Skip to main content
SpringerLink
Log in

Non-compaction Cardiomyopathy: Prevalence, Prognosis, Pathoetiology, Genetics, and Risk of Cardioembolism

  • Prevention of Heart Failure (M St. John Sutton, Section Editor)
  • Published:
Current Heart Failure Reports Aims and scope Submit manuscript

Abstract

Left ventricular non-compaction (LVNC) is thought to arise from arrest of the normal process of trabecular remodeling or “compaction” that takes place during embryonic life and is characterized by the presence of a two-layered ventricular wall, with a compact epicardial layer and a non-compacted endocardial layer. It is an uncommon condition that can occur isolated or in association with other disorders, including congenital heart anomalies and mitochondrial or musculoskeletal disorders. Both familial and sporadic forms are recognized, and several responsible genes have been identified, although only a minority of patients can be successfully genotyped. The diagnosis is usually made by echocardiography, but cardiac magnetic resonance imaging has been used increasingly. Management is mainly empirical and directed at the major clinical manifestations: heart failure, arrhythmias, and systemic embolic events. This article will review the major features of LVNC and present new trends in the diagnosis and management of this intriguing condition.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2
Fig. 3
Fig. 4

Similar content being viewed by others

Abbreviations

ICD:

Implantable cardioverter-defibrillator

LGE:

Late gadolinium enhancement

LV:

Left ventricle

LVNC:

Left ventricular non-compaction

CMR:

Cardiac magnetic resonance imaging

References

Papers of particular interest published recently have been highlighted as: • Of importance

  1. Oechslin E, Jenni R. Left ventricular non-compaction revisited: a distinct phenotype with genetic heterogeneity? Eur Heart J. 2011;32:1446–56.

    Article  PubMed  Google Scholar 

  2. Weiford BC, Subbarao VD, Mulhern KM. Noncompaction of the ventricular myocardium. Circulation. 2004;109:2965–71.

    Article  PubMed  Google Scholar 

  3. Agarwal A, Khandheria BK, Paterick TE, Treiber SC, Bush M, Tajik AJ. Left ventricular noncompaction in patients with bicuspid aortic valve. J Am Soc Echocardiogr. 2013;26:1306–13.

    Article  PubMed  Google Scholar 

  4. Vermeer AM, van Engelen K, Postma AV, Baars MJ, Christiaans I, De Haij S, et al. Ebstein anomaly associated with left ventricular noncompaction: an autosomal dominant condition that can be caused by mutations in MYH7. Am J Med Genet C: Semin Med Genet. 2013;163C:178–84.

    Article  Google Scholar 

  5. Nakashima K, Kusakawa I, Yamamoto T, Hirabayashi S, Hosoya R, Shimizu W, et al. A left ventricular noncompaction in a patient with long QT syndrome caused by a KCNQ1 mutation: a case report. Heart Vessels. 2013;28:126–9.

    Article  PubMed  Google Scholar 

  6. 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.

    Article  CAS  PubMed  Google Scholar 

  7. 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.

    Article  CAS  PubMed  Google Scholar 

  8. 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.

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  9. Ichida F, Tsubata S, Bowles KR, Haneda N, Uese K, Miyawaki T, et al. Novel gene mutations in patients with left ventricular noncompaction or Barth syndrome. Circulation. 2001;103:1256–63.

    Article  CAS  PubMed  Google Scholar 

  10. Kimura K, Takenaka K, Ebihara A, Uno K, Morita H, Nakajima T, et al. Prognostic impact of left ventricular noncompaction in patients with Duchenne/Becker muscular dystrophy—prospective multicenter cohort study. Int J Cardiol. 2013;168:1900–4.

    Article  PubMed  Google Scholar 

  11. Statile CJ, Taylor MD, Mazur W, Cripe LH, King E, Pratt J, et al. Left ventricular noncompaction in Duchenne muscular dystrophy. J Cardiovasc Magn Reson. 2013;15:67.

    Article  PubMed Central  PubMed  Google Scholar 

  12. 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.

