Long-term prognosis of patients with left ventricular noncompaction: Comparison between infantile and juvenile types

Ce Wang, Asami Takasaki, Sayaka Watanabe Ozawa, Hideyuki Nakaoka, Mako Okabe, Nariaki Miyao, Kazuyoshi Saito, Keijiro Ibuki, Keiichi Hirono, Naoki Yoshimura, Xianyi Yu, Fukiko Ichida

研究成果: Article査読

17 被引用数 (Scopus)

抄録

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.

本文言語English
ページ(範囲)694-700
ページ数7
ジャーナルCirculation Journal
81
5
DOI
出版ステータスPublished - 2017
外部発表はい

All Science Journal Classification (ASJC) codes

  • 循環器および心血管医学

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