Detection of Progressive Cardiac Dysfunction by Serial Evaluation of Circumferential Strain in Patients With Duchenne Muscular Dystrophy

• Published in: The American journal of cardiology 2010-05, Vol.105 (10), p.1451-1455
• Main Authors: Hagenbuch, Sean C., MD, Gottliebson, William M., MD, MS, Wansapura, Janaka, PhD, Mazur, Wojciech, MD, Fleck, Robert, MD, Benson, D. Woodrow, MD, PhD, Hor, Kan N., MD
• Format: Article
• Language: English
• Subjects: Adolescent; Age Factors; Biological and medical sciences; Cardiology; Cardiology. Vascular system; Cardiomyopathies - diagnosis; Cardiomyopathies - etiology; Cardiovascular; Cardiovascular disease; Child; Cohort Studies; Coronary vessels; Disease Progression; Diseases of striated muscles. Neuromuscular diseases; Female; Fibrosis - diagnosis; Fibrosis - etiology; Humans; Magnetic Resonance Imaging - methods; Male; Medical sciences; Monitoring, Physiologic - methods; Muscular dystrophy; Muscular Dystrophy, Duchenne - complications; Muscular Dystrophy, Duchenne - diagnosis; Neurology; Probability; Prognosis; Risk Assessment; Severity of Illness Index; Sex Factors; Statistics, Nonparametric; Stroke Volume; Survival Rate; Time Factors; Ventricular Dysfunction, Left - diagnosis; Ventricular Dysfunction, Left - etiology
• ISSN: 0002-9149; 1879-1913
• DOI: 10.1016/j.amjcard.2009.12.070

The present study evaluated progressive cardiac dysfunction using serial circumferential strain (εcc ) measurements in patients with Duchenne muscular dystrophy (DMD). DMD is characterized by progressive cardiac dysfunction and myocardial fibrosis late in the disease process. We hypothesized that serial εcc changes could be detected in individual patients with DMD during a time when the left ventricular ejection fraction (EF) changes are insignificant. Cardiac magnetic resonance imaging data from patients with DMD were evaluated. The left ventricular EF was calculated from steady-state free precession cine images and the composite εcc measurement from tagged cine images. The serial εcc and EF values for each patient were analyzed using the Wilcoxon sign rank test. Data from 51 patients with DMD (2 studies per patient, mean age at the initial study 11.8 ± 3.5 years, range 7.4 to 25.4) were analyzed, with a mean interval between cardiac magnetic resonance studies of 15.6 ± 6.0 months (range 6.2 to 28.1). In the interval between studies, the εcc had decreased in all patients with DMD. The average decrease was 1.8 ± 1.3 (p