Portal de Periódicos da CAPES

Isolated Noncompaction of the Myocardium

2002; Lippincott Williams & Wilkins; Volume: 106; Issue: 6 Linguagem: Inglês

10.1161/01.cir.0000028961.66881.2d

1524-4539

Jane McCrohon, D. Richmond, Dudley J. Pennell, Raad Mohiaddin,

Neurogenetic and Muscular Disorders Research

HomeCirculationVol. 106, No. 6Isolated Noncompaction of the Myocardium Free AccessReview ArticlePDF/EPUBAboutView PDFView EPUBSections ToolsAdd to favoritesDownload citationsTrack citationsPermissions ShareShare onFacebookTwitterLinked InMendeleyReddit Jump toFree AccessReview ArticlePDF/EPUBIsolated Noncompaction of the MyocardiumA Rarity or Missed Diagnosis? J. A. McCrohon, FRACP, PhD, D. R. Richmond, MBChB, MSc, FRACP, D. J. Pennell, FRCP, MD and R. H. Mohiaddin, FRCR, PhD J. A. McCrohonJ. A. McCrohon From the Department of Cardiovascular Magnetic Resonance, Royal Brompton Hospital, London, UK (J.A.M., D.J.P., R.H.M.); and Department of Cardiology, St George Hospital (J.A.M.), and Royal Prince Alfred Hospital (D.R.R.), Sydney, Australia. , D. R. RichmondD. R. Richmond From the Department of Cardiovascular Magnetic Resonance, Royal Brompton Hospital, London, UK (J.A.M., D.J.P., R.H.M.); and Department of Cardiology, St George Hospital (J.A.M.), and Royal Prince Alfred Hospital (D.R.R.), Sydney, Australia. , D. J. PennellD. J. Pennell From the Department of Cardiovascular Magnetic Resonance, Royal Brompton Hospital, London, UK (J.A.M., D.J.P., R.H.M.); and Department of Cardiology, St George Hospital (J.A.M.), and Royal Prince Alfred Hospital (D.R.R.), Sydney, Australia. and R. H. MohiaddinR. H. Mohiaddin From the Department of Cardiovascular Magnetic Resonance, Royal Brompton Hospital, London, UK (J.A.M., D.J.P., R.H.M.); and Department of Cardiology, St George Hospital (J.A.M.), and Royal Prince Alfred Hospital (D.R.R.), Sydney, Australia. Originally published6 Aug 2002https://doi.org/10.1161/01.CIR.0000028961.66881.2DCirculation. 2002;106:e22–e23A 31-year-old man presented to the emergency department with central chest tightness, shortness of breath, and presyncope. His admission ECG was abnormal (Figure 1), with inferior and lateral Q waves, left ventricular hypertrophy, repolarization changes in leads II, III, AVF, and V4 to V6, and nonspecific ST elevation in V1 to V3. A physical examination was unremarkable except for a fourth heart sound. Baseline blood studies showed mildly elevated troponin and creatine kinase-MB levels and hypercholesterolemia. A cardiac ultrasound showed the upper limit of normal wall thickness and normal valvular flows, biventricular size, and function. Cardiac catheterization revealed a mildly abnormal contraction of the anterobasal wall of the left ventricle and normal epicardial coronary vessels. Cardiac magnetic resonance imaging was performed to help exclude myocardial/pericardial disease. Download figureDownload PowerPointFigure 1. ECG showing left ventricular hypertrophy and T-wave abnormalities.Magnetic resonance documented intramyocardial recesses of the inferior and anterobasal left ventricular (LV) wall. These recesses were in communication with the LV lumen (Figure 2, A and B). In addition, prominent trabeculation extended into the LV cavity (Figure 3). Ventricular mass, size, and systolic function were normal. There was no evidence of myocardial hyperenhancement after gadolinium injection. Serology was normal. Download figureDownload PowerPointFigure 2. T1-weighted turbo spin echo (A) and true fast imaging with steady-state free precession (true FISP) diastolic cine image (B) in the vertical long axis of the left ventricle, showing multiple inferior wall intertrabecular recesses in communication with the LV cavity (arrows).Download figureDownload PowerPointFigure 3. Short-axis true FISP cine image apical to the papillary muscles showing unusual muscle trabeculations extending into the LV cavity.These appearances are consistent with myocardial noncompaction, a congenital disorder of endomyocardial embryogenesis. This example is less florid than cases detailed in the limited number of echo and pathology series currently published and, in fact, would have been missed by ultrasound criteria. A diagnosis of noncompaction has important implications because of the need for familial screening and the possible association with other cardiac anomalies and/or muscle disorders, progressive LV dysfunction, risk of systemic embolism, and life-threatening arrhythmias. Techniques such as magnetic resonance imaging may improve detection rates and provide new insights into the prevalence, spectrum, and natural course of this potentially not-so-rare condition.The editor of Images in Cardiovascular Medicine is Hugh A. McAllister, Jr, MD, Chief, Department of Pathology, St Luke's Episcopal Hospital and Texas Heart Institute, and Clinical Professor of Pathology, University of Texas Medical School and Baylor College of Medicine.Circulation encourages readers to submit cardiovascular images to the Circulation Editorial Office, St Luke's Episcopal Hospital/Texas Heart Institute, 6720 Bertner Ave, MC1-267, Houston, TX 77030.This work was supported by Coronary Artery Disease Research Association (CORDA; Marian Silcock Fellowship).FootnotesCorrespondence to Dr Jane McCrohon, Department of Cardiology, St George Hospital, Gray St, Kogarah, Sydney, 2217, Australia. E-mail [email protected] Previous Back to top Next FiguresReferencesRelatedDetailsCited By Mavrogeni S, Markousis-Mavrogenis G, Vartela V, Manolopoulou D, Abate E, Hamadanchi A, Rigopoulos A, Kolovou G and Noutsias M (2019) The pivotal role of cardiovascular imaging in the identification and risk stratification of non-compaction cardiomyopathy patients, Heart Failure Reviews, 10.1007/s10741-019-09898-8, 25:6, (1007-1015), Online publication date: 1-Nov-2020. 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. LEE W, KUCHAR D, WALKER B and SUBBIAH R (2012) Isolated Ventricular Noncompaction: Implications for Mechanisms of Sudden Cardiac Death, Pacing and Clinical Electrophysiology, 10.1111/j.1540-8159.2012.03344.x, 36:5, (e153-e155), Online publication date: 1-May-2013. Finsterer J, Stöllberger C, Blazek G and Sehnal E (2013) Familal left ventricular hypertrabeculation (noncompaction) is myopathic, International Journal of Cardiology, 10.1016/j.ijcard.2011.07.012, 164:3, (312-317), Online publication date: 1-Apr-2013. Madan S, Mandal S, Bost J, Mishra M, Bailey A, Willaman D, Jonnalagadda P, Pisapati K and Tadros S (2011) Noncompaction Cardiomyopathy in Children With Congenital Heart Disease: Evaluation Using Cardiovascular Magnetic Resonance Imaging, Pediatric Cardiology, 10.1007/s00246-011-0111-9, 33:2, (215-221), Online publication date: 1-Feb-2012. Bogaert J and Taylor A (2011) Heart Muscle Diseases Clinical Cardiac MRI, 10.1007/174_2011_358, (275-353), . Erol C, Koplay M, Olcay A, Kivrak A, Ozbek S, Seker M and Paksoy Y (2012) Congenital left ventricular wall abnormalities in adults detected by gated cardiac multidetector computed tomography: Clefts, aneurysms, diverticula and