Background: Isolated noncompaction of the left ventricular myocardium, a rare congenital disorder, is thought to be due to an arrest of myocardial morphogenesis. It is characterized by an excessively prominent trabecular meshwork and by deep intertrabecular recesses. The aim of this study was to clarify the late outcome of this malformation, reporting our experience on 5 pediatric patients observed for a period of 11 years.
Methods: We describe the clinical, electrocardiographic and echocardiographic findings of 5 pediatric cases, with an age ranging from 1 month to 10 years. The mean follow-up was 4.5 years (> 10 years in 1 patient).
Results: In no case did life-threatening ventricular arrhythmias or systemic embolic events occur. Four patients who developed heart failure have been submitted to digoxin, diuretic, angiotensin-converting enzyme and anticoagulant therapy. One of them is still asymptomatic and in good health. One patient died while on the waiting list for cardiac transplantation.
Conclusions: The appropriate and early diagnosis and treatment of this disorder can improve the outcome even though further studies will be needed to elucidate its long-term prognosis. At the same time, in view of the high incidence of a positive family history, evaluation of all members of the family is warranted.