Idiopathic Restrictive Cardiomyopathy in Children

• Ishida Hidekazu

  Department of Pediatrics, Osaka University Graduate School of Medicine

Bibliographic Information

Other Title

• 小児期発症の特発性拘束型心筋症

Abstract

Restrictive cardiomyopathy (RCM) is characterized by impaired ventricular diastolic function with preserved ventricular contraction and wall thickness. The prognosis of young children with idiopathic RCM is poor, and the 2-year transplant-free survival rate has been reported to be approximately 40%. No medical treatment has been established for ventricular diastolic dysfunction; thus, heart transplantation is the only way to cure end-stage heart failure in patients with RCM. Herein, the pathology, etiology, clinical examinations, and medical management of pediatric patients with RCM were reviewed. This article includes a clinical case review of pediatric patients with RCM in our institute and the results of in vitro experiments using cardiac fibroblasts derived from patients with RCM and exome sequencing.

Journal

• Pediatric Cardiology and Cardiac Surgery

  Pediatric Cardiology and Cardiac Surgery 37 (3), 184-192, 2021-11-01

  Japanese Society of Pediatric Cardiology and Cardiac Surgery

Keywords

• restrictive cardiomyopathy
• pediatric
• heart transplantation
• ventricular assist device
• exome sequence

Details

• CRID: 1390853719881505152
• NII Article ID: 130008140097
• DOI: 10.9794/jspccs.37.184
• ISSN: 21872988
• ISSN: 09111794
• Web Site: https://www.jstage.jst.go.jp/article/jspccs/37/3/37_2021-0003/_pdf
• Web Site: https://search.jamas.or.jp/link/ui/2022172505
• Text Lang: ja
• Data Source:
  • JaLC
  • Crossref
  • CiNii Articles
  • KAKEN
• Abstract License Flag: Disallowed