Clinical features of isolated noncompaction of the myocardium in children

Isolated noncompaction of the myocardium, also known as “spongy myocardium”, is a rare disease in children and adults. It is suggested that, during early development of the heart, the primary spongy structure persists due to an arrest of compaction. No other cardiac malformations are found, but ther...

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Bibliographic Details
Published in:Cardiology in the young 2001-07, Vol.11 (4), p.439-442
Main Authors: Neudorf, Ulrich E., Hussein, Ahmad, Trowitzsch, Eckart, Schmaltz, Achim A.
Format: Article
Language:English
Subjects:
absence of the spleen
cardiac transplantation
Child
Child Welfare
Child, Preschool
Echocardiography
Female
Germany
Heart Defects, Congenital - diagnosis
Humans
Infant
Infant Welfare
Male
Original Article
Radiography
Severity of Illness Index
Spongy myocardium
Survival Analysis
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Summary:Isolated noncompaction of the myocardium, also known as “spongy myocardium”, is a rare disease in children and adults. It is suggested that, during early development of the heart, the primary spongy structure persists due to an arrest of compaction. No other cardiac malformations are found, but there are familial occurences, relations to genetic disorders or syndromes such as Melnick-needles-syndrome or Xq28-linked cardiomyopathy, and reports of conduction disorders. We have now diagnosed isolated noncompaction in seven children aged between five weeks and 5.5 years. Three are doing well with anticongestive therapy, while transplantation of the heart was performed in one. Three of the children have died, but in only one case due to cardiac failure. Our experience emphasises the need rapidly to establish the diagnosis, to search for associated extracardiac abnormalities, and to consider transplantation at an early stage.
ISSN:1047-9511
1467-1107
DOI:10.1017/S1047951101000567