核素心肌灌注显像评价FH纯合子患者心肌血供异常临床意义初步研究

A study on the clinical significance of abnormal myocardial blood supply in homozygote patients with familial hypercholesterolemia by myocardial perfusion imaging

目的：利用核素心肌灌注显像（MPI）研究家族性高胆固醇血症（FH）纯合子患者病情进展对心肌血供的影响及临床意义.方法：以经染色体检测确诊并来我院检查的28例FH纯合子患者为研究对象,均行药物负荷/静息MPI,根据心肌血供异常与否,将患者分成两组,并对两组患者的基本资料、血脂水平及心肌血流灌注情况进行对比分析,其中8例患者随诊1～2年.结果：28例FH患者,男性17例,女性11例,平均年龄12.12岁,TC （16.00 ±2.92） mmol/L,LDLC（13.82 ±2.73） mmol/L;MPI示15例阴性（男/女=11/4例）,13例阳性（男/女=6/7例）.负荷试验中6例出现心电图ST段下移性改变.MPI显示：13例MPI阳性患者共计221个心肌节段中,29个节段出现心肌缺血（13.1％）;其中左前降支、回旋支、右冠状动脉供血区分别有21个节段（21/29,72.4％）、5个节段（5/29,17.2％）、3个节段（3/29,10.3％）.MPI阳性与阴性两组中,患者的年龄（P =0.621）、身高（P =0.219）、体质量（P =0.847）、体质量指数（P=0.155）、治疗时间（P=0.189）、性别（P =0.246）、心电图ST段有无下移（P =0.372）、TC（P=0.088）、LDL-C（P=0.082）、TG（P =0.062）、HDL-C（P=0.652）均差异无统计学意义.8例随诊患者复查MPI结果提示：4例原无心肌缺血现仍为阴性;4例原有心肌缺血者,3例心肌缺血范围和程度均无明显变化,1例因心肌缺血程度加重导致心力衰竭后死亡.结论：FH纯合子患者心肌缺血好发于左前降支供血区,且患者的年龄、身高、体质量、治疗时间、血脂等因素在MPI阳性组与阴性组中的差异无统计学意义;最好早期对FH纯合子患者行MPI以评估心血管病危险度分层、患者治疗效果和预后.

Objective：Investigating the effect and clinical value to myocardial blood supply in homozy- gous patients with the progress of familial hypercholesterolemia（FH） by myocardial perfusion imaging （MPI）. Methods：As the objects,28 FH patients diagnosed by gene underwent drug induced stress/rest MPI. Put the patients into 2 groups according to whether the myocardial blood supply is normal or not and analyze the basic data, blood lipid and MPI data of patients statistically. 8 patients were followed in 1-2 years. Results： Among 28 patients,17 males and 11 females, mean age （ 12. 12 ± 6. 4） years, TC （ 16. 00 ± 2. 92 ） mmol/L, LDL-C （ 13.82 ± 2. 73 ） mmol/L ; MPI showed 15 uegtive （ male/female = 11/4 ） and 13 positive （ male/female = 6/7 ）. ST segments of ECG moved down in 6 patients in stress test. There were myocardial ischemia in 29 myocardial segments（ 13. 12% ） in all 221 ones of 13 positive patients with MPI, and among 29 myocardial segments,21 （21/29,72. 41% ）,5（5/29,17.24% ）,3（3/29,10. 34% ）ones belonged to the blood-supply areas of the left anterior descending, the left circumflex artery and the right coronary artery respectively. In the positive and neg- tire groups of MPI, there were no significant differences in the patients of age, height, weight, BMI, treatment time,gender,moving down of ST segment in ECG,TC,LDLC,TG and HDLC. The MPI of 8 followed patients showed that：the results of 4 patients who underwent MPI with no myocardial ischemia previously were still neg- tire, and with the positive MPI patients, the area, extent of ischemia in 3 patients had no change, 1 patient got more serious myocardial ischemia and was dead subsequently because of heart failure. Conclusion： The ische- mia area with FH homozygous patients was easy uccur in left anterior descending artery, and the myocardial is- chemia had no obvious correlation with age, height, weight, treatment time and blood lipid and so on. They should be undergone MPI as earlier as possibIe to evaluate cardiovascular disease risk stratification, treatment ef- fectiveness and prognosis.