Left ventricular noncompaction, morphological, and clinical features for an integrated diagnosis

  • Francesco NegriEmail author
  • Antonio De Luca
  • Enrico Fabris
  • Renata Korcova
  • Carlo Cernetti
  • Chrysanthos Grigoratos
  • Giovanni Donato Aquaro
  • Gaetano Nucifora
  • Paolo G. Camici
  • Gianfranco Sinagra


The presence of myocardial noncompaction (NC), regardless of the criterion used, does not identify cardiomyopathy per se. The distinction between a morphological variant and the presence of an NC cardiomyopathy is challenging. However, thanks to larger cohorts of patients and longer periods of follow-up, better clinical characterization and prognostic evaluation are becoming available. Indeed, the physician is required to integrate the evidence of NC with the clinical history of the patient, which is supplemented by necessary advanced instrumental investigations before a definite diagnosis of NC cardiomyopathy can be made. Therefore, we extensively revised the current literature in order to help the clinicians to identify clinical features which are pivotal supporting diagnostic element for the correct recognition of Left ventricular noncompaction cardiomyopathy and thus highlighting the difference between a form of cardiomyopathy and a mere intraventricular hypertrabeculation.


Left ventricular noncompaction Cardiomyopathy Echocardiography Cardiac magnetic resonance Late gadolinium enhancement Genetics 



This study was supported by the CRTrieste, FINCANTIERI, and GENERALI Foundations to Heart Muscle Diseases Research at Cardiovascular Department, University Hospital of Trieste.

Compliance with ethical standards

Conflict of interest

The authors declare that they have no conflict of interest.


