Noncompaction of the right ventricle following Senning repair

A three-year-old boy presented with generalized edema, respiratory distress, prominent right ventricular impulse and hepatosplenomegaly. He had undergone Senning repair at one year of age. On his echocardiography, there were numerous prominent trabeculations and deep intertrabecular recesses measuring approximately 5 mm in depth along the free wall and right ventricular apex. Echogenicity of the endocardial surface was increased suggesting a fibrotic process in progress. Intertrabecular recesses were observed to be filling from the ventricle by color Doppler which is consistent with noncompaction. Various semilunar valve obstructions were shown to be responsible for the persistence of deep endomyocardial spaces surrounded by exeggerated hypertrophy of the trabeculae. This report presents the echocardiographic findings of right ventricular cardiomyopathy associated with dextroposition of the great arteries following Senning operation resembling noncompaction. Thus, this rare entity needs to be clarified regarding morphological criteria in distinction from other cardiomyopathies.