© Davlouros et al; licensee BioMed Central Ltd. 2009
Received: 22 September 2009
Accepted: 16 December 2009
Published: 16 December 2009
We present an unusual case of cardiomyopathy in a two month old male infant with a grade-I systolic murmur. Echocardiographic examination disclosed left ventricular (LV), dysplasia with saw-tooth like inwards myocardial projections extending from the lateral walls towards the LV cavity. There was mild LV systolic dysfunction with apical hypokinesia. Cardiovascular magnetic resonance demonstrated in detail these cross bridging muscular projections originating from the inferior interventricular septum and lateral LV wall, along with areas of hypokinesis at the LV septum and apex in a noncoronary distribution, without any late gadolinium enhancement. We have termed this condition saw-tooth cardiomyopathy because of the very characteristic appearance.
The incidence of heart muscle disease induced new-onset heart failure in the absence of congenital heart disease in children, has been recently reported in a nationwide multicenter study from UK and Ireland to be 87 per 100,000 population less than 16 years. The median age at presentation was 1 year in that study and the most common causes of heart failure included dilated cardiomyopathy, probable myocarditis, occult arrhythmia, anthracycline toxicity, metabolic disease and LV non-compaction. Our case constitutes the first report of echocardiographic and CMR of a form of heart muscle disease accompanied by LV systolic dysfunction, characterized by formation of successive open muscular rings at the inferior half of the LV. The relationship of the latter with the reduced LV systolic function-if any-remains obscure; however a presumably deranged myofibril organization pattern might link gross morphology with altered systolic function.
We present a form of LV dysplasia of the newborn with unique morphologic features, which we have termed saw-tooth cardiomyopathy because of the very characteristic appearance.
Written informed consent was obtained from the mother of the infant for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.
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