    Article  PubMed  Google Scholar 

  13. Richardson P, McKenna W, Bristow M, Maisch B, Mautner B, O’Connell J, et al. Report of the 1995 world health organization/international society and federation of cardiology task force on the definition and classification of cardiomyopathies. Circulation. 1996;93:841–2.

    Article  CAS  PubMed  Google Scholar 

  14. Maron BJ, Towbin JA, Thiene G, Antzelevitch C, Corrado D, Arnett D, 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;113:1807–16.

    Article  PubMed  Google Scholar 

  15. Elliott P, Andersson B, Arbustini E, Bilinska Z, Cecchi F, Charron P, 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;29:270–6.

    Article  PubMed  Google Scholar 

  16. Sedmera D, Pexieder T, Vuillemin M, Thompson RP, Anderson RH. Developmental patterning of the myocardium. Anat Rec. 2000;258:319–37.

    Article  CAS  PubMed  Google Scholar 

  17. Ritter M, Oechslin E, Sutsch G, Attenhofer C, Schneider J, Jenni R. Isolated noncompaction of the myocardium in adults. Mayo Clin Proc. 1997;72:26–31.

    Article  CAS  PubMed  Google Scholar 

  18. Zambrano E, Marshalko SJ, Jaffe CC, Hui P. Isolated noncompaction of the ventricular myocardium: clinical and molecular aspects of a rare cardiomyopathy. Lab Invest. 2002;82:117–22.

    Article  PubMed  Google Scholar 

  19. Nucifora G, Aquaro GD, Masci PG, Pingitore A, Lombardi M. Magnetic resonance assessment of prevalence and correlates of right ventricular abnormalities in isolated left ventricular noncompaction. Am J Cardiol. 2014;113:142–6.

    Article  PubMed  Google Scholar 

  20. Stacey RB, Andersen M, Haag J, Hall ME, McLeod G, Upadhya B, et al. Right ventricular morphology and systolic function in left ventricular noncompaction cardiomyopathy. Am J Cardiol. 2014;113:1018–23. A study on the structural abnormalities found in the right ventricle and its prognostic significance.

    Article  PubMed  Google Scholar 

  21. Bleyl SB, Mumford BR, Brown-Harrison MC, Pagotto LT, Carey JC, Pysher TJ, et al. Xq28-linked noncompaction of the left ventricular myocardium: prenatal diagnosis and pathologic analysis of affected individuals. Am J Med Genet. 1997;72:257–65.

    Article  CAS  PubMed  Google Scholar 

  22. Almeida AG, Pinto FJ. Non-compaction cardiomyopathy. Heart. 2013;99:1535–42.

    Article  PubMed  Google Scholar 

  23. 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.

    Article  CAS  PubMed  Google Scholar 

  24. Pignatelli RH, McMahon CJ, Dreyer WJ, Denfield SW, Price J, Belmont JW, et al. Clinical characterization of left ventricular noncompaction in children: a relatively common form of cardiomyopathy. Circulation. 2003;108:2672–8.

    Article  PubMed  Google Scholar 

  25. Toste A, Branco LM, Galrinho A, Lousinha A, Fiarresga A, Oliveira MM, et al. Noncompaction cardiomyopathy—a review of eight cases. Rev Port Cardiol. 2010;29:1847–64.

    PubMed  Google Scholar 

  26. Ichida F, Hamamichi Y, Miyawaki T, Ono Y, Kamiya T, Akagi T, et al. Clinical features of isolated noncompaction of the ventricular myocardium: long-term clinical course, hemodynamic properties, and genetic background. J Am Coll Cardiol. 1999;34:233–40.

    Article  CAS  PubMed  Google Scholar 

  27. Stollberger C, Blazek G, Winkler-Dworak M, Finsterer J. Sex differences in left ventricular noncompaction in patients with and without neuromuscular disorders. Rev Esp Cardiol (Engl Ed). 2008;61:130–6.

    Article  Google Scholar 

  28. Ackerman MJ, Priori SG, Willems S, Berul C, Brugada R, Calkins H, et al. HRS/EHRA expert consensus statement on the state of genetic testing for the channelopathies and cardiomyopathies: this document was developed as a partnership between the Heart Rhythm Society (HRS) and the European Heart Rhythm Association (EHRA). Europace. 2011;13:1077–109.

