Serial Cardiopulmonary Exercise Testing in Patients after Extracardiac Conduit Total Cavopulmonary Connection for Single-Ventricle Hearts: An Observational Study.

OBJECTIVE: To analyze the aerobic fitness and evolution of exercise tolerance in patients with single-ventricle physiology after total cavopulmonary connection (TCPC) with an extracardiac conduit (ECC). STUDY DESIGN: This retrospective cohort study included patients with previous ECC-TCPC who underwent cardiopulmonary exercise testing (CPET) between September 2010 and September 2019. Patients who completed at least 2 tests (≥6 months apart) with adequate levels of effort were recruited for the serial CPET evaluation. RESULTS: We identified 70 patients (50% male) with a mean age of 6.45 ± 5.14 years at ECC-TCPC and 15.67 ± 5.03 years at the initial CPET. The peak oxygen consumption (peak VO2) to predicted value (peak PD) was 55.90 ± 16.81%. Twenty of the 70 identified patients (50% male) were recruited for serial analysis. The average number of CPETs was 2.6 per patient. The average duration from the first CPET to the last CPET was 3.64 years. The peak VO2 and PD increased slowly, with mean rates of 38.77 ± 129.01 mL/min and 1.66 ± 6.40%, respectively, during the study period. CONCLUSIONS: Although the patients had lower exercise tolerance after ECC-TCPC compared with their normal peers, exercise tolerance appears to have been preserved over the adolescent period in those who underwent serial testing after ECC-TCPC.

Analysis of Exercise Capacity of Children with Kawasaki Disease by a Coronary Artery z Score Model (ZSP Version 4) Derived by the Lambda-Mu-Sigma Method.

OBJECTIVE: To compare exercise capacity measured by direct cardiopulmonary exercise testing (CPET) of children with Kawasaki disease with different coronary artery diameter z scores (CA z score). STUDY DESIGN: This was a retrospective study that recruited children with Kawasaki disease after the acute stage receiving CPETs determined by CPET with treadmill. CA z score was based on a model using the Lambda-Mu-Sigma method. Max-Z was defined as the maximum z score of the proximal left anterior descending CA (LCA) or right CA (RCA). Children with Kawasaki disease with a Max z <2.0 and ≥2.0 were defined as Kawasaki disease group 1 and Kawasaki disease group 2, respectively. RESULTS: We recruited 32 boys and 17 girls with a mean age of 12.39 ± 3.61 years. Kawasaki disease group 1 (n = 36) had significantly higher peak metabolic equivalent (peak-MET) and peak rate pressure product (PRPP) than Kawasaki disease group 2 (n-13) (P = .046, P < .001). Max-Z correlated with peak-MET moderately and negatively (P < .001, Spearman rho= - .506). Max-Z correlated with PRPP modestly and negatively (P = .011, Spearman rho= - .360). CONCLUSIONS: Children after Kawasaki disease with a coronary artery Max-Z ≥ 2.0 had significantly lower peak exercise capacity than those with a Max-Z < 2.0. Max-Z might be used as an indicator of CA reserve and exercise capacity during peak exercise after the acute stage of Kawasaki disease.