- Case report
- Open Access
© 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.
- Cardiovascular Magnetic Resonance
- Late Gadolinium Enhancement
- Brain Natriuretic Peptide
- Interventricular Septum
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.
- Koch A, Singer H: Normal values of B type natriuretic peptide in infants, children, and adolescent. Heart. 2003, 89: 875-878. 10.1136/heart.89.8.875.PubMed CentralView ArticlePubMedGoogle Scholar
- Jenni R, Oechslin E, Schneider J, Attenhofer Jost C, Kaufmann PA: Echocardiographic and pathoanatomical characteristics of isolated left ventricular non-compaction: a step towards classification as a distinct cardiomyopathy. Heart. 2001, 86: 666-671. 10.1136/heart.86.6.666.PubMed CentralView ArticlePubMedGoogle Scholar
- Petersen SE, Selvanayagam JB, Wiesmann F, Robson MD, Francis JM, Anderson RH, Watkins H, Neubauer S: Left ventricular non-compaction: insights from cardiovascular magnetic resonance imaging. Journal of the American College of Cardiology. 2005, 46: 101-105. 10.1016/j.jacc.2005.03.045.View ArticlePubMedGoogle Scholar
- Andrews RE, Fenton MJ, Ridout DA, Burch M: New-onset heart failure due to heart muscle disease in childhood: a prospective study in the United kingdom and Ireland. Circulation. 2008, 117: 79-84. 10.1161/CIRCULATIONAHA.106.671735.View ArticlePubMedGoogle Scholar
This article is published under license to BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.