    Article  PubMed  Google Scholar 

  29. Hoedemaekers YM, Caliskan K, Michels M, Frohn-Mulder I, van der Smagt JJ, Phefferkorn JE, et al. The importance of genetic counseling, DNA diagnostics, and cardiologic family screening in left ventricular noncompaction cardiomyopathy. Circ Cardiovasc Genet. 2010;3:232–9.

    Article  PubMed  Google Scholar 

  30. Cortez-Dias N, Varela MG, Sargento L, Brito D, Almeida A, Cerqueira R, et al. Left ventricular non-compaction: a new mutation predisposing to reverse remodeling? Rev Port Cardiol. 2009;28:185–94.

    PubMed  Google Scholar 

  31. Vatta M, Mohapatra B, Jimenez S, Sanchez X, Faulkner G, Perles Z, et al. Mutations in Cypher/ZASP in patients with dilated cardiomyopathy and left ventricular non-compaction. J Am Coll Cardiol. 2003;42:2014–27.

    Article  CAS  PubMed  Google Scholar 

  32. Finsterer J, Zarrouk-Mahjoub S. Left ventricular noncompaction associated with a compound heterozygous MYBPC3 mutation. Eur J Med Genet. 2014;57:349.

    Article  PubMed  Google Scholar 

  33. Finsterer J, Stollberger C, Brandau O, Laccone F, Bichler K, Laing NG. Novel MYH7 mutation associated with mild myopathy but life-threatening ventricular arrhythmias and noncompaction. Int J Cardiol. 2014;173:532–5.

    Article  PubMed  Google Scholar 

  34. Schaefer E, Helms P, Marcellin L, Desprez P, Billaud P, Chanavat V, et al. Next-generation sequencing (NGS) as a fast molecular diagnosis tool for left ventricular noncompaction in an infant with compound mutations in the MYBPC3 gene. Eur J Med Genet. 2014;57:129–32.

    Article  PubMed  Google Scholar 

  35. Klaassen S, Probst S, Oechslin E, Gerull B, Krings G, Schuler P, et al. Mutations in sarcomere protein genes in left ventricular noncompaction. Circulation. 2008;117:2893–901.

    Article  CAS  PubMed  Google Scholar 

  36. Luxan G, Casanova JC, Martinez-Poveda B, Prados B, D’Amato G, MacGrogan D, et al. Mutations in the NOTCH pathway regulator MIB1 cause left ventricular noncompaction cardiomyopathy. Nat Med. 2013;19:193–201. A report of a new gene associated with LVNC.

    Article  CAS  PubMed  Google Scholar 

  37. Habib G, Charron P, Eicher JC, Giorgi R, Donal E, Laperche T, et al. 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.

    Article  PubMed  Google Scholar 

  38. Agmon Y, Connolly HM, Olson LJ, Khandheria BK, Seward JB. Noncompaction of the ventricular myocardium. J Am Soc Echocardiogr. 1999;12:859–63.

    Article  CAS  PubMed  Google Scholar 

  39. Stanton C, Bruce C, Connolly H, Brady P, Syed I, Hodge D, et al. Isolated left ventricular noncompaction syndrome. Am J Cardiol. 2009;104:1135–8.

    Article  PubMed  Google Scholar 

  40. Murphy RT, Thaman R, Blanes JG, Ward D, Sevdalis E, Papra E, et al. Natural history and familial characteristics of isolated left ventricular non-compaction. Eur Heart J. 2005;26:187–92.

    Article  PubMed  Google Scholar 

  41. Lofiego C, Biagini E, Pasquale F, Ferlito M, Rocchi G, Perugini E, et al. Wide spectrum of presentation and variable outcomes of isolated left ventricular non-compaction. Heart. 2007;93:65–71.

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  42. Aras D, Tufekcioglu O, Ergun K, Ozeke O, Yildiz A, Topaloglu S, 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;12:726–33.

    Article  PubMed  Google Scholar 

  43. Jorge C, Marques JS, Miltenberger-Miltenyi G, Pinto FJ. Hypertrophic cardiomyopathy or non-compaction? How the first impression can be wrong. Int J Cardiol. 2012;158:e53–4.

