肌聚糖病的99Tc^m—MIBI门控心肌灌注断层显像评价

目的探讨99Tc^m一MIBIG—MPI评价肌聚糖病患者心肌受损的价值。方法临床、分子病理确诊的肌聚糖病患者8例（男3例，女5例，年龄10—30岁）、对照者4名均接受99TcmMIBIG—MPI。原始数据按常规处理成断层图像，再应用定量门控分析软件（QGS）进行左室功能定量分析。采用将左心室分为7个节段、20个亚段的5分制（0—4分）半定量方法，用于评估心肌受损的程度。结果8例患者99Tcm-MIBIG—MPI阳性7例。7例阳性患者140个心肌亚段中共检出59个亚段异常。按病变累及室壁节段（7个节段）分为：单一室壁节段异常1例，2个室壁节段异常2例，3个及3个以上室壁节段异常、病灶呈散在性分布4例。8例肌聚糖患者中5例患者左心室扩大，其中3例左室射血分数为（43．1±2．8）％。结论99Tcm-MIBIG—MPI能直观探测肌聚糖病患者的心肌损害，可作为肌聚糖病患者心肌损害早期诊断和远期随访的辅助诊断方法。

Detection of sarcoglycanopathy with 99Tcm-MIBI gated myocardial perfusion imaging

Objective To investigate the clinical value of 99Tcm-MIBI gated G-MPI in detecting the myocardial damage in sarcoglycanopathy.Methods 99 Tcm - MIBI G - MPI was performed in 8 patients (3 males,5 females,age ranged from 10 to 30 y) with sarcoglycanopathy confirmed by clinical results and molecular pathology and 4 healthy persons as control group.Quantitative gated SPECT (QGS) software was used for processing and interpretation.Myocardium of left ventricle was divided into 7 segments and 20 subsegments.A five-point scoring system was used to evaluate the myocardial damage.Results Seven patients showed positive results in G-MPI (7/8).Fifty-nine sub-segments of injured myocardium were detected in the 140 sub-segments of 7 abnormal patients.One abnormal segment was observed in 1 patient,two abnormal segments were detected in 2 patients,and ≥3 abnormal segments were observed in 4 patients.Enlarged left ventricles were detected in 5 patients (5/8),and the LVEF of 3 patients among them decreased to (43.1 ±2.8)％.Conclusion 99Tcm-MIBI G-MPI can detect myocardial damage in sarcoglycanopathy as a direct and non-invasive method,and can be used in the early diagnosis and long-term follow-up in sarcoglycanopathy.