European Heart Journal Advance Access originally published online on December 12, 2006
European Heart Journal 2007 28(1):139-140; doi:10.1093/eurheartj/ehl418
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Myopathic background of non-compaction in children: reply
Kinderkardiologie
Universitäres Herzzentrum Hamburg
Martinistr. 52
D-20246 Hamburg, Germany
Ped. Cardiology
Tulane University
1430 Tulane Ave
New Orleans, LA 70112, USA
Tel: +49 40 42803 3718
Fax: +49 40 42803 6826
E-mail address: lilje{at}europe.com
Kinderkardiologie
Universitäres Herzzentrum Hamburg
Martinistr. 52
D-20246 Hamburg, Germany
Ped. Cardiology
Tulane University
1430 Tulane Ave
New Orleans, LA 70112, USA
Pharmakologie/Toxikologie
Universitäts-Klinikum Hamburg
Martinistr. 52
D-20246 Hamburg. Germany
Clinical Pathology/Pediatrics
Universitäts-Klinikum Hamburg
Martinistr. 52
D-20246 Hamburg, Germany
Diagnost. Radiologie
Universitäts-Klinikum Hamburg
Martinistr. 52
D-20246 Hamburg, Germany
Diagnost. Radiologie
Universitäts-Klinikum Hamburg
Martinistr. 52
D-20246 Hamburg, Germany
Biostatistics
Tulane University
1430 Tulane Ave
New Orleans
LA 70112, USA
Kinderkardiologie
Universitäres Herzzentrum Hamburg
Martinistr. 52
D-20246 Hamburg, Germany
We appreciate the interest of Finsterer et al. in our article.1 Unfortunately, not all of their numerous concerns can be addressed here, some of which have been raised and replied to previously.
- Chin et al.2 were among the first to describe NCVM. It was considered to result from an arrest in the process of embryonic myocardial compaction. There is much evidence, including excellent work on animal models, to support this pathogenic concept and the term NCVM.3 Both have been widely accepted. The evidence for ‘acquired’ NCVM is scarce. A few reports, mostly by Finsterer et al., have been published on single patients with ‘acquired’ NCVM. Although there is a variety of reasons for secondary left ventricular hypertrabeculation (LVHT), the recognition of NCVM is frequently delayed.3–6
- In 1990, Chin et al.2 introduced diagnostic criteria for NCVM, including the X/Y ratio taken at the mentioned three ventricular levels.2 The subsequent introduction of both a competitive (N/C) ratio6 and a new non-specific term (LVHT) was confusing. Others and we realized that measurements taken in diastole enhance diagnostic specificity. In paediatrics, the mitral valve and papillary muscles are not usually areas with poor echocardiographic windows. False tendons or aberrant bands are easily discriminated against NCVM (numerous trabeculations, deep recesses, two-layered myocardium). There was no investigator disagreement on NCVM criteria.
- Most previous studies exclusively addressed isolated NCVM (i-NCVM). But NCVM accompanies diverse forms of structural congenital heart disease (CHD).4,5 There is initial evidence that NCVM subgroups may be different in several regards.5 We attempted to characterize i-NCVM and non-isolated NCVM (ni-NCVM) in regard to cardio-vascular complications. Surprisingly, the outcome was similarly poor. Structural CHD is well defined. ECG abnormalities or relative valve insufficiencies certainly do not qualify. Besides the defects stated, ni-NCVM comprised atrial and atrioventricular septal defects, anomalous pulmonary venous connections, atrial isomerism, Ebstein's anomaly, tricuspid atresia, left ventricle (LV)-aortic tunnel, persisting ductus, and double aortic arch.
- Not attempting a study on all aspects of NCVM, the investigation of genetics or neuro-muscular disorders was not included in our protocol. We introduced examples of the presumably heterogeneous genetic background, since some gene mutations were exclusively found in i-NCVM but not in ni-NCVM and vice versa.
- ECG abnormalities comprised bundle branch block, supraventricular and ventricular tachycardia, ST-segment depression, and abnormal T waves. Patients presented for heart murmurs, failure to thrive, excessive sweating, exercise intolerance, palpitations, (pre-) syncope, and suspected or known CHD or a positive family history, among others. Reduced systolic function or ventricular dilation (56.3 and 50.0% of patients at follow-up, respectively) was subsumed within the described CHF score. The anticongestive therapy comprised digoxin, diuretics, ACE-inhibitors, and beta-blockers. Seven patients presented with a hypertrophied non-obstrucive LV.
Coronary abnormalities commonly associated with right ventricular outflow obstruction were seen in four ni-NCVM patients. Coronary abnormalities affecting the LV function were not found. Follow-up data on six patients (9.1%) were incomplete. Neither localization nor degree of non-compaction changed during follow-up in any patient. However, NCVM was missed in several patients on previous presentations.
References
- Lilje C, Razek V, Joyce J, Rau T, Finckh B, Weiss F, Habermann C, Rice J, Weil J. (2006) Complications of non-compaction of the left ventricular myocardium in a paediatric population: a prospective study. Eur Heart J 27:1855–1860.
[Abstract/Free Full Text] - Chin T, Perloff J, Williams R, Jue K, Mohrmann R. (1990) Isolated noncompaction of left ventricular myocardium. A study of eight cases. Circulation 82:507–513.
- Ostadal B. (1999) Comparative aspects of the cardiac blood supply. In Rakusan K (Ed.). Coronary Angiogenesis(JAI Press, Stamford, Conn) pp. p91–110.
- Freedom R, Yoo SJ, Perrin D, Taylor G, Petersen S, Anderson R. (2005) The morphological spectrum of ventricular noncompaction. Cardiol Young 15:345–364.[CrossRef][Web of Science][Medline]
- Burke A, Mont E, Kutys R, Virmani R. (2005) Left ventricular noncompaction: a pathological study. Hum Pathol 36:403–411.[CrossRef][Web of Science][Medline]
- Oechslin E, Attenhofer C, Jost C, Rojas J, Kaufmann P, Jenni R. (2000) Long-term follow-up of 34 adults with isolated left ventricular noncompaction: a distinct cardiomyopathy with poor prognosis. J Am College Cardiol 36:493–500.
[Abstract/Free Full Text]
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