ABSTRACT
Uhl's anomaly is a rare cardiomyopathy characterized by a thin walled dilated right ventricle due to the absence of myocardium. This could be due to an alteration in embriological development or the presence of massive apoptosis in the right ventricle myocardium. It generally manifests as cardiac failure, and the diagnosis is made by echocardiography and magnetic resonance. The response to medical treatment is poor and there is no known ideal treatment. Isolated cases of different surgical options have been described that include cardiac transplant, but there is still a poor prognosis and a high mortality rate. The case of a 10 year old girl with six months of thoracic pain and decreased exercise tolerance with echocardiographic and resonance findings characteristic of Uhl's anomaly is presented.
Subject(s)
Child , Female , Humans , Cardiomyopathy, Dilated , Heart Defects, Congenital , Heart Failure , Chest Pain , Diagnosis, Differential , Echocardiography , Heart Defects, Congenital , Heart Defects, Congenital , Heart Defects, Congenital , Magnetic Resonance Imaging , PrognosisABSTRACT
Uhl's anomaly is a rare cardiomyopathy characterized by a thin walled dilated right ventricle due to the absence of myocardium. This could be due to an alteration in embriological development or the presence of massive apoptosis in the right ventricle myocardium. It generally manifests as cardiac failure, and the diagnosis is made by echocardiography and magnetic resonance. The response to medical treatment is poor and there is no known ideal treatment. Isolated cases of different surgical options have been described that include cardiac transplant, but there is still a poor prognosis and a high mortality rate. The case of a 10 year old girl with six months of thoracic pain and decreased exercise tolerance with echocardiographic and resonance findings characteristic of Uhl's anomaly is presented.
