BENTALL OPERATION FOR A CHILD WITH MARFAN-SYNDROME - A CASE-REPORT

Children with Marfan syndrome rarely undergo surgery for annuloaortic ectasia and aortic regurgitation in the first decade. A 7-year-old gir l presented with congestive heart failure due to severe aortic regurgi tation associated with annuloaortic ectasia (6 cm). She also had funne l chest. She underwent a Bentall operation and sternal turn-over with a satisfactory result. Since the aortic valve cusps had rolled edges, the aortic valve was not spared. Histology of the aortic valve cusps s howed myxoid degeneration and fragmentation of elastic fibers.