Abstract
Loeys-Dietz syndrome involves vascular abnormalities and aortic root aneurysms. This study described a 10-year-old boy who presented with progressively enlarging ascending aortic aneurysm and mild aortic valve regurgitation. He was diagnosed with Loeys-Dietz syndrome and underwent aortic valve-sparing repair. However, 6 months after the surgery, aortic root pseudoaneurysms were detected. Thus, aortic root repair was performed by transseptal approach. The orifices of pseudoaneurysms were closed with double-layer patches composed of autologous and bovine pericardial patches. Based on the findings of this study, we conclude that the transseptal approach is useful for closing aneurysms at the aortic root.