Isolated non compaction of left ventricular myocardium is a rare cardiomyopathy due to abnormal endomyocardialmorphogenesis.The incidence of non compaction cardiomyopathy in the general population has been estimated at 0.05% to 0.25% per year.NCCMP is a heart muscle disorder that is still little known among physicians and was first described in 1984 by engberding and bender.Echocardiography is diagnostic method of choice.We report our recent experience with six NCCM patients who present at our hospital between September2011and April 2012. Three patients were with severe symptoms, two patients with mild symptoms and one patient was asymptomatic. serial follow up and medical management is advisable in these patients for improving outcome.

BACKGROUND: Although isolated left ventricular noncompaction (ILVNC) has been described almost two decades ago, our knowledge about its diagnosis, presentation, echocardiographic features and clinical outcome is sparse. We aimed to assess the echocardiographic and clinical characteristics of ILVNC in a group of patients referred to our center.METHODS: Patients who were referred to a tertiary referral center, affiliated with Mashhad University of Medical Sciences, with primary diagnosis of dilated cardiomyopathy underwent comprehensive echocardiographic evaluation. The diagnosis of ILVNC was made based on the presence of two-structural layer in myocardium; ratio of noncompacted to compacted layers more than 2, and excessive trabeculation in the left ventricle.RESULTS: Final diagnoses of ILVNC were made in 42 patients. Mean age of patients was 32.9±15.6 years (ranging from 9 to 70 years). Females comprised a higher proportion of patients (61.9%) and shortness of breath was the most reported symptom among patients (47.6%). Non-compacted layers were detected in inferior and lateral segments of apex in 97.6% of patients. A total of 26 (61.9%) patients had left ventricle (LV) dysfunction (defined as ejection fraction less than 50%). The only factor that showed significant association with LV dysfunction was the number of affected segments with noncompaction (P=0.008). Reduced ejection fraction was not associated with either age or sex (P=0.437 and P=0.206, respectively).CONCLUSION: Based on the result of the current study, it can be suggested that apex of the heart is the most common site of noncompaction and increasing numbers of affected segments might be associated with LV dysfunction.