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Purpose  Cystic fibrosis (CF) is commonly recognized as a pulmonary disease associated with reduced airway function. Another primary symptom of CF is low exercise capacity where ventilation and gas-exchange are exacerbated. However, an independent link between pathophysiology of the pulmonary system and abnormal ventilatory and gas-exchange responses during cardiopulmonary exercise testing (CPET) has not been established in CF. Complicating this understanding, accumulating evidence suggests CF demonstrate abnormal peripheral vascular function; although, the clinical implications are unclear. We hypothesized that compared to controls, relative to total work performed (Work TOT ), CF would demonstrate increased ventilation accompanied by augmented systolic blood pressure (SBP) during CPET.    Methods  16 CF and 23 controls (age: 23±4 vs. 27±4 years,  P  = 0.11; FEV 1 %predicted: 73±14 vs. 96±5,  P <0.01) participated in CPET. Breath-by-breath oxygen uptake (V  ˙    O  2), ventilation (V  ˙   E), and carbon dioxide output (V  ˙   C   O  2) were measured continuously during incremental 3-min stage step-wise cycle ergometry CPET. SBP was measured via manual sphygmomanometry. Linear regression was used to calculateV  ˙   E   /   V  ˙   C   O  2slope from rest to peak-exercise.    Results  Compared to controls, CF performed less Work TOT  during CPET (90±19 vs. 43±14 kJ, respectively,  P< 0.01). With Work TOT  as a covariate, peakV  ˙   E(62±8 vs. 90±4 L/min,  P  = 0.76),V  ˙   C   O  2(1.8±0.3 vs. 2.7±0.1 L/min,  P  = 0.40), and SBP (144±13 vs. 152±6 mmHg,  P  = 0.88) were similar between CF and controls, respectively; whereas CF demonstrated increasedV  ˙   E   /   V  ˙   C   O  2slope (38±4 vs. 28±2,  P  = 0.02) but lower peakV  ˙    O  2versus controls (22±5 vs. 33±4 mL/kg/min,  P< 0.01). There were modest-to-moderate correlations between peak SBP withV  ˙    O  2( r  = 0.30),V  ˙   E( r  = 0.70), andV  ˙   C   O  2( r  = 0.62) in CF.    Conclusions  These data suggest that relative to Work TOT , young adults with mild-to-moderate severity CF demonstrate augmentedV  ˙   E   /   V  ˙   C   O  2slope accompanied by increased SBP during CPET. Although the underlying mechanisms remain unclear, the coupling of ventilatory inefficiency with increased blood pressure suggest important contributions from peripheral pathophysiology to low exercise capacity in CF.

The Coupling of Peripheral Blood Pressure and Ventilatory Responses during Exercise in Young Adults with Cystic Fibrosis