Cardiac involvement in Fukuyama muscular dystrophy is less severe than in Duchenne muscular dystrophy

Tetsushi Yamamoto, Mariko Ikeda, Hiroyuki Awano, Masaaki Matsumoto, Tomoko Lee, Risa Harada, Takamitsu Imanishi, Nobuhide Hayashi, Yoshitada Sakai, Ichiro Morioka, Yasuhiro Takeshima, Kazumoto Iijima, Jun Saegusa, Tatsushi Toda

Research output: Contribution to journalArticle

2 Citations (Scopus)

Abstract

Background One of the main complications in patients with muscular dystrophies is cardiac dysfunction. The literature on cardiac involvement in patients with Fukuyama congenital muscular dystrophy (FCMD) is limited. Aim To compare cardiac involvement between patients with FCMD and Duchenne muscular dystrophy (DMD). Methods We compared cardiac involvement between 30 patients with FCMD and 181 patients with DMD using echocardiography and serum biomarkers. All patients were receiving regular checkups at Kobe University Hospital. We used single regression analysis to compare echocardiographic parameters, age, and serum biomarkers. Results Almost all clinical and echocardiographic parameters were lower in patients with FCMD than DMD. The brain natriuretic peptide concentration in patients with FCMD showed no correlation with age or left ventricular ejection fraction (r = 0.231, p = 0.22 and r = 0.058, p = 0.76, respectively). A log-rank test revealed that the risk of left ventricular systolic dysfunction was lower in patients with FCMD than DMD (p = 0.046, hazard ratio = 0.348). Conclusion The clinical progression of cardiac dysfunction is significantly milder in patients with FCMD than DMD, while skeletal muscle involvement is significantly worse in patients with FCMD. These data suggest that the pathophysiological findings of FCMD can be explained by less severe cardiac dysfunction in FCMD than DMD.

Original languageEnglish
Pages (from-to)861-868
Number of pages8
JournalBrain and Development
Volume39
Issue number10
DOIs
Publication statusPublished - 01-11-2017

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Walker-Warburg Syndrome
Duchenne Muscular Dystrophy
Biomarkers
Muscular Dystrophies
Brain Natriuretic Peptide
Left Ventricular Dysfunction
Serum
Stroke Volume
Echocardiography

All Science Journal Classification (ASJC) codes

  • Pediatrics, Perinatology, and Child Health
  • Developmental Neuroscience
  • Clinical Neurology

Cite this

Yamamoto, Tetsushi ; Ikeda, Mariko ; Awano, Hiroyuki ; Matsumoto, Masaaki ; Lee, Tomoko ; Harada, Risa ; Imanishi, Takamitsu ; Hayashi, Nobuhide ; Sakai, Yoshitada ; Morioka, Ichiro ; Takeshima, Yasuhiro ; Iijima, Kazumoto ; Saegusa, Jun ; Toda, Tatsushi. / Cardiac involvement in Fukuyama muscular dystrophy is less severe than in Duchenne muscular dystrophy. In: Brain and Development. 2017 ; Vol. 39, No. 10. pp. 861-868.
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abstract = "Background One of the main complications in patients with muscular dystrophies is cardiac dysfunction. The literature on cardiac involvement in patients with Fukuyama congenital muscular dystrophy (FCMD) is limited. Aim To compare cardiac involvement between patients with FCMD and Duchenne muscular dystrophy (DMD). Methods We compared cardiac involvement between 30 patients with FCMD and 181 patients with DMD using echocardiography and serum biomarkers. All patients were receiving regular checkups at Kobe University Hospital. We used single regression analysis to compare echocardiographic parameters, age, and serum biomarkers. Results Almost all clinical and echocardiographic parameters were lower in patients with FCMD than DMD. The brain natriuretic peptide concentration in patients with FCMD showed no correlation with age or left ventricular ejection fraction (r = 0.231, p = 0.22 and r = 0.058, p = 0.76, respectively). A log-rank test revealed that the risk of left ventricular systolic dysfunction was lower in patients with FCMD than DMD (p = 0.046, hazard ratio = 0.348). Conclusion The clinical progression of cardiac dysfunction is significantly milder in patients with FCMD than DMD, while skeletal muscle involvement is significantly worse in patients with FCMD. These data suggest that the pathophysiological findings of FCMD can be explained by less severe cardiac dysfunction in FCMD than DMD.",
author = "Tetsushi Yamamoto and Mariko Ikeda and Hiroyuki Awano and Masaaki Matsumoto and Tomoko Lee and Risa Harada and Takamitsu Imanishi and Nobuhide Hayashi and Yoshitada Sakai and Ichiro Morioka and Yasuhiro Takeshima and Kazumoto Iijima and Jun Saegusa and Tatsushi Toda",
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Yamamoto, T, Ikeda, M, Awano, H, Matsumoto, M, Lee, T, Harada, R, Imanishi, T, Hayashi, N, Sakai, Y, Morioka, I, Takeshima, Y, Iijima, K, Saegusa, J & Toda, T 2017, 'Cardiac involvement in Fukuyama muscular dystrophy is less severe than in Duchenne muscular dystrophy', Brain and Development, vol. 39, no. 10, pp. 861-868. https://doi.org/10.1016/j.braindev.2017.05.008

