Tricuspid aortic stenosis with systolic dysfunction in a 14-year-old boy: Critical stenosis, noncompaction or secondary fibroelastosis?

A bicuspid aortic valve is the most common cause of isolated valvular aortic stenosis in the pediatric age group; another cause is annular hypoplasia, with the tricuspid valve being the least commonly involved. In aortic stenosis, causes of systolic dysfunction are critical stenosis, secondary fibroelastosis and noncompaction. Critical stenosis is diagnosed in the usual fashion. Secondary fibroelastosis is characterized by endocardial thickening, systolic dysfunction and distinctive bright echoes originating from the endocardium. Noncompaction is a congenital cardiomyopathy due to embryonic arrest of the normal development of the myocardium, leading to persistence of fetal myocardium in postnatal life. Although described in infants with aortic stenosis or atresia, the presence of noncompaction beyond infancy associated with aortic valve disease is exceedingly rare. The echocardiographic appearance of the ventricular myocardium in noncompaction is the distinctive presence of deep intramyocardial recesses. A case of left ventricular noncompaction in association with a tricuspid aortic valve with severe aortic stenosis with systolic dysfunction in a 14-year-old boy is reported.