  1. 1.
    Sedmera D, Pexieder T, Vuillemin M, Thompson RP, Anderson RH (2000) Developmental patterning of the myocardium. Anat Rec 258:319–337CrossRefGoogle Scholar
  2. 2.
    Pignatelli RH, McMahon CJ, Dreyer WJ, Denfield SW, Price J, Belmont JW, Craigen WJ, Wu J, el Said H, Bezold LI, Clunie S, Fernbach S, Bowles NE, Towbin JA (2003) Clinical characterization of left ventricular noncompaction in children: a relatively common form of cardiomyopathy. Circulation 108:2672–2678CrossRefGoogle Scholar
  3. 3.
    Ichida F, Hamamichi Y, Miyawaki T, Ono Y, Kamiya T, Akagi T, Hamada H, Hirose O, Isobe T, Yamada K, Kurotobi S, Mito H, Miyake T, Murakami Y, Nishi T, Shinohara M, Seguchi M, Tashiro S, Tomimatsu H (1999) Clinical features of isolated noncompaction of the ventricular myocardium: long-term clinical course, hemodynamic properties, and genetic background. J Am Coll Cardiol 34:233–240CrossRefGoogle Scholar
  4. 4.
    Andreini D, Pontone G, Bogaert J, Roghi A, Barison A, Schwitter J, Mushtaq S, Vovas G, Sormani P, Aquaro GD, Monney P, Segurini C, Guglielmo M, Conte E, Fusini L, dello Russo A, Lombardi M, Gripari P, Baggiano A, Fiorentini C, Lombardi F, Bartorelli AL, Pepi M, Masci PG (2016) Long-term prognostic value of cardiac magnetic resonance in left ventricle noncompaction: a prospective multicenter study. J Am Coll Cardiol 68:2166–2181CrossRefGoogle Scholar
  5. 5.
    Nucifora G, Aquaro GD, Masci PG, Pingitore A, Lombardi M (2014) Magnetic resonance assessment of prevalence and correlates of right ventricular abnormalities in isolated left ventricular noncompaction. Am J Cardiol 113:142–146CrossRefGoogle Scholar
  6. 6.
    Arbustini E, Narula N, Dec GW, Reddy KS, Greenberg B, Kushwaha S, Marwick T, Pinney S, Bellazzi R, Favalli V, Kramer C, Roberts R, Zoghbi WA, Bonow R, Tavazzi L, Fuster V, Narula J (2013) The MOGE(S) classification for a phenotype-genotype nomenclature of cardiomyopathy: endorsed by the World Heart Federation. J Am Coll Cardiol 62:2046–2072CrossRefGoogle Scholar
  7. 7.
    Aras D, Tufekcioglu O, Ergun K, Ozeke O, Yildiz A, Topaloglu S, Deveci B, Sahin O, Kisacik HL, Korkmaz S (2006) Clinical features of isolated ventricular noncompaction in adults long-term clinical course, echocardiographic properties, and predictors of left ventricular failure. J Card Fail 12:726–733CrossRefGoogle Scholar
  8. 8.
    Stanton C, Bruce C, Connolly H, Brady P, Syed I, Hodge D, Asirvatham S, Friedman P (2009) Isolated left ventricular noncompaction syndrome. Am J Cardiol 104:1135–1138CrossRefGoogle Scholar
  9. 9.
    Oechslin E, Jenni R (2018) Left ventricular noncompaction. J Am Coll Cardiol 71:723–726CrossRefGoogle Scholar
  10. 10.
    Gati S, Papadakis M, Papamichael ND, Zaidi A, Sheikh N, Reed M, Sharma R, Thilaganathan B, Sharma S (2014) Reversible de novo left ventricular trabeculations in pregnant women: implications for the diagnosis of left ventricular noncompaction in low-risk populations. Circulation 130:475–483CrossRefGoogle Scholar
  11. 11.
    Gati S, Chandra N, Bennett RL, Reed M, Kervio G, Panoulas VF, Ghani S, Sheikh N, Zaidi A, Wilson M, Papadakis M, Carré F, Sharma S (2013) 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 99:401–408CrossRefGoogle Scholar
  12. 12.
    Minamisawa M, Koyama J, Kozuka A, Miura T, Ebisawa S, Motoki H, Okada A, Izawa A, Ikeda U (2016) Regression of left ventricular hypertrabeculation is associated with improvement in systolic function and favorable prognosis in adult patients with non-ischemic cardiomyopathy. J Cardiol 68:431–438CrossRefGoogle Scholar
  13. 13.
    Caselli S, Attenhofer Jost CH, Jenni R, Pelliccia A (2015) Left ventricular noncompaction diagnosis and management relevant to pre-participation screening of athletes. Am J Cardiol 116:801–808CrossRefGoogle Scholar
  14. 14.
    Arbustini E, Weidemann F, Hall JL (2014) Left ventricular noncompaction. J Am Coll Cardiol 64:1840–1850CrossRefGoogle Scholar
  15. 15.
    Ciolli A, de Matteis G, Trambaiolo P, Castro A, Stingone A, Altamura G (2015) Is left ventricular noncompaction only a morphological feature? A case of disappearance of noncompaction after surgical correction of aorto-right ventricular fistula, interventricular septal defect and aortic stenosis. J Cardiovasc Echogr 25:26–28CrossRefGoogle Scholar
