Aortic Valve Replacement for Aortic Regurgitation with Rare Left Ventricular Non-Compaction

Left ventricular noncompaction cardiomyopathy is a rare type of congenital cardiomyopathy characterized by prematurely arrested compaction of the endocardial and myocardial fibers and the progressive deterioration of left ventricular contractility. This entity is a genetically heterogeneous disorder and has a wide spectrum of presentation from no symptoms to critical disabling congestive heart failure, which can appear at any age. The prognosis is therefore varied. An elderly patient with left ventricular noncompaction underwent aortic valve replacement for associated aortic regurgitation. Follow-up at two years after surgery revealed an improved clinical condition and recovered cardiac function. This is the fourth known aortic valve replacement in a patient with left ventricular noncompaction.