    Article  PubMed  Google Scholar 

  44. Jenni R, Wyss CA, Oechslin EN, Kaufmann PA. Isolated ventricular noncompaction is associated with coronary microcirculatory dysfunction. J Am Coll Cardiol. 2002;39:450–4.

    Article  PubMed  Google Scholar 

  45. 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.

    Article  PubMed  Google Scholar 

  46. Steffel J, Hurlimann D, Namdar M, Despotovic D, Kobza R, Wolber T, et al. Long-term follow-up of patients with isolated left ventricular noncompaction: role of electrocardiography in predicting poor outcome. Circ J. 2011;75:1728–34.

    Article  PubMed  Google Scholar 

  47. Tamborini G, Pepi M, Celeste F, Muratori M, Susini F, Maltagliati A, et al. Incidence and characteristics of left ventricular false tendons and trabeculations in the normal and pathologic heart by second harmonic echocardiography. J Am Soc Echocardiogr. 2004;17:367–74.

    Article  PubMed  Google Scholar 

  48. Stollberger C, Finsterer J. Left ventricular hypertrabeculation/noncompaction. J Am Soc Echocardiogr. 2004;17:91–100.

    Article  PubMed  Google Scholar 

  49. Saleeb SF, Margossian R, Spencer CT, Alexander ME, Smoot LB, Dorfman AL, et al. Reproducibility of echocardiographic diagnosis of left ventricular noncompaction. J Am Soc Echocardiogr. 2012;25:194–202. A report on the limitations of echocardiography for the diagnosis of LVNC.

    Article  PubMed  Google Scholar 

  50. Kohli SK, Pantazis AA, Shah JS, Adeyemi B, Jackson G, McKenna WJ, 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;29:89–95.

    Article  PubMed  Google Scholar 

  51. Koo BK, Choi D, Ha JW, Kang SM, Chung N, Cho SY. Isolated noncompaction of the ventricular myocardium: contrast echocardiographic findings and review of the literature. Echocardiography. 2002;19:153–6.

    Article  PubMed  Google Scholar 

  52. Eidem BW. Noninvasive evaluation of left ventricular noncompaction: what’s new in 2009? Pediatr Cardiol. 2009;30:682–9.

    Article  PubMed  Google Scholar 

  53. McMahon CJ, Pignatelli RH, Nagueh SF, Lee VV, Vaughn W, Valdes SO, 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;93:676–81.

    Article  PubMed Central  PubMed  Google Scholar 

  54. Kalapos A, Domsik P, Forster T, Nemes A (2013) Left ventricular strain reduction is not confined to the noncompacted segments in noncompaction cardiomyopathy—insights from the three-dimensional speckle tracking echocardiographic MAGYAR-Path Study. Echocardiography

  55. van Dalen BM, Caliskan K, Soliman OI, Nemes A, Vletter WB, Ten Cate FJ, et al. 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.

    Article  PubMed  Google Scholar 

  56. Peters F, Khandheria BK, Libhaber E, Maharaj N, Dos Santos C, Matioda H, et al. Left ventricular twist in left ventricular noncompaction. Eur Heart J Cardiovasc Imaging. 2014;Study on the prevalence and impact of the abnormalities of left ventricular rotation:48–55. A study on the prevalence and impact of the abnormalities of left ventricular rotation.

    Article  Google Scholar 

  57. Caselli S, Autore C, Serdoz A, Santini D, Musumeci MB, Pelliccia A, et al. Three-dimensional echocardiographic characterization of patients with left ventricular noncompaction. J Am Soc Echocardiogr. 2012;25:203–9. Characterization of LVNC with three-dimensional echocardiography.

    Article  PubMed  Google Scholar 

  58. Petersen SE, Selvanayagam JB, Wiesmann F, Robson MD, Francis JM, Anderson RH, et al. Left ventricular non-compaction: insights from cardiovascular magnetic resonance imaging. J Am Coll Cardiol. 2005;46:101–5.

    Article  PubMed  Google Scholar 

  59. Jacquier A, Thuny F, Jop B, Giorgi R, Cohen F, Gaubert JY, 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;31:1098–104.

