Key result
Left ventricular end-diastolic diameter ≥ 55 mm predicted significantly higher mortality from heart failure compared to ≤ 54 mm (57.1% vs 0.8%, p<0.001) in patients with Duchenne muscular dystrophy.
Why the study?
It was unknown whether left ventricular end-diastolic diameter (LVDd) could predict mortality from heart failure in patients with Duchenne muscular dystrophy receiving standard cardio-protective therapies.
Does left ventricular end-diastolic diameter (LVDd) ≥ 55 mm predict mortality from heart failure in patients with Duchenne muscular dystrophy?
Cohort (n=133)
Does left ventricular end-diastolic diameter (LVDd) ≥ 55 mm predict mortality from heart failure in patients with Duchenne muscular dystrophy?
Absolute Event Rate: 57.1% vs 0.8%
p-value: p=<0.001
An LVDd ≥ 55 mm on echocardiography is a strong predictor of heart failure mortality in patients with Duchenne muscular dystrophy.
LVDd ≥55 mm may flag high HF mortality risk in DMD; hypothesis-generating and should not yet change practice.
PURPOSE: This study aimed to examine weather left ventricular end-diastolic diameter (LVDd) could predict mortality from heart failure in patients with Duchenne muscular dystrophy (DMD) receiving standard cardio-protective therapies. PATIENTS AND METHODS: One hundred thirty-three patients with DMD aged ≥10 years who underwent echocardiography from 2011 to 2015 were included in this study and retrospectively followed until August 2018. Patients were divided into two groups according to LVDd at initial echocardiography: ≤ 54 mm (Group 1: n=119) and ≥ 55 mm (Group 2: n=14). To identify factors other than LVDd that may affect heart failure-related mortality, Group 2 patients who developed no left atrial (LA) enlargement, moderate mitral regurgitation (MR), or pulmonary hypertension (PH) during the observation period (Group 2A: n=5) were compared with those who newly developed one or more of those complications (Group 2B: n=7). Clinical outcomes were all-cause mortality and mortality from heart failure. RESULTS: Mean observation period was 5.5±1.5 years in Group 1 and 4.4±1.9 years in Group 2. A total of 14 patients (10.5%) died, including 6 of 119 (5.0%) patients in Group 1 and 8 of 14 (57.1%) patients in Group 2 (p<0.001). Among these, 1 (0.8%) patient in Group 1 and 8 (57.1%) patients in Group 2 died from heart failure (p<0.001). Group 2B patients had shorter survival compared to Group 2A patients (p=0.006). CONCLUSION: LVDd ≥ 55 mm is a predictive factor for mortality from heart failure in patients with DMD. Complications including LA enlargement, moderate MR, and PH were found to be predictive factors for mortality from heart failure in a short period.
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Segawa et al. (2020) conducted a cohort in Duchenne muscular dystrophy (n=133). Left ventricular end-diastolic diameter (LVDd) ≥ 55 mm vs. LVDd ≤ 54 mm was evaluated on Mortality from heart failure (p=<0.001). Left ventricular end-diastolic diameter ≥ 55 mm predicted significantly higher mortality from heart failure compared to ≤ 54 mm (57.1% vs 0.8%, p<0.001) in patients with Duchenne muscular dystrophy.
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