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

A patient who underwent cardiac surgery for pulmonary valve stenosis at the age of nine years subsequently presented as an adult with signs and symptoms of severe congestive heart failure. At echocardiography, the left ventricle showed an increased trabecular pattern, mainly in the region of the apex, the mitral annulus was severely dilated with mitral incompetence, the right ventricular outflow 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 of left ventricular non-compaction in patients with congenital left or right ventricular outflow tract 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 in familial cases. Often, the condition is characterized by arrhythmias, thromboembolic events, and heart failure, though affected individuals may not be symptomatic. The peculiarity of the present case was a strange association between the 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.