© 2007 European Society of Cardiology
Reply to the letter from Finsterer and Stöllberger
* Corresponding author. University Medical Centre Utrecht, Dept. of Cardiology, Heidelberglaan 100 3508 GA, Utrecht Netherlands. Tel: +31 30 2506176; fax: +31 30 2505471. E-mail address: A.Lorsheyd{at}hli.azu.nl
Key Words: Non-compaction • Cardiomyopathy
Received August 21, 2006; With great interest we read the extensive comments and remarks made by Finsterer and Stöllberger with regard to our article entitled ‘Familial occurrence of isolated non-compaction cardiomyopathy’ [1]. We would like to comment on a few remarks.
First, left ventricular non-compaction cardiomyopathy (LVNC) is not described as a subtype of hypertrophic cardiomyopathy (HCM) because of the absence of muscular disarray, the histopathological characteristic in HCM.
Secondly, we are aware of the genetic heterogeneity of LVNC. As described in our article, the patients in both families were screened for alfa-dystrobrevin and G4.5 mutation. The lamin A/C gene was also evaluated and no mutations were found. In addition, no evidence of neuromuscular disease or facial dysmorphism was observed in either of the families.
Furthermore, the syncopal episodes in patient II-2 (family A) were considered to be the result of the AV conduction disturbances (HV interval 85 ms) in combination with a left-bundle branch block. On exercise testing and electrophysiological study no arrhythmias could be provoked. The syncopal episodes were not suspect for seizures or orthostatic hypotension. It is very unlikely that thrombo-embolic events resulting from a cardiac embolus or carotid artery disease would have resulted in the complete loss of consciousness in the absence of any other neurological sign.
In patient II-2 (family B) a monomorphic ventricular tachycardia was induced during electrophysiological study which deteriorated into ventricular fibrillation. Syncope is considered to be the result of cardiac arrest with loss of consciousness because of a sustained ventricular tachycardia. This patient's son (III-1) was considered to have the identical underlying disease. The sudden death in the brother and sister of patient II-2 potentially resulted from a ventricular tachycardia based on the diagnosis LVNC in two family members. However, this is an assumption since post-mortem investigation has not been performed.
In LVNC there are three potential mechanisms for thrombo-embolic complications. First, when heart failure occurs with dilation of the left ventricle, an intra-cavitary thrombus can be formed. Secondly, atrial flutter or atrial fibrillation can lead to cardiac embolism. And thirdly, the changing haemostasis in the deep endomyocardial recesses makes patients with LVNC more vulnerable to thrombus formation [2-5]. Anticoagulation diminishes the risk of thrombo-embolic complications. The exact individual attribution of oral anticoagulation on each of these three mechanisms remains unclear. Moreover, Oechslin et al. described thrombo-embolic events in 8 out of 34 patients (24%) with LVNC [2]. In our opinion, this is a considerable number of patients. Therefore patient III-1 (family B) started anticoagulation therapy.
The distribution of LVNC did not differ between the two families; as shown in Table 1 in Ref. [1], the apico-lateral wall was most severely affected. However, the morphology was different between the two families; in family A, there was a fine network of non-compacted myocardium versus a coarser network of non-compacted myocardium in family B. This probably reflects the heterogeneous nature of this condition.
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- Lorsheyd A., Cramer M.M., Velthuis B.K., Vonken E.P., van der Smagt J., van Tintelen P., Hauer R.N.W. Familial occurrence of isolated non-compaction cardiomyopathy. Eur J Heart Fail (2006) 8:826–831.
[Abstract/Free Full Text] - Oechslin E.N., Attenhofer Jost C.H., Rojas J.R., Kaufmann P.A., Jenni R. Long-term follow-up of 34 adults with left ventricular noncompaction: a distinct cardiomyopathy with poor prognosis. J Am Coll Cardiol (2000) 36:493–500.
[Abstract/Free Full Text] - Ritter M., Oeschlin E., Sutsch G., Attenhofer C., Schneider J., Jenni R. Isolated noncompaction of the myocardium in adults. Mayo Clin Proc (1997) 72:26–31.[Abstract]
- Ichida F., Hamamichi Y., Miyawaki T., et al. Clinical features of isolated noncompaction of the ventricular myocardium. J Am Coll Cardiol (1999) 34:233–240.
[Abstract/Free Full Text] - Weiford B.C., Subbarao V.D., Mulhern K.M. Noncompaction of the ventricular myocardium. Circulation (2004) 109:2965–2971.
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