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Isolated left ventricular noncompaction

Heidi M Connolly, MD, FASE
Christine H Attenhofer-Jost, MD
Section Editor
William J McKenna, MD
Deputy Editor
Susan B Yeon, MD, JD, FACC


Left ventricular noncompaction (LVNC) is a cardiomyopathy characterized by prominent left ventricular trabeculae and deep intertrabecular recesses [1,2]. The 2008 European Society of Cardiology Working Group on Myocardial and Pericardial Diseases categorized LVNC as an "unclassified" cardiomyopathy (table 1A-B) [2]. LVNC was previously also called spongy myocardium or hypertrabeculation syndrome but these terms should not be used interchangeably with LVNC [3].

This review will focus on LVNC as an isolated disorder distinct from other clinical settings in which non-compacted myocardium may be seen in association with other cardiac and non-cardiac abnormalities. The classification of cardiomyopathies is discussed separately. (See "Definition and classification of the cardiomyopathies".)


Left ventricular noncompaction (LVNC; or "non-compaction") is characterized by the following features:

An altered myocardial wall with prominent trabeculae and deep intertrabecular recesses, resulting in thickened myocardium with two layers consisting of non-compacted myocardium and a thin compacted layer of myocardium (picture 1) [4-6].

Continuity between the left ventricular cavity and the deep intratrabecular recesses, which are filled with blood from the ventricular cavity without evidence of communication to the epicardial coronary artery system.

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Literature review current through: Nov 2017. | This topic last updated: Sep 12, 2016.
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  1. Maron BJ, Towbin JA, Thiene G, et al. 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.
  2. Elliott P, Andersson B, Arbustini E, et al. Classification of the cardiomyopathies: a position statement from the European Society Of Cardiology Working Group on Myocardial and Pericardial Diseases. Eur Heart J 2008; 29:270.
  3. Stöllberger C, Finsterer J. Outcome of left ventricular hypertrabeculation/noncompaction in children. Am J Cardiol 2005; 96:607; author reply 607.
  4. Ritter M, Oechslin E, Sütsch G, et al. Isolated noncompaction of the myocardium in adults. Mayo Clin Proc 1997; 72:26.
  5. Weiford BC, Subbarao VD, Mulhern KM. Noncompaction of the ventricular myocardium. Circulation 2004; 109:2965.
  6. Arbustini E, Weidemann F, Hall JL. Left ventricular noncompaction: a distinct cardiomyopathy or a trait shared by different cardiac diseases? J Am Coll Cardiol 2014; 64:1840.
  7. Henderson DJ, Anderson RH. The development and structure of the ventricles in the human heart. Pediatr Cardiol 2009; 30:588.
  8. Chen H, Zhang W, Li D, et al. Analysis of ventricular hypertrabeculation and noncompaction using genetically engineered mouse models. Pediatr Cardiol 2009; 30:626.
  9. Piga A, Longo F, Musallam KM, et al. Left ventricular noncompaction in patients with β-thalassemia: uncovering a previously unrecognized abnormality. Am J Hematol 2012; 87:1079.
  10. Pfammatter JP, Paul T, Flik J, et al. [Q-fever associated myocarditis in a 14-year-old boy]. Z Kardiol 1995; 84:947.
  11. Wlodarska EK, Wozniak O, Konka M, et al. Isolated ventricular noncompaction mimicking arrhythmogenic right ventricular cardiomyopathy--a study of nine patients. Int J Cardiol 2010; 145:107.
  12. Pignatelli RH, McMahon CJ, Dreyer WJ, et al. Clinical characterization of left ventricular noncompaction in children: a relatively common form of cardiomyopathy. Circulation 2003; 108:2672.
  13. Aras D, Tufekcioglu O, Ergun K, et al. Clinical features of isolated ventricular noncompaction in adults long-term clinical course, echocardiographic properties, and predictors of left ventricular failure. J Card Fail 2006; 12:726.
  14. Stanton C, Bruce C, Connolly H, et al. Isolated left ventricular noncompaction syndrome. Am J Cardiol 2009; 104:1135.
  15. Oechslin EN, Attenhofer Jost CH, Rojas JR, et al. Long-term follow-up of 34 adults with isolated left ventricular noncompaction: a distinct cardiomyopathy with poor prognosis. J Am Coll Cardiol 2000; 36:493.
  16. Kovacevic-Preradovic T, Jenni R, Oechslin EN, et al. Isolated left ventricular noncompaction as a cause for heart failure and heart transplantation: a single center experience. Cardiology 2009; 112:158.
