Ceylan Özben, Özgür Senem, Örün Utku Arman, Doğan Vehbi, Yılmaz Osman, Keskin Mahmut, Arı Mehmet Emre, Erdoğan Özlem, Karademir Selmin
Department of Pediatric Cardiology, Dr. Sami Ulus Children's Hospital; Ankara-Turkey.
Anatol J Cardiol. 2015 Aug;15(8):663-8. doi: 10.5152/akd.2014.5544. Epub 2014 Aug 19.
This study assessed the early changes in regional and global systolic and diastolic myocardial functions in patients with familial Mediterranean fever without any cardiovascular symptoms using tissue Doppler and strain and strain rate echocardiography and compared them to the results of a control group.
This study has a cross-sectional and observational design. FMF patients with normal left ventricular function were included in the study. We excluded patients who had arrhythmia, acquired/congenital heart disease, pericarditis, or acute attack. We compared 45 children with familial Mediterranean fever on colchicine therapy and 45 age- and sex-matched healthy children.
The 45 patients with familial Mediterranean fever included 24 (55.3%) girls and 21 (46.7%) boys with a mean age of 11.3 ± 3.7 (range 2-18) years. The mean disease duration was 4.6 ± 2.4 (range 0.5-10) years. In the patient group, the homozygous M694V mutation was the most common (64.4%) mutation. The patients with familial Mediterranean fever had statistically lower longitudinal global strain, radial global strain, and strain rates (-14.44 ± 4.77%, 14.80 ± 6.29%, and 0.59 ± 0.24 s, respectively) than the controls (-17.40 ± 1.79%, 17.53 ± 4.63%, and 0.83 ± 0.51 s) (p < 0.05). The circumferential global strain did not differ significantly between the groups.
Patients with familial Mediterranean fever who are subclinical from a cardiac aspect might have normal left ventricular function as measured by conventional echocardiography. However, the disease affects their myocardial tissue, and these patients should be followed with conventional, strain, and strain rate echocardiography techniques regularly.
本研究使用组织多普勒、应变及应变率超声心动图评估无任何心血管症状的家族性地中海热患者局部和整体收缩及舒张期心肌功能的早期变化,并将其与对照组结果进行比较。
本研究采用横断面观察性设计。纳入左心室功能正常的家族性地中海热患者。排除患有心律失常、获得性/先天性心脏病、心包炎或急性发作的患者。我们比较了45例接受秋水仙碱治疗的家族性地中海热儿童和45例年龄及性别匹配的健康儿童。
45例家族性地中海热患者中,女孩24例(55.3%),男孩21例(46.7%),平均年龄11.3±3.7(2 - 18)岁。平均病程为4.6±2.4(0.5 - 10)年。患者组中,纯合子M694V突变最为常见(64.4%)。家族性地中海热患者的纵向整体应变、径向整体应变及应变率(分别为-14.44±4.77%、14.80±6.29%和0.59±0.24 s)在统计学上低于对照组(-17.40±1.79%、17.53±4.63%和0.83±0.51 s)(p<0.05)。两组间圆周整体应变无显著差异。
从心脏方面来看处于亚临床状态的家族性地中海热患者,通过传统超声心动图测量其左心室功能可能正常。然而,该疾病会影响其心肌组织,这些患者应定期采用传统、应变及应变率超声心动图技术进行随访。