European Heart Journal Advance Access originally published online on April 30, 2007
European Heart Journal 2007 28(10):1269-1270; doi:10.1093/eurheartj/ehm099
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The chronic heart failure is not so frequent in non-compaction: reply
Kinderkardiologie, Univ. Herzzentrum
Martinistr. 52, D-20246 Hamburg
Germany; and
Ped. Cardiology, Tulane University
1430 Tulane Ave
New Orleans, LA 70112
USA
Kinderkardiologie, Herzzentrum
Univ. Leipzig
Leipzig
Germany
Ped. Cardiovasc. Center
University of Florida
Jacksonville
USA
Pharmakologie/Toxikologie
Univ.-Klinikum Hamburg-Eppendorf
Hamburg
Germany
Klin. Pathologie/Pädiatrie
Univ.-Klinikum Hamburg-Eppendorf
Hamburg
Germany
Diagnost. Radiologie
Univ.-Klinikum Hamburg-Eppendorf
Hamburg
Germany
Diagnost. Radiologie
Univ.-Klinikum Hamburg-Eppendorf
Hamburg
Germany
Biostatistics
Tulane University
New Orleans
USA
Kinderkardiologie
Univ. Herzzentrum
Hamburg
Germany
Tel: +49 40 42803 3718 fax: +49 40 42803 6826 E-mail address: lilje{at}uke.uni-hamburg.de
We appreciate the interest of Fazio et al. in our paper. We agree with their valuable comments in many regards.
1. Prognosis. Presumably by accident, Fazio et al. cited our data as describing both a bad and a good prognosis. In fact, we do consider the outcome concerning. At 12 months follow-up, the occurrence of congestive heart failure (CHF), arrhythmias, and thrombo-embolic events was 68.0, 20.0, and 13.9%, respectively. The mortality was 7.1%.1 These figures are even higher in several other studies.
2. CHF. The incidence of CHF in the population studied by Fazio et al. was 50–61%. These data are likewise concerning. They support our and previous findings of a high incidence of CHF in non-compaction of the left ventricular myocardium (NCVM), as do additional recent studies.2,3 We discussed several theories and findings explaining why NCVM may lead to CHF.1 Further data have since been published in support of this discussion.4
3. Selection bias. As mentioned within the paragraph ‘study limitations’ of our paper, our population—like the one studied by Fazio et al.—was undoubtedly prone to a referral bias of a tertiary care medical centre. However, not all patients were symptomatic. We evaluated all consecutive patients sent for echocardiography during a specified period of time.1 Reasons for presentation were innocent heart murmurs, a family history of congenital heart disease, rule-out of cardiac side effects of non-cardiac, medications, among others.
4. Patient populations. While comparing study populations, we would be interested to know the age of the patients and relatives studied by Fazio et al. Presumably most of them were adults. By which criteria did patients enter the registry; was it hospital admissions only? How many NCVM patients were entered with and without congenital heart disease? NCVM subpopulations may carry a different cardio-vascular risk.1
5. Incidence. We do not know the total number of patients entered into the highly specialized Italian registry. But certainly, the number of NCVM patients entered in 1 year (>230) is remarkable; as is the number of first-degree relatives detected with NCVM (48/31). It has been repeatedly stated that NCVM appears to have been previously under-diagnosed all together.1,2,5
Pretty much all existing data on NCVM has been prone to a selection bias. We do not know the prevalence and the natural history in a truly non-selected population. Based on existing data, including our own study and the one by Fazio et al., the occurrence of CHF in NCVM is concerning. It will be highly interesting to learn about details of the investigation by Fazio et al. addressing first-degree relatives and future similar data. The outlook may be more encouraging in incidental or familial discovery of NCVM.3 Unfortunately, even such populations are prone to a selection bias.
References
- Lilje C, Rázek V, Joyce J, Rau T, Finckh B, Weiss F, Habermann C, Rice J, Weil J. Complications of noncompaction of the left ventricular myocardium in a pediatric population: a prospective study. Euro Heart J (2006) 27:1855–1860.
[Abstract/Free Full Text] - Alehan D. Clinical features of isolated left ventricular noncompaction in children. Int J Cardiol (2004) 97:233–237.[CrossRef][Web of Science][Medline]
- Lofiego C, Biagini E, Pasquale F, Ferlito M, Rocchi G, Perugini E, Bacchi-Reggiani L, Boriani G, Leone O, Caliskan K, ten Cate F, Picchio F, Branzi A, Rapezzi C. Wide spectrum of presentation and variable outcomes of isolated left ventricular non-compaction. Heart (2007) 93:65–71.
[Abstract/Free Full Text] - Tufekcioglu O, Aras D, Ozeke O, Maden O, Topaloglu S. Comparison of regional systolic myocardial velocities in patients with isolated left ventricular noncompaction and patients with idiopathic dilated cardiomyopathy. J Am Soc Echocardiogr (2006) 19:1320–1325.[CrossRef][Web of Science][Medline]
- Jenni R, Oechslin E, van der Loo B. Isolated ventricular non-compaction of the myocardium in adults. Heart (2007) 93:11–15.
[Abstract/Free Full Text]
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