Author + information
- Shaida Khan,
- Daniel Cheeran,
- Sonia Garg,
- Justin Grodin,
- Robert Morlend,
- Faris Araj,
- Alpesh Amin,
- Jennifer Thibodeau,
- Mark Drazner and
- Pradeep Mammen
Background: DMD-associated cardiomyopathy is the primary cause of death in DMD, but the mechanism has not been well characterized. In DMD, skeletal muscle degeneration and atrophy occurs. Therefore, we hypothesize that cardiac remodeling occurs secondary to cardiac atrophy rather than pathological cardiac hypertrophy leading to dilated cardiomyopathy. We compared MRI derived LV parameters between DMD and non-ischemic cardiomyopathy (NICM) patients.
Methods: Demographic data and cardiac MRI parameters were collected via retrospective chart review on 20 DMD patients and 20 age-and sex-matched patients with NICM. The LV mass index was calculated as the LV mass divided by the BSA. Data were expressed as mean ± standard error.
Results: DMD patients had a significantly lower LV mass index as compared to NICM patients (Figure: 54.8 ± 3.4 vs 76.4 ± 5.9, p<0.005). All volumetric variables were significantly lower in the DMD cohort as compared to the NICM cohort (LVEDV 133.76 ± 14.85 ml vs 245.3 ± 20.61 ml, p<0.001, normal: 136 ± 30 ml; LVESV: 73.2 ± 12.84 ml vs 170.35 ± 24.36 ml, p<0.005, normal: 45 ± 14 ml). Both cohorts had lower than normal EF; however, the NICM patients had a lower LVEF as compared to the DMD cohort (39 ± 5% vs 51 ± 3%, p<0.05, normal: 67 ± 5%).
Conclusions: DMD patients have significantly lower LV mass index as compared to subjects with NICM, suggesting that cardiac atrophy may be a potential mode of cardiac remodeling. These data identify a novel pathophysiologic mechanism of DMD-associated cardiomyopathy.
Poster Hall, Hall C
Sunday, March 19, 2017, 9:45 a.m.-10:30 a.m.
Session Title: The Evolving World of LVADs, Transplant and Other Novel Discoveries
Abstract Category: 13. Heart Failure and Cardiomyopathies: Clinical
Presentation Number: 1294-281
- 2017 American College of Cardiology Foundation