  17. Patrianakos AP, Parthenakis FI, Nyktari EG, Vardas PE. Noncompaction myocardium imaging with multiple echocardiographic modalities. Echocardiography 2008; 25:898.
  18. Murphy RT, Thaman R, Blanes JG, et al. Natural history and familial characteristics of isolated left ventricular non-compaction. Eur Heart J 2005; 26:187.
  19. Zaragoza MV, Arbustini E, Narula J. Noncompaction of the left ventricle: primary cardiomyopathy with an elusive genetic etiology. Curr Opin Pediatr 2007; 19:619.
  20. Gati S, Rajani R, Carr-White GS, Chambers JB. Adult left ventricular noncompaction: reappraisal of current diagnostic imaging modalities. JACC Cardiovasc Imaging 2014; 7:1266.
  21. Kelley-Hedgepeth A, Towbin JA, Maron MS. Images in cardiovascular medicine. Overlapping phenotypes: left ventricular noncompaction and hypertrophic cardiomyopathy. Circulation 2009; 119:e588.
  22. Monserrat L, Barriales-Villa R, Hermida-Prieto M. Apical hypertrophic cardiomyopathy and left ventricular non-compaction: two faces of the same disease. Heart 2008; 94:1253.
  23. http://omim.org/ (Accessed on December 10, 2014).
  24. Ichida F, Tsubata S, Bowles KR, et al. Novel gene mutations in patients with left ventricular noncompaction or Barth syndrome. Circulation 2001; 103:1256.
  25. Gati S, Papadakis M, Papamichael ND, et al. Reversible de novo left ventricular trabeculations in pregnant women: implications for the diagnosis of left ventricular noncompaction in low-risk populations. Circulation 2014; 130:475.
  26. Luxán G, Casanova JC, Martínez-Poveda B, et al. Mutations in the NOTCH pathway regulator MIB1 cause left ventricular noncompaction cardiomyopathy. Nat Med 2013; 19:193.
  27. Parent JJ, Towbin JA, Jefferies JL. Fibrillin-1 Gene Mutations in Left Ventricular Non-compaction Cardiomyopathy. Pediatr Cardiol 2016; 37:1123.
  28. Bleyl SB, Mumford BR, Thompson V, et al. Neonatal, lethal noncompaction of the left ventricular myocardium is allelic with Barth syndrome. Am J Hum Genet 1997; 61:868.
  29. Xing Y, Ichida F, Matsuoka T, et al. Genetic analysis in patients with left ventricular noncompaction and evidence for genetic heterogeneity. Mol Genet Metab 2006; 88:71.
  30. Spencer CT, Bryant RM, Day J, et al. Cardiac and clinical phenotype in Barth syndrome. Pediatrics 2006; 118:e337.
  31. Monserrat L, Hermida-Prieto M, Fernandez X, et al. Mutation in the alpha-cardiac actin gene associated with apical hypertrophic cardiomyopathy, left ventricular non-compaction, and septal defects. Eur Heart J 2007; 28:1953.
  32. Klaassen S, Probst S, Oechslin E, et al. Mutations in sarcomere protein genes in left ventricular noncompaction. Circulation 2008; 117:2893.
  33. Probst S, Oechslin E, Schuler P, et al. Sarcomere gene mutations in isolated left ventricular noncompaction cardiomyopathy do not predict clinical phenotype. Circ Cardiovasc Genet 2011; 4:367.
  34. Hoedemaekers YM, Caliskan K, Majoor-Krakauer D, et al. Cardiac beta-myosin heavy chain defects in two families with non-compaction cardiomyopathy: linking non-compaction to hypertrophic, restrictive, and dilated cardiomyopathies. Eur Heart J 2007; 28:2732.
  35. Hermida-Prieto M, Monserrat L, Castro-Beiras A, et al. Familial dilated cardiomyopathy and isolated left ventricular noncompaction associated with lamin A/C gene mutations. Am J Cardiol 2004; 94:50.
  36. Vatta M, Mohapatra B, Jimenez S, et al. Mutations in Cypher/ZASP in patients with dilated cardiomyopathy and left ventricular non-compaction. J Am Coll Cardiol 2003; 42:2014.
  37. Chang B, Nishizawa T, Furutani M, et al. Identification of a novel TPM1 mutation in a family with left ventricular noncompaction and sudden death. Mol Genet Metab 2011; 102:200.
