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J Appl Physiol (1985). 2014 May 1;116(9):1230-7. doi: 10.1152/japplphysiol.01063.2013. Epub 2014 Mar 20.

The "second wind" in McArdle's disease patients during a second bout of constant work rate submaximal exercise.

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Institute of Bioimaging and Molecular Physiology, National Research Council, Segrate, Italy;


Patients with McArdle's disease (McA) typically show the "second-wind" phenomenon, a sudden decrease in heart rate (HR) and an improved exercise tolerance occurring after a few minutes of exercise. In the present study, we investigated whether in McA a first bout of exercise determines a second wind during a second bout, separated by the first by a few minutes of recovery. Eight McA (44 ± 4 yr) and a control group of six mitochondrial myopathy patients (51 ± 6 yr) performed two repetitions (CWR1 and CWR2) of 6-min constant work rate exercise (∼50% of peak work rate) separated by 6-min (SHORT) or 18-min (LONG) recovery. Pulmonary O2 uptake (Vo2), HR, cardiac output, rates of perceived exertion, vastus lateralis oxygenation {changes in deoxygenated Hb and myoglobin Mb concentrations, Δ[deoxy(Hb+Mb)], by near-infrared spectroscopy} were determined. In McA, Vo2 (0.86 ± 0.2 vs. 0.95 ± 0.1 l/min), HR (113 ± 10 vs. 150 ± 13 beats/min), cardiac output (11.6 ± 0.6 vs. 15.0 ± 0.8 l/min), and rates of perceived exertion (11 ± 2 vs. 14 ± 3) were lower, whereas Δ[deoxy(Hb+Mb)] was higher (14.7 ± 2.3 vs. -0.1 ± 4.6%) in CWR2-SHORT vs. CWR1; the "overshoot" of Δ[deoxy(Hb+Mb)] and the "slow component" of Vo2 kinetics disappeared in CWR2-SHORT. No differences (vs. CWR1) were observed in McA during CWR2-LONG, or in mitochondrial myopathy patients during both CWR2-SHORT and -LONG. A second-wind phenomenon was observed in McA during the second of two consecutive 6-min constant-work rate submaximal exercises. The second wind was associated with changes of physiological variables, suggesting an enhanced skeletal muscle oxidative metabolism. The second wind was not described after a longer (18-min) recovery period.


Vo2 kinetics slow component; exercise tolerance; myophoshorylase deficiency; near-infrared spectroscopy

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