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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 V̇ o 2 ), ventilation per unit of carbon dioxide production (V̇ e /V̇ co 2 slope), and heart rate reserve were measured. During a median follow-up of 5.8 years, 117 patients died. Peak V̇ o 2 , heart rate reserve, and V̇ e /V̇ co 2 slope were related to midterm survival in adult patients with congenital heart disease. Risk of death increased with lower peak V̇ o 2 and heart rate reserve. A higher V̇ e /V̇ co 2 slope was also related to increased risk of death in noncyanotic patients, whereas the V̇ e /V̇ co 2 slope was not predictive of mortality in cyanotic patients. The combination of peak V̇ o 2 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 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.

We thank Guazzi et al for their thoughtful comments regarding our article.1 Exertional oscillatory breathing (EOB) is recognized as an abnormal cardiopulmonary response in patients with heart failure. Although the precise pathophysiologic mechanism of EOB remains somewhat unclear, it is considered to reflect the deregulation of mechanical and neural feedback control mechanisms of the cardiopulmonary system. Several studies have demonstrated that EOB is a strong, effort-independent …