title: Acute rupture of chordae tendineae of the mitral valve in infants: a nationwide survey in Japan exploring a new syndrome.
abstract: BACKGROUND
  Recently, infant cases of acute heart failure attributable to rupture of the mitral chordae tendineae have been reported. However, little is known about the pathogenesis and clinical course of this condition.
  
  
  RESULTS
  Ninety-five children with rupture of mitral chordae tendineae were identified in nationwide surveys of Japan diagnosed from 1995 to 2013. The clinical manifestations, management strategies, and prognosis were investigated. Eighty-one (85%) patients were between 4 and 6 months (median, 5 months) of age. In 63 (66%) patients, rupture occurred during the spring or summer. The underlying conditions before rupture included Kawasaki disease (10 cases), maternally derived anti-SSA antibodies (2 cases), and infective endocarditis (1 case). Surgery was performed in 80 patients (94 operations), and the final operations included plasty of mitral chordae in 52 cases and mechanical valve replacement in 26 cases. The histopathologic examinations of the mitral valves and chordae (n=28) revealed inflammatory reactions with predominant mononuclear cell infiltration in 18 cases (64%) and increased fibrous and myxoid tissue in 11 cases (39%), suggesting that nonbacterial infectious or autoimmune endocarditis and myxoid changes are involved in the pathogenesis. Eight patients (8.4%) died before (n=6) and shortly after (n=2) the operation, and significant neurological complications persisted in 10 cases (11%).
  
  
  CONCLUSIONS
  Acute heart failure attributable to rupture of the mitral chordae tendineae in infants is a unique disease resulting from diverse causes. This condition should be recognized as a significant cardiovascular disorder that may cause sudden onset of cardiogenic shock and death in infants.
authors: From the Department of Pediatric Cardiology, National Cerebral and Cardiovascular Center, Osaka, Japan (I.S., H.S., T.A., M.K., K. Kurosaki); the Department of Preventive Cardiology, National Cerebral and Cardiovascular Center, Osaka, Japan (K.N.); the Department of Cardiology, National Center for Child Health and Development, Tokyo, Japan (H.K.); the Department of Pediatric Cardiology, Tokyo Women's Medical University, Tokyo, Japan (T.N.); the Department of Pediatrics, Keio University School of Medicine, Tokyo, Japan (H.Y.); the Department of Cardiology, Fukuoka Children's Hospital, Fukuoka, Japan (K.S.); the Department of Clinical Pathology, National Cerebral and Cardiovascular Center, Osaka, Japan (Y.I.); the Department of Bioscience and Genetics, National Cerebral and Cardiovascular Center, Osaka, Japan (T.M.); and the Department of Pediatric Cardiac Surgery, National Cerebral and Cardiovascular Center, Osaka, Japan (T.H., K. Kagisaki, H.I.). shiraishi.isao.hp@ncvc.go.jp.
authors: Isao
authors: I
authors: Shiraishi
authors: From the Department of Pediatric Cardiology, National Cerebral and Cardiovascular Center, Osaka, Japan (I.S., H.S., T.A., M.K., K. Kurosaki); the Department of Preventive Cardiology, National Cerebral and Cardiovascular Center, Osaka, Japan (K.N.); the Department of Cardiology, National Center for Child Health and Development, Tokyo, Japan (H.K.); the Department of Pediatric Cardiology, Tokyo Women's Medical University, Tokyo, Japan (T.N.); the Department of Pediatrics, Keio University School of Medicine, Tokyo, Japan (H.Y.); the Department of Cardiology, Fukuoka Children's Hospital, Fukuoka, Japan (K.S.); the Department of Clinical Pathology, National Cerebral and Cardiovascular Center, Osaka, Japan (Y.I.); the Department of Bioscience and Genetics, National Cerebral and Cardiovascular Center, Osaka, Japan (T.M.); and the Department of Pediatric Cardiac Surgery, National Cerebral and Cardiovascular Center, Osaka, Japan (T.H., K. Kagisaki, H.I.).
