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European Journal of Echocardiography 2006 7(1):7-8; doi:10.1016/j.euje.2005.10.004
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Copyright © 2005, The European Society of Cardiology

Response

Dursun Aras*, Omac Tufekcioglu, Serkan Topaloglu, Kumral Ergun, Ozcan Ozeke, Ali Yildiz and Sule Korkmaz

Turkiye Yuksek Ihtisas Hospital, Department of Cardiology, Ankara, Turkey

Received 3 October 2005; .

* Corresponding author. Ballibaba sokak No:52/3, 06700 Kucukesat, Ankara, Turkey. Tel.: +903124302825; fax: +903124175315. sevgidursun06{at}yahoo.com

We appreciate the comments of Dr. Finsterer and Stollberger regarding our article,1 which raise several important points.

1. Many cases of left ventricular noncompaction are caused by other cardiac anomalies that generate intraventricular pressure overload, as for example in pulmonary atresia with intact ventricular septum.2 In these hearts, the deep recesses are in continuity with the ventricular cavity and with the coronary arteries and are therefore more accurately described as persistent intramyocardial sinusoids. In contrast, isolated ventricular noncompaction (IVNC) has no associated cardiac lesions and persistent sinusoids are not seen.2–4
2. Today there is no consensus on the treatment of IVNC patients. In our patient carvedilol was primarily used for nonsustained ventricular tachycardia (VT), which did not recur after treatment on follow-up. Since beneficial effects of carvedilol on left ventricular function, hypertrophy, and both metabolic and adrenergic abnormalities have been described previously,5 we thought that it would also provide these benefits in addition to suppressing ventricular arrhythmias. An implantable cardiac defibrillator was not implanted in this patient with preserved left ventricular systolic function and without documented sustained VT.
3. IVNC is an unclassified congenital cardiomyopathy that is thought to be related to the arrest of myocardial development, resulting in persistence of multiple prominent ventricular trabeculations and deep intertrabecular recesses. The basic hypothesis about its pathogenesis may be the arrest of normal compaction of the loosely interwoven mesh of myocardial fibers in the embryo.3,4,6 Furthermore, the genetic basis for isolated IVNC is now becoming established and both X-linked and an autosomal inheritance is described.7,8
4. We thought that, in past, this patient was misdiagnosed as apical hypertrophic cardiomyopathy rather than to be late development of IVNC. We suppose that hypertrabeculation and IVNC are two distinct entities and these terms are not interchangeable. Hypertrabeculation may be a normal variant and it may be seen in patients with left ventricular pressure or volume overload for any reason.
5. Stöllberger et al. use the term ‘hypertrabeculation/ventricular noncompaction’. They are the only authors in the literature who use this term. The 1995 report of the World Health Organisation/International Society and Federation of Cardiology Task Force on the definition and classification of cardiomyopathies does not use this term either.9 According to this report cardiomyopathies of patients with a neuromuscular disorder are classified as specific cardiomyopathies. Since Stollberger's patient population cannot be accepted as having IVNC, it is very hard to say that it is the largest patient population.10 In past the patient population reported by Oechslin et al. has been the largest series.11 However, during the review process of our case report, a recent report describing 45 IVNC patients was published in European Heart Journal by Murphy et al.12
6. Previously described echocardiographic criteria4,11,13 were used to establish the diagnosis of IVNC in this case. The accuracy of these criteria has been confirmed by pathoanatomical studies.13
7. Both extension of the noncompaction morphology and left ventricular systolic function showed no important change during follow-up.
8. The oldest patient reported by Finsterer et al. was an 80-year-old female, not 84 years.14 To the best of our knowledge, in literature, the oldest IVNC patient was 83-year-old.15 However the oldest patient with IVNC and preserved LV systolic function is our patient.1
9. Acetyl-salicylic acid was used to minimize the risk of thrombosis.
10. Neuropathy and myopathy were not suspected in our patient and her relatives with history and neurological examination.


