title: Mechanism of aortic root dilation and cardiovascular function in tetralogy of Fallot.
abstract: The aortic root dilation in tetralogy of Fallot (TOF) is a long-term clinical problem, because a severely dilated aorta can lead to aortic regurgitation, dissection, or rupture, which can be fatal, necessitating surgical intervention. The details of the mechanism of aortic root dilation, however, are unclear. We have shown that aortic stiffness is increased in patients with repaired TOF, and may mirror the histological abnormality of elastic fiber disruption and matrix expansion. This aortic stiffness is related closely to the aortic dilation, indicating that aortic stiffness may be a predictor of outcome of aortic dilation. Furthermore, the aortic volume overload is a very important determinant of aortic diameter in TOF patients before corrective surgery. In addition, a chromosomal abnormality and the transforming growth factor-β signaling pathway, a major contributor to aortic dilation in Marfan syndrome, also affect this mechanism. In this way, aortic dilation in TOF patients is suggested to be a multifactorial disorder. The aim of this review was therefore to clarify the mechanism of aortic dilation in TOF, focusing on recent research findings. Studies linking histopathology, mechanical properties, molecular/cellular physiology, and clinical manifestations of aortic dilation facilitate appropriate treatment intervention and improvement of long-term prognosis of TOF.
authors.affiliation: Division of Pediatric Cardiology, Saitama Medical Center, Saitama Medical University, Kawagoe, Saitama, Japan.|Department of Pediatrics, Gunma University School of Medicine, Maebashi, Gunma, Japan.
authors.forename: Mitsuru
authors.initials: M
authors.lastname: Seki
authors.affiliation: Division of Pediatric Cardiology, Saitama Medical Center, Saitama Medical University, Kawagoe, Saitama, Japan.
authors.forename: Seiko
authors.initials: S
authors.lastname: Kuwata
authors.affiliation: Division of Pediatric Cardiology, Saitama Medical Center, Saitama Medical University, Kawagoe, Saitama, Japan.|Department of Pediatric Cardiology, Tokyo Women's Medical University, Shinjuku, Tokyo, Japan.
authors.forename: Clara
authors.initials: C
authors.lastname: Kurishima
authors.affiliation: Division of Pediatric Cardiology, Saitama Medical Center, Saitama Medical University, Kawagoe, Saitama, Japan.|Johns Hopkins Medical Institute, Baltimore, Maryland, USA.
authors.forename: Ryo
authors.initials: R
authors.lastname: Nakagawa
authors.affiliation: Department of Pediatrics, University of Tokyo Hospital, Bunkyo, Tokyo, Japan.
authors.forename: Ryo
authors.initials: R
authors.lastname: Inuzuka
authors.affiliation: Department of Pediatrics, Asahikawa Medical University, Asahikawa, Hokkaido, Japan.
authors.forename: Masaya
authors.initials: M
authors.lastname: Sugimoto
authors.affiliation: Division of Pediatric Cardiology, Saitama Medical Center, Saitama Medical University, Kawagoe, Saitama, Japan.|Mayo Clinic, Rochester, Minnesota, USA.
authors.forename: Hirofumi
authors.initials: H
authors.lastname: Saiki
authors.affiliation: Division of Pediatric Cardiology, Saitama Medical Center, Saitama Medical University, Kawagoe, Saitama, Japan.
authors.forename: Yoichi
authors.initials: Y
authors.lastname: Iwamoto
authors.affiliation: Division of Pediatric Cardiology, Saitama Medical Center, Saitama Medical University, Kawagoe, Saitama, Japan.
authors.forename: Hirotaka
authors.initials: H
authors.lastname: Ishido
authors.affiliation: Division of Pediatric Cardiology, Saitama Medical Center, Saitama Medical University, Kawagoe, Saitama, Japan.
authors.forename: Satoshi
authors.initials: S
authors.lastname: Masutani
authors.affiliation: Division of Pediatric Cardiology, Saitama Medical Center, Saitama Medical University, Kawagoe, Saitama, Japan.
authors.forename: Hideaki
authors.initials: H
authors.lastname: Senzaki
mesh_terms: D001011:Aorta / Q000473:pathology* / Q000503:physiopathology; D004108:Dilatation, Pathologic; D006801:Humans; D013771:Tetralogy of Fallot / Q000235:genetics / Q000473:pathology* / Q000503:physiopathology; D059289:Vascular Stiffness
keywords: aortic dilation; aortopathy; mechanism; stiffness; tetralogy of Fallot