European Heart Journal Advance Access originally published online on July 3, 2006
European Heart Journal 2006 27(15):1855-1860; doi:10.1093/eurheartj/ehl112
| ||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
Complications of non-compaction of the left ventricular myocardium in a paediatric population: a prospective study
1 Departments of Kinderkardiologie, Universitäts-Klinikum Hamburg-Eppendorf, Martinistr. 52, D-20246 Hamburg, Germany
2 Experimentelle & Klinische Pharmakologie & Toxikologie, Universitäts-Klinikum Hamburg-Eppendorf, Hamburg, Germany
3 Clinical Pathology/Pediatrics, Universitäts-Klinikum Hamburg-Eppendorf, Hamburg, Germany
4 Diagnostische Radiologie, Universitäts-Klinikum Hamburg-Eppendorf, Hamburg, Germany
5 Section of Pediatric Cardiology, Tulane University Health Sciences Center, New Orleans, LA, USA
6 Department of Biostatistics, Tulane University School of Public Health & Tropical Medicine, New Orleans, LA, USA
Received 28 September 2004; revised 5 April 2006; accepted 1 June 2006; online publish-ahead-of-print 3 July 2006.
* Corresponding author. Tel: +49 40 42803 3718; fax: +49 40 42803 6826. E-mail address: lilje{at}europe.com
Aims Non-compaction of the left ventricular myocardium (NCVM) is reportedly exceedingly rare and associated with a high morbidity and mortality. A different genetic background has been suggested for NCVM with [non-isolated NCVM (ni-NCVM)] and without [isolated NCVM (i-NCVM)] other congenital heart defects. We prospectively evaluated both the NCVM subgroups regarding frequency of occurrence and cardiovascular complications in a paediatric population.
Results In a prospective, single-centre study, 66/5220 consecutive patients (1.26%) were diagnosed (25 i-NCVM, 41 ni-NCVM). The median age was 4 years (range 0–21), the median follow-up 12 months (range 0–51). The occurrence of congestive heart failure (CHF) at follow-up was 68.0%. CHF was as frequently seen in i-NCVM and ni-NCVM patients (77.5 vs. 62.1%, P = 0.322). The occurrence of arrhythmias (20.0%) and thrombo-embolic events (13.9%) was not different between subgroups. The cardiomyopathy related mortality was 7.1%, with three and one deaths in the i-NCVM and ni-NCVM groups, respectively (P = 0.126).
Conclusion When prospectively evaluated, NCVM appears to have been previously under-diagnosed. Whereas arrhythmias and thrombo-embolic events were rare, CHF was frequently found. An equally aggressive anticongestive treatment regimen would seem indicated for both the NCVM subgroups.
Key Words: Heart defects, congenital • Cardiomyopathy • Heart failure, congestive • Echocardiography • Non-compaction
CiteULike 
Connotea 
Del.icio.us What's this?
This article has been cited by other articles:
|
|
 |
|
  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]
|
|
|
|
 |
|
 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]
|
|
|
|
|
|
C. Lilje, V. Razek, J. J. Joyce, T. Rau, B. F. Finckh, F. Weiss, C. R. Habermann, J. C. Rice, and J. Weil The chronic heart failure is not so frequent in non-compaction: reply Eur. Heart J., May 2, 2007; 28(10): 1269 - 1270. [Full Text] [PDF]
|
|
|
|
|
|
J. Finsterer and C. Stollberger Myopathic background of non-compaction in children Eur. Heart J., January 1, 2007; 28(1): 139 - 139. [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 Myopathic background of non-compaction in children: reply Eur. Heart J., January 1, 2007; 28(1): 139 - 140. [Full Text] [PDF]
|
|