The neuromuscular group at Great Ormond Street Hospital, London UK, have recently published their study comparing the effectiveness of echocardiogram with cardiac MRI in the assessment Duchenne cardiomyopathy in patients preparing for surgical procedures. This study was a retrospective evaluation of 35 patients, ages 12-18 years who were undergoing pre-operative cardiac assessments prior to scoliosis or lower limb surgeries. 20% of patients had cardiac dysfunction seen on cardiac MRI that was not evident by echocardiogram. The results clearly indicated that cardiac MRI was superior to echocardiography in the pre-operative assessment of Duchenne cardiomyopathy, particularly when images from the echocardiogram were not as clear as needed (“suboptimal”).
PPMD has been supportive of cardiac MRI over echocardiogram (when appropriate and available) for the assessment of Duchenne cardiomyopathy, both as part of cardiac evaluation and care, as well as in clinical trials. As you may recall, subsequent to PPMD’s submission of draft guidance to the FDA, PPMD and the National Heart, Lung and Blood Institute (NHLBI) of the National Institutes of Health (NIH) collaborated in convening a working group to explore contemporary issues in Duchenne cardiology (read a recap here). Attendees at that meeting included experts in pediatric and adult cardiology, clinical research, patient advocacy, and representatives of the FDA. During discussions at that meeting, the FDA indicated interest in receiving recommendations for cardiac surveillance and cardiac markers, to be included in current and future Duchenne clinical trials. In response to those questions, recommendations were developed by the Expert Working Group and submitted to the FDA for consideration for inclusion in the draft guidance.
How does this impact your cardiac care?
This article was sent to all center directors and cardiologists in the Certified Duchenne Care Centers. The information has been shared, as well, with the members of the NHLBI small group developing lifelong cardiac care guidelines in coordination with the Center for Disease Control and Prevention’s efforts to update the current guidelines for Duchenne care, and should be reflected in the updated guidelines.
It is our hope that as studies continue to demonstrate the improved value of cardiac MRI over echocardiogram, that these diagnostic tests will be ordered and financially covered more often. Parents should be encouraged to discuss cardiac MRI with their cardiac providers, if MRI is not routinely ordered.
For questions on cardiac MRI, please watch the attached webinar, which was part of PPMD’s cardiac webinar series: