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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.

Author information

1
Department of Pediatrics, Fukui Cardiovascular Center, Fukui, Japan.
2
Department of Cardiovascular Surgery, Fukui Cardiovascular Center, Fukui, Japan.
3
Department of Pediatric Cardiovascular Surgery, Children's Medical Center, Kyoto Prefectural University of Medicine, Kyoto, Japan.
4
Division of Tumor Pathology, Department of Pathological Sciences, School of Medical Sciences, University of Fukui, Fukui, Japan.
5
Department of Bioscience and Genetics, National Cerebral and Cardiovascular Center Research Institute, Osaka, Japan.

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.

KEYWORDS:

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

PMID:
25521989
DOI:
10.1111/ped.12430
[Indexed for MEDLINE]

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