  38. Sasse-Klaassen S, Probst S, Gerull B, et al. Novel gene locus for autosomal dominant left ventricular noncompaction maps to chromosome 11p15. Circulation 2004; 109:2720.
  39. Ohno S, Omura M, Kawamura M, et al. Exon 3 deletion of RYR2 encoding cardiac ryanodine receptor is associated with left ventricular non-compaction. Europace 2014; 16:1646.
  40. Duru F, Candinas R. Noncompaction of ventricular myocardium and arrhythmias. J Cardiovasc Electrophysiol 2000; 11:493.
  41. Brescia ST, Rossano JW, Pignatelli R, et al. Mortality and sudden death in pediatric left ventricular noncompaction in a tertiary referral center. Circulation 2013; 127:2202.
  42. Menon SC, O'Leary PW, Wright GB, et al. Fetal and neonatal presentation of noncompacted ventricular myocardium: expanding the clinical spectrum. J Am Soc Echocardiogr 2007; 20:1344.
  43. Bhatia NL, Tajik AJ, Wilansky S, et al. Isolated noncompaction of the left ventricular myocardium in adults: a systematic overview. J Card Fail 2011; 17:771.
  44. Stöllberger C, Wegner C, Finsterer J. CHADS2- and CHA2DS2VASc scores and embolic risk in left ventricular hypertrabeculation/noncompaction. J Stroke Cerebrovasc Dis 2013; 22:709.
  45. Salerno JC, Chun TU, Rutledge JC. Sinus bradycardia, Wolff Parkinson White, and left ventricular noncompaction: an embryologic connection? Pediatr Cardiol 2008; 29:679.
  46. Ergul Y, Nisli K, Varkal MA, et al. Electrocardiographic findings at initial diagnosis in children with isolated left ventricular noncompaction. Ann Noninvasive Electrocardiol 2011; 16:184.
  47. Kohli SK, Pantazis AA, Shah JS, et al. Diagnosis of left-ventricular non-compaction in patients with left-ventricular systolic dysfunction: time for a reappraisal of diagnostic criteria? Eur Heart J 2008; 29:89.
  48. Rapezzi C, Leone O, Ferlito M, et al. Isolated ventricular non-compaction with restrictive cardiomyopathy. Eur Heart J 2006; 27:1927.
  49. Biagini E, Ragni L, Ferlito M, et al. Different types of cardiomyopathy associated with isolated ventricular noncompaction. Am J Cardiol 2006; 98:821.
  50. Johnson MT, Zhang S, Gilkeson R, et al. Intrafamilial variability of noncompaction of the ventricular myocardium. Am Heart J 2006; 151:1012.e7.
  51. Jenni R, Oechslin E, Schneider J, et al. Echocardiographic and pathoanatomical characteristics of isolated left ventricular non-compaction: a step towards classification as a distinct cardiomyopathy. Heart 2001; 86:666.
  52. Frischknecht BS, Attenhofer Jost CH, Oechslin EN, et al. Validation of noncompaction criteria in dilated cardiomyopathy, and valvular and hypertensive heart disease. J Am Soc Echocardiogr 2005; 18:865.
  53. Gebhard C, Stähli BE, Greutmann M, et al. Reduced left ventricular compacta thickness: a novel echocardiographic criterion for non-compaction cardiomyopathy. J Am Soc Echocardiogr 2012; 25:1050.
  54. Chin TK, Perloff JK, Williams RG, et al. Isolated noncompaction of left ventricular myocardium. A study of eight cases. Circulation 1990; 82:507.
  55. Stöllberger C, Finsterer J, Blazek G. Left ventricular hypertrabeculation/noncompaction and association with additional cardiac abnormalities and neuromuscular disorders. Am J Cardiol 2002; 90:899.
  56. Caselli S, Autore C, Serdoz A, et al. Three-dimensional echocardiographic characterization of patients with left ventricular noncompaction. J Am Soc Echocardiogr 2012; 25:203.
  57. Lowery MH, Martel JA, Zambrano JP, et al. Noncompaction of the ventricular myocardium: the use of contrast-enhanced echocardiography in diagnosis. J Am Soc Echocardiogr 2003; 16:94.
  58. Gati S, Chandra N, Bennett RL, et al. Increased left ventricular trabeculation in highly trained athletes: do we need more stringent criteria for the diagnosis of left ventricular non-compaction in athletes? Heart 2013; 99:401.
  59. Zemrak F, Ahlman MA, Captur G, et al. The relationship of left ventricular trabeculation to ventricular function and structure over a 9.5-year follow-up: the MESA study. J Am Coll Cardiol 2014; 64:1971.
