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Table 1 Description of parameters in lung function analysis and exercise testing

From: Primary mitochondrial disease as a rare cause of unclear breathlessness and distinctive performance degradation – a case report

parameter

time point

       

Aug 2017

 

Sep 2018

 

Aug 2020

 

Aug 2022

 

pulmonary function (unit)

value

%predicted

value

%predicted

value

%predicted

value

%predicted

vital capacity VC (l)

3.9

97.4

4.1

103.2

4.1

104.8

4.0

94.0

Forced vital capacity FVC (l)

3.9

99.3

4.0

101.2

4.2

108.2

4.0

94.0

FEV1 (l)

3.4

98.4

3.5

103.0

3.5

104.0

3.5

98.0

FEV1 / FVC (%)

86.4

 

88.7

 

83.8

 

87.2

 

total lung capacity TLC (l)

5.7

102.8

6.0

109.2

6.0

108.4

5.8

106.0

inspiratory capacity (l)

2.6

95.1

2.9

108.3

2.8

103.4

2.4

90.0

residual volume (l)

1.7

111.7

1.9

120.9

1.8

110.6

1.9

115.0

RV / TLC (%)

30.8

106.9

31.4

108.9

29.6

100.2

32.0

106.0

DLCOc (mmol/min/kPa)

8.2

83.9

8.4

85.9

8.2

84.7

7.2

74.0

DLCOc / VA (mmol/min/kPa/l)

1.6

91.2

1.7

92.8

1.6

88.2

1.4

77.0

cardiopulmonary exercise testing

        

body weight (Kg)

65

 

66

 

66

 

56

 

maximum power (Watt)

84

60

68

49

84

62

84

65

VO2@VT1 (ml/min)

507

 

443

 

312

 

397

 

VO2@VT1 / VO2 peak pred. (%)

25.6

 

22.8

 

16.2

 

22

 

peakVO2 (ml/min)

655

29

537

26

736

38

560

31

peakVO2 / weight (ml/min/kg)

10.1

29

8.5

30.8

11.1

29.2

10

31

peakVO2 / HR (ml)

3.6

29

4.4

28

4.1

40

4.3

27

maximum ventilation (l/min)

76

 

68

 

73

 

57

 

maximum Vtex (l)

1.73

 

1.51

 

1.59

 

1.46

 

breathing reserve (%)

45

 

53

 

49

 

60

 

VE/VO2@rest

30.9

 

29.3

 

36.1

 

24.5

 

VE/VO2@VT1

30.8

 

33.7

 

31

 

37.8

 

VE/VO2@max

95

 

75

 

90

 

n.a.

 

petO2 @rest (mmHg)

115.26

 

122.67

 

116.56

 

111.13

 

petO2 @VT1 (mmHg)

116.24

 

120.87

 

113.33

 

121.86

 

petO2 @max (mmHg)

140.29

 

140.55

 

137.26

 

138.1

 

VE/VCO2@rest

35.5

 

39.7

 

41.3

 

34.3

 

VE/VCO2@VT1

35.7

 

40.5

 

38.2

 

33.9

 

VE/VCO2@max

65.8

 

73.2

 

60.8

 

59

 

petCO2 @rest (mmHg)

30.28

 

21.74

 

30

 

30.77

 

petCO2 @VT1 (mmHg)

29.65

 

25.47

 

30.69

 

30.73

 

petCO2 @max (mmHg)

14.9

 

22.68

 

n.a.

 

19.01

 

maximum RER

1.96

 

1.86

 

1.59

 

1.93

 

blood gas analysis (at rest)

        

AaDO2 (mmHg)

36.14

 

11.47

 

27.1

 

29.32

 

pa−etCO2 (mmHg)

0.66

 

4.34

 

-1.32

 

-1.12

 

paO2 (mmHg)

77.1

 

99.6

 

85

 

82

 

paCO2 (mmHg)

31.3

 

28.8

 

29

 

31.6

 

SaO2 (%)

96.05

 

97.88

 

96.96

 

95.9

 

lactate (mmol/l)

2.4

 

1.9

 

1

 

1.6

 

HCO3 (mmol/l)

22.3

 

21.8

 

21.9

 

23

 

base excess (mmol/l)

-6.3

 

-4.6

 

-4.6

 

-4

 

VO2 (ml/min)

377

 

329

 

215

 

216

 

blood gas analysis (at 46 W)

        

AaDO2 (mmHg)

15.66

 

15.46

 

18.01

 

10.56

 

pa−etCO2 (mmHg)

3.29

 

3.61

 

4.31

 

-1.12

 

paO2 (mmHg)

113

 

116

 

109

 

119

 

paCO2 (mmHg)

30.4

 

25.9

 

28.1

 

29.3

 

SaO2 (%)

98.9

 

98.5

 

98.5

 

99.1

 

lactate (mmol/l)

6.2

 

4.1

 

2.3

 

5.4

 

HCO3 (mmol/l)

19.4

 

20.7

 

20.7

 

19.2

 

base excess (mmol/l)

-6.3

 

-4.6

 

-4.6

 

-4

 

VO2 (ml/min)

519

 

433

 

544

 

425

 

blood gs analysis (at 68 W)

        

AaDO2 (mmHg)

15.98

 

17.72

 

17.46

 

n.a.

 

pa−etCO2 (mmHg)

3.82

 

6.71

 

4.66

 

5.13

 

paO2 (mmHg)

120

 

117

 

112

 

135

 

paCO2 (mmHg)

21.6

 

22.6

 

24.8

 

25.7

 

SaO2 (%)

98.9

 

98.5

 

98.3

 

99.1

 

lactate (mmol/l)

14.4

 

7.5

 

6.3

 

10.8

 

HCO3 (mmol/l)

14.8

 

18.7

 

18.1

 

14.7

 

base excess (mmol/l)

-13

 

-7.2

 

-8

 

-8.7

 

VO2 (ml/min)

570

 

478

 

640

 

448

 
  1. Abbreviations: AaDO2 arterial-alveolar difference for oxygen, VT1 ventilatory threshold, DLCOc diffusion capacity of carbon monoxide corrected by haemoglobin, DLCOc / VA Krogh factor, FEV1 forced expiratory volume in one second, FVC forced vital capacity, HCO3 bicarbonate ion, HR heart rate, peakVO2 peak oxygen uptake, paCO2 arterial partial pressure of carbon dioxide, paO2 arterial partial pressure of oxygen, pa−etCO2 arterial - end-tidal difference for carbon dioxide, petCO2 end- tidal pressure of carbon dioxide, petCO2@VT1 end tidal pressure of carbon dioxide at ventilatory threshold, RER respiratory ratio (quotient of carbon dioxide output and oxygen uptake), SaO2 arterial oxygen saturation, TLC total lung capacity, VE/VCO2@VT1 ratio ventilation to carbon dioxide output at ventilatory threshold, VE/VCO2@max ratio ventilation to carbon dioxide output at maximum exercise, VE/VCO2@rest ratio ventilation to carbon dioxide output at rest, VE/VO2@VT1 ratio ventilation to oxygen uptake at ventilatory threshold, VE/VO2@max ratio ventilation to oxygen uptake at maximum exercise, VE/VO2@rest ratio ventilation to oxygen uptake at rest, VO2 oxygen uptake per minute, VO2@VT1 oxygen uptake at ventilatory threshold, VO2/HR oxygen uptake per heart rate, Vtex expiratory tidal volume