Does Restrictive Lung Function Affect the Exercise Capacity in Patients with Repaired Tetralogy of Fallot?

Patients with repaired Tetralogy of Fallot (rTOF) have decreased exercise capacity (XC) and restrictive lung function (RLF). Our objective was to determine the association between RLF and impaired XC in patients with rTOF. This was a single center retrospective review of patients with rTOF who underwent a cardiopulmonary treadmill exercise testing and spirometry from 2005 to 2015. Patients with a respiratory exchange ratio >= 1.05 and peak heart rate > 90% of predicted value were included. Forced vital capacity (FVC) and Forced expiratory volume in 1st second of forceful expiration (FEV1) were used to classify the lung function. Exercise parameters such as peak oxygen uptake (VO2), % of predicted VO2 (%VO2), Metabolic equivalents (METS), and exercise time (ET) were compared between the two groups (i) compared patients with normal lung function (normal FEV1, FVC, and FEV1/FVC > 80%) (ii) RLF (FVC < 80%, normal or increased FEV1/FVC > 80%). In our cohort (n = 151, 52% male, mean age +/- SD of 22.3 +/- 9.1 years), patients with RLF (n = 73) compared to those with normal lung function (n = 86) had a lower peak VO2 (30.8 +/- 8.6 vs. 36.6 +/- 9.8 mL/kg/min; p < 0.001) and shorter exercise time (9:23 +/- 1:78 vs. 10:23 +/- 1:62 min, p < 0.001). On multivariate regression analysis, RLF was independently associated with reduced XC (VO2%) (beta-coefficient - 0.182, p < 0.02) after controlling for age and gender. RLF is common in patients with rTOF and is associated with decreased XC. The contribution of RLF to reduced XC in this population should be considered prior to therapeutic decisions.