title: Postoperative mitral leaflet rupture in an infant with Loeys-Dietz syndrome.
abstract: This report describes an infantile case of Loeys-Dietz syndrome (LDS) with spontaneous mitral leaflet rupture. The patient was diagnosed with a type B interruption of the aortic arch. Bilateral pulmonary artery banding was performed 5 days after birth. On the 53rd day, intra-cardiac repair was performed without valvuloplasty. Although the operation was successful, mitral regurgitation deteriorated at 4 weeks after operation. On the 88th day, a mitral valvuloplasty was performed and a severely ruptured anterior leaflet was observed. Seven days after valvuloplasty, the mitral valve insufficiency again worsened and a fourth operation was performed. Two tears were observed in the anterior and posterior mitral valve leaflets, and a mitral valve replacement was required. Subsequently, the patient was diagnosed with LDS according to gene mutational status. LDS is known to have a poor prognosis with cardiovascular complications, but valve rupture has not been previously reported in other cases.
authors.affiliation: Department of Pediatrics, Fukui Cardiovascular Center, Fukui, Japan.
authors.forename: Koichi
authors.initials: K
authors.lastname: Nishida
authors.affiliation: Department of Pediatrics, Fukui Cardiovascular Center, Fukui, Japan.
authors.forename: Satoshi
authors.initials: S
authors.lastname: Tamura
authors.affiliation: Department of Cardiovascular Surgery, Fukui Cardiovascular Center, Fukui, Japan.
authors.forename: Sachiko
authors.initials: S
authors.lastname: Yamazaki
authors.affiliation: Department of Cardiovascular Surgery, Fukui Cardiovascular Center, Fukui, Japan.
authors.forename: Ryo
authors.initials: R
authors.lastname: Sugita
authors.affiliation: Department of Pediatric Cardiovascular Surgery, Children's Medical Center, Kyoto Prefectural University of Medicine, Kyoto, Japan.
authors.forename: Masaaki
authors.initials: M
authors.lastname: Yamagishi
authors.affiliation: Division of Tumor Pathology, Department of Pathological Sciences, School of Medical Sciences, University of Fukui, Fukui, Japan.
authors.forename: Sakon
authors.initials: S
authors.lastname: Noriki
authors.affiliation: Department of Bioscience and Genetics, National Cerebral and Cardiovascular Center Research Institute, Osaka, Japan.
authors.forename: Hiroko
authors.initials: H
authors.lastname: Morisaki
mesh_terms: D002675:Child, Preschool; D006349:Heart Valve Diseases / Q000175:diagnosis* / Q000209:etiology* / Q000601:surgery; D006801:Humans; D055947:Loeys-Dietz Syndrome / Q000150:complications*; D008297:Male; D008943:Mitral Valve; D012422:Rupture, Spontaneous
keywords: Loeys-Dietz syndrome; Marfan syndrome; congenital heart disease; interruption of aortic arch; mitral valve rupture