Massive mitral and pulmonary valve incompetence in a patient with left ventricular, non-compacted myocardium

The case is reported of a patient who underwent cardiac surgery for pulmonary valve stenosis as a child, and presented as an adult with signs and symptoms of severe congestive heart failure. The left ventricle showed an increased trabecular pattern in the region of the apex, the mitral annulus was severely dilated with mitral incompetence, the right ventricular out-flow tract (RVOT) was largely dilated with aneurysm of both pulmonary arteries, and there was evidence of pulmonary valve incompetence. Previously, rare cases have been reported of persistent left ventricular non-compaction in patients with congenital left or RVOT obstruction. Non-compaction of the ventricular myocardium is an inherited autosomal dominant disorder; to date, four genes and one genetic locus have been found to be associated with non-compacted ventricular myocardium. The condition is characterized by arrhythmias, thromboembolic events and heart failure, but affected individuals may not be symptomatic. The present case represented a strange association between non-compacted left ventricle, mitral annular dilation with persistence of a normal leaflet and subvalvular mitral valve apparatus, and RVOT dilation with pulmonary artery aneurysms.