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Pediatr Int. 2014 Dec;56(6):e82-e85. doi: 10.1111/ped.12430.

Postoperative mitral leaflet rupture in an infant with Loeys-Dietz syndrome.

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Department of Pediatrics, Fukui Cardiovascular Center, Fukui, Japan.
Department of Cardiovascular Surgery, Fukui Cardiovascular Center, Fukui, Japan.
Department of Pediatric Cardiovascular Surgery, Children's Medical Center, Kyoto Prefectural University of Medicine, Kyoto, Japan.
Division of Tumor Pathology, Department of Pathological Sciences, School of Medical Sciences, University of Fukui, Fukui, Japan.
Department of Bioscience and Genetics, National Cerebral and Cardiovascular Center Research Institute, Osaka, Japan.


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.


Loeys-Dietz syndrome; Marfan syndrome; congenital heart disease; interruption of aortic arch; mitral valve rupture

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