myostatin is elevated in congenital heart disease and after mechanical unloading先天性心脏病和肌肉生长抑制素水平升高后机械卸载.pdfVIP

Myostatin Is Elevated in Congenital Heart Disease and After Mechanical Unloading 1 . 2. 3 2 2 Lawrence T. Bish * , Isaac George , Simon Maybaum , Jonathan Yang , Jonathan M. Chen , H. Lee Sweeney1 1 Department of Physiology, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania, United States of America, 2 Department of Surgery, Division of Cardiothoracic Surgery, College of Physicians and Surgeons, Columbia University, New York, New York, United States of America, 3 Department of Medicine, Division of Cardiology, Albert Einstein College of Medicine, New York, New York, United States of America Abstract Background: Myostatin is a negative regulator of skeletal muscle mass whose activity is upregulated in adult heart failure (HF); however, its role in congenital heart disease (CHD) is unknown. Methods: We studied myostatin and IGF-1 expression via Western blot in cardiac tissue at varying degrees of myocardial dysfunction and after biventricular support in CHD by collecting myocardial biopsies from four patient cohorts: A) adult subjects with no known cardiopulmonary disease (left ventricle, LV), (Adult Normal), (n = 5); B) pediatric subjects undergoing congenital cardiac surgery with normal RV size and function (right ventricular outflow tract, RVOT), (n = 3); C) pediatric subjects with worsening but hemodynamically stable LV failure [LV and right ventricle (LV, RV,)] with biopsy collected at the time of orthotopic heart transplant (OHT), (n = 7); and D) pediatric subjects with decompensated bi-ventricular failure on BiVAD support with biopsy collected at OHT (LV, RV, BiVAD), (n = 3). Results: The duration of HF was longest in OHT patients compared to BIVAD. The duration of BiVAD support was 4.3 61.9