A Unique Association of Left Ventricular Noncompaction With Rheumatic Heart Disease

Left ventricular noncompaction cardiomyopathy (LVNC) is a type of primary genetic cardiomyopathy, which occurs during embryogenesis by the arrest in the ventricular myocardium compaction. LVNC is characterized by prominent wall trabeculations and intertrabecular recesses that communicate with the ventricular cavity. There are 2 types of cardiomyopathy: the first one is associated with other primary cardiac structural abnormalities like malfunctional cardiac valves as mentioned in the case report below and the second type is in which there are no other associated cardiac structural abnormalities also called isolated LV noncompaction cardiomyopathy. We report an association of severe rheumatic mitral valve disease and LV noncompaction with significantly reduced ejection fraction which is rare.