Background: Ventricular noncompaction is a rare unclassified cardiomyopathy due to intrauterin arrest of compaction of the loose interwoven meshwork with limited data regarding diagnosis and outcome in children.
Methods: In this study we describe clinical features of isolated left ventricular noncompaction (IVNC) in children and compare our findings with those previously reported. A diagnosis of ventricular noncompaction was made according to the characteristic echocardiographic appearance of two-layered myocardial wall consisting of a thin compacted epicardial and a thick noncompacted endocardial layer with numerous, prominent trabeculations and deep intertrabecular recesses communicating with left ventricular cavity.
Results: Nine children, eight male and one female, with ages ranging from 10 days to 12 years and follow-up as long as 5 years were included into the study. The primary diagnosis of IVNC had been missed in four of the patients. Clinical manifestations were heart failure in five patients, cardiac murmur in two, dizziness in one, and palpitation in one patient. All patients, except two asymptomatic ones, had electrocardiographic abnormalities. Four patients required hospital admission for decompansated heart failure. Two patients died during follow-up while waiting for heart transplantation. Familial occurrence, ventricular tachycardia and thromboembolic events were not observed in any of the patients.
Conclusions: Although IVNC is present at birth, it may become clinically overt at any time from infancy through adolescence. Physicians and echocardiographers should be familiar with the diagnostic pattern of ventricular noncompaction in order to prevent any delay in diagnosis. Since associated morbidity and mortality rates are high, these patients require regular follow-up.