Noncompaction cardiomyopathy echocardiography

Overview
On echocardiography there is ventricular hypertrophy with deep recesses which tend to be located apically. Unless there is an awareness of the syndrome, the diagnosis is often missed in up to 89% of children. Likewise, the time from onset of symptoms to the diagnosis was more than 3 years in adult patients.

Conditions that Noncompaction Cardiomyopathy should be Distinguished form on Echocardiography

 * Normal varaint with < 3 prominent trabeculations
 * Left ventricular thrombus
 * An apical variant of hypertrophic obstructive cardiomyopathy
 * Endocardial fibroelastosis
 * Dilated cardiomyopathy
 * Arrhythmogenic right ventricular dysplasia
 * Metastases to the heart

==Transthoracic Echocardiographic Findings ==
 * There are deep trabeculations in the ventricular wall
 * There is systolic dysfunction with an average LVEF of 33%
 * It is notable that there is hypokinesis in both the noncompacted as well as the compacted segments. It has been speculated that this is due to the microcirculatory defect observed in these patients.
 * There was diastolic dysfunction in all 17 patients in one series, with 36% of these patients having a restrictive filling pattern.
 * The left ventricular wall is thick with a two layered appearance
 * The epicardial layer is compacted and thin and the endocardial layer is non-compacted and thick
 * The ratio of the non-compacted endocardial layer to the epicardial layer is > 2 in adults and >1.4 in children
 * 41% of patients will have involvement of the RV apex

Role of Transesophageal and Contrast Echocardiography in Improving Sensitivity and Specificity of the Diagnosis of Noncompaction Cardiomyopathy
Transesophageal echocardiography and contrast echocardiography have been advocated as methods for improving the sensitivity and specificity of detecting noncompaction cardiomyopathy.