Abstract
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.
Similar content being viewed by others
References
Sedmera D, Pexieder T, Vuillemin M, Thompson RP, Anderson RH (2000) Developmental patterning of the myocardium. Anat Rec 258:319–337
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 108:2672–2678
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 34:233–240
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 68:2166–2181
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 113:142–146
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 62:2046–2072
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 12:726–733
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 104:1135–1138
Oechslin E, Jenni R (2018) Left ventricular noncompaction. J Am Coll Cardiol 71:723–726
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 130:475–483
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 99:401–408
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 68:431–438
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 116:801–808
Arbustini E, Weidemann F, Hall JL (2014) Left ventricular noncompaction. J Am Coll Cardiol 64:1840–1850
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 25:26–28
Stähli BE et al (2013) Left ventricular non-compaction: prevalence in congenital heart disease. Int J Cardiol 167:2477–2481
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 27:28–32
Arunamata A, Punn R, Cuneo B, Bharati S, Silverman NH (2012) Echocardiographic diagnosis and prognosis of fetal left ventricular noncompaction. J Am Soc Echocardiogr 25:112–120
Hussein A, Karimianpour A, Collier P, Krasuski RA (2015) Isolated noncompaction of the left ventricle in adults. J Am Coll Cardiol 66:578–585
Chin TK, Perloff JK, Williams RG, Jue K, Mohrmann R (1990) Isolated noncompaction of left ventricular myocardium. A study of eight cases. Circulation 82:507–513
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 86:666–671
Stöllberger C, Finsterer J, Blazek G (2002) Left ventricular hypertrabeculation/noncompaction and association with additional cardiac abnormalities and neuromuscular disorders. Am J Cardiol 90:899–902
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 102:92–96
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 46:101–105
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 31:1098–1104
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 22:2699–2709
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 15:36
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 6:931–940
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 5:357–366
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 68:2157–2165
Oechslin E, Jenni R (2011) Left ventricular non-compaction revisited: a distinct phenotype with genetic heterogeneity? Eur Heart J 32:1446–1456
Towbin JA, Lorts A, Jefferies JL (2015) Left ventricular non-compaction cardiomyopathy. Lancet 386:813–825
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 167:598–599
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 86:452–460
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 25:203–209
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 13:170–176
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 15:20
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 19:567–573
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 33:2013–2020
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 117:1516–1523
Stöllberger C, Finsterer J (2006) Pitfalls in the diagnosis of left ventricular hypertrabeculation/non-compaction. Postgrad Med J 82:679–683
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:e006174
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 246:68–73
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):102
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. https://doi.org/10.1016/j.jcmg.2018.02.006
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 11:48–59
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:e003285
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 80:1210–1216
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. https://doi.org/10.1093/ehjci/jey022
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) 14:677–680
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 29:60–66
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 17:405–424
Moric-Janiszewska E, Markiewicz-Łoskot G (2008) Genetic heterogeneity of left-ventricular noncompaction cardiomyopathy. Clin Cardiol 31:201–204
Zaragoza MV, Arbustini E, Narula J (2007) Noncompaction of the left ventricle: primary cardiomyopathy with an elusive genetic etiology. Curr Opin Pediatr 19:619–627
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 38:3449–3460
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:e006210
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 71:711–722
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:e39
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 36:493–500
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 14:166–175
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 108:1021–1023
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):18
Acknowledgments
This study was supported by the CRTrieste, FINCANTIERI, and GENERALI Foundations to Heart Muscle Diseases Research at Cardiovascular Department, University Hospital of Trieste.
Author information
Authors and Affiliations
Corresponding author
Ethics declarations
Conflict of interest
The authors declare that they have no conflict of interest.
Additional information
Publisher’s Note
Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.
Rights and permissions
About this article
Cite this article
Negri, F., De Luca, A., Fabris, E. et al. Left ventricular noncompaction, morphological, and clinical features for an integrated diagnosis. Heart Fail Rev 24, 315–323 (2019). https://doi.org/10.1007/s10741-018-9763-3
Published:
Issue Date:
DOI: https://doi.org/10.1007/s10741-018-9763-3