Owing to advances in cardiac surgery, intensive care, and diagnostic capabilities, 88% of children born with a congenital heart disease (CHD) survive into adulthood. 1-3 Besides arrhythmias, (end-stage) heart failure is the primary