Abstract 11876: A Prospective Cohort Study of Left Ventricular Noncompaction in Patients with Duchenne/Becker Muscular Dystrophy
BACKGROUND: Although studies have shown that neuromuscular diseases are most frequently associated with LV noncompaction (LVNC), the prevalence of LVNC varies widely and its prognostic impact remains controversial because most studies conducted to date have been retrospective and lacked adequate controls.
METHODS: Patients with Duchenne/Becker muscular dystrophy (DMD/BMD) aged 4-64 years old (n=186) were evaluated by echocardiography at the study entry and prospectively followed-up their subsequent courses. The study endpoint was all-cause death and the presence of LVNC was blinded until the end of the study (median follow-up: 46 months; interquartile range: 41-48 months).
RESULTS: LVNC was diagnosed in 35 (19%) out of the 186 patients. There were no significant differences in the baseline distributions of DMD/BMD type, age, blood pressure, heart rate, medications administered, and follow-up duration between patients with and without LVNC, with the exception of LV function. A worse prognosis was observed (Figure) in patients with LVNC (13/35 died) than in patients without LVNC (22/151 died). Multivariate Cox analysis revealed that LVNC is an independent prognostic factor (relative hazard 2.7 [95% CI: 1.2-6.0]).
CONCLUSION: LVNC was prevalent in patients with DMD/BMD. The presence of LVNC was significantly associated with higher mortality rate. Neurologists and cardiologists should pay more careful attention to the presence of LVNC.
- © 2012 by American Heart Association, Inc.