T1 mapping is abnormal before decline in EF in patients with Becker and Duchenne muscular dystrophy
نویسندگان
چکیده
Background Patients with Duchenne muscular dystrophy (DMD) develop cardiomyopathy (CM) at an earlier age compared to Becker muscular dystrophy (BMD), but the age of onset within each diagnosis is variable. A method to predict CM onset associated with dystrophinopathy could alter therapeutic approaches and improve outcomes. Shortened post-contrast T1 relaxation times are an early marker of myocardial fibrosis and are abnormal in patients with DMD as described in our previous work. To our knowledge, T1 mapping has not been evaluated in BMD patients. We hypothesized that patients with BMD would also have abnormal T1 times and that these abnormalities would precede LV dysfunction.
منابع مشابه
T1 mapping in Becker muscular dystrophy patients detects diffuse microfibrosis prior to evidence of late gadolinium enhancement or cardiac dysfunction
Background Becker muscular dystrophy (BMD) is allelic with Duchenne muscular dystrophy and represents a milder skeletal muscle phenotype; however, cardiac dysfunction remains a significant clinical problem. Cardiac involvement in BMD precedes skeletal muscular decline, and cardiomyopathy often leads to death before the age of 60 years. Cardiac magnetic resonance (CMR) is used for ventricular fu...
متن کاملDetection of the Duplication in Exons 56-63 of Duchenne Muscular Dystrophy Patients with MLPA
Background Duchenne Muscular Dystrophy (DMD) is a deadly X-linked recessive disorder. This genetic disorder affects 1 among 3,500-5,000 males in the world. The majority of the patients are male, due to the type of inheritance. It affects most of the skeletal, the respiratory, and cardiac muscles, causing these vital organs to contract and eventually mortality.<br...
متن کاملMyocardial characterisation in Becker muscular dystrophy using T1 and T2 mapping
Methods From April 2012 to May 2014, 84 consecutive patients (age=38,6 ± 13,6 years) with Becker muscular dystrophy (BMD) and 12 control subjects underwent cardiac magnetic imaging (MRI) on a 3-T clinical scanner (Magnetom Trio Tim, Siemens Healthcare). T1 maps and apparent extracellular volume fraction (appECV) preand post-GdDOTA injection was performed with a prototype modified Look-Locker se...
متن کاملQuantitative left ventricular rotational mechanics in Duchenne and Becker muscular dystrophy patients
Background Duchenne/Becker muscular dystrophy (D/BMD) is associated with early onset cardiomyopathy, with cardiac fibrosis amongst the earliest manifestations. Changes in conventional estimates of cardiac function (e.g. ejection fraction, EF) may occur late in Duchenne and Becker muscular dystrophy (DMD/BMD). Cardiac MRI tagging is a noninvasive imaging biomarker for quantifying ventricular dys...
متن کاملP164: Adeno-Associated Viral Vectors in Duchenne Muscular Dystrophy
Duchenne muscular dystrophy (BMD) is an inherited X-link disease. The incidence of this muscle-wasting disease is 1:5000 male live births. Mutation in the gene coding for dystrophin is the main cause of BMD. Most cases of this disease succumb to respiratory and cardiac failure in 3rd to 4th decades. The slow progression of BMD and recent achievement of gene therapies make it as an appropriate c...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
عنوان ژورنال:
دوره 15 شماره
صفحات -
تاریخ انتشار 2013