Multimodality Imaging, Diagnostic Challenges and Proposed Diagnostic Algorithm for Noncompaction Cardiomyopathy

  • Osama I. Soliman
  • Jackie McGhie
  • Folkert J. ten Cate
  • Bernard P. Paelinck
  • Kadir Caliskan


Noncompaction cardiomyopathy (NCCM) is a clinical-patho-anatomic entity, disputably believed to result from abnormal arrest in embryonic endomyocardial morphogenesis. Introduction of echocardiography has led to recognition of NCCM for the first time in 1984. During almost 35 years of technical improvements in imaging alongside a more dedicated research of NCCM, more knowledge has developed alongside uncertainties concerning every aspect of the disease. In this chapter, we present the role of multimodality imaging in NCCM alongside diagnostic uncertainties and practical aspects on how to overcome these challenges. Limitations of the current diagnostic criteria will be reviewed. The inter-technique differences will be contrasted. Finally, future aspirations will be outlined in providing a more thoughtful appraisal towards NCCM diagnosis.

There is paucity of data regarding NCCM due to the etiology, natural history, pathophysiology, heterogeneity of disease spectrum and phenotype-genotype overlap with other cardiac anomalies. There are three research aspects in the fields of NCCM: patho-anatomic studies, imaging studies, and genetic screening. Besides conventional echocardiography, imaging should include both structural (cardiac magnetic resonance imaging (MRI), cardiovascular computed tomography, contrast echocardiography and 3D echocardiography) and functional diagnosis such as deformation imaging. Moreover, it holds the promise of the detection of earliest stages of the disease. Currently, several efforts are underway including large registries as well as the use of novel imaging modalities in better understanding and diagnosis of NCCM. Genotype-phenotype studies for better risk stratification of sudden cardiac death are underway. Importantly, a clear patho-anatomic cut-off definition of NCCM should be the initial step towards a uniform imaging diagnosis.


Compacted myocardium Noncompacted myocardium Noncompaction Excessive trabeculations Left ventricular noncompaction Cardiomyopathy Left ventricular dysfunction Heart failure Echocardiography Cardiac magnetic resonance imaging Computed tomography Isolated noncompaction cardiomyopathy (NCCM) 