terminology problems, European Journal of Radiology, 10.1016/j.ejrad.2012.03.025, 81:11, (3276-3281), Online publication date: 1-Nov-2012. Wein M, Wolf-Puetz A, Niehues R, Klein T, Kilner P and Klein R (2011) Multiple left ventricular inferoseptal cleftsMultiple linksventrikuläre inferoseptale Clefts, Herz, 10.1007/s00059-011-3424-9, 36:5, (438-443), Online publication date: 1-Aug-2011. Slavich M, Florian A and Bogaert J (2011) The emerging role of magnetic resonance imaging and multidetector computed tomography in the diagnosis of dilated cardiomyopathy, Insights into Imaging, 10.1007/s13244-011-0101-8, 2:4, (453-469), Online publication date: 1-Aug-2011. Captur G and Nihoyannopoulos P (2010) Left ventricular non-compaction: Genetic heterogeneity, diagnosis and clinical course, International Journal of Cardiology, 10.1016/j.ijcard.2009.07.003, 140:2, (145-153), Online publication date: 1-Apr-2010. Kaski J and Elliott P (2010) Cardiomyopathies Paediatric Cardiology, 10.1016/B978-0-7020-3064-2.00052-7, (1003-1034), . Sarma R, Chana A and Elkayam U (2010) Left Ventricular Noncompaction, Progress in Cardiovascular Diseases, 10.1016/j.pcad.2009.11.001, 52:4, (264-273), Online publication date: 1-Jan-2010. Fazio G, Corrado G, Novo G, Zachara E, Rapezzi C, Sulafa A, Sutera L, D'angelo L, Visconti C, Stollberger C, Sormani L, Finsterer J, Cavusoglu Y, Di Gesaro G, Grassedonio E, Ferrara F, Galia M, Midiri M, Pipitone S, Carerj S and Novo S (2010) Ventricular dysfunction and number of non compacted segments in non compaction: Non-independent predictors, International Journal of Cardiology, 10.1016/j.ijcard.2008.11.199, 141:3, (250-253), Online publication date: 1-Jun-2010. Varadarajan P, Pai R, Nayak K, Kim H and Pohost G (2010) Cardiovascular Magnetic Resonance: Evaluation of Myocardial Function, Perfusion, and Viability Cardiac CT, PET and MR, 10.1002/9781444323894.ch8, (196-245) Fazio G, Novo G, D'angelo L, Visconti C, Sutera L, Grassedonio E, Galia M, Ferrara F, Midiri M and Novo S (2010) Magnetic resonance in isolated noncompaction of the ventricular myocardium, International Journal of Cardiology, 10.1016/j.ijcard.2008.11.080, 140:3, (367-369), Online publication date: 1-Apr-2010. Germans T, Nijveldt R and van Rossum A (2008) A More Detailed View Calls for More Detailed Definition: Description of Cardiac Morphology with High-Resolution CT and MRI, American Journal of Roentgenology, 10.2214/AJR.07.2980, 190:2, (W169-W169), Online publication date: 1-Feb-2008. OGINOSAWA Y, NOGAMI A, SOEJIMA K, AONUMA K, KUBOTA S, SATO T, SUGIYASU A, YOSHIDA K, KOWASE S, SAKAMAKI M, KUROSAKI K and KATO K (2008) Effect of Cardiac Resynchronization Therapy in Isolated Ventricular Noncompaction in Adults: Follow-Up of Four Cases, Journal of Cardiovascular Electrophysiology, 10.1111/j.1540-8167.2008.01161.x, 19:9, (935-938), Online publication date: 1-Sep-2008. Stöllberger C, Kopsa W, Tscherney R and Finsterer J (2008) Diagnosing Left Ventricular Noncompaction by Echocardiography and Cardiac Magnetic Resonance Imaging and Its Dependency on Neuromuscular Disorders, Clinical Cardiology, 10.1002/clc.20253, 31:8, (383-387), Online publication date: 1-Aug-2008. Mueller G and Attili A (2008) Cardiomyopathy: Magnetic Resonance Imaging Evaluation, Seminars in Roentgenology, 10.1053/j.ro.2008.02.006, 43:3, (204-222), Online publication date: 1-Jul-2008. Teaching File Case 46 Cardiovascular MRI in Practice, 10.1007/978-1-84800-090-2_54, (146-147) Nakamura T, Colbert M, Krenz M, Molkentin J, Hahn H, Dorn G and Robbins