  60. Jacquier A, Thuny F, Jop B, et al. Measurement of trabeculated left ventricular mass using cardiac magnetic resonance imaging in the diagnosis of left ventricular non-compaction. Eur Heart J 2010; 31:1098.
  61. Garcia-Pavia P, de la Pompa JL. Left ventricular noncompaction: a genetic cardiomyopathy looking for diagnostic criteria. J Am Coll Cardiol 2014; 64:1981.
  62. Stöllberger C, Gerecke B, Engberding R, et al. Interobserver Agreement of the Echocardiographic Diagnosis of LV Hypertrabeculation/Noncompaction. JACC Cardiovasc Imaging 2015; 8:1252.
  63. McMahon CJ, Pignatelli RH, Nagueh SF, et al. Left ventricular non-compaction cardiomyopathy in children: characterisation of clinical status using tissue Doppler-derived indices of left ventricular diastolic relaxation. Heart 2007; 93:676.
  64. Towbin JA. Left ventricular noncompaction: a new form of heart failure. Heart Fail Clin 2010; 6:453.
  65. Petersen SE, Selvanayagam JB, Wiesmann F, et al. Left ventricular non-compaction: insights from cardiovascular magnetic resonance imaging. J Am Coll Cardiol 2005; 46:101.
  66. Fazio G, Novo G, D'Angelo L, et al. Magnetic resonance in isolated noncompaction of the ventricular myocardium. Int J Cardiol 2010; 140:367.
  67. Stöllberger C, Kopsa W, Tscherney R, Finsterer J. Diagnosing left ventricular noncompaction by echocardiography and cardiac magnetic resonance imaging and its dependency on neuromuscular disorders. Clin Cardiol 2008; 31:383.
  68. Petersen SE. CMR and LV noncompaction: does it matter how we measure trabeculations? JACC Cardiovasc Imaging 2013; 6:941.
  69. Captur G, Muthurangu V, Cook C, et al. Quantification of left ventricular trabeculae using fractal analysis. J Cardiovasc Magn Reson 2013; 15:36.
  70. Stacey RB, Andersen MM, St Clair M, et al. Comparison of systolic and diastolic criteria for isolated LV noncompaction in CMR. JACC Cardiovasc Imaging 2013; 6:931.
  71. Thuny F, Jacquier A, Jop B, et al. Assessment of left ventricular non-compaction in adults: side-by-side comparison of cardiac magnetic resonance imaging with echocardiography. Arch Cardiovasc Dis 2010; 103:150.
  72. Dodd JD, Holmvang G, Hoffmann U, et al. Quantification of left ventricular noncompaction and trabecular delayed hyperenhancement with cardiac MRI: correlation with clinical severity. AJR Am J Roentgenol 2007; 189:974.
  73. Wan J, Zhao S, Cheng H, et al. Varied distributions of late gadolinium enhancement found among patients meeting cardiovascular magnetic resonance criteria for isolated left ventricular non-compaction. J Cardiovasc Magn Reson 2013; 15:20.
  74. Stacey RB, Andersen M, Haag J, et al. Right ventricular morphology and systolic function in left ventricular noncompaction cardiomyopathy. Am J Cardiol 2014; 113:1018.
  75. Sidhu MS, Uthamalingam S, Ahmed W, et al. Defining left ventricular noncompaction using cardiac computed tomography. J Thorac Imaging 2014; 29:60.
  76. Burke A, Mont E, Kutys R, Virmani R. Left ventricular noncompaction: a pathological study of 14 cases. Hum Pathol 2005; 36:403.
  77. Azevedo O, Gaspar P, Sá Miranda C, et al. Left ventricular noncompaction in a patient with fabry disease: overdiagnosis, morphological manifestation of fabry disease or two unrelated rare conditions in the same patient? Cardiology 2011; 119:155.
  78. Oechslin E, Jenni R. Left ventricular non-compaction revisited: a distinct phenotype with genetic heterogeneity? Eur Heart J 2011; 32:1446.
  79. Gati S, Papadakis M, Van Niekerk N, et al. Increased left ventricular trabeculation in individuals with sickle cell anaemia: physiology or pathology? Int J Cardiol 2013; 168:1658.
  80. Paterick TE, Tercius AJ, Agarwal A, et al. Double jeopardy in the echocardiography laboratory: coexistence of two distinct cardiomyopathies? Echocardiography 2014; 31:931.