  1. 1.
    Engberding R, Bender F. Identification of a rare congenital anomaly of the myocardium by two-dimensional echocardiography: persistence of isolated myocardial sinusoids. Am J Cardiol. 1984;53:1733–4.CrossRefGoogle Scholar
  2. 2.
    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;113:1807–16.CrossRefGoogle Scholar
  3. 3.
    Elliott P, Andersson B, Arbustini E, Bilinska Z, Cecchi F, Charron P, Dubourg O, Kuhl U, Maisch B, McKenna WJ, Monserrat L, Pankuweit S, Rapezzi C, Seferovic P, Tavazzi L, Keren A. Classification of the cardiomyopathies: a position statement from the European Society Of Cardiology Working Group on Myocardial and Pericardial Diseases. Eur Heart J. 2008;29:270–6.CrossRefGoogle Scholar
  4. 4.
    Habib G, Charron P, Eicher JC, Giorgi R, Donal E, Laperche T, Boulmier D, Pascal C, Logeart D, Jondeau G, Cohen-Solal A. Isolated left ventricular non-compaction in adults: clinical and echocardiographic features in 105 patients. Results from a French registry. Eur J Heart Fail. 2011;13:177–85.CrossRefGoogle Scholar
  5. 5.
    Stollberger C, Blazek G, Dobias C, Hanafin A, Wegner C, Finsterer J. Frequency of stroke and embolism in left ventricular hypertrabeculation/noncompaction. Am J Cardiol. 2011;108:1021–3.CrossRefGoogle Scholar
  6. 6.
    Ergul Y, Nisli K, Varkal MA, Oner N, Dursun M, Dindar A, Aydogan U, Omeroglu RE. Electrocardiographic findings at initial diagnosis in children with isolated left ventricular noncompaction. Ann Noninvasive Electrocardiol. 2011;16:184–91.CrossRefGoogle Scholar
  7. 7.
    Steffel J, Kobza R, Oechslin E, Jenni R, Duru F. Electrocardiographic characteristics at initial diagnosis in patients with isolated left ventricular noncompaction. Am J Cardiol. 2009;104:984–9.CrossRefGoogle Scholar
  8. 8.
    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. Clinical characterization of left ventricular noncompaction in children: a relatively common form of cardiomyopathy. Circulation. 2003;108:2672–8.CrossRefGoogle Scholar
  9. 9.
    Salerno JC, Chun TU, Rutledge JC. Sinus bradycardia, Wolff Parkinson White, and left ventricular noncompaction: an embryologic connection? Pediatr Cardiol. 2008;29:679–82.CrossRefGoogle Scholar
  10. 10.
    Oechslin EN, Attenhofer Jost CH, Rojas JR, Kaufmann PA, Jenni R. Long-term follow-up of 34 adults with isolated left ventricular noncompaction: a distinct cardiomyopathy with poor prognosis. J Am Coll Cardiol. 2000;36:493–500.CrossRefGoogle Scholar
  11. 11.
    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;86:666–71.CrossRefGoogle Scholar
  12. 12.
    Chin TK, Perloff JK, Williams RG, Jue K, Mohrmann R. Isolated noncompaction of left ventricular myocardium. A study of eight cases. Circulation. 1990;82:507–13.CrossRefGoogle Scholar
  13. 13.
    Belanger AR, Miller MA, Donthireddi UR, Najovits AJ, Goldman ME. New classification scheme of left ventricular noncompaction and correlation with ventricular performance. Am J Cardiol. 2008;102:92–6.CrossRefGoogle Scholar
  14. 14.
    Stollberger C, Finsterer J, Blazek G. Left ventricular hypertrabeculation/noncompaction and association with additional cardiac abnormalities and neuromuscular disorders. Am J Cardiol. 2002;90:899–902.CrossRefGoogle Scholar
  15. 15.
    Stollberger C, Gerecke B, Finsterer J, Engberding R. Refinement of echocardiographic criteria for left ventricular noncompaction. Int J Cardiol. 2011;165(3):463–7.CrossRefGoogle Scholar
  16. 16.
    Stollberger C, Winkler-Dworak M, Blazek G, Finsterer J. Prognosis of left ventricular hypertrabeculation/noncompaction is dependent on cardiac and neuromuscular comorbidity. Int J Cardiol. 2007;121:189–93.CrossRefGoogle Scholar
  17. 17.
    Stollberger C, Gerecke B, Finsterer J, Engberding R. Refinement of echocardiographic criteria for left ventricular noncompaction. Int J Cardiol. 2013;165:463–7.CrossRefGoogle Scholar
