Long-Term Prognosis of Patients With Left Ventricular Noncompaction　― Comparison Between Infantile and Juvenile Types ―

• Wang Ce

  Department of Pediatrics, Faculty of Medicine, University of Toyama Department of Pediatrics, Shengjing Hospital of China Medical University

• Takasaki Asami

  Department of Pediatrics, Faculty of Medicine, University of Toyama

• Watanabe Ozawa Sayaka

  Department of Pediatrics, Faculty of Medicine, University of Toyama

• Nakaoka Hideyuki

  Department of Pediatrics, Faculty of Medicine, University of Toyama

• Okabe Mako

  Department of Pediatrics, Faculty of Medicine, University of Toyama

• Miyao Nariaki

  Department of Pediatrics, Faculty of Medicine, University of Toyama

• Saito Kazuyoshi

  Department of Pediatrics, Faculty of Medicine, University of Toyama

• Ibuki Keijiro

  Department of Pediatrics, Faculty of Medicine, University of Toyama

• Hirono Keiichi

  Department of Pediatrics, Faculty of Medicine, University of Toyama

• Yoshimura Naoki

  Department of Thoracic and Cardiovascular Surgery, Faculty of Medicine, University of Toyama

• Yu Xianyi

  Department of Pediatrics, Shengjing Hospital of China Medical University

• Ichida Fukiko

  Department of Pediatrics, Faculty of Medicine, University of Toyama

Abstract

Background:The natural history of left ventricular noncompaction (LVNC) is largely unsolved, so the aim of the present study was to clarify the clinical features and long-term prognosis of children with LVNC until adulthood. Methods and Results:We conducted a nationwide survey over 20 years and compared the clinical features, anatomical characteristics and long-term prognosis of 205 patients divided into 2 classifications: infantile type (diagnosed at <1 year of age: 108 cases) and juvenile type (diagnosed 1–15 years of age: 97 cases). Most patients diagnosed during infancy had heart failure (HF) at initial presentation (60.19%), while the majority of juvenile cases were asymptomatic (53.61%) but their event-free survival rate decreased gradually, because of later HF, thromboembolism and fatal arrhythmias. The initial LVEF was significantly lower in the infantile type and correlated with the thickness of the compacted layer in the LV posterior wall (LVPWC) and LV end-diastolic dimension (LVDD) Z-score, but not to the noncompacted to compacted layer (N/C) ratio. Survival analysis showed prognosis was similarly poor for both types after 2 decades. The significant risk factors for death, heart transplantation or implantable cardioverter-defibrillator insertion were congestive HF at diagnosis and lower LVPWC Z-score but not age of onset. Conclusions:LVNC of both types showed poor long-term prognosis, therefore ongoing follow-up is recommended into adulthood. HF at diagnosis and LVPWC hypoplasia are major determinants of poor prognosis.

Journal

• Circulation Journal

  Circulation Journal 81 (5), 694-700, 2017

  The Japanese Circulation Society

Keywords

• Heart failure
• Infants
• Juveniles
• Noncompaction
• Prognosis

Details

• CRID: 1390282680085384576
• NII Article ID: 130005612070
• NII Book ID: AA11591968
• DOI: 10.1253/circj.cj-16-1114
• ISSN: 13474820
• ISSN: 13469843
• NDL BIB ID: 028129430
• PubMed: 28154298
• Web Site: https://ndlsearch.ndl.go.jp/books/R000000004-I028129430
• Web Site: https://www.jstage.jst.go.jp/article/circj/81/5/81_CJ-16-1114/_pdf
• Web Site: https://search.jamas.or.jp/link/ui/2017381432
• Text Lang: en
• Data Source:
  • JaLC
  • NDL
  • Crossref
  • PubMed
  • CiNii Articles
  • KAKEN
• Abstract License Flag: Disallowed