  82. Bonnet D, Gautier-Lhermitte I, Bonhoeffer P, Sidi D. Right ventricular myocardial sinusoidal-coronary artery connections in critical pulmonary valve stenosis. Pediatr Cardiol 1998; 19:269.
  83. Stähli BE, Gebhard C, Biaggi P, et al. Left ventricular non-compaction: prevalence in congenital heart disease. Int J Cardiol 2013; 167:2477.
  84. Dias V, Cabral S, Vieira M, et al. Noncompaction cardiomyopathy and multiple coronary arterioventricular fistulae: 1 or 2 distinct disease entities? J Am Coll Cardiol 2011; 57:e377.
  85. Agarwal A, Khandheria BK, Paterick TE, et al. Left ventricular noncompaction in patients with bicuspid aortic valve. J Am Soc Echocardiogr 2013; 26:1306.
  86. Ali SK. Unique features of non-compaction of the ventricular myocardium in Arab and African patients. Cardiovasc J Afr 2008; 19:241.
  87. Attenhofer Jost CH, Connolly HM, O'Leary PW, et al. Left heart lesions in patients with Ebstein anomaly. Mayo Clin Proc 2005; 80:361.
  88. Friedberg MK, Ursell PC, Silverman NH. Isomerism of the left atrial appendage associated with ventricular noncompaction. Am J Cardiol 2005; 96:985.
  89. Lilje C, Rázek V, Joyce JJ, et al. Complications of non-compaction of the left ventricular myocardium in a paediatric population: a prospective study. Eur Heart J 2006; 27:1855.
  90. Corrado G, Checcarelli N, Santarone M, et al. Left ventricular hypertrabeculation/noncompaction with PMP22 duplication-based Charcot-Marie-Tooth disease type 1A. Cardiology 2006; 105:142.
  91. Gerger D, Stöllberger C, Grassberger M, et al. Pathomorphologic findings in left ventricular hypertrabeculation/noncompaction of adults in relation to neuromuscular disorders. Int J Cardiol 2013; 169:249.
  92. Finsterer J, Kothari S. Cardiac manifestations of primary mitochondrial disorders. Int J Cardiol 2014; 177:754.
  93. Finsterer J, Stöllberger C, Kopsa W. Noncompaction on cardiac MRI in a patient with nail-patella syndrome and mitochondriopathy. Cardiology 2003; 100:48.
  94. Greutmann M, Mah ML, Silversides CK, et al. Predictors of adverse outcome in adolescents and adults with isolated left ventricular noncompaction. Am J Cardiol 2012; 109:276.
  95. Stöllberger C, Wegner C, Benatar A, et al. Postnatal Outcome of Fetal Left Ventricular Hypertrabeculation/Noncompaction. Pediatr Cardiol 2016; 37:919.
  96. Stamou SC, Lefrak EA, Athari FC, et al. Heart transplantation in a patient with isolated noncompaction of the left ventricular myocardium. Ann Thorac Surg 2004; 77:1806.
  97. Weir RA, MacKenzie N, Petrie CJ. Cheating the CHA2DS2-VASc Score: Thromboembolism in Apical Hypertrophic Cardiomyopathy. Case Rep Cardiol 2014; 2014:189895.
  98. Czosek RJ, Spar DS, Khoury PR, et al. Outcomes, arrhythmic burden and ambulatory monitoring of pediatric patients with left ventricular non-compaction and preserved left ventricular function. Am J Cardiol 2015; 115:962.
  99. Authors/Task Force members, Elliott PM, Anastasakis A, et al. 2014 ESC Guidelines on diagnosis and management of hypertrophic cardiomyopathy: the Task Force for the Diagnosis and Management of Hypertrophic Cardiomyopathy of the European Society of Cardiology (ESC). Eur Heart J 2014; 35:2733.
  100. Cetin MS, Ozcan Cetin EH, Canpolat U, et al. Usefulness of Fragmented QRS Complex to Predict Arrhythmic Events and Cardiovascular Mortality in Patients With Noncompaction Cardiomyopathy. Am J Cardiol 2016; 117:1516.
  101. Caselli S, Attenhofer Jost CH, Jenni R, Pelliccia A. Left Ventricular Noncompaction Diagnosis and Management Relevant to Pre-participation Screening of Athletes. Am J Cardiol 2015; 116:801.
  102. Hershberger RE, Lindenfeld J, Mestroni L, et al. Genetic evaluation of cardiomyopathy--a Heart Failure Society of America practice guideline. J Card Fail 2009; 15:83.