  18. 18.
    Engberding R, Stollberger C, Ong P, Yelbuz TM, Gerecke BJ, Breithardt G. Isolated non-compaction cardiomyopathy. Dtsch Arztebl Int. 2010;107:206–13.PubMedPubMedCentralGoogle Scholar
  19. 19.
    Gebhard C, Stahli BE, Greutmann M, Biaggi P, Jenni R, Tanner FC. Reduced left ventricular compacta thickness: a novel echocardiographic criterion for non-compaction cardiomyopathy. J Am Soc Echocardiogr. 2012;25:1050–7.CrossRefGoogle Scholar
  20. 20.
    Paterick TE, Umland MM, Jan MF, Ammar KA, Kramer C, Khandheria BK, Seward JB, Tajik AJ. Left ventricular noncompaction: a 25-year odyssey. J Am Soc Echocardiogr. 2012;25:363–75.CrossRefGoogle Scholar
  21. 21.
    Jacquier A, Thuny F, Jop B, Giorgi R, Cohen F, Gaubert JY, Vidal V, Bartoli JM, Habib G, Moulin G. Measurement of trabeculated left ventricular mass using cardiac magnetic resonance imaging in the diagnosis of left ventricular non-compaction. Eur Heart J. 2010;31:1098–104.CrossRefGoogle Scholar
  22. 22.
    Kohli SK, Pantazis AA, Shah JS, Adeyemi B, Jackson G, McKenna WJ, Sharma S, Elliott PM. Diagnosis of left-ventricular non-compaction in patients with left-ventricular systolic dysfunction: time for a reappraisal of diagnostic criteria? Eur Heart J. 2008;29:89–95.CrossRefGoogle Scholar
  23. 23.
    Stollberger C, Finsterer J. Superiority of contrast echocardiography over conventional echocardiography in diagnosing left ventricular hypertrabeculation/noncompaction. Am J Cardiol. 2007;99:145–6.CrossRefGoogle Scholar
  24. 24.
    de Laat LE, Galema TW, Krenning BJ, Roelandt JR. Diagnosis of non-compaction cardiomyopathy with contrast echocardiography. Int J Cardiol. 2004;94:127–8.CrossRefGoogle Scholar
  25. 25.
    Nemes A, Caliskan K, Geleijnse ML, Soliman OI, Vletter WB, ten Cate FJ. Reduced regional systolic function is not confined to the noncompacted segments in noncompaction cardiomyopathy. Int J Cardiol. 2009;134:366–70.CrossRefGoogle Scholar
  26. 26.
    Tufekcioglu O, Aras D, Yildiz A, Topaloglu S, Maden O. Myocardial contraction properties along the long and short axes of the left ventricle in isolated left ventricular non-compaction: pulsed tissue Doppler echocardiography. Eur J Echocardiogr. 2008;9:344–50.PubMedGoogle Scholar
  27. 27.
    Bellavia D, Michelena HI, Martinez M, Pellikka PA, Bruce CJ, Connolly HM, Villarraga HR, Veress G, Oh JK, Miller FA. Speckle myocardial imaging modalities for early detection of myocardial impairment in isolated left ventricular non-compaction. Heart. 2010;96:440–7.CrossRefGoogle Scholar
  28. 28.
    van Dalen BM, Caliskan K, Soliman OI, Kauer F, van der Zwaan HB, Vletter WB, van Vark LC, Ten Cate FJ, Geleijnse ML. Diagnostic value of rigid body rotation in noncompaction cardiomyopathy. J Am Soc Echocardiogr. 2011;24:548–55.CrossRefGoogle Scholar
  29. 29.
    Nemes A, Anwar AM, Caliskan K, Soliman OI, van Dalen BM, Geleijnse ML, ten Cate FJ. Non-compaction cardiomyopathy is associated with mitral annulus enlargement and functional impairment: a real-time three-dimensional echocardiographic study. J Heart Valve Dis. 2008;17:31–5.PubMedGoogle Scholar
  30. 30.
    Caselli S, Autore C, Serdoz A, Santini D, Musumeci MB, Pelliccia A, Agati L. Three-dimensional echocardiographic characterization of patients with left ventricular noncompaction. J Am Soc Echocardiogr. 2011;25(2):203–9.CrossRefGoogle Scholar
  31. 31.
    Nemes A, Geleijnse ML, Krenning BJ, Soliman OI, Anwar AM, Vletter WB, Ten Cate FJ. Usefulness of ultrasound contrast agent to improve image quality during real-time three-dimensional stress echocardiography. Am J Cardiol. 2007;99:275–8.CrossRefGoogle Scholar
  32. 32.
