[Isolated abnormality ("noncompaction") of the myocardium in 3 children].

UNLABELLED: In three asymptomatic children an isolated myocardial noncompaction was detected by echocardiography at age 11 months, 5 weeks and 5.5 years. In the first male infant both ventricles and septum were severely affected and myocardial function was depressed. Nevertheless, during a follow up of 16 months he remained asymptomatic on anticongestive therapy. In the other two children apex and lateral wall of the left ventricle were affected and myocardial function was still normal. The second boy had also an infantile epilepsy-encephalopathy syndrome and the third child (a girl) had a Wolff-Parkinson-White syndrome; an association of either syndromes with myocardial noncompaction has not been reported earlier. DISCUSSION: Myocardial noncompaction (spongy myocardium) is a rare maldevelopment, which occurs either associated with certain congenital heart defects or, even more rarely, isolated, as the two cases reported here. Myocardial failure, severe arrhythmias or thromboembolism may occur at any age and determine the outcome. Clinical course, therapy and prognosis are similar to dilatative cardiomyopathy, which represents an important differential diagnosis.