Mehmood Muddassir, Ambach Stephanie A, Taylor Michael D, Jefferies John L, Raman Subha V, Taylor Robin J, Sawani Hemant, Mathew Jacob, Mazur Wojciech, Hor Kan N, Al-Khalidi Hussein R
Allegheny General Hospital, Pittsburgh, PA, USA.
University of Cincinnati, Cincinnati, OH, USA.
Pediatr Cardiol. 2016 Jun;37(5):878-83. doi: 10.1007/s00246-016-1362-2. Epub 2016 Mar 2.
The relationship between pulmonary function and right ventricle (RV) in Duchenne muscular dystrophy (DMD) has not been evaluated. Using cardiac magnetic resonance (CMR), we describe the relationship of RV size and function with spirometry in a DMD cohort. Fifty-seven boys undergoing CMR and pulmonary function testing within 1 month at a single center (2013-2015) were enrolled. Comparisons of RV ejection fraction (RVEF) and end-diastolic volume index (RVEDVI) were made across categories of percent forced vital capacity (FVC%), and relationships were assessed. Mean age was 15.5 ± 3.5 years. Spirometry and CMR were performed within 3.9 ± 4.1 days. Median FVC% was 92.0 % (67.5-116.5 %). Twenty-three (40 %) patients had abnormal FVC% (<80 %) of which 13 (57 %) had mild (FVC% 60-79 %), 6 (26 %) had moderate (FVC% 40-59 %), and 4 (17 %) had severe (FVC <40 %) reductions. Mean RVEF was 58.3 ± 3.7 %. Patients with abnormal FVC% were older and had lower RVEF and RVEDVI. Both RVEF and RVEDVI were significantly associated with FVC% (r = 0.31, p = 0.02 and r = 0.39, p = 0.003, respectively). In a large DMD cohort, RVEF and RVEDVI were related to FVC%. Worsening respiratory status may guide monitoring of cardiac function in these patients.
杜氏肌营养不良症（DMD）患者的肺功能与右心室（RV）之间的关系尚未得到评估。我们使用心脏磁共振成像（CMR）技术，在一个DMD队列中描述了RV大小和功能与肺活量测定之间的关系。纳入了在2013年至2015年期间于单个中心1个月内接受CMR和肺功能测试的57名男孩。对不同类别用力肺活量百分比（FVC%）下的右心室射血分数（RVEF）和舒张末期容积指数（RVEDVI）进行比较，并评估它们之间的关系。平均年龄为15.5±3.5岁。肺活量测定和CMR在3.9±4.1天内完成。FVC%中位数为92.0%（67.5 - 116.5%）。23名（40%）患者的FVC%异常（<80%），其中13名（57%）为轻度（FVC% 60 - 79%），6名（26%）为中度（FVC% 40 - 59%），4名（17%）为重度（FVC<40%）降低。平均RVEF为58.3±3.7%。FVC%异常的患者年龄较大，RVEF和RVEDVI较低。RVEF和RVEDVI均与FVC%显著相关（r分别为0.31，p = 0.02和r = 0.39，p = 0.003）。在一个大型DMD队列中，RVEF和RVEDVI与FVC%相关。呼吸状态恶化可能指导对这些患者心脏功能的监测。
