Cardiorespiratory failure is a common cause of death for patients with Duchenne muscular dystrophy (DMD). Current therapies may delay the progression of heart failure for this population. Measuring left ventricular ejection fraction (LVEF) regularly can show the rate of decline in left ventricle function for a patient.[1]
This section illustrates how the results of several systolic function tests, and accompanying data, are represented using the SDTM.
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Add text on Ejection Fraction and DMD.
- "Duchenne muscular dystrophy is associated with progressive cardiorespiratory failure, including left ventricular dysfunction." (ref: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8941672/)
- "The heart is also affected by the lack of dystrophin, beginning with myocardial fibrosis in the left ventricular wall behind the mitral valve, to eventually affect the entire left ventricle." (ref: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8960570/#:~:text=DMD%20leads%20to%20loss%20of,failure%20%5B9%E2%80%9311%5D.)
- "Cardiomyopathy with systolic heart failure is the leading cause of death and lower LVEF is an independent predictor of mortality at younger ages in patients with DMD." (ref: https://openres.ersjournals.com/content/9/5/00176-2023)
The example below shows a cardiac magnetic resonance imaging procedure highlighting basic cardiac magnetic resonance systolic function tests such as ejection fraction and similar cardiovascular tests. Measurements such as cardiac index and spirometry are affected by body surface area (ref: https://www.ahajournals.org/doi/10.1161/CIRCIMAGING.115.003810). It is important in the DMD population to ensure the height and weight are current for accurate data collection (especially as the participant becomes wheelchair bound). Height and weight are represented in the vital signs domain; see Section 6.3.13 of the SDTMIG v3.4 (https://www.cdisc.org/standards/foundational/sdtmig) for more information.
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For more information on the domains used in this section, see:
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| SDTM. Ejection Fraction | SDTM. Ejection Fraction
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