J (2007) Mediating ERK1/2 signaling rescues congenital heart defects in a mouse model of Noonan syndrome, Journal of Clinical Investigation, 10.1172/JCI30756, 117:8, (2123-2132), Online publication date: 1-Aug-2007. Macedo R, Schmidt A, Rochitte C, Lima J and Bluemke D (2007) MRI to Assess Arrhythmia and Cardiomyopathies: Relationship to Echocardiography, Echocardiography, 10.1111/j.1540-8175.2007.00376.x, 24:2, (194-206), Online publication date: 1-Feb-2007. Hughes M, Carstensen B, Wilkinson J and Weintraub R (2006) Angiographic diagnosis, prevalence and outcomes for left ventricular noncompaction in children with congenital cardiac disease, Cardiology in the Young, 10.1017/S1047951106001351, 17:01, (56), Online publication date: 1-Feb-2007. Martínez Dolz L and Almenar Bonet L (2006) Técnicas de imagen en la insuficiencia cardiaca, Revista Española de Cardiología Suplementos, 10.1016/S1131-3587(06)74832-4, 6:6, (27F-45F), Online publication date: 1-Jan-2006. Gabrielli F, Lombardo A, Natale L, Galiuto L, Porcelli A, Rebuzzi A and Crea F (2006) Myocardial infarction in isolated ventricular non-compaction: Contrast echo and MRI, International Journal of Cardiology, 10.1016/j.ijcard.2005.09.054, 111:2, (315-317), Online publication date: 1-Aug-2006. Lilje C, Rázek V, Schäfer H and Weil J (2006) Noncompaction of the ventricular myocardium, The Journal of Pediatrics, 10.1016/j.jpeds.2005.10.006, 148:4, (562), Online publication date: 1-Apr-2006. Neo H, Wong R, Seto K, Yip J, Yang H and Ling L (2006) Noncompaction cardiomyopathy presenting with congestive heart failure during intercurrent dengue viral illness: Importance of phenotypic recognition, International Journal of Cardiology, 10.1016/j.ijcard.2005.01.064, 107:1, (123-125), Online publication date: 1-Feb-2006. Alhabshan F, Smallhorn J, Golding F, Musewe N, Freedom R and Yoo S (2005) Extent of myocardial noncompaction: comparison between MRI and echocardiographic evaluation, Pediatric Radiology, 10.1007/s00247-005-1551-2, 35:11, (1147-1151), Online publication date: 1-Nov-2005. Bogaert J and Taylor A Nonischemic Myocardial Disease Clinical Cardiac MRI, 10.1007/3-540-26997-5_9, (217-270) Errando C, Tatay J, Serrano-Romero A, Gudín-Uriel M, Revert M and Peiró C (2005) Splenic rupture and haemoperitoneum in a patient with non-compaction of the left ventricular myocardium, British Journal of Anaesthesia, 10.1093/bja/aei207, 95:3, (358-361), Online publication date: 1-Sep-2005. Blaauw Y, Gögelein H, Tieleman R, van Hunnik A, Schotten U and Allessie M (2004) "Early" Class III Drugs for the Treatment of Atrial Fibrillation, Circulation, 110:13, (1717-1724), Online publication date: 28-Sep-2004. Alberto García-Lledó J, de Juan C, Balaguer J and Borrallo M (2004) Miocardiopatía espongiforme aislada del adulto, Medicina Clínica, 10.1016/S0025-7753(04)74323-7, 122:15, (599), Online publication date: 1-Jan-2004. van der Loo B and Jenni R (2003) Isolated Noncompaction of the Myocardium, Circulation, 107:7, (e50-e50), Online publication date: 25-Feb-2003.Pignatelli R, McMahon C, Dreyer W, Denfield S, Price J, Belmont J, Craigen W, Wu J, El Said H, Bezold L, Clunie S, Fernbach S, Bowles N and Towbin J (2003) Clinical Characterization of Left Ventricular Noncompaction in Children, Circulation, 108:21, (2672-2678), Online publication date: 25-Nov-2003. August 6, 2002Vol 106, Issue 6 Advertisement Article InformationMetrics https://doi.org/10.1161/01.CIR.0000028961.66881.2DPMID: 12163441 Originally publishedAugust 6, 2002 PDF download Advertisement