   References
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 References
 

  1. Aras D., Tufekcioglu O., Topaloglu S., Ergun K., Ozeke O., Yildiz A., et al. Preserved systolic function with isolated left ventricular noncompaction in an elderly patient. Eur J Echocardiogr (2006) 7:71–74.[Abstract/Free Full Text]
  2. Lauer R.M., Fink H.P., Petry E.L., Dunn M.I., Diehl A.M. Angiographic demonstration of intramyocardial sinusoids in pulmonary valve atresia with intact ventricular septum. N Engl J Med (1964) 271:68–72.[Web of Science][Medline]
  3. Chin T.K., Perloff J.K., Williams R.G., Jue K., Mohrmann R. Isolated noncompaction of left ventricular myocardium. A study of eight cases. Circulation (1990) 82:507–513.[Abstract/Free Full Text]
  4. Ritter M., Oechslin E., Sutsch G., Attenhofer C., Schneider J., Jenni R. Isolated noncompaction of the myocardium in adults. Mayo Clin Proc (1997) 72:26–31.[Abstract]
  5. Toyono M., Kondo C., Nakajima Y., Nakazawa M., Momma K., Kusakabe K., et al. Effects of carvedilol on left ventricular function, mass, and scintigraphic findings in isolated left ventricular non-compaction. Heart (2001) 86(1):e4.[Abstract/Free Full Text]
  6. Jenni R., Goebel N., Tartini R., Schneider J., Arbenz U., Oelz O. Persisting myocardial sinusoids of both ventricles as an isolated anomaly: echocardiographic, angiographic and pathologic anatomical findings. Cardiovasc Intervent Radiol (1986) 9:127–131.[Web of Science][Medline]
  7. Bleyl S.B., Mumford B.R., Brown-Harrison M.C., Pagotto L.T., Carey J.C., Pysher T.J., et al. Xq28-linked noncompaction of the left ventricular myocardium: prenatal diagnosis and pathologic analysis of affected individuals. Am J Med Genet (1997) 72(3):257–265.[CrossRef][Web of Science][Medline]
  8. Ichida F., Tsubata S., Bowles K.R., Haneda N., Uese K., Miyawaki T., et al. Novel gene mutations in patients with left ventricular noncompaction or Barth syndrome. Circulation (2001) 103(9):1256–1263.[Abstract/Free Full Text]
  9. Richardson P., McKenna W., Bristow M., Maisch B., Mautner B., O'Connell J., et al. Report of the 1995 World Health Organization/International Society and Federation of Cardiology Task Force on the Definition and Classification of Cardiomyopathies. Circulation (1996) 93(5):841–842.[Free Full Text]
  10. Stollberger C., Finsterer J. Cardiologic and neurologic findings in left ventricular hypertrabeculation/non-compaction related to wall thickness, size and systolic function. Eur J Heart Fail (2005) 7(1):95–97.[CrossRef][Web of Science][Medline]
  11. Oechslin E.N., Attenhofer Jost C.H., Rojas J.R., Kaufmann P.A., Jenni R. Long-term follow-up of 34 adults with isolated left ventricular noncompaction: a distinct cardiomyopathy with poor prognosis. J Am Coll Cardiol (2000) 36:493–500.[Abstract/Free Full Text]
  12. Murphy R.T., Thaman R., Blanes J.G., Ward D., Sevdalis E., Papra E., et al. Natural history and familial characteristics of isolated left ventricular non-compaction. Eur Heart J (2005) 26(2):187–192.[Abstract/Free Full Text]
  13. Jenni R., Oechslin E., Schneider J., Attenhofer Jost C., Kaufmann P.A. Echocardiographic and pathoanatomical characteristics of isolated left ventricular non-compaction: a step towards classification as a distinct cardiomyopathy. Heart (2001) 86(6):666–671.[Abstract/Free Full Text]
  14. Finsterer J., Stollberger C., Feichtinger H. Non-compaction on echocardiography and autopsy. Acta Cardiol (2003) 58(2):165–168.[CrossRef][Web of Science][Medline]
  15. Takashima A., Shimizu M., Tatsumi K., Shima T., Miwa Y. Isolated left ventricular noncompaction in the elderly: a case report. J Cardiol (2004) 44(1):21–25.[Medline]

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