    Krenning BJ, Kirschbaum SW, Soliman OI, Nemes A, van Geuns RJ, Vletter WB, Veltman CE, Ten Cate FJ, Roelandt JR, Geleijnse ML. Comparison of contrast agent-enhanced versus non-contrast agent-enhanced real-time three-dimensional echocardiography for analysis of left ventricular systolic function. Am J Cardiol. 2007;100:1485–9.CrossRefGoogle Scholar
  33. 33.
    van Dalen BM, Bosch JG, Kauer F, Soliman OI, Vletter WB, ten Cate FJ, Geleijnse ML. Assessment of mitral annular velocities by speckle tracking echocardiography versus tissue Doppler imaging: validation, feasibility, and reproducibility. J Am Soc Echocardiogr. 2009;22:1302–8.CrossRefGoogle Scholar
  34. 34.
    McMahon CJ, Pignatelli RH, Nagueh SF, Lee VV, Vaughn W, Valdes SO, Kovalchin JP, Jefferies JL, Dreyer WJ, Denfield SW, Clunie S, Towbin JA, Eidem BW. Left ventricular non-compaction cardiomyopathy in children: characterisation of clinical status using tissue Doppler-derived indices of left ventricular diastolic relaxation. Heart. 2007;93:676–81.CrossRefGoogle Scholar
  35. 35.
    van Dalen BM, Caliskan K, Soliman OI, Nemes A, Vletter WB, Ten Cate FJ, Geleijnse ML. Left ventricular solid body rotation in non-compaction cardiomyopathy: a potential new objective and quantitative functional diagnostic criterion? Eur J Heart Fail. 2008;10:1088–93.CrossRefGoogle Scholar
  36. 36.
    Pacileo G, Baldini L, Limongelli G, Di Salvo G, Iacomino M, Capogrosso C, Rea A, D’Andrea A, Russo MG, Calabro R. Prolonged left ventricular twist in cardiomyopathies: a potential link between systolic and diastolic dysfunction. Eur J Echocardiogr. 2011;12:841–9.CrossRefGoogle Scholar
  37. 37.
    Araujo-Filho JAB, Assuncao AN Jr, Tavares de Melo MD, Biere L, Lima CR, Dantas RN Jr, Nomura CH, Salemi VMC, Jerosch-Herold M, Parga JR. Myocardial T1 mapping and extracellular volume quantification in patients with left ventricular non-compaction cardiomyopathy. Eur Heart J Cardiovasc Imaging. 2018;19:888–95.CrossRefGoogle Scholar
  38. 38.
    Zhou H, Lin X, Fang L, Zhao X, Ding H, Chen W, Xu R, Bai X, Wang Y, Fang Q. 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. 2016;80:1210–6.CrossRefGoogle Scholar
  39. 39.
    Wan J, Zhao S, Cheng H, Lu M, Jiang S, Yin G, Gao X, Yang Y. 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.CrossRefGoogle Scholar
  40. 40.
    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;46:101–5.CrossRefGoogle Scholar
  41. 41.
    Nucifora G, Aquaro GD, Lombardi M. Cardiac magnetic resonance for early detection and risk stratification of patients with non-compaction cardiomyopathy: reply. Eur J Heart Fail. 2011;13:1154.CrossRefGoogle Scholar
  42. 42.
    Dawson DK, Maceira AM, Raj VJ, Graham C, Pennell DJ, Kilner PJ. Regional thicknesses and thickening of compacted and trabeculated myocardial layers of the normal left ventricle studied by cardiovascular magnetic resonance. Circ Cardiovasc Imaging. 2011;4:139–46.CrossRefGoogle Scholar
  43. 43.
    Weir-McCall JR, Yeap PM, Papagiorcopulo C, Fitzgerald K, Gandy SJ, Lambert M, Belch JJ, Cavin I, Littleford R, Macfarlane JA, Matthew SZ, Nicholas RS, Struthers AD, Sullivan F, Waugh SA, White RD, Houston JG. Left ventricular noncompaction: anatomical phenotype or distinct cardiomyopathy? J Am Coll Cardiol. 2016;68:2157–65.CrossRefGoogle Scholar
  44. 44.
    Melendez-Ramirez G, Castillo-Castellon F, Espinola-Zavaleta N, Meave A, Kimura-Hayama ET. Left ventricular noncompaction: a proposal of new diagnostic criteria by multidetector computed tomography. J Cardiovasc Comput Tomogr. 2012;6:346–54.CrossRefGoogle Scholar
  45. 45.
    Anderson RH, Jensen B, Mohun TJ, Petersen SE, Aung N, Zemrak F, Planken RN, MacIver DH. Key questions relating to left ventricular noncompaction cardiomyopathy: is the emperor still wearing any clothes? Can J Cardiol. 2017;33:747–57.CrossRefGoogle Scholar
  46. 46.
