Objective: This quality improvement project is ongoing to evaluate the impact of cardiologists joining the MDA clinic in August 2025 and adherence to cardiac guidelines of care.
Background: Guidelines for cardiac monitoring and care of patients with DMD include imaging with electrocardiogram (EKG), echocardiogram (ECHO) and magnetic resonance imaging (cardiac MRI). Cardioprotective treatment is recommended to be started at age 10 years and augmented as needed for signs of left ventricular dysfunction. Lab monitoring per prescribing guidelines should be conducted at regular intervals. Due to cardiac remodeling, the utility and significance of troponin-I levels is still being investigated.
Methods: Retrospective chart review was conducted for the cohort of patients (N=46) with DMD seen from 2023-July 2025. Seven (n=7) patients are followed by outside cardiologist. Statistical analysis of data conducted to determine adherence to guidelines and correlation with lab monitoring, imaging results and treatment.
Results: 74% (29/39) of patients had their first cardiology visit at or before age 10 years old. In the 30 DMD patients 10 years and older, 63% (19/30) of patients have seen cardiology in the past year. 66.7% (20/30) of these patients are prescribed a cardioprotective medication. 16% (5/30) of patients were started on this medication before age 11 years old. 19% (7/37) of patients demonstrated left ventricular dysfunction by ECHO. No patients in this cohort have had a cardiac MRI. 12 patients had troponin-I levels measured, but only 7 patients have had multiple troponin-I levels recorded.
Conclusion: Majority of patients demonstrate adherence to guidelines for cardiac visits and monitoring with EKG and ECHO. On average, patients were started on cardioprotective medications later than age 10 years. Additional basic metabolic, cystatin-C, and troponin measurements are needed to investigate whether there is a longitudinal correlation with heart function or medication management. Data from the pre-intervention phase will be used to inform resource allocation.