Rare Case Report Of Uhl’s Anomaly Versus Arrythmogenic Right Ventricular Dysplasia (Arvd)

Research Article
Vijay Basayya Metgudmath., Suresh Vijaybasappa Patted., Prabhu Channabasappa Halkati., Sanjay Champalal Porwal., Sameer Suresh Ambar., Prasad Murigendrappa Renuka and Vaibhav Bansidhar Patil
DOI: 
xxx-xxxxx-xxxx
Subject: 
science
KeyWords: 
Uhl’s anomaly, Arrythmogenic right ventricular dysplasia/ cardiomyopathy (ARVD/C), Cardiomegaly.
Abstract: 

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.