Exercise Physiology Comprehensive Use of Cardiopulmonary Exercise Testing Identifies Adults With Congenital Heart Disease at Increased Mortality Risk in the Medium Term

Background—Parameters of cardiopulmonary exercise testing were recently identified as strong predictors of mortality in adults with congenital heart disease. We hypothesized that combinations of cardiopulmonary exercise testing parameters may provide optimal prognostic information on midterm survival in this population. Methods and Results—A total of 1375 consecutive adult patients with congenital heart disease (age, 33 13 years) underwent cardiopulmonary exercise testing at a single center over a period of 10 years. Peak oxygen consumption (peak V̇O2), ventilation per unit of carbon dioxide production (V̇E/V̇CO2 slope), and heart rate reserve were measured. During a median follow-up of 5.8 years, 117 patients died. Peak V̇O2, heart rate reserve, and V̇E/V̇CO2 slope were related to midterm survival in adult patients with congenital heart disease. Risk of death increased with lower peak V̇O2 and heart rate reserve. A higher V̇E/V̇CO2 slope was also related to increased risk of death in noncyanotic patients, whereas the V̇E/V̇CO2 slope was not predictive of mortality in cyanotic patients. The combination of peak V̇O2 and heart rate reserve provided the greatest predictive information after adjustment for clinical parameters such as negative chronotropic agents, age, and presence of cyanosis. However, the incremental value of these exercise parameters was reduced in patients with peak respiratory exchange ratio 1.0. Conclusions—Cardiopulmonary exercise testing provides strong prognostic information in adult patients with congenital heart disease. Prognostication should be approached differently, depending on the presence of cyanosis, use of rate-lowering medications, and achieved level of exercise. We provide 5-year survival prospects based on cardiopulmonary exercise testing parameters in this growing population. (Circulation. 2012;125:250-259.)