    Boyd MT, Seward JB, Tajik AJ, Edwards WD. Frequency and location of prominent left ventricular trabeculations at autopsy in 474 normal human hearts: implications for evaluation of mural thrombi by two-dimensional echocardiography. J Am Coll Cardiol. 1987;9:323–6.CrossRefGoogle Scholar
  47. 47.
    Loukas M, Louis RG Jr, Black B, Pham D, Fudalej M, Sharkees M. False tendons: an endoscopic cadaveric approach. Clin Anat. 2007;20:163–9.CrossRefGoogle Scholar
  48. 48.
    Keren A, Billingham ME, Popp RL. Ventricular aberrant bands and hypertrophic trabeculations. A clinical pathological correlation. Am J Cardiovasc Pathol. 1988;1:369–78.PubMedGoogle Scholar
  49. 49.
    Bhattacharya IS, Dweck M, Gardner A, Jones M, Francis M. Isolated ventricular noncompaction syndrome in a nigerian male: case report and review of the literature. Cardiol Res Pract. 2010;2010:539538.CrossRefGoogle Scholar
  50. 50.
    Towbin JA, Lorts A, Jefferies JL. Left ventricular non-compaction cardiomyopathy. Lancet. 2015;386:813–25.CrossRefGoogle Scholar
  51. 51.
    Saleeb SF, Margossian R, Spencer CT, Alexander ME, Smoot LB, Dorfman AL, Bergersen L, Gauvreau K, Marx GR, Colan SD. Reproducibility of echocardiographic diagnosis of left ventricular noncompaction. J Am Soc Echocardiogr. 2011;25(2):194–202.CrossRefGoogle Scholar
  52. 52.
    Fernandez-Golfin C, Pachon M, Corros C, Bustos A, Cabeza B, Ferreiros J, de Isla LP, Macaya C, Zamorano J. Left ventricular trabeculae: quantification in different cardiac diseases and impact on left ventricular morphological and functional parameters assessed with cardiac magnetic resonance. J Cardiovasc Med. 2009;10:827–33.CrossRefGoogle Scholar
  53. 53.
    Sandhu R, Finkelhor RS, Gunawardena DR, Bahler RC. Prevalence and characteristics of left ventricular noncompaction in a community hospital cohort of patients with systolic dysfunction. Echocardiography. 2008;25:8–12.PubMedGoogle Scholar
  54. 54.
    Elliott P. The 2006 American Heart Association classification of cardiomyopathies is not the gold standard. Circ Heart Fail. 2008;1:77–9.CrossRefGoogle Scholar
  55. 55.
    Maron BJ. The 2006 American Heart Association classification of cardiomyopathies is the gold standard. Circ Heart Fail. 2008;1:72–5.CrossRefGoogle Scholar
  56. 56.
    Gianfagna P, Badano LP, Faganello G, Tosoratti E, Fioretti PM. Additive value of contrast echocardiography for the diagnosis of noncompaction of the left ventricular myocardium. Eur J Echocardiogr. 2006;7:67–70.CrossRefGoogle Scholar
  57. 57.
    Nemes A, Caliskan K, Soliman OI, McGhie JS, Geleijnse ML, ten Cate FJ. Diagnosis of biventricular non-compaction cardiomyopathy by real-time three-dimensional echocardiography. Eur J Echocardiogr. 2009;10:356–7.PubMedGoogle Scholar
  58. 58.
    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. Long-term prognostic value of cardiac magnetic resonance in left ventricle noncompaction: a prospective multicenter study. J Am Coll Cardiol. 2016;68:2166–81.CrossRefGoogle Scholar
  59. 59.
    Caliskan K, Soliman OI, Nemes A, van Domburg RT, Simoons ML, Geleijnse ML. No relationship between left ventricular radial wall motion and longitudinal velocity and the extent and severity of noncompaction cardiomyopathy. Cardiovasc Ultrasound. 2012;10:9.CrossRefGoogle Scholar

Copyright information

© Springer Nature Switzerland AG 2019

Authors and Affiliations

  • Osama I. Soliman
    • 1
  • Jackie McGhie
    • 2
    • 3
  • Folkert J. ten Cate
    • 5
  • Bernard P. Paelinck
    • 4
  • Kadir Caliskan
    • 5
  1. 1.Erasmus MC University Medical CenterThe ThoraxcenterRotterdamThe Netherlands
  2. 2.Thoraxcenter, Department of CardiologyErasmus MC University Medical CenterRotterdamThe Netherlands
  3. 3.The Euro Heart FoundationBrusselsBelgium
  4. 4.Department of CardiologyUniversity of AntwerpAntwerpBelgium
  5. 5.Erasmus MC University Medical Center, Department of CardiologyRotterdamThe Netherlands

Personalised recommendations