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Uhl’s anomaly: A rare form of congestive cardiac failure in early childhood


Uhl’s anomaly is a rare congenital anomaly of the heart in which there is a congenital absence of the right ventricular myocardium. Only a few cases have been reported in the literature. It normally presents in infancy with features of congestive cardiac failure; however, cases have been reported in adults with right heart failure. Although the prognosis is not good, palliative surgical modalities are being done in this population. We report below 2 cases of children with Uhl’s anomaly who presented to us with this rare condition and are un- der medical management.


uhl’s anomaly, congestive cardiac failure, right ventricle, myocardium, right ventricular dysfunction