European Heart Journal Advance Access published online on November 30, 2004
European Heart Journal, doi:10.1093/eurheartj/ehi025
Copyright © 2004 by the European Society of Cardiology.
| ||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
1 The Heart Hospital, University College London, 16-18 Westmoreland Street, London W1G 8PH, UK
* To whom correspondence should be addressed. Aims Non-compaction of the left ventricle (LVNC) is a disorder of endomyocardial morphogenesis that results in multiple trabeculations in the left ventricular myocardium. The current literature suggests that LVNC in adults is rare and associated with a poor prognosis. Given that the disorder is present at birth and that several studies have reported asymptomatic familial disease in some patients, we hypothesized that there is a long pre-clinical phase of the disease. The aim of this study was to define the prognosis and familial incidence of LVNC. Methods and results This study cohort comprised 45 patients (mean age at diagnosis 37 years) consecutively identified at a referral centre for cardiomyopathy over a 10-year period. Twenty-eight patients (62%) had dyspnoea at presentation; 41 (91%) an abnormal ECG; and 30 (66%) left ventricular dilatation and impaired systolic function. Nine patients (20%) had non-sustained ventricular tachycardia on 24 h Holter monitoring. Mean survival from death or transplantation was 97% at 46 months. There were three thromboembolic events in two patients (4%). On systematic family screening, 8 of 32 (25%) asymptomatic relatives had a range of echocardiographic abnormalities, including LVNC, LVNC with impaired systolic function, and left ventricular enlargement without LVNC. Conclusion This study demonstrates that LVNC is associated with a better prognosis than previously reported. In patients with familial disease, relatives may have features consistent with dilated cardiomyopathy rather than LVNC.
Clinical research
Natural history and familial characteristics of isolated left ventricular non-compaction
Perry M. Elliott, E-mail: pelliott{at}doctors.org.uk
Abstract 
CiteULike 
Connotea 
Del.icio.us What's this?
This article has been cited by other articles:
|
|
 |
|
  L. M. Dellefave, P. Pytel, S. Mewborn, B. Mora, D. L. Guris, S. Fedson, D. Waggoner, I. Moskowitz, and E. M. McNally Sarcomere Mutations in Cardiomyopathy With Left Ventricular Hypertrabeculation Circ Cardiovasc Genet, October 1, 2009; 2(5): 442 - 449. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 G. Paparella, L. Capulzini, C. de Asmundis, A. Francesconi, A. Sarkozy, G. Chierchia, and P. Brugada Electro-anatomical mapping in a patient with isolated left ventricular non-compaction and left ventricular tachycardia Europace, September 1, 2009; 11(9): 1227 - 1229. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
K. Caliskan, A. Kardos, and T. Szili-Torok Empty handed: a call for an international registry of risk stratification to reduce the 'sudden-ness' of death in patients with non-compaction cardiomyopathy Europace, September 1, 2009; 11(9): 1138 - 1139. [Full Text] [PDF]
|
|
|
|
 |
|
 A. Garnier and G. Girod Cardiac re-synchronization therapy in a patient with isolated ventricular non-compaction: a case report Eur J Echocardiogr, July 1, 2009; 10(5): 713 - 715. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 S B Nair and R S Khattar Isolated left ventricular non-compaction: an emerging cause of heart failure in adults Postgrad. Med. J., April 1, 2009; 85(1002): 202 - 207. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 E. R. Behr, C. Dalageorgou, M. Christiansen, P. Syrris, S. Hughes, M. T. Tome Esteban, E. Rowland, S. Jeffery, and W. J. McKenna Sudden arrhythmic death syndrome: familial evaluation identifies inheritable heart disease in the majority of families Eur. Heart J., July 1, 2008; 29(13): 1670 - 1680. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 S. Klaassen, S. Probst, E. Oechslin, B. Gerull, G. Krings, P. Schuler, M. Greutmann, D. Hurlimann, M. Yegitbasi, L. Pons, et al. Mutations in Sarcomere Protein Genes in Left Ventricular Noncompaction Circulation, June 3, 2008; 117(22): 2893 - 2901. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
