[Marfan-like aortic aneurysm syndrome; A surgical experience with Loeys-Dietz syndrome].

A 17-year-old female patient complaining of chest pain was transferred to our hospital to treat annuloaortic ectasia associated with severe aortic valve insufficiency. She underwent a valve sparing operation with graft replacement of the ascending aorta and the proximal portion of the aortic arch. After the 1st operation, she was diagnosed with Loeys-Dietz syndrome (LDS), which is recently described as an autosomal dominant aortic aneurysm syndrome caused by heterozygous mutations in the transforming growth factor-beta receptor type 1 and 2 genes. Only 2 months after the 1st operation, she developed heart failure due to recurrence of aortic regurgitation. Computed tomography (CT) scan showed an expansion of the aortic arch. Total arch replacement using the elephant trunk technique and aortic valve replacement were performed successfully 4 months after the 1st operation. The rapidly progressive nature of the aortic disease in patients with LDS underscores the importance of meticulous surveillance of the entire aorta and the need for early surgical management.