European Journal of Echocardiography 2008 9(2):321-322; doi:10.1093/ejechocard/jen065
Published on behalf of the European Society of Cardiology. All rights reserved. © The Authors 2008. For permissions please email: journals.permissions@oxfordjournals.org
Left ventricular non-compaction: is it really isolated?
Stefano Muzzarelli*,
Peter Buser,
Alain Bernheim and
Andre Linka
Department of Cardiology, University Hospital, CH-4031 Basel, Switzerland
Received 12 September 2007; accepted after revision 30 September 2007.
* Corresponding author. Tel: +41 612 655214; fax: +41 612 654598. E-mail address: smuzzarelli{at}uhbs.ch
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Abstract
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Isolated ventricular non-compaction (IVNC) is a genetically
determined cardiac disease mainly involving the left ventricle.
Since the first description and recognition of IVNC as a distinct
cardiomyopathy, many cases have been described. However, the
associated right-sided cardiac anomalies are unusual. Two cases
of IVNC showing associated right-sided pathology on transthoracic
echocardiography are presented.
Keywords: Left ventricular non-compaction; Cardiomyopathy
A 46-year-old woman was evaluated in the emergency room because of dyspnoea and abdominal pain secondary to a pelvic mass. The presence of a systolic murmur led to further cardiological investigation. Transthoracic echocardiography demonstrated apical hypertrophy of both ventricles with a prominent trabecular meshwork and deep intertrabecular sinusoids. The ratio between this non-compacted portion of the myocardium and the normally structured myocardium was >2. Additionally, an apical displacement of the septal tricuspid leaflet (15 mm) with mild ventricular atrialization was present. Therefore, the findings are consistent with non-compaction of the left ventricle with right ventricular involvement and a mild form of Ebstein's anomaly (Figure 1).
A 58-year-old woman with exertional dyspnoea had a transient ischaemic attack and multiple cutaneous embolization. Transthoracic echocardiography revealed a dilated left ventricle with severely reduced ejection fraction and typical findings of left ventricular non-compaction in the apical and infero-lateral myocardium (Figure 2). In addition, a diverticulum in the subtricuspidal lateral right ventricular wall was seen.
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Figure 1 Transthoracic echocardiography: four-chamber view depicting the anomaly in both ventricles (arrows) and the minor form of Ebstein's anomaly (asterisk). RV, right ventricle; LV, left ventricle; RA, right atrium; LA, left atrium.
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Figure 2 (A) Apical view of the left ventricle demonstrating non-compaction of the infero-apical wall (arrows). (B) End-diastolic (D) and end-systolic (S) stop frame showing the subtricuspidal diverticulum within the right ventricle (asterisk). RV, right ventricle; LV, left ventricle; RA, right atrium; LA, left atrium.
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Discussion
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After the first description of non-compaction cardiomyopathy,
an isolated left ventricular involvement was supposed.
1 Over
the following years, some cases with right-sided cardiac abnormalities
have been reported.
2,3 Nevertheless, diagnostic criteria of
isolated ventricular non-compaction (IVNC) are focused on description
of the left ventricle. Most notably, the absence of coexisting
cardiac abnormalities have been supposed as one of the criteria
for diagnosis of IVNC.
4 The two presented cases delineate that
right-sided and valvular cardiac abnormalities may accompany
IVNC. Therefore, comprehensive echocardiographic investigation
is mandatory in case of IVNC.
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References
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- Engberding R, Bender F. Identification of a rare congenital anomaly of the myocardium by two-dimensional echocardiography: persistence of isolated myocardial sinusoids. Am J Cardiol (1984) 53:1733–4.[CrossRef][Web of Science][Medline]
- Jenni R, Oechslin E, Schneider J, Attenhofer JC, Kaufmann PA. Echocardiographic and pathoanatomical characteristics of isolated left ventricular non-compaction: a step towards classification as a distinct cardiomyopathy. Heart (2001) 86:666–71.[Abstract/Free Full Text]
- Attenhofer Jost CH, Connolly HM, Warnes CA, et al. Noncompacted myocardium in Ebstein's anomaly: initial description in three patients. J Am Soc Echocardiogr (2004) 17:677–80.[CrossRef][Web of Science][Medline]
- Engberding R, Yelbuz TM, Breithardt G. Isolated noncompaction of the left ventricular myocardium: a review of the literature two decades after the initial case description. Clin Res Cardiol (2007) 96:481–8.[CrossRef][Web of Science][Medline]
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