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紫绀型先天性心脏病患者的心肌功能障碍和脂肪酸代谢降低

Myocardial dysfunction and depressed fatty acid metabolism in patients with cyanotic congenital heart disease.

作者信息

Kondo C, Nakazawa M, Kusakabe K, Momma K

机构信息

Department of Pediatric Cardiology, Tokyo Women's Medical College, Japan.

出版信息

J Nucl Cardiol. 1996 Jan-Feb;3(1):30-6. doi: 10.1016/s1071-3581(96)90021-5.

DOI:10.1016/s1071-3581(96)90021-5
PMID:8799225
Abstract

BACKGROUND

Myocardial contractile dysfunction has been frequently observed in adolescents or adults with cyanotic congenital heart disease. Impaired energy metabolism may be present in such dysfunctional myocardium.

METHODS AND RESULTS

To evaluate the findings of myocardial free fatty acid metabolism, and its relations to ventricular wall motion and myocardial perfusion in cyanotic congenital heart disease, we performed a combined study of iodine 123-labeled 15-(p-iodophenyl)-3-(R,S)-methylpentadecanoic acid (BMIPP) scintigraphy, thallium scintigraphy, and contrast cine-angiography in seven patients with single right or left ventricle. The results showed that wall motion was reduced in 17 of 35 ventricular segments (49%), which were mostly identical in location to decreased BMIPP uptake. The severity of BMIPP uptake deficit correlated positively with the degree of impairment of wall motion. On the other hand, thallium uptake was abnormal only in 5 of 35 segments (14%), and the severity of the perfusion defect did not correlate with the degree of wall motion abnormality.

CONCLUSIONS

Contractile dysfunction in cyanotic heart disease was primarily linked to impaired free fatty acid metabolism rather than to myocardial scar as represented by perfusion defect on thallium imaging.

摘要

背景

在患有紫绀型先天性心脏病的青少年或成人中,经常观察到心肌收缩功能障碍。这种功能失调的心肌中可能存在能量代谢受损。

方法与结果

为了评估紫绀型先天性心脏病中心肌游离脂肪酸代谢的结果及其与心室壁运动和心肌灌注的关系,我们对7名单一右心室或左心室患者进行了碘123标记的15 - (对碘苯基)-3 - (R,S)-甲基十五烷酸(BMIPP)闪烁扫描、铊闪烁扫描和对比电影血管造影的联合研究。结果显示,35个心室节段中有17个(49%)壁运动减弱,其位置大多与BMIPP摄取减少的位置相同。BMIPP摄取缺陷的严重程度与壁运动受损程度呈正相关。另一方面,35个节段中只有5个(14%)铊摄取异常,灌注缺损的严重程度与壁运动异常程度无关。

结论

紫绀型心脏病中的收缩功能障碍主要与游离脂肪酸代谢受损有关,而非与铊显像上灌注缺损所代表的心肌瘢痕有关。

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本文引用的文献

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Myocardial accumulation of iodinated beta-methyl-branched fatty acid analog, [125I](p-iodophenyl)-3-(R,S)-methylpentadecanoic acid (BMIPP), and correlation to ATP concentration--II. Studies in salt-induced hypertensive rats.碘化β-甲基支链脂肪酸类似物[125I](对碘苯基)-3-(R,S)-甲基十五烷酸(BMIPP)在心肌中的蓄积及其与三磷酸腺苷(ATP)浓度的相关性——II. 盐诱导高血压大鼠的研究
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I-123脂肪酸与铊-201联合研究评估缺血心肌:与铊再分布及葡萄糖代谢的比较
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Relationship between blood flow and fatty acid metabolism in subacute myocardial infarction: a study by means of 99mTc-Sestamibi and 123I-beta-methyl-iodo-phenyl pentadecanoic acid.
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