Description
Uhl’s Syndrome is a rare cardiac condition characterised by a congenitally acquired total absence of right ventricular myocardium, with the left ventricular myocardium and atrial tissues remaining normal. This myocardial atrophy may also be associated with complete atrioventricular block attributable to absence of the His bundle, the AV node remaining intact. The mechanism by which this extremely selective muscle loss occurs is unknown, histological examination of the right heart revealing only adipose and fibrous tissue with apposition of right ventricular endocardium to the epicardium but no evidence of an inflammatory or infiltrative process.