  16. 16.
    Stähli BE et al (2013) Left ventricular non-compaction: prevalence in congenital heart disease. Int J Cardiol 167:2477–2481CrossRefGoogle Scholar
  17. 17.
    Tian T, Yang Y, Zhou L, Luo F, Li Y, Fan P, Dong X, Liu Y, Cui J, Zhou X (2018) Left ventricular non-compaction: a cardiomyopathy with acceptable prognosis in children. Heart Lung Circ 27:28–32CrossRefGoogle Scholar
  18. 18.
    Arunamata A, Punn R, Cuneo B, Bharati S, Silverman NH (2012) Echocardiographic diagnosis and prognosis of fetal left ventricular noncompaction. J Am Soc Echocardiogr 25:112–120CrossRefGoogle Scholar
  19. 19.
    Hussein A, Karimianpour A, Collier P, Krasuski RA (2015) Isolated noncompaction of the left ventricle in adults. J Am Coll Cardiol 66:578–585CrossRefGoogle Scholar
  20. 20.
    Chin TK, Perloff JK, Williams RG, Jue K, Mohrmann R (1990) Isolated noncompaction of left ventricular myocardium. A study of eight cases. Circulation 82:507–513CrossRefGoogle Scholar
  21. 21.
    Jenni R, Oechslin E, Schneider J, Attenhofer Jost C, Kaufmann PA (2001) Echocardiographic and pathoanatomical characteristics of isolated left ventricular non-compaction: a step towards classification as a distinct cardiomyopathy. Heart 86:666–671CrossRefGoogle Scholar
  22. 22.
    Stöllberger C, Finsterer J, Blazek G (2002) Left ventricular hypertrabeculation/noncompaction and association with additional cardiac abnormalities and neuromuscular disorders. Am J Cardiol 90:899–902CrossRefGoogle Scholar
  23. 23.
    Belanger AR, Miller MA, Donthireddi UR, Najovits AJ, Goldman ME (2008) New classification scheme of left ventricular noncompaction and correlation with ventricular performance. Am J Cardiol 102:92–96CrossRefGoogle Scholar
  24. 24.
    Petersen SE, Selvanayagam JB, Wiesmann F, Robson MD, Francis JM, Anderson RH, Watkins H, Neubauer S (2005) Left ventricular non-compaction. J Am Coll Cardiol 46:101–105CrossRefGoogle Scholar
  25. 25.
    Jacquier A, Thuny F, Jop B, Giorgi R, Cohen F, Gaubert JY, Vidal V, Bartoli JM, Habib G, Moulin G (2010) Measurement of trabeculated left ventricular mass using cardiac magnetic resonance imaging in the diagnosis of left ventricular non-compaction. Eur Heart J 31:1098–1104CrossRefGoogle Scholar
  26. 26.
    Grothoff M, Pachowsky M, Hoffmann J, Posch M, Klaassen S, Lehmkuhl L, Gutberlet M (2012) Value of cardiovascular MR in diagnosing left ventricular non-compaction cardiomyopathy and in discriminating between other cardiomyopathies. Eur Radiol 22:2699–2709CrossRefGoogle Scholar
  27. 27.
    Captur G, Muthurangu V, Cook C, Flett AS, Wilson R, Barison A, Sado DM, Anderson S, McKenna WJ, Mohun TJ, Elliott PM, Moon JC (2013) Quantification of left ventricular trabeculae using fractal analysis. J Cardiovasc Magn Reson 15:36CrossRefGoogle Scholar
  28. 28.
    Stacey RB, Andersen MM, St Clair M, Hundley WG, Thohan V (2013) Comparison of systolic and diastolic criteria for isolated LV noncompaction in CMR. JACC Cardiovasc Imaging 6:931–940CrossRefGoogle Scholar
  29. 29.
    Kawel N, Nacif M, Arai AE, Gomes AS, Hundley WG, Johnson WC, Prince MR, Stacey RB, Lima JAC, Bluemke DA (2012) Trabeculated (noncompacted) and compact myocardium in adults: the multi-ethnic study of atherosclerosis. Circ. Cardiovasc. Imaging 5:357–366CrossRefGoogle Scholar
  30. 30.
    Weir-McCall JR, Yeap PM, Papagiorcopulo C, Fitzgerald K, Gandy SJ, Lambert M, Belch JJF, Cavin I, Littleford R, Macfarlane JA, Matthew SZ, Nicholas RS, Struthers AD, Sullivan F, Waugh SA, White RD, Houston JG (2016) Left ventricular noncompaction. J Am Coll Cardiol 68:2157–2165CrossRefGoogle Scholar
  31. 31.
    Oechslin E, Jenni R (2011) Left ventricular non-compaction revisited: a distinct phenotype with genetic heterogeneity? Eur Heart J 32:1446–1456CrossRefGoogle Scholar
  32. 32.
    Towbin JA, Lorts A, Jefferies JL (2015) Left ventricular non-compaction cardiomyopathy. Lancet 386:813–825CrossRefGoogle Scholar
  33. 33.
    Mavrogeni S, Sfendouraki E, Theodorakis G, Kolovou G (2013) Diagnosis, severity grading and prognosis of left ventricular non-compaction using cardiovascular magnetic resonance. Int J Cardiol 167:598–599CrossRefGoogle Scholar
  34. 34.
