Dilated cardiomyopathy associated with elephant trunk in Loeys-Dietz syndrome

A 14-year-old boy presented to our institution with a diagnosis of acute type A dissection. He was diagnosed with Loeys-Dietz syndrome and underwent aortic valve sparing repair at the age of 9 years. Emergency total arch repair with elephant trunk (ET) was performed successfully; echocardiogram before discharge showed normal left ventricular function and size. However, he was readmitted 1 month after discharge with significant left ventricular dysfunction and dilatation. The small folded ET caused a pressure gradient between the upper and lower body, which might deteriorate left ventricular function. Urgent balloon arterioplasty was performed to unfold the ET graft, resulting in no improvement of left ventricular function. ET removal and descending aorta replacement with an 18-mm graft was performed eventually. Left ventricular function and brain natriuretic peptide gradually improved after approximately 2 years of follow-up.