authors: Kunihiro
authors: K
authors: Nishimura
authors: From the Department of Pediatric Cardiology, National Cerebral and Cardiovascular Center, Osaka, Japan (I.S., H.S., T.A., M.K., K. Kurosaki); the Department of Preventive Cardiology, National Cerebral and Cardiovascular Center, Osaka, Japan (K.N.); the Department of Cardiology, National Center for Child Health and Development, Tokyo, Japan (H.K.); the Department of Pediatric Cardiology, Tokyo Women's Medical University, Tokyo, Japan (T.N.); the Department of Pediatrics, Keio University School of Medicine, Tokyo, Japan (H.Y.); the Department of Cardiology, Fukuoka Children's Hospital, Fukuoka, Japan (K.S.); the Department of Clinical Pathology, National Cerebral and Cardiovascular Center, Osaka, Japan (Y.I.); the Department of Bioscience and Genetics, National Cerebral and Cardiovascular Center, Osaka, Japan (T.M.); and the Department of Pediatric Cardiac Surgery, National Cerebral and Cardiovascular Center, Osaka, Japan (T.H., K. Kagisaki, H.I.).
authors: Heima
authors: H
authors: Sakaguchi
authors: From the Department of Pediatric Cardiology, National Cerebral and Cardiovascular Center, Osaka, Japan (I.S., H.S., T.A., M.K., K. Kurosaki); the Department of Preventive Cardiology, National Cerebral and Cardiovascular Center, Osaka, Japan (K.N.); the Department of Cardiology, National Center for Child Health and Development, Tokyo, Japan (H.K.); the Department of Pediatric Cardiology, Tokyo Women's Medical University, Tokyo, Japan (T.N.); the Department of Pediatrics, Keio University School of Medicine, Tokyo, Japan (H.Y.); the Department of Cardiology, Fukuoka Children's Hospital, Fukuoka, Japan (K.S.); the Department of Clinical Pathology, National Cerebral and Cardiovascular Center, Osaka, Japan (Y.I.); the Department of Bioscience and Genetics, National Cerebral and Cardiovascular Center, Osaka, Japan (T.M.); and the Department of Pediatric Cardiac Surgery, National Cerebral and Cardiovascular Center, Osaka, Japan (T.H., K. Kagisaki, H.I.).
authors: Tadaaki
authors: T
authors: Abe
authors: From the Department of Pediatric Cardiology, National Cerebral and Cardiovascular Center, Osaka, Japan (I.S., H.S., T.A., M.K., K. Kurosaki); the Department of Preventive Cardiology, National Cerebral and Cardiovascular Center, Osaka, Japan (K.N.); the Department of Cardiology, National Center for Child Health and Development, Tokyo, Japan (H.K.); the Department of Pediatric Cardiology, Tokyo Women's Medical University, Tokyo, Japan (T.N.); the Department of Pediatrics, Keio University School of Medicine, Tokyo, Japan (H.Y.); the Department of Cardiology, Fukuoka Children's Hospital, Fukuoka, Japan (K.S.); the Department of Clinical Pathology, National Cerebral and Cardiovascular Center, Osaka, Japan (Y.I.); the Department of Bioscience and Genetics, National Cerebral and Cardiovascular Center, Osaka, Japan (T.M.); and the Department of Pediatric Cardiac Surgery, National Cerebral and Cardiovascular Center, Osaka, Japan (T.H., K. Kagisaki, H.I.).
