Abstract
This review summarises various applications of how ventilatory equivalent (ventilatory efficiency or better still ventilatory inefficiency) and the minute ventilation (V'(E))/carbon dioxide production (V'(CO(2)) ) slope obtained from cardiopulmonary exercise testing (CPET) can be used in the diagnostic or prognostic workup of patients with congenital heart disease.The field of congenital heart disease comprises not only a very heterogeneous patient group with various heart diseases, but also various conditions in different stages of repair, as well as the different residuals seen in long-term follow-up. As such, various physiologic disarrangements must be considered in the analysis of increased V'(E)/V'(CO(2)) slope from CPET in patients with congenital heart disease. In addition to congestive heart failure (CHF), cyanosis, unilateral pulmonary stenosis and pulmonary hypertension (PH) provide the background for this finding. The predictive value of increased V'(E)/V'(CO(2)) slope on prognosis seems to be more important in conditions where circulatory failure is associated with failure of the systemic ventricle. In cyanotic patients, those with Fontan circulation, or those with substantial mortality from arrhythmia, the impact of V'(E)/V'(CO(2)) on prognosis is not that important.