Objective – Uhl’s anomaly, a very rare anomaly with unknown aetiology, is characterized by complete or partial absence of the myocardium of the right ventricle. Arrythmogenic right ventricular dysplasia/ cardiomyopathy (ARVD/C) is characterized by local deficiency or fibro-fatty replacement of the right ventricular myocardium. ARVD/C is an inherited cardiomyopathy with right ventricular dysfunction due to fibro-fatty replacement of myocardium, predisposing to ventricular tachycardia and death. ARVD/C and Uhl’s anomaly are considered as different manifestations of the same disease. Here we report a rare case of cardiomyopathy encountered at our tertiary hospital, aim is to discuss the clinical findings and the imagining methods.
Case report – A 12-year old girl presented with progressive breathlessness and palpitations. Patient was admitted with right-sided heart failure symptoms. She was treated for cardiac failure. X-ray shows gross cardiomegaly, Echocardiography showed a hugely dilated and diffuse hypokinetic right ventricle
Conclusion – Uhl’s anomaly is a rare cause of cardiomegaly, and echocardiography Is beneficial in the diagnostic process of this anomaly.