    Luedde M, Ehlermann P, Weichenhan D, Will R, Zeller R, Rupp S, Müller A, Steen H, Ivandic BT, Ulmer HE, Kern M, Katus HA, Frey N (2010) Severe familial left ventricular non-compaction cardiomyopathy due to a novel troponin T (TNNT2) mutation. Cardiovasc Res 86:452–460CrossRefGoogle Scholar
  35. 35.
    Caselli S, Autore C, Serdoz A, Santini D, Musumeci MB, Pelliccia A, Agati L (2012) Three-dimensional echocardiographic characterization of patients with left ventricular noncompaction. J Am Soc Echocardiogr 25:203–209CrossRefGoogle Scholar
  36. 36.
    Nucifora G, Aquaro GD, Pingitore A, Masci PG, Lombardi M (2011) Myocardial fibrosis in isolated left ventricular non-compaction and its relation to disease severity. Eur J Heart Fail 13:170–176CrossRefGoogle Scholar
  37. 37.
    Wan J, Zhao S, Cheng H, Lu M, Jiang S, Yin G, Gao X, Yang Y (2013) Varied distributions of late gadolinium enhancement found among patients meeting cardiovascular magnetic resonance criteria for isolated left ventricular non-compaction. J Cardiovasc Magn Reson 15:20CrossRefGoogle Scholar
  38. 38.
    Stöllberger C, Gerger D, Jirak P, Wegner C, Finsterer J (2014) Evolution of electrocardiographic abnormalities in association with neuromuscular disorders and survival in left ventricular hypertrabeculation/noncompaction. Ann Noninvasive Electrocardiol 19:567–573CrossRefGoogle Scholar
  39. 39.
    Zhou H, Lin X, Fang L, Zhu W, Zhao X, Ding H, Jiang M, Ge H, Fang Q, He B (2017) Prolonged QTc indicates the clinical severity and poor prognosis in patients with isolated left ventricular non-compaction. Int J Cardiovasc Imaging 33:2013–2020CrossRefGoogle Scholar
  40. 40.
    Cetin MS, Ozcan Cetin EH, Canpolat U, Cay S, Topaloglu S, Temizhan A, Aydogdu S (2016) Usefulness of fragmented QRS complex to predict arrhythmic events and cardiovascular mortality in patients with noncompaction cardiomyopathy. Am J Cardiol 117:1516–1523CrossRefGoogle Scholar
  41. 41.
    Stöllberger C, Finsterer J (2006) Pitfalls in the diagnosis of left ventricular hypertrabeculation/non-compaction. Postgrad Med J 82:679–683CrossRefGoogle Scholar
  42. 42.
    Ivanov A et al (2017) Prevalence and prognostic significance of left ventricular noncompaction in patients referred for cardiac magnetic resonance imaging. Circ Cardiovasc Imaging 10:e006174CrossRefGoogle Scholar
  43. 43.
    Nucifora G, Sree Raman K, Muser D, Shah R, Perry R, Awang Ramli KA, Selvanayagam JB (2017) Cardiac magnetic resonance evaluation of left ventricular functional, morphological, and structural features in children and adolescents vs. young adults with isolated left ventricular non-compaction. Int J Cardiol 246:68–73CrossRefGoogle Scholar
  44. 44.
    Cai J, Bryant JA, le TT, Su B, de Marvao A, O’Regan DP, Cook SA, Chin CWL (2017) Fractal analysis of left ventricular trabeculations is associated with impaired myocardial deformation in healthy Chinese. J Cardiovasc Magn Reson 19(102):102CrossRefGoogle Scholar
  45. 45.
    Martinez-Naharro A, Kotecha T, Norrington K, Boldrini M, Rezk T, Quarta C, Treibel TA, Whelan CJ, Knight DS, Kellman P, Ruberg FL, Gillmore JD, Moon JC, Hawkins PN, Fontana M (2018) Native T1 and extracellular volume in transthyretin amyloidosis. JACC Cardiovasc Imaging.
  46. 46.
    Nakamori S, Dohi K, Ishida M, Goto Y, Imanaka-Yoshida K, Omori T, Goto I, Kumagai N, Fujimoto N, Ichikawa Y, Kitagawa K, Yamada N, Sakuma H, Ito M (2018) Native T1 mapping and extracellular volume mapping for the assessment of diffuse myocardial fibrosis in dilated cardiomyopathy. JACC Cardiovasc Imaging 11:48–59CrossRefGoogle Scholar
  47. 47.
    Hinojar R et al (2015) T1 mapping in discrimination of hypertrophic phenotypes: hypertensive heart disease and hypertrophic cardiomyopathy: findings from the international T1 multicenter cardiovascular magnetic resonance study. Circ. Cardiovasc. Imaging 8:e003285CrossRefGoogle Scholar
  48. 48.
    Zhou H, Lin X, Fang L, Zhao X, Ding H, Chen W, Xu R, Bai X, Wang Y, Fang Q (2016) Characterization of compacted myocardial abnormalities by cardiac magnetic resonance with native T1 mapping in left ventricular non-compaction patients—a comparison with late gadolinium enhancement. Circ J 80:1210–1216CrossRefGoogle Scholar
  49. 49.
