Segawa Kazuhiko, Sugawara Norio, Maruo Kazushi, Kimura Koichi, Komaki Hirofumi, Takahashi Yuji, Sasaki Masayuki
Department of Cardiology, National Center Hospital, National Center of Neurology and Psychiatry, Tokyo, Japan.
Department of Psychiatry, Dokkyo Medical University School of Medicine, Tochigi, Japan.
Neuropsychiatr Dis Treat. 2020 Jan 16;16:171-178. doi: 10.2147/NDT.S235166. eCollection 2020.
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.
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.
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).
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.
本研究旨在探讨左心室舒张末期内径（LVDd）能否预测接受标准心脏保护治疗的杜氏肌营养不良症（DMD）患者的心力衰竭死亡率。
本研究纳入了2011年至2015年间接受超声心动图检查的133例年龄≥10岁的DMD患者，并对其进行回顾性随访直至2018年8月。根据初次超声心动图检查时的LVDd将患者分为两组：≤54mm（第1组：n = 119）和≥55mm（第2组：n = 14）。为了确定可能影响心力衰竭相关死亡率的LVDd以外的因素，将观察期内未出现左心房（LA）扩大、中度二尖瓣反流（MR）或肺动脉高压（PH）的第2组患者（第2A组：n = 5）与新出现一种或多种这些并发症的患者（第2B组：n = 7）进行比较。临床结局为全因死亡率和心力衰竭死亡率。
第1组的平均观察期为5.5±1.5年，第2组为4.4±1.9年。共有14例患者（10.5%）死亡，其中第1组119例患者中有6例（5.0%），第2组14例患者中有8例（57.1%）（p<0.001）。其中，第1组有1例（0.8%）患者和第2组有8例（57.1%）患者死于心力衰竭（p<0.001）。第2B组患者的生存期比第2A组患者短（p = 0.006）。
LVDd≥55mm是DMD患者心力衰竭死亡率的预测因素。发现包括LA扩大、中度MR和PH在内的并发症是短期内心力衰竭死亡率的预测因素。
