Use of CMR in patients with Duchenne disease
Duchenne muscular dystrophy (DMD) is a congenital disease caused by a mutation in the gene encoding the dystrophin protein. It is a deadly, as yet incurable disease that occurs in boys. DMD is manifested mainly by skeletal muscle damage, but also by heart dysfunction. Assessment of the early stages of cardiac involvement by echocardiography is very important in these patients, but often very difficult. Researchers from the International Clinical Research Center of St. Anne’s University Hospital Brno (FNUSA-ICRC) therefore aimed to test the use of cardiac magnetic resonance imaging.
The results of their study “Quantitative assessment of left ventricular longitudinal function and myocardial deformity in Duchenne muscular dystrophy” were published on the server of the Orphanet Journal of Rare Diseases (Impact Factor 3,52).
The study involved 51 patients with Duchenne muscular dystrophy and 18 follow-up observations. The aim of the study was to use cardiac magnetic resonance (CMR) analysis of strains (myocardial deformation within the cardiac cycle) and mitral annular plane systolic excursion (MAPSE) in the detection of early left ventricular development.
These methods have been shown to be effective in monitoring the early stages of heart disease in patients with DMD. “The results of cardiac magnetic resonance imaging showed that the studied patients had reduced left ventricular systolic function as measured by MAPSE,” said doc. MUDr. Roman Panovský Ph.D., Faculty of Medicine, Masaryk University, head of the FNUSA-ICRC Cardiovascular Magnetic Resonance Research Team, added: “Even if the ejection fraction was completely normal.”