    Article  PubMed  Google Scholar 

  60. Stacey RB, Andersen MM, St Clair M, Hundley WG, Thohan V. Comparison of systolic and diastolic criteria for isolated LV noncompaction in cmr. JACC Cardiovasc Imaging. 2013;6:931–40.

    Article  PubMed  Google Scholar 

  61. Captur G, Muthurangu V, Cook C, Flett AS, Wilson R, Barison A, et al. Quantification of left ventricular trabeculae using fractal analysis. J Cardiovasc Magn Reson. 2013;15:36. Describes a novel technique for the diagnosis of LVNC using CMR.

    Article  PubMed Central  PubMed  Google Scholar 

  62. Nucifora G, Aquaro GD, Pingitore A, Masci PG, Lombardi M. Myocardial fibrosis in isolated left ventricular non-compaction and its relation to disease severity. Eur J Heart Fail. 2011;13:170–6. Significance of late gadolinium enhancement.

    Article  PubMed  Google Scholar 

  63. Conces Jr DJ, Ryan T, Tarver RD. Noncompaction of ventricular myocardium: CT appearance. AJR Am J Roentgenol. 1991;156:717–8.

    Article  PubMed  Google Scholar 

  64. Hamamichi Y, Ichida F, Hashimoto I, Uese KH, Miyawaki T, Tsukano S, et al. Isolated noncompaction of the ventricular myocardium: ultrafast computed tomography and magnetic resonance imaging. Int J Cardiovasc Imaging. 2001;17:305–14.

    Article  CAS  PubMed  Google Scholar 

  65. Sidhu MS, Uthamalingam S, Ahmed W, Engel LC, Vorasettakarnkij Y, Lee AM, et al. Defining left ventricular noncompaction using cardiac computed tomography. J Thorac Imaging. 2014;29:60–6.

    Article  PubMed  Google Scholar 

  66. 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.

    Article  PubMed  Google Scholar 

  67. Erer HB, Guvenc TS, Kemik AS, Yilmaz H, Kul S, Altay S, et al. Assessment of tenascin-C levels in ventricular noncompaction/hypertrabeculation patients: A cross-sectional study. Echocardiography. 2014;31:203–8.

    Article  PubMed  Google Scholar 

  68. 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.

    Article  CAS  PubMed  Google Scholar 

  69. Delgado A, Moreira D, Rodrigues B, Correia E, Gama P, Cabral C, et al. Hypertrophic cardiomyopathy associated with left ventricular noncompaction cardiomyopathy and coronary fistulae: a case report. One genotype, three phenotypes? Rev Port Cardiol. 2013;32:919–24.

    PubMed  Google Scholar 

  70. McMurray JJ, Adamopoulos S, Anker SD, Auricchio A, Bohm M, Dickstein K, et al. ESC guidelines for the diagnosis and treatment of acute and chronic heart failure 2012. Eur Heart J. 2012;33:1787–847.

    Article  PubMed  Google Scholar 

  71. Toyono M, Kondo C, Nakajima Y, Nakazawa M, Momma K, Kusakabe K. Effects of carvedilol on left ventricular function, mass, and scintigraphic findings in isolated left ventricular non-compaction. Heart. 2001;86:E4.

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  72. Penela D, Bijnens B, Doltra A, Silva E, Castel MA, Berruezo A, et al. Noncompaction cardiomyopathy is associated with mechanical dyssynchrony: a potential underlying mechanism for favorable response to cardiac resynchronization therapy. J Card Fail. 2013;19:80–6.

    Article  PubMed  Google Scholar 

  73. Conraads V, Paelinck B, Vorlat A, Goethals M, Jacobs W, Vrints C. Isolated non-compaction of the left ventricle: a rare indication for transplantation. J Heart Lung Transplant. 2001;20:904–7.

    Article  CAS  PubMed  Google Scholar 

  74. Brignole M, Auricchio A, Baron-Esquivias G, Bordachar P, Boriani G, Breithardt O-A, et al. 2013 ESC guidelines on cardiac pacing and cardiac resynchronization therapy. Eur Heart J. 2013;34:2281–329.

