A case with Uhl's anomaly presenting with severe right heart failure.
Uhl's anomaly was first reported by Uhl in 1952 and is characterized by congenital partial or complete absence of right ventricular myocardium. It is a very rare anomaly with unknown aetiology. Associations with other congenital heart diseases, familial occurrency, sudden death and arrhythmia with Uhl's anomaly have been reported. Pathologic findings vary with the patient's age and severity of the right ventricular disorder. In infancy, it may occur with severe right-sided heart failure as well as asymptomatic cardiomegaly. Despite its rarity, Uhl's anomaly may be considered in patients with right ventricular failure due to dilated cardiomyopathy of the right ventricle. We report the case of six-year-old boy presenting with striking ascites due to severe right heart failure of Uhl's anomaly.
Department of Pediatrics, Gaziantep University, Gaziantep Medical Faculty, Turkey. mkilinc@gantep.edu.tr
M
M
Kilinc
I
I
Akdemir
E
E
Sivasli
D002316:Cardiotonic Agents / Q000627:therapeutic use; D002648:Child; D003937:Diagnosis, Differential; D004077:Digoxin / Q000627:therapeutic use; D004232:Diuretics / Q000627:therapeutic use; D004359:Drug Therapy, Combination; D004452:Echocardiography; D006325:Heart Atria / Q000002:abnormalities* / Q000000981:diagnostic imaging; D006330:Heart Defects, Congenital / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy; D006333:Heart Failure / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology*; D006801:Humans; D008297:Male; D011859:Radiography; D018497:Ventricular Dysfunction, Right / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology
D002316:Cardiotonic Agents; D004232:Diuretics; D004077:Digoxin