Imaging in cardiology M.R.M. Jongbloed, J.J. Bax, M.J. Schalij, E.E. van der Wall

Noncompaction cardlomyopathy A 37-year-old man presented with acute occlusion of the right ciliary artery. He had a history of syncope and a family history of sudden cardiac death. The ECG showed left ventricular hypertrophy, 24-hour Holter monitoring revealed frequent premature (supra)ventricular beats and one episode of nonsustained ventricular tachycardia. Transthoracic two-dimensional echocardiography showed an enlarged left atrium (47 mm), normal LV dimensions and preserved systolic function. Diastolic function was abnormal (impaired relaxation), confirmed by tissue Doppler myocardial imaging at the mitral valve annulus (showing a reduced E wave, 5.0 cm/sec). The basal septum was hypertrophic (19 mm) but nonobstructive. The left ventricle and to a lesser extent the right ventricle demonstrated prominent trabeculations with deep intertrabecular recesses. Communication between the deep intertrabecular recesses and the left ventricular cavity was confirmed by colour flow imaging. A thin epicardial layer could be differentiated from the thick, noncompacted endocardial layer, which had a 'spongy' appearance (figures 1 and 2). A subsequent transoesophageal echocardiogram revealed a thrombus in the left atrial appendage, whereas no thrombi were observed in the left ventricular recesses. Based on the echocardiographic findings, the patient was diagnosed with nonM.R.M. Jongbloed. J.J. Bax. MJ. Schalij. E.E. van der Wall. Leiden University Medical Centre, Department of Cardiology, PO Box 9100, 2600 RC Leiden.

Figurcs I and 2. Apicalfour-chambcr images showing thc tyical prominent trabeculations located in the left ventricular apex, extending to the lateral wall, with deep intertrabecular recesses (apex). Moreover, the thin epicardial layer and the thick 'spongy' endocardial layer can be differentiated.

Address for correspondence: M.R.M Jongbloed. E-mail: [email protected]

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Netherlands Heart Journal, Volume I1, Number 10, October 2003

Imaging in cardiology

cardiomyopathy. Noncompaction of the myocardium is a rare congenital disorder (estimated incidence 0.05%).12 During early embryogenesis, the primitive left ventricle consists of trabeculations with deep intertrabecular recesses which communicate with the ventricular cavity, giving it a 'spongy' appearance. During week 5 to 8 of foetal life, gradual compaction of the ventricular myocardium normally occurs. Noncompaction is the resultant of an arrest in this compaction

process.

Patients may be asymptomatic or may present with heart failure (both diastolic and systolic), tachyarrhythmias, conduction disorders and/or thromboembolic events2 and therapy should be directed at the prevention and management of these entities. In the current patient, medication consisted of statins, ACE inhibition, oral anticoagulants and 5-blockade. ICD implantation will be considered.

Nbedrands Heart Journal, Volume 11, Number 10, October 2003

References 1 2

Agmon Y, Connolly HM, Olson LJ, Khandhenia BK, Seward JB. Noncompaction of the ventricular myocardium. JAm Soc Echocardiogr 1999;12:859-63. Oechslin EN, Attenhofer Jost CH, Rojas JR, Kauffmann PA, Jenni R. Long-term follow-up of 34 patients with isolated left ventricular noncompaction: A distinct cardiomyopathy with poor prognosis. JAm Coll Cardiol 2000;36:493-500.

In this section a remarkable 'image' is presented and a short comment is given. We invite you to send in images (in triplicate) with a short comment (one to two pages at the most) to Mediselect bv, Editorial Office Netherlands Heart Journal, PO Box 63, 3830 AB Leusden, the Netherlands. This section is edited by M.J.M. Cramer.

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