Turan Özlem, Kocabaş Abdullah
Department of Pediatric Cardiology, Antalya Training and Research Hospital, University of Health Sciences, Antalya, Türkiye.
Anatol J Cardiol. 2024 Sep 18;28(10):493-8. doi: 10.14744/AnatolJCardiol.2024.4599.
Duchenne muscular dystrophy (DMD)-related cardiomyopathy is associated with hemodynamic and conduction abnormalities and begins at an early age with subtle symptoms.
The study population included 55 patients with DMD and 54 healthy controls. We compared electrocardiogram (ECG), conventional echocardiography, and tissue Doppler imaging (TDI) assessments between patients with DMD and healthy controls. Also, we investigated atrial electromechanical delay, which has not been previously studied in DMD patients. Mitral, septal, and tricuspid segments were analyzed by TDI.
The mean age was 13.6 ± 2.5 years (range, 9.3-17.9 years) in the patient group and 12.8 ± 2.6 years (range, 8-17.5 years) in the control group (P = .1). Patients had higher heart rates, longer QTc intervals, and P-wave dispersion (PWD) than controls (P < .001, P = .004, P < .001, respectively). The patient group had larger left ventricular end-systolic dimension (P < .001), lower left ventricular ejection fraction (EF) (P < .001), MAPSE (P < .001), TAPSE (P < .001), and mitral-E/A (P = .029) values than control subjects. Myocardial performance index (P < .001) was higher, and the E'/A' ratio (P < .001) was lower at all 3 segments in the patient group. Also, atrial electromechanical delay was longer in the patient group at these segments (P < .001). Patients had significantly longer interatrial (P = .033) electromechanical conduction delays. EF was negatively correlated with atrial conduction time variables.
We have shown deterioration in systolic and diastolic function in both ventricles, PWD, and atrial conduction in children with DMD. Patients with DMD may be at risk of atrial arrhythmias due to disturbed atrial conduction.
杜兴氏肌营养不良症（DMD）相关的心肌病与血流动力学和传导异常有关，且在幼年时就开始出现细微症状。
研究人群包括55例DMD患者和54名健康对照者。我们比较了DMD患者与健康对照者之间的心电图（ECG）、传统超声心动图和组织多普勒成像（TDI）评估结果。此外，我们还研究了心房机电延迟，此前尚未在DMD患者中进行过此项研究。通过TDI分析二尖瓣、室间隔和三尖瓣节段。
患者组的平均年龄为13.6±2.5岁（范围9.3 - 17.9岁），对照组为12.8±2.6岁（范围8 - 17.5岁）（P = 0.1）。患者的心率更高，QTc间期和P波离散度（PWD）比对照组更长（分别为P < 0.001、P = 0.004、P < 0.001）。患者组的左心室收缩末期内径更大（P < 0.001），左心室射血分数（EF）更低（P < 0.001），心肌运动幅度（MAPSE）更低（P < 0.001），三尖瓣环平面收缩期位移（TAPSE）更低（P < 0.001），二尖瓣E/A值更低（P = 0.029）。患者组的心肌做功指数更高（P < 0.001），所有3个节段的E'/A'比值更低（P < 0.001）。此外，患者组这些节段的心房机电延迟更长（P < 0.001）。患者的房间（P = 0.033）机电传导延迟明显更长。EF与心房传导时间变量呈负相关。
我们已经证明DMD患儿的左右心室收缩和舒张功能、PWD以及心房传导均出现恶化。由于心房传导紊乱，DMD患者可能有发生房性心律失常的风险。
