Pitkänen O P, Nuutila P, Raitakari O T, Porkka K, Iida H, Nuotio I, Rönnemaa T, Viikari J, Taskinen M R, Ehnholm C, Knuuti J
Departments of Medicine and Clinical Physiology, Turku PET Centre, Turku University, Turku, Finland.
Circulation. 1999 Apr 6;99(13):1678-84. doi: 10.1161/01.cir.99.13.1678.
Familial combined hyperlipidemia (FCHL) is a common hereditary disorder of lipoprotein metabolism estimated to cause 10% to 20% of premature coronary heart disease. We investigated whether functional abnormalities exist in coronary reactivity in asymptomatic patients with FCHL.
We studied 21 male FCHL patients (age, 34.8+/-5.4 years) and a matched group of 21 healthy control subjects. Myocardial blood flow (MBF) was measured at baseline and during dipyridamole-induced hyperemia with PET and 15O-labeled water. The baseline MBF was similar in patients and control subjects (0.79+/-0.19 versus 0.88+/-0.20 mL. g-1. min-1, P=NS). An increase in MBF was seen in both groups after dipyridamole infusion, but MBF at maximal vasodilation was lower in FCHL patients (3.54+/-1.59 versus 4.54+/-1.17 mL. g-1. min-1, P=0.025). The difference in coronary flow reserve (CFR) was not statistically significant (4.7+/-2.2 versus 5.3+/-1.6, P=NS, patients versus control subjects). Considerable variability in CFR values was detected within the FCHL group. Patients with phenotype IIB (n=8) had lower flow during hyperemia (2.5+/-1.2 versus 4.2+/-1.5 mL. g-1. min-1, P<0.05) and lower CFR (3.4+/-2.1 versus 5.4+/-2.0, P<0.05) compared with phenotype IIA (n=13).
Abnormalities in coronary flow regulation exist in young asymptomatic FCHL patients expressing phenotype IIB (characterized by abnormalities in both serum cholesterol and triglyceride concentrations). This is in line with previous observations suggesting that the metabolic abnormalities related to the pathophysiology of FCHL are associated with the phenotype IIB.
家族性混合型高脂血症(FCHL)是一种常见的脂蛋白代谢遗传性疾病,估计导致10%至20%的早发性冠心病。我们研究了无症状FCHL患者的冠状动脉反应性是否存在功能异常。
我们研究了21名男性FCHL患者(年龄34.8±5.4岁)和21名匹配的健康对照者。使用PET和15O标记水在基线和双嘧达莫诱导的充血期间测量心肌血流量(MBF)。患者和对照者的基线MBF相似(0.79±0.19对0.88±0.20 mL·g-1·min-1,P=无显著性差异)。双嘧达莫输注后两组的MBF均增加,但FCHL患者最大血管舒张时的MBF较低(3.54±1.59对4.54±1.17 mL·g-1·min-1,P=0.025)。冠状动脉血流储备(CFR)的差异无统计学意义(4.7±2.2对5.3±1.6,P=无显著性差异,患者对对照者)。在FCHL组中检测到CFR值有相当大的变异性。与IIA型(n=13)相比,IIB型(n=8)患者在充血期间的血流量较低(2.5±1.2对4.2±1.5 mL·g-1·min-1,P<0.05),CFR也较低(3.4±2.1对5.4±2.0,P<0.05)。
表达IIB型(以血清胆固醇和甘油三酯浓度异常为特征)的年轻无症状FCHL患者存在冠状动脉血流调节异常。这与先前的观察结果一致,表明与FCHL病理生理学相关的代谢异常与IIB型相关。