    Araujo-Filho JAB, Assuncao AN Jr, Tavares de Melo MD, Bière L, Lima CR, Dantas RN Jr, Nomura CH, Salemi VMC, Jerosch-Herold M, Parga JR (2018) Myocardial T1 mapping and extracellular volume quantification in patients with left ventricular non-compaction cardiomyopathy. Eur Heart J Cardiovasc Imaging 19:888–895. CrossRefPubMedGoogle Scholar
  50. 50.
    Grillone S, Nucifora G, Piccoli G, Gianfagna P, Hysko F, Pavoni D, Slavich G, Proclemer A, Gasparini D (2013) Biventricular non-compaction demonstrated on multi-slice computed tomography with echocardiographic correlation. J Cardiovasc Med (Hagerstown) 14:677–680CrossRefGoogle Scholar
  51. 51.
    Sidhu MS, Uthamalingam S, Ahmed W, Engel LC, Vorasettakarnkij Y, Lee AM, Hoffmann U, Brady T, Abbara S, Ghoshhajra BB (2014) Defining left ventricular noncompaction using cardiac computed tomography. J Thorac Imaging 29:60–66CrossRefGoogle Scholar
  52. 52.
    Richards S et al (2015) Standards and guidelines for the interpretation of sequence variants: a joint consensus recommendation of the American College of Medical Genetics and Genomics and the Association for Molecular Pathology. Genet Med 17:405–424CrossRefGoogle Scholar
  53. 53.
    Moric-Janiszewska E, Markiewicz-Łoskot G (2008) Genetic heterogeneity of left-ventricular noncompaction cardiomyopathy. Clin Cardiol 31:201–204CrossRefGoogle Scholar
  54. 54.
    Zaragoza MV, Arbustini E, Narula J (2007) Noncompaction of the left ventricle: primary cardiomyopathy with an elusive genetic etiology. Curr Opin Pediatr 19:619–627CrossRefGoogle Scholar
  55. 55.
    Sedaghat-Hamedani F, Haas J, Zhu F, Geier C, Kayvanpour E, Liss M, Lai A, Frese K, Pribe-Wolferts R, Amr A, Li DT, Samani OS, Carstensen A, Bordalo DM, Müller M, Fischer C, Shao J, Wang J, Nie M, Yuan L, Haßfeld S, Schwartz C, Zhou M, Zhou Z, Shu Y, Wang M, Huang K, Zeng Q, Cheng L, Fehlmann T, Ehlermann P, Keller A, Dieterich C, Streckfuß-Bömeke K, Liao Y, Gotthardt M, Katus HA, Meder B (2017) Clinical genetics and outcome of left ventricular non-compaction cardiomyopathy. Eur Heart J 38:3449–3460CrossRefGoogle Scholar
  56. 56.
    Wang C et al (2017) A wide and specific spectrum of genetic variants and genotype-phenotype correlations revealed by next-generation sequencing in patients with left ventricular noncompaction. J Am Heart Assoc 6:e006210PubMedPubMedCentralGoogle Scholar
  57. 57.
    van Waning JI, Caliskan K, Hoedemaekers YM, van Spaendonck-Zwarts KY, Baas AF, Boekholdt SM, van Melle JP, Teske AJ, Asselbergs FW, Backx APCM, du Marchie Sarvaas GJ, Dalinghaus M, Breur JMPJ, Linschoten MPM, Verlooij LA, Kardys I, Dooijes D, Lekanne Deprez RH, IJpma AS, van den Berg MP, Hofstra RMW, van Slegtenhorst MA, Jongbloed JDH, Majoor-Krakauer D (2018) Genetics, clinical features, and long-term outcome of noncompaction cardiomyopathy. J Am Coll Cardiol 71:711–722CrossRefGoogle Scholar
  58. 58.
    Muser D, Nucifora G, Gianfagna E, Pavoni D, Rebellato L, Facchin D, Daleffe E, Proclemer A (2014) Clinical spectrum of isolated left ventricular noncompaction. J Am Coll Cardiol 63:e39CrossRefGoogle Scholar
  59. 59.
    Oechslin EN, Attenhofer Jost CH, Rojas JR, Kaufmann PA, Jenni R (2000) Long-term follow-up of 34 adults with isolated left ventricular noncompaction: a distinct cardiomyopathy with poor prognosis. J Am Coll Cardiol 36:493–500CrossRefGoogle Scholar
  60. 60.
    Muser D, Liang JJ, Witschey WRT, Pathak RK, Castro S, Magnani S, Zado ES, Garcia FC, Desjardins B, Callans DJ, Frankel DS, Marchlinski FE, Santangeli P (2017) Ventricular arrhythmias associated with left ventricular noncompaction: electrophysiologic characteristics, mapping, and ablation. Hearth Rhythm 14:166–175CrossRefGoogle Scholar
  61. 61.
    Stöllberger C, Blazek G, Dobias C, Hanafin A, Wegner C, Finsterer J (2011) Frequency of stroke and embolism in left ventricular hypertrabeculation/noncompaction. Am J Cardiol 108:1021–1023CrossRefGoogle Scholar
  62. 62.
    Ponikowski P et al (2016) ESC guidelines for the diagnosis and treatment of acute and chronic heart failure: the Task Force for the diagnosis and treatment of acute and chronic heart failure of the European Society of Cardiology (ESC). Developed with the special contribution of the Heart Failure Association (HFA) of the ESC. Eur J Heart Fail 891–975(2016):18Google Scholar

