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Europace Advance Access originally published online on November 13, 2006
Europace 2006 8(12):1064-1067; doi:10.1093/europace/eul125
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© The European Society of Cardiology 2006. All rights reserved. For Permissions, please e-mail: journals.permissions@oxfordjournals.org

GENETICS

Isolated ventricular non-compaction: clinical study and genetic review

Grazyna Markiewicz-Loskot1,*, Ewa Moric-Janiszewska2, Maria Loskot1, Leslaw Szydlowski1, Ludmila Weglarz2 and Andrzej Hollek2

1 Department of Pediatric Cardiology, Medical University of Silesia, Medyków 16, 40-752 Katowice, Poland; 2 Department of Biochemistry, Medical University of Silesia, Narcyzów 1, 41-200 Sosnowiec, Poland

Isolated non-compaction of the ventricular myocardium (INVM), sometimes referred to as ‘spongy myocardium’, is a congenital and exceedingly rare cardiomyopathy. Isolated ventricular non-compaction occurs in the absence of other structural heart diseases and, hypothetically, it is due to the arrest of myocardial morphogenesis. Isolated non-compaction of the ventricular myocardium may manifest itself from infancy to young adulthood with a high mortality rate. Both sexes are affected. In our study, we present a case of INVM (left and right ventricles) in a 3-year-old girl, diagnosed by two-dimensional echocardiography. The anomaly presented as a restrictive cardiomyopathy. The girl was admitted to our hospital with heart failure, when she was 10 months old. She was treated with dopamine, digoxin, furosemide, spironolactone, and acenocoumarol and her condition improved. Presently, the girl remains asymptomatic and for 3 years of follow-up, her development has been almost normal. We here describe the genetic background of this disorder (based on a literature review).

Key Words: Congenital heart anomaly, Ventricular non-compaction, Restrictive cardiomyopathy, Heart failure, G 4.5 (tafazzin gene), {alpha}-dystrobrevin gene (DTNA), FKBP-12 gene, Lamin A/C gene

* Corresponding author. Tel: +48 32 207 18 55; fax: +48 32 207 18 61. E-mail address: ejaniszewska{at}slam.katowice.pl


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