    Article  PubMed  Google Scholar 

  75. Kobza R, Jenni R, Erne P, Oechslin E, Duru F. Implantable cardioverter-defibrillators in patients with left ventricular noncompaction. Pacing Clin Electrophysiol. 2008;31:461–7.

    Article  PubMed  Google Scholar 

  76. 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.

    Article  PubMed  Google Scholar 

  77. Stollberger C, Wegner C, Finsterer J. CHADS2- and CHA2DS2VASc scores and embolic risk in left ventricular hypertrabeculation/noncompaction. J Stroke Cerebrovasc Dis. 2013;22:709–12.

    Article  PubMed  Google Scholar 

  78. Caliskan K, Michels M, Geleijnse ML, van Domburg RT, van der Boon R, Balk AH, et al. Frequency of asymptomatic disease among family members with noncompaction cardiomyopathy. Am J Cardiol. 2012;110:1512–7.

    Article  PubMed  Google Scholar 

  79. Tian T, Liu Y, Gao L, Wang J, Sun K, Zou Y, Wang L, Zhang L, Li Y, Xiao Y, Song L, Zhou X. Isolated left ventricular noncompaction: clinical profile and prognosis in 106 adult patients. Heart Vessels. 2013. One of the latest published series.

  80. Oliveira SM, Martins E, Oliveira A, Pinho T, Gavina C, Faria T, et al. Cardiac 123I-MIBG scintigraphy and arrhythmic risk in left ventricular noncompaction. Rev Port Cardiol. 2012;31:247–50.

    PubMed  Google Scholar 

  81. Captur G, Flett AS, Jacoby DL, Moon JC. Left ventricular non-noncompaction: the mitral valve prolapse of the 21st century? Int J Cardiol. 2013;164:3–6.

    Article  PubMed  Google Scholar 

  82. Luedde M, Ehlermann P, Weichenhan D, Will R, Zeller R, Rupp S, et al. Severe familial left ventricular non-compaction cardiomyopathy due to a novel troponin T (TNNT2) mutation. Cardiovasc Res. 2010;86:452–60.

    Article  CAS  PubMed  Google Scholar 

Download references

Compliance with Ethics Guidelines

Conflict of Interest

Pedro Carrilho-Ferreira, Ana G. Almeida, and Fausto J. Pinto declare that they have no conflict of interest.

Human and Animal Rights and Informed Consent

This article does not contain any studies with human or animal subjects performed by any of the authors.

Author information

Authors and Affiliations

  1. Lisbon School of Medicine, Lisbon Academic Medical Center, CCUL, Lisbon University, Lisbon, Portugal

    Pedro Carrilho-Ferreira, Ana G. Almeida & Fausto J. Pinto

  2. Department of Cardiology, Santa Maria University Hospital, Centro Hospitalar Lisboa Norte, Lisbon, Portugal

    Pedro Carrilho-Ferreira, Ana G. Almeida & Fausto J. Pinto

Authors
  1. Pedro Carrilho-Ferreira
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Ana G. Almeida
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Fausto J. Pinto
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Fausto J. Pinto.

Electronic Supplementary Material

Below is the link to the electronic supplementary material.

Cine 4-chamber view from a transthoracic echocardiogram showing the typical two-layered myocardium, with prominent trabeculations and deep recesses involving the apical segments of both the septal and lateral walls and the mid-lateral segment (AVI 7.02 MB)

Cine zoomed 4-chamber view from a contrast echocardiogram demonstrating the presence of contrast as expressing blood flow in the myocardial recesses (AVI 12.1 MB)

Cine 2-chamber view from a cardiac magnetic resonance imaging showing non-compaction with the two-layered myocardium at the apical segments of both the inferior and anterior walls and the mid-anterior segment (AVI 2.89 MB)

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Carrilho-Ferreira, P., Almeida, A.G. & Pinto, F.J. Non-compaction Cardiomyopathy: Prevalence, Prognosis, Pathoetiology, Genetics, and Risk of Cardioembolism. Curr Heart Fail Rep 11, 393–403 (2014). https://doi.org/10.1007/s11897-014-0227-3

Download citation

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s11897-014-0227-3

Keywords

Search

Navigation