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Current Heart Failure Reports

, Volume 11, Issue 4, pp 393–403 | Cite as

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

  • Pedro Carrilho-Ferreira
  • Ana G. Almeida
  • Fausto J. PintoEmail author
Prevention of Heart Failure (M St. John Sutton, Section Editor)

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.

Keywords

Left ventricular non-compaction Pathogenesis Epidemiology Genetics Clinical presentation Diagnosis Management Natural history Prognosis 

Abbreviations

ICD

Implantable cardioverter-defibrillator

LGE

Late gadolinium enhancement

LV

Left ventricle

LVNC

Left ventricular non-compaction

CMR

Cardiac magnetic resonance imaging

Notes

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.

Supplementary material

Video 1

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)

Video 2

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)

Video 3

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)

References

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

  1. 1.
    Oechslin E, Jenni R. Left ventricular non-compaction revisited: a distinct phenotype with genetic heterogeneity? Eur Heart J. 2011;32:1446–56.PubMedCrossRefGoogle Scholar
  2. 2.
    Weiford BC, Subbarao VD, Mulhern KM. Noncompaction of the ventricular myocardium. Circulation. 2004;109:2965–71.PubMedCrossRefGoogle Scholar
  3. 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.PubMedCrossRefGoogle Scholar
  4. 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.CrossRefGoogle Scholar
  5. 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.PubMedCrossRefGoogle Scholar
  6. 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.PubMedCrossRefGoogle Scholar
  7. 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.PubMedCrossRefGoogle Scholar
  8. 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.PubMedCentralPubMedCrossRefGoogle Scholar
  9. 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.PubMedCrossRefGoogle Scholar
  10. 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.PubMedCrossRefGoogle Scholar
  11. 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.PubMedCentralPubMedCrossRefGoogle Scholar
  12. 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.PubMedCrossRefGoogle Scholar
  13. 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.PubMedCrossRefGoogle Scholar
  14. 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.PubMedCrossRefGoogle Scholar
  15. 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.PubMedCrossRefGoogle Scholar
  16. 16.
    Sedmera D, Pexieder T, Vuillemin M, Thompson RP, Anderson RH. Developmental patterning of the myocardium. Anat Rec. 2000;258:319–37.PubMedCrossRefGoogle Scholar
  17. 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.PubMedCrossRefGoogle Scholar
  18. 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.PubMedCrossRefGoogle Scholar
  19. 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.PubMedCrossRefGoogle Scholar
  20. 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. PubMedCrossRefGoogle Scholar
  21. 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.PubMedCrossRefGoogle Scholar
  22. 22.
    Almeida AG, Pinto FJ. Non-compaction cardiomyopathy. Heart. 2013;99:1535–42.PubMedCrossRefGoogle Scholar
  23. 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.PubMedCrossRefGoogle Scholar
  24. 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.PubMedCrossRefGoogle Scholar
  25. 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.PubMedGoogle Scholar
  26. 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.PubMedCrossRefGoogle Scholar
  27. 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.CrossRefGoogle Scholar
  28. 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.PubMedCrossRefGoogle Scholar
  29. 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.PubMedCrossRefGoogle Scholar
  30. 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.PubMedGoogle Scholar
  31. 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.PubMedCrossRefGoogle Scholar
  32. 32.
    Finsterer J, Zarrouk-Mahjoub S. Left ventricular noncompaction associated with a compound heterozygous MYBPC3 mutation. Eur J Med Genet. 2014;57:349.PubMedCrossRefGoogle Scholar
  33. 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.PubMedCrossRefGoogle Scholar
  34. 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.PubMedCrossRefGoogle Scholar
  35. 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.PubMedCrossRefGoogle Scholar
  36. 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.PubMedCrossRefGoogle Scholar
  37. 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.PubMedCrossRefGoogle Scholar
  38. 38.
    Agmon Y, Connolly HM, Olson LJ, Khandheria BK, Seward JB. Noncompaction of the ventricular myocardium. J Am Soc Echocardiogr. 1999;12:859–63.PubMedCrossRefGoogle Scholar
  39. 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.PubMedCrossRefGoogle Scholar
  40. 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.PubMedCrossRefGoogle Scholar
  41. 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.PubMedCentralPubMedCrossRefGoogle Scholar
  42. 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.PubMedCrossRefGoogle Scholar
  43. 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.PubMedCrossRefGoogle Scholar
  44. 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.PubMedCrossRefGoogle Scholar
  45. 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.PubMedCrossRefGoogle Scholar
  46. 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.PubMedCrossRefGoogle Scholar
  47. 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.PubMedCrossRefGoogle Scholar
  48. 48.
    Stollberger C, Finsterer J. Left ventricular hypertrabeculation/noncompaction. J Am Soc Echocardiogr. 2004;17:91–100.PubMedCrossRefGoogle Scholar
  49. 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.PubMedCrossRefGoogle Scholar
  50. 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.PubMedCrossRefGoogle Scholar
  51. 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.PubMedCrossRefGoogle Scholar
  52. 52.
    Eidem BW. Noninvasive evaluation of left ventricular noncompaction: what’s new in 2009? Pediatr Cardiol. 2009;30:682–9.PubMedCrossRefGoogle Scholar
  53. 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.PubMedCentralPubMedCrossRefGoogle Scholar
  54. 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 Google Scholar
  55. 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.PubMedCrossRefGoogle Scholar
  56. 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. CrossRefGoogle Scholar
  57. 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. PubMedCrossRefGoogle Scholar
  58. 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.PubMedCrossRefGoogle Scholar
  59. 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.PubMedCrossRefGoogle Scholar
  60. 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.PubMedCrossRefGoogle Scholar
  61. 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. PubMedCentralPubMedCrossRefGoogle Scholar
  62. 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. PubMedCrossRefGoogle Scholar
  63. 63.
    Conces Jr DJ, Ryan T, Tarver RD. Noncompaction of ventricular myocardium: CT appearance. AJR Am J Roentgenol. 1991;156:717–8.PubMedCrossRefGoogle Scholar
  64. 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.PubMedCrossRefGoogle Scholar
  65. 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.PubMedCrossRefGoogle Scholar
  66. 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.PubMedCrossRefGoogle Scholar
  67. 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.PubMedCrossRefGoogle Scholar
  68. 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.PubMedCrossRefGoogle Scholar
  69. 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.PubMedGoogle Scholar
  70. 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.PubMedCrossRefGoogle Scholar
  71. 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.PubMedCentralPubMedCrossRefGoogle Scholar
  72. 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.PubMedCrossRefGoogle Scholar
  73. 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.PubMedCrossRefGoogle Scholar
  74. 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.PubMedCrossRefGoogle Scholar
  75. 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.PubMedCrossRefGoogle Scholar
  76. 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.PubMedCrossRefGoogle Scholar
  77. 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.PubMedCrossRefGoogle Scholar
  78. 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.PubMedCrossRefGoogle Scholar
  79. 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. Google Scholar
  80. 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.PubMedGoogle Scholar
  81. 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.PubMedCrossRefGoogle Scholar
  82. 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.PubMedCrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media New York 2014

Authors and Affiliations

  • Pedro Carrilho-Ferreira
    • 1
    • 2
  • Ana G. Almeida
    • 1
    • 2
  • Fausto J. Pinto
    • 1
    • 2
    Email author
  1. 1.Lisbon School of Medicine, Lisbon Academic Medical Center, CCULLisbon UniversityLisbonPortugal
  2. 2.Department of CardiologySanta Maria University Hospital, Centro Hospitalar Lisboa NorteLisbonPortugal

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