Cardiac involvement in Fukuyama muscular dystrophy is less severe than in Duchenne muscular dystrophy. / Yamamoto, Tetsushi; Ikeda, Mariko; Awano, Hiroyuki; Matsumoto, Masaaki; Lee, Tomoko; Harada, Risa; Imanishi, Takamitsu; Hayashi, Nobuhide; Sakai, Yoshitada; Morioka, Ichiro; Takeshima, Yasuhiro; Iijima, Kazumoto; Saegusa, Jun; Toda, Tatsushi.

In: Brain and Development, Vol. 39, No. 10, 01.11.2017, p. 861-868.

Research output: Contribution to journalArticle

TY - JOUR

T1 - Cardiac involvement in Fukuyama muscular dystrophy is less severe than in Duchenne muscular dystrophy

AU - Yamamoto, Tetsushi

AU - Ikeda, Mariko

AU - Awano, Hiroyuki

AU - Matsumoto, Masaaki

AU - Lee, Tomoko

AU - Harada, Risa

AU - Imanishi, Takamitsu

AU - Hayashi, Nobuhide

AU - Sakai, Yoshitada

AU - Morioka, Ichiro

AU - Takeshima, Yasuhiro

AU - Iijima, Kazumoto

AU - Saegusa, Jun

AU - Toda, Tatsushi

PY - 2017/11/1

Y1 - 2017/11/1

N2 - Background One of the main complications in patients with muscular dystrophies is cardiac dysfunction. The literature on cardiac involvement in patients with Fukuyama congenital muscular dystrophy (FCMD) is limited. Aim To compare cardiac involvement between patients with FCMD and Duchenne muscular dystrophy (DMD). Methods We compared cardiac involvement between 30 patients with FCMD and 181 patients with DMD using echocardiography and serum biomarkers. All patients were receiving regular checkups at Kobe University Hospital. We used single regression analysis to compare echocardiographic parameters, age, and serum biomarkers. Results Almost all clinical and echocardiographic parameters were lower in patients with FCMD than DMD. The brain natriuretic peptide concentration in patients with FCMD showed no correlation with age or left ventricular ejection fraction (r = 0.231, p = 0.22 and r = 0.058, p = 0.76, respectively). A log-rank test revealed that the risk of left ventricular systolic dysfunction was lower in patients with FCMD than DMD (p = 0.046, hazard ratio = 0.348). Conclusion The clinical progression of cardiac dysfunction is significantly milder in patients with FCMD than DMD, while skeletal muscle involvement is significantly worse in patients with FCMD. These data suggest that the pathophysiological findings of FCMD can be explained by less severe cardiac dysfunction in FCMD than DMD.

AB - Background One of the main complications in patients with muscular dystrophies is cardiac dysfunction. The literature on cardiac involvement in patients with Fukuyama congenital muscular dystrophy (FCMD) is limited. Aim To compare cardiac involvement between patients with FCMD and Duchenne muscular dystrophy (DMD). Methods We compared cardiac involvement between 30 patients with FCMD and 181 patients with DMD using echocardiography and serum biomarkers. All patients were receiving regular checkups at Kobe University Hospital. We used single regression analysis to compare echocardiographic parameters, age, and serum biomarkers. Results Almost all clinical and echocardiographic parameters were lower in patients with FCMD than DMD. The brain natriuretic peptide concentration in patients with FCMD showed no correlation with age or left ventricular ejection fraction (r = 0.231, p = 0.22 and r = 0.058, p = 0.76, respectively). A log-rank test revealed that the risk of left ventricular systolic dysfunction was lower in patients with FCMD than DMD (p = 0.046, hazard ratio = 0.348). Conclusion The clinical progression of cardiac dysfunction is significantly milder in patients with FCMD than DMD, while skeletal muscle involvement is significantly worse in patients with FCMD. These data suggest that the pathophysiological findings of FCMD can be explained by less severe cardiac dysfunction in FCMD than DMD.

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