authors: Masataka
authors: M
authors: Kitano
authors: From the Department of Pediatric Cardiology, National Cerebral and Cardiovascular Center, Osaka, Japan (I.S., H.S., T.A., M.K., K. Kurosaki); the Department of Preventive Cardiology, National Cerebral and Cardiovascular Center, Osaka, Japan (K.N.); the Department of Cardiology, National Center for Child Health and Development, Tokyo, Japan (H.K.); the Department of Pediatric Cardiology, Tokyo Women's Medical University, Tokyo, Japan (T.N.); the Department of Pediatrics, Keio University School of Medicine, Tokyo, Japan (H.Y.); the Department of Cardiology, Fukuoka Children's Hospital, Fukuoka, Japan (K.S.); the Department of Clinical Pathology, National Cerebral and Cardiovascular Center, Osaka, Japan (Y.I.); the Department of Bioscience and Genetics, National Cerebral and Cardiovascular Center, Osaka, Japan (T.M.); and the Department of Pediatric Cardiac Surgery, National Cerebral and Cardiovascular Center, Osaka, Japan (T.H., K. Kagisaki, H.I.).
authors: Kenichi
authors: K
authors: Kurosaki
authors: From the Department of Pediatric Cardiology, National Cerebral and Cardiovascular Center, Osaka, Japan (I.S., H.S., T.A., M.K., K. Kurosaki); the Department of Preventive Cardiology, National Cerebral and Cardiovascular Center, Osaka, Japan (K.N.); the Department of Cardiology, National Center for Child Health and Development, Tokyo, Japan (H.K.); the Department of Pediatric Cardiology, Tokyo Women's Medical University, Tokyo, Japan (T.N.); the Department of Pediatrics, Keio University School of Medicine, Tokyo, Japan (H.Y.); the Department of Cardiology, Fukuoka Children's Hospital, Fukuoka, Japan (K.S.); the Department of Clinical Pathology, National Cerebral and Cardiovascular Center, Osaka, Japan (Y.I.); the Department of Bioscience and Genetics, National Cerebral and Cardiovascular Center, Osaka, Japan (T.M.); and the Department of Pediatric Cardiac Surgery, National Cerebral and Cardiovascular Center, Osaka, Japan (T.H., K. Kagisaki, H.I.).
authors: Hitoshi
authors: H
authors: Kato
authors: From the Department of Pediatric Cardiology, National Cerebral and Cardiovascular Center, Osaka, Japan (I.S., H.S., T.A., M.K., K. Kurosaki); the Department of Preventive Cardiology, National Cerebral and Cardiovascular Center, Osaka, Japan (K.N.); the Department of Cardiology, National Center for Child Health and Development, Tokyo, Japan (H.K.); the Department of Pediatric Cardiology, Tokyo Women's Medical University, Tokyo, Japan (T.N.); the Department of Pediatrics, Keio University School of Medicine, Tokyo, Japan (H.Y.); the Department of Cardiology, Fukuoka Children's Hospital, Fukuoka, Japan (K.S.); the Department of Clinical Pathology, National Cerebral and Cardiovascular Center, Osaka, Japan (Y.I.); the Department of Bioscience and Genetics, National Cerebral and Cardiovascular Center, Osaka, Japan (T.M.); and the Department of Pediatric Cardiac Surgery, National Cerebral and Cardiovascular Center, Osaka, Japan (T.H., K. Kagisaki, H.I.).
authors: Toshio
authors: T
authors: Nakanishi
authors: From the Department of Pediatric Cardiology, National Cerebral and Cardiovascular Center, Osaka, Japan (I.S., H.S., T.A., M.K., K. Kurosaki); the Department of Preventive Cardiology, National Cerebral and Cardiovascular Center, Osaka, Japan (K.N.); the Department of Cardiology, National Center for Child Health and Development, Tokyo, Japan (H.K.); the Department of Pediatric Cardiology, Tokyo Women's Medical University, Tokyo, Japan (T.N.); the Department of Pediatrics, Keio University School of Medicine, Tokyo, Japan (H.Y.); the Department of Cardiology, Fukuoka Children's Hospital, Fukuoka, Japan (K.S.); the Department of Clinical Pathology, National Cerebral and Cardiovascular Center, Osaka, Japan (Y.I.); the Department of Bioscience and Genetics, National Cerebral and Cardiovascular Center, Osaka, Japan (T.M.); and the Department of Pediatric Cardiac Surgery, National Cerebral and Cardiovascular Center, Osaka, Japan (T.H., K. Kagisaki, H.I.).
