title: Prognostic impact of left ventricular noncompaction in patients with Duchenne/Becker muscular dystrophy--prospective multicenter cohort study.
abstract: BACKGROUND
  The reported prevalence of left ventricular noncompaction (LVNC) varies widely and its prognostic impact remains controversial. We sought to clarify the prevalence and prognostic impact of LVNC in patients with Duchenne/Becker muscular dystrophy (DMD/BMD).
  
  
  METHODS
  We evaluated the presence of LNVC in patients with DMD/BMD aged 4-64 years old at the study entry (from July 2007 to December 2008) and prospectively followed-up their subsequent courses (n=186). The study endpoint was all-cause death and the presence of LVNC was blinded until the end of the study (median follow-up: 46 months; interquartile range: 41-48 months).
  
  
  RESULTS
  There were no significant differences in baseline characteristics between patients with LVNC (n=35) and control patients without LVNC (n=151), with the exception of LV function. Patients with LVNC showed, in comparison with patients without LVNC, a significant negative correlation between age and LVEF (R=-0.7 vs. R=-0.4) at baseline; and showed a significantly greater decrease in absolute LVEF (-8.6 ± 4.6 vs. -4.3 ± 4.5, p<0.001) during the follow-up. A worse prognosis was observed in patients with LVNC (13/35 died) than in patients without LVNC (22/151 died, Log-rank p<0.001). Multivariate Cox analysis revealed that LVNC is an independent prognostic factor (relative hazard 2.67 [95% CI: 1.19-5.96]).
  
  
  CONCLUSIONS
  LVNC was prevalent in patients with DMD/BMD. The presence of LVNC is significantly associated with a rapid deterioration in LV function and higher mortality. Neurologists and cardiologists should pay more careful attention to the presence of LVNC.
authors: Cardiovascular Medicine, The University of Tokyo, Tokyo, Japan; Pharmacoepidemiology, The University of Tokyo, Tokyo, Japan. Electronic address: kimurak-int@h.u-tokyo.ac.jp.
authors: Koichi
authors: K
authors: Kimura
authors: Katsu
authors: K
authors: Takenaka
authors: Aya
authors: A
authors: Ebihara
authors: Kansei
authors: K
authors: Uno
authors: Hiroyuki
authors: H
authors: Morita
authors: Takashi
authors: T
authors: Nakajima
authors: Tetsuo
authors: T
authors: Ozawa
authors: Izumi
authors: I
authors: Aida
authors: Yosuke
authors: Y
authors: Yonemochi
authors: Shinya
authors: S
authors: Higuchi
authors: Yasufumi
authors: Y
authors: Motoyoshi
authors: Takashi
authors: T
authors: Mikata
authors: Idai
authors: I
authors: Uchida
authors: Tadayuki
authors: T
authors: Ishihara
authors: Tetsuo
authors: T
authors: Komori
authors: Ruriko
authors: R
authors: Kitao
authors: Tetsuya
authors: T
authors: Nagata
authors: Shin'ichi
authors: S
authors: Takeda
authors: Yutaka
authors: Y
authors: Yatomi
authors: Ryozo
authors: R
authors: Nagai
authors: Issei
authors: I
authors: Komuro
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D006352:Heart Ventricles / Q000000981:diagnostic imaging*; D006801:Humans; D056830:Isolated Noncompaction of the Ventricular Myocardium / Q000150:complications* / Q000175:diagnosis / Q000453:epidemiology; D007564:Japan / Q000453:epidemiology; D008297:Male; D008875:Middle Aged; D020388:Muscular Dystrophy, Duchenne / Q000150:complications*; D015995:Prevalence; D011379:Prognosis; D011446:Prospective Studies; D012307:Risk Factors; D015996:Survival Rate / Q000639:trends; D014463:Ultrasonography; D055815:Young Adult
keywords: Cardiomyopathy; Left ventricular noncompaction; Muscular dystrophy; Neuromuscular disease; Prognosis