Metabolic control of cardiac output response to exercise in McArdle's disease.

S F Lewis, R G Haller, J D Cook, C G Blomqvist
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引用次数: 40

Abstract

During dynamic exercise cardiac output (Q) normally increases approximately 5 liters per liter of increase in O2 uptake (Vo2) (i.e., delta Q/delta Vo2 approximately equal to 5), indicative of a tight coupling between systemic O2 transport and utilization. We studied four patients with muscle phosphorylase deficiency (McArdle's disease) in whom Q was normal at rest, but delta Q/delta Vo2 was 14.1 +/- 1.3 during bicycle exercise. Procedures designed to alter the availability of substrates were employed to test the hypothesis that the increased delta Q/delta Vo2 is linked to the abnormal metabolic state of skeletal muscle. Fasting plus prolonged moderate exercise was used to increase the availability of plasma free fatty acid (FFA) and resulted in a normalization of delta Q/delta Vo2 (5.3 +/- 0.4). Hyperglycemia (70% above control levels) partially normalized delta Q/delta Vo2. Nicotinic acid lowered plasma FFA concentration and dramatically increased delta Q/delta Vo2 (4.6 to 13.7) when administered after fasting plus prolonged exercise in one patient. Glucose infusion after nicotinic acid administration markedly lowered delta Q/delta Vo2. The results support the hypothesis and suggest that the metabolic state of skeletal muscle, possibly via activation of muscle afferents, participates in the regulation of systemic O2 transport.

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McArdle病运动后心输出量的代谢控制
在动态运动中,心输出量(Q)通常每增加1升氧摄取(Vo2)增加约5升(即Q/ Vo2约等于5),表明全身氧运输和利用之间存在紧密耦合。我们研究了4例肌肉磷酸化酶缺乏症(McArdle's disease)患者,他们在休息时Q正常,但在自行车运动时Q/ Vo2为14.1 +/- 1.3。设计程序来改变底物的可用性,以验证增加的δ Q/ δ Vo2与骨骼肌异常代谢状态有关的假设。禁食加长时间的适度运动可以增加血浆游离脂肪酸(FFA)的可用性,并导致δ Q/ δ Vo2正常化(5.3 +/- 0.4)。高血糖(比对照水平高70%)Q/ Vo2部分正常化。一名患者在禁食加长时间运动后服用烟酸可降低血浆FFA浓度,并显著增加δ Q/ δ Vo2(4.6至13.7)。烟酸给药后葡萄糖输注显著降低Q/ Vo2。结果支持了这一假设,表明骨骼肌的代谢状态可能通过激活肌肉传入神经参与了全身氧运输的调节。
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