authors: Hiroyuki
authors: H
authors: Yamagishi
authors: From the Department of Pediatric Cardiology, National Cerebral and Cardiovascular Center, Osaka, Japan (I.S., H.S., T.A., M.K., K. Kurosaki); the Department of Preventive Cardiology, National Cerebral and Cardiovascular Center, Osaka, Japan (K.N.); the Department of Cardiology, National Center for Child Health and Development, Tokyo, Japan (H.K.); the Department of Pediatric Cardiology, Tokyo Women's Medical University, Tokyo, Japan (T.N.); the Department of Pediatrics, Keio University School of Medicine, Tokyo, Japan (H.Y.); the Department of Cardiology, Fukuoka Children's Hospital, Fukuoka, Japan (K.S.); the Department of Clinical Pathology, National Cerebral and Cardiovascular Center, Osaka, Japan (Y.I.); the Department of Bioscience and Genetics, National Cerebral and Cardiovascular Center, Osaka, Japan (T.M.); and the Department of Pediatric Cardiac Surgery, National Cerebral and Cardiovascular Center, Osaka, Japan (T.H., K. Kagisaki, H.I.).
authors: Koichi
authors: K
authors: Sagawa
authors: From the Department of Pediatric Cardiology, National Cerebral and Cardiovascular Center, Osaka, Japan (I.S., H.S., T.A., M.K., K. Kurosaki); the Department of Preventive Cardiology, National Cerebral and Cardiovascular Center, Osaka, Japan (K.N.); the Department of Cardiology, National Center for Child Health and Development, Tokyo, Japan (H.K.); the Department of Pediatric Cardiology, Tokyo Women's Medical University, Tokyo, Japan (T.N.); the Department of Pediatrics, Keio University School of Medicine, Tokyo, Japan (H.Y.); the Department of Cardiology, Fukuoka Children's Hospital, Fukuoka, Japan (K.S.); the Department of Clinical Pathology, National Cerebral and Cardiovascular Center, Osaka, Japan (Y.I.); the Department of Bioscience and Genetics, National Cerebral and Cardiovascular Center, Osaka, Japan (T.M.); and the Department of Pediatric Cardiac Surgery, National Cerebral and Cardiovascular Center, Osaka, Japan (T.H., K. Kagisaki, H.I.).
authors: Yoshihiko
authors: Y
authors: Ikeda
authors: From the Department of Pediatric Cardiology, National Cerebral and Cardiovascular Center, Osaka, Japan (I.S., H.S., T.A., M.K., K. Kurosaki); the Department of Preventive Cardiology, National Cerebral and Cardiovascular Center, Osaka, Japan (K.N.); the Department of Cardiology, National Center for Child Health and Development, Tokyo, Japan (H.K.); the Department of Pediatric Cardiology, Tokyo Women's Medical University, Tokyo, Japan (T.N.); the Department of Pediatrics, Keio University School of Medicine, Tokyo, Japan (H.Y.); the Department of Cardiology, Fukuoka Children's Hospital, Fukuoka, Japan (K.S.); the Department of Clinical Pathology, National Cerebral and Cardiovascular Center, Osaka, Japan (Y.I.); the Department of Bioscience and Genetics, National Cerebral and Cardiovascular Center, Osaka, Japan (T.M.); and the Department of Pediatric Cardiac Surgery, National Cerebral and Cardiovascular Center, Osaka, Japan (T.H., K. Kagisaki, H.I.).
