Ventricular Arrhythmia in the X-linked Cardiomyopathy Barth Syndrome

  title={Ventricular Arrhythmia in the X-linked Cardiomyopathy Barth Syndrome},
  author={Carolyn T. Spencer and Barry Byrne and Michael H. Gewitz and Stephanie Burns Wechsler and Andrew C Kao and Edward P Gerstenfeld and A. D. Merliss and Michael P. Carboni and Randall M. Bryant},
  journal={Pediatric Cardiology},
Barth syndrome is an X-linked disorder characterized by dilated cardiomyopathy, cyclic neutropenia, skeletal myopathy, abnormal mitochondria, and growth deficiency. The primary defect is a mutation in the TAZ gene on the X chromosome at Xq28, resulting in abnormal phospholipid biosynthesis and cardiolipin deficiency. To date, there has been no systematic evaluation of the cardiac phenotype. We report five cases of cardiac arrest and/or placement of an internal cardiac defibrillator with… CONTINUE READING
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