NON-COMPACTION OF VENTRICLES by Dr. SATISH KUMAR

Non-compaction of the ventricular myocardium is a recently described ungrouped cardiomyopathy caused by the arrest of normal embryogenesis of the ventricles (intrauterine arrest of compaction of the left ventricular endocardial myocytes). It is further classified as isolated non-compaction of the ventricles (most frequently of the left one) and ventricular non-compaction associated with other congenital anomalies of the endocardium and myocardium, such as obstruction of the right or left ventricular outflow tracts, complex cyanotic congenital heart disease, and coronary artery anomalies.
In this anomaly, the recesses and clefts (trabeculations) formed by non-compaction of the endocardial tissue become filled with blood that is derived from the left ventricular cavity. These trabeculae can be identified by echocardio-graphy and communication with the cavity .can be shown with doppler imaging.
Patients with non-compaction of the left ventricle can develop heart failure, embolic events from left ventricular thrombi, and ventricular arrhythmias. The mortality rate for the condition is high, as it is with other types of cardiomyopathy. Management includes treatment of heart failure, arrhythmia, and emboli. Some patients may need an implanted internal defibrillator, and others may need cardiac transplantation.
We report a fully worked-up case of this rare but important disorder presenting as Dilated Cardiomyopathy with refractory heart failure.