authors: Takayuki
authors: T
authors: Morisaki
authors: From the Department of Pediatric Cardiology, National Cerebral and Cardiovascular Center, Osaka, Japan (I.S., H.S., T.A., M.K., K. Kurosaki); the Department of Preventive Cardiology, National Cerebral and Cardiovascular Center, Osaka, Japan (K.N.); the Department of Cardiology, National Center for Child Health and Development, Tokyo, Japan (H.K.); the Department of Pediatric Cardiology, Tokyo Women's Medical University, Tokyo, Japan (T.N.); the Department of Pediatrics, Keio University School of Medicine, Tokyo, Japan (H.Y.); the Department of Cardiology, Fukuoka Children's Hospital, Fukuoka, Japan (K.S.); the Department of Clinical Pathology, National Cerebral and Cardiovascular Center, Osaka, Japan (Y.I.); the Department of Bioscience and Genetics, National Cerebral and Cardiovascular Center, Osaka, Japan (T.M.); and the Department of Pediatric Cardiac Surgery, National Cerebral and Cardiovascular Center, Osaka, Japan (T.H., K. Kagisaki, H.I.).
authors: Takaya
authors: T
authors: Hoashi
authors: From the Department of Pediatric Cardiology, National Cerebral and Cardiovascular Center, Osaka, Japan (I.S., H.S., T.A., M.K., K. Kurosaki); the Department of Preventive Cardiology, National Cerebral and Cardiovascular Center, Osaka, Japan (K.N.); the Department of Cardiology, National Center for Child Health and Development, Tokyo, Japan (H.K.); the Department of Pediatric Cardiology, Tokyo Women's Medical University, Tokyo, Japan (T.N.); the Department of Pediatrics, Keio University School of Medicine, Tokyo, Japan (H.Y.); the Department of Cardiology, Fukuoka Children's Hospital, Fukuoka, Japan (K.S.); the Department of Clinical Pathology, National Cerebral and Cardiovascular Center, Osaka, Japan (Y.I.); the Department of Bioscience and Genetics, National Cerebral and Cardiovascular Center, Osaka, Japan (T.M.); and the Department of Pediatric Cardiac Surgery, National Cerebral and Cardiovascular Center, Osaka, Japan (T.H., K. Kagisaki, H.I.).
authors: Koji
authors: K
authors: Kagisaki
authors: From the Department of Pediatric Cardiology, National Cerebral and Cardiovascular Center, Osaka, Japan (I.S., H.S., T.A., M.K., K. Kurosaki); the Department of Preventive Cardiology, National Cerebral and Cardiovascular Center, Osaka, Japan (K.N.); the Department of Cardiology, National Center for Child Health and Development, Tokyo, Japan (H.K.); the Department of Pediatric Cardiology, Tokyo Women's Medical University, Tokyo, Japan (T.N.); the Department of Pediatrics, Keio University School of Medicine, Tokyo, Japan (H.Y.); the Department of Cardiology, Fukuoka Children's Hospital, Fukuoka, Japan (K.S.); the Department of Clinical Pathology, National Cerebral and Cardiovascular Center, Osaka, Japan (Y.I.); the Department of Bioscience and Genetics, National Cerebral and Cardiovascular Center, Osaka, Japan (T.M.); and the Department of Pediatric Cardiac Surgery, National Cerebral and Cardiovascular Center, Osaka, Japan (T.H., K. Kagisaki, H.I.).
authors: Hajime
authors: H
authors: Ichikawa
mesh_terms: D013504:Cardiovascular Surgical Procedures; D002815:Chordae Tendineae; D015331:Cohort Studies; D005260:Female; D006306:Health Surveys; D006333:Heart Failure / Q000209:etiology; D006349:Heart Valve Diseases / Q000175:diagnosis* / Q000453:epidemiology* / Q000601:surgery; D019918:Heart Valve Prosthesis Implantation; D006801:Humans; D015994:Incidence; D007223:Infant; D007564:Japan / Q000453:epidemiology; D008297:Male; D008943:Mitral Valve; D011379:Prognosis; D012189:Retrospective Studies; D012422:Rupture, Spontaneous / Q000175:diagnosis / Q000453:epidemiology / Q000601:surgery; D013577:Syndrome
keywords: heart failure; mitral regurgitation; pathology, surgical; pediatrics