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European Journal of Echocardiography Advance Access published online on April 30, 2009
European Journal of Echocardiography, doi:10.1093/ejechocard/jep040
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Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2009. For permissions please email: journals.permissions@oxfordjournals.org

Cardiac re-synchronization therapy in a patient with isolated ventricular non-compaction: a case report

Antoine Garnier* and Grégoire Girod

Department of Cardiology, Hôpital de Sion, 1950 Sion, Switzerland

Received 19 November 2008; accepted after revision 29 March 2009.

* Corresponding author. Tel: +41 21 314 0565. E-mail address: Antoine.Garnier{at}bluewin.ch


  Abstract

Isolated ventricular non-compaction (IVNC) is a rare, congenital, unclassified cardiomyopathy characterized by prominent trabecular meshwork and deep recesses. Major clinical manifestations of IVNC are heart failure, atrial and ventricular arrhythmias, and thrombo-embolic events. We describe a case of a 69-year-old woman in whom the diagnosis of IVNC was discovered late, whereas former echocardiographic examinations were considered normal. She was known for systolic left ventricular dysfunction for 3 years and then became symptomatic (NYHA III). In the past, she suffered from multiple episodes of deep vein thrombosis and pulmonary embolism. Electrocardiogram revealed a wide QRS complex, and transthoracic echocardiography showed typical apical thickening of the left and right ventricular myocardial wall with two distinct layers. The ratio of non-compacted to compacted myocardium was >2:1. Cardiac MRI confirmed the echocardiographic images. Cerebral MRI revealed multiple ischaemic sequellae. In view of the persistent refractory, heart failure in medical treatment of patients with classical criteria for cardiac re-synchronization therapy, as well as the ventricular arrhythmias, a biventricular automatic intracardiac defibrillator (biventricular ICD) was implanted. The 2-year follow-up period was characterized by improvement of NYHA functional class from III to I and increasing in left ventricular function. We hereby present a case of IVNC with favourable outcome after biventricular ICD implantation. Cardiac re-synchronization therapy could be considered in the management of this pathology.

Keywords: Isolated ventricular non-compaction; Cardiac re-synchronization therapy; Heart failure; Arrhythmia


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