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© Springer Science+Business Media, LLC, part of Springer Nature 2019

Authors and Affiliations

  • Francesco Negri
    • 1
    Email author return OK on get
  • Antonio De Luca
    • 1
  • Enrico Fabris
    • 1
  • Renata Korcova
    • 1
  • Carlo Cernetti
    • 2
  • Chrysanthos Grigoratos
    • 3
    • 4
  • Giovanni Donato Aquaro
    • 3
  • Gaetano Nucifora
    • 5
    • 6
  • Paolo G. Camici
    • 7
  • Gianfranco Sinagra
    • 1
  1. 1.Cardiovascular Department, Cardiomyopathy CenterAzienda Sanitaria Universitaria Integrata di TriesteTriesteItaly
  2. 2.Head of Cardio-Neuro-Vascular Department Ca’ Foncello and San Giacomo Hospital Azienda N 2TrevisoItaly
  3. 3.Fondazione G. Monastiero CNR-Regione ToscanaPisaItaly
  4. 4.Scuola Superiore Sant’AnnaInstitute of Life SciencesPisaItaly
  5. 5.Northwest Heart Centre, Wythenshawe HospitalManchester University NHS Foundation TrustManchesterUK
  6. 6.Flinders University, Bedford ParkAdelaideAustralia
  7. 7.Vita-Salute University and San Raffaele HospitalMilanItaly

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