Lévèque (M.L., M.M., V.L., O.C., X.I., F.L.), Pessac, France; and Laboratoire de Physiologie Cellulaire Respiratoire, INSERM, E356 (F.L.), Bordeaux, France. , Xavier IriartXavier Iriart From the Hospital Privado de Córdoba (R.H.B.), Córdoba, Argentina; Hôpital cardiologique du Haut-Lévèque (M.L., M.M., V.L., O.C., X.I., F.L.), Pessac, France; and Laboratoire de Physiologie Cellulaire Respiratoire, INSERM, E356 (F.L.), Bordeaux, France. and François LaurentFrançois Laurent From the Hospital Privado de Córdoba (R.H.B.), Córdoba, Argentina; Hôpital cardiologique du Haut-Lévèque (M.L., M.M., V.L., O.C., X.I., F.L.), Pessac, France; and Laboratoire de Physiologie Cellulaire Respiratoire, INSERM, E356 (F.L.), Bordeaux, France. Originally published14 Oct 2008https://doi.org/10.1161/CIRCULATIONAHA.108.767822Circulation. 2008;118:e662–e664A 56-year-old woman with gradually progressing exertional dyspnea was referred to our hospital. She had a known diagnosis of Ebstein anomaly and patent foramen ovale since childhood. One year previously, she had presented with a transient ischemic attack that was thought to be secondary to a paradoxal embolism through the patent foramen ovale, so an Amplatzer occluder had been implanted.Clinical examination revealed a normal sinus rhythm at 56 bpm and a New York Heart Association class II dyspnea without cyanosis. ECG (Figure 1) showed a first- degree atrioventricular block with a pattern of complete right bundle-branch block. Chest radiography (Figure 2) demonstrated mild cardiomegaly and clear lung fields. Two-dimensional echocardiography showed malposition of the anterior and septal tricuspid valve leaflets with grade 2 tricuspid regurgitation. The atrial septal occluder was in correct position without residual shunt. In addition, echocardiography revealed a small left ventricle with a noncompacted apical wall appearance that had not been depicted before. Download figureDownload PowerPointFigure 1. Twelve-lead ECG demonstrating a first-degree atrioventricular block with a complete right bundle-branch block.Download figureDownload PowerPointFigure 2. Frontal posterior–anterior chest x-ray showing cardiac enlargement with no other abnormality.In order to provide precise morphological and functional characteristics of this noncompacted left ventricle, the patient underwent cardiovascular magnetic resonance (CMR) imaging. Four-chamber (Figure 3 and online-only Data Supplement Movie I), apical short-axis (Figure 4 and online-only Data Supplement Movie II), and long-axis (Figure 5 and online-only Data Supplement Movie III) cine images were obtained using steady-state free precession (SSFP) cine sequences. Four-chamber and short-axis views confirmed downward displacement of the hinge points of the leaflets of the tricuspid valve and subsequent "atrialization" of the right ventricle with normal function (ejection fraction, 57%). CMR also demonstrated left ventricular apical noncompaction. On 4-chamber, apical short-axis, and long-axis views, the diastolic noncompacted to compacted (NC/C) ratios were 3.3, 3.4, and 3.5, respectively. However, left ventricular function was not significantly impaired (ejection fraction, 60%). Download figureDownload PowerPointFigure 3. Four-chamber SSFP CMR imaging shows marked apical displacement of the tricuspid valve leaflets (arrows) resulting in "atrialization" of the right ventricle. Amplatzer occluder appears as a large area of low signal intensity due to susceptibility effects (asterisk). Left ventricle demonstrates apical compacted and noncompacted walls (double arrows) with a NC/C ratio of 3.3. RA indicates right atrium; LA, left atrium; and RV, right ventricle.Download figureDownload PowerPointFigure 4. Apical short-axis SSFP CMR imaging shows a coarse and hypertrabeculated noncompacted left ventricle with a 3.4 NC/C ratio (double arrows). Arrow indicates tricuspid valve leaflets.Download figureDownload PowerPointFigure 5. Long-axis SSFP CMR imaging shows apical noncompacted left ventricle with a 3.5 NC/C ratio (double arrows).Previously known as "spongy left ventricular myocardium" or left ventricular hypertrabeculation, left ventricular noncompaction (LVNC) is a congenital cardiomyopathy thought to be caused by an arrest in normal embryogenesis. It is characterized by prominent myocardial trabeculations and deep intertrabecular recesses that lie in continuity with the left ventricular cavity.1 According to magnetic resonance imaging criteria,2 a NC/C ratio >2.3 in diastole distinguishes pathological noncompaction with a sensitivity of 86% and a specificity of 99%.The natural history of LVNC is variable, ranging from asymptomatic left ventricular dysfunction to severe congestive heart failure with complications such as arrhythmias and embolic events. LVNC may be an isolated finding or may be associated with other congenital cardiopathies such as complex cyanotic heart diseases and especially Ebstein anomaly.The pathology of the LV myocardium is observed in 39% of patients with Ebstein anomaly, and within that subgroup, LVNC might occur in 18% of patients.3 The pathophysiological mechanism that might explain this association remains unknown. However, it is expected that the genetic change that is responsible for Ebstein anomaly might play a role in the differentiation of the LV myocardium.Identification of LVNC in Ebstein anomaly has prognostic implications because it may be associated with LV systolic dysfunction. CMR appears to be the imaging technique of choice to precisely define structural and functional abnormalities of these 2 rare conditions, as well as to indicate the best follow-up care. Three-dimensional echocardiography might also offer new insights into the morphology and function of both diseases.4The online-only Data Supplement can be found with this article at http://circ.ahajournals.org/cgi/content/full/118/16/e•••/DC1.DisclosuresNone.FootnotesCorrespondence to Dr M. Lederlin, MD, Unité d'imagerie thoracique et cardiovasculaire, Hôpital Haut-Lévèque, avenue de Magellan, 33604 Pessac, France. E-mail [email protected]References1 Maron BJ, Towbin JA, Thiene G, Antzelevitch C, Corrado D, Arnett D, Moss AJ, Seidman CE, Young JB. 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. Circulation. 2006; 113: 1807–1816.LinkGoogle Scholar2 Petersen SE, Selvanayagam JB, Wiesmann F, Robson MD, Francis JM, Anderson RH, Watkins H, Neubauer S. Left ventricular non-compaction: insights from cardiovascular magnetic resonance imaging. J Am Coll Cardiol. 2005; 46: 101–105.CrossrefMedlineGoogle Scholar3 Attenhofer Jost CH, Connolly HM, O'Leary PW, Warnes CA, Tajik AJ, Seward JB. Left heart lesions in patients with Ebstein anomaly. 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Authors of the article cited in the comment will be invited to reply, as appropriate.Comments and feedback on AHA/ASA Scientific Statements and Guidelines should be directed to the AHA/ASA Manuscript Oversight Committee via its Correspondence page.Sign In to Submit a Response to This Article Previous Back to top Next FiguresReferencesRelatedDetailsCited By Jiménez K, Castillo L, Sandoval A, Aguilar H and Alcazar R (2023) Anomalía de Ebstein y miocardiopatía no compacta, un caso excepcional, South Florida Journal of Development, 10.46932/sfjdv4n1-039, 4:1, (546-550) Zhou Z, He W, Li X, Bai W, Huang W, Hou R, Wang Y and Guo Y (2022) Comparison of cardiovascular magnetic resonance characteristics and clinical prognosis in left ventricular noncompaction patients with and without arrhythmia, BMC Cardiovascular Disorders, 10.1186/s12872-022-02470-7, 22:1, Online publication date: 1-Dec-2022. 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Hoedemaekers Y, Caliskan K and Majoor-Krakauer D (2011) Noncompaction Cardiomyopathy Clinical Cardiogenetics, 10.1007/978-1-84996-471-5_6, (97-122), . O'Neill A, McDermott S, Ridge C, McDonald K, Keane D and Dodd J (2010) Uncharted waters: rare and unclassified cardiomyopathies characterized on cardiac magnetic resonance imaging, Insights into Imaging, 10.1007/s13244-010-0045-4, 1:5-6, (293-308), Online publication date: 1-Nov-2010. October 14, 2008Vol 118, Issue 16 Advertisement Article InformationMetrics https://doi.org/10.1161/CIRCULATIONAHA.108.767822PMID: 18852371 Originally publishedOctober 14, 2008 PDF download Advertisement SubjectsComputerized Tomography (CT)Developmental Biology
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