O. Tufekcioglu, D. Aras, A. Yildiz, S. Topaloglu, and O. Maden 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, May 1, 2008; 9(3): 344 - 350. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 J. D. Dodd and R. C. Cury Reply Am. J. Roentgenol., April 1, 2008; 190(4): W274 - W274. [Full Text] [PDF]
|
|
|
|
|
|
S. K. Kohli, A. A. Pantazis, J. S. Shah, B. Adeyemi, G. Jackson, W. J. McKenna, S. Sharma, and P. M. Elliott Diagnosis of left-ventricular non-compaction in patients with left-ventricular systolic dysfunction: time for a reappraisal of diagnostic criteria? Eur. Heart J., January 1, 2008; 29(1): 89 - 95. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
J. D. Dodd, G. Holmvang, U. Hoffmann, M. Ferencik, S. Abbara, T. J. Brady, and R. C. Cury Quantification of Left Ventricular Noncompaction and Trabecular Delayed Hyperenhancement with Cardiac MRI: Correlation with Clinical Severity Am. J. Roentgenol., October 1, 2007; 189(4): 974 - 980. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 N. H. Robin, P. B. Tabereaux, R. Benza, and B. R. Korf Genetic Testing in Cardiovascular Disease J. Am. Coll. Cardiol., August 21, 2007; 50(8): 727 - 737. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
L. Monserrat, M. Hermida-Prieto, X. Fernandez, I. Rodriguez, C. Dumont, L. Cazon, M. G. Cuesta, C. Gonzalez-Juanatey, J. Peteiro, N. Alvarez, et al. Mutation in the alpha-cardiac actin gene associated with apical hypertrophic cardiomyopathy, left ventricular non-compaction, and septal defects Eur. Heart J., August 2, 2007; 28(16): 1953 - 1961. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
G. Fazio, L. Sutera, G. Corrado, and S. Novo The chronic heart failure is not so frequent in non-compaction Eur. Heart J., May 2, 2007; 28(10): 1269 - 1269. [Full Text] [PDF]
|
|
|
|
|
|
J. Finsterer and C. Stollberger Genetic background of left ventricular hypertrabeculation/non-compaction with stroke Europace, May 1, 2007; 9(5): 333 - 333. [Full Text] [PDF]
|
|
|
|
|
|
M. A. Friedman, S. Wiseman, L. Haramati, G. M. Gordon, and D. M. Spevack Noncompaction of the left ventricle in a patient with dextroversion Eur J Echocardiogr, January 1, 2007; 8(1): 70 - 73. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 C Lofiego, E Biagini, F Pasquale, M Ferlito, G Rocchi, E Perugini, L Bacchi-Reggiani, G Boriani, O Leone, K Caliskan, et al. Wide spectrum of presentation and variable outcomes of isolated left ventricular non-compaction Heart, January 1, 2007; 93(1): 65 - 71. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 A. Lorsheyd, M.-J. M. Cramer, B. K. Velthuis, E.-J. P. Vonken, J. van der Smagt, P. van Tintelen, and R. N.W. Hauer Familial occurrence of isolated non-compaction cardiomyopathy Eur J Heart Fail, December 1, 2006; 8(8): 826 - 831. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
C. Lilje, V. Razek, J. J. Joyce, T. Rau, B. F. Finckh, F. Weiss, C. R. Habermann, J. C. Rice, and J. Weil Complications of non-compaction of the left ventricular myocardium in a paediatric population: a prospective study Eur. Heart J., August 1, 2006; 27(15): 1855 - 1860. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
D. Aras, O. Tufekcioglu, S. Topaloglu, K. Ergun, O. Ozeke, A. Yildiz, and S. Korkmaz Response Eur J Echocardiogr, January 1, 2006; 7(1): 7 - 8. [Full Text] [PDF]
|
|
|
|
|
|
D. Ward, R. Murphy, D. Pellerin, and P. Elliott Evident, but poorly defined: left ventricular hypertrabeculation/noncompaction and its diagnostic criteria: reply Eur. Heart J., June 1, 2005; 26(11): 1144 - 1145. [Full Text] [PDF]
|
|
|
|
|
|
C. Stollberger and J. Finsterer Evident, but poorly defined: left ventricular hypertrabeculation/noncompaction and its diagnostic criteria Eur. Heart J., June 1, 2005; 26(11): 1143 - 1144. [Full Text] [PDF]
|
|