1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20 21 22 23 24 25 26 27 28 29 30 31 32 33 34 35 36 37 38 39 40 41 42 43 44 45 46 47 48 49 50 51 52 53 54 55 56 57 58 59 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20 21 22 23 24 25 26 27 28 29 30 31 32 33 34 35 36 37 38 39 40 41 42 43 44 45 46 47 48 49 50 51 52 53 54 55 56 57 58 59 (RPE 11, 13, 15, 17 and 20), following an incremental format. Changes in PO 115were facilitated by the participants manually adjusting the cycle ergometer air brake and 116 cadence at their own free will in order to produce a level of resistance that allowed them to 117 match the target RPE for each stage of the SPV. 119The sCPET from study 2 was completed on an electro magnetically braked cycle ergometer 120 (Lode Corival), so that PO for each stage could be fixed according to the test requirements. 121The test followed a standard incremental ramp design. for three of the patients in study 2, and so data from only SPV1 has been used in these cases. 158The reasons for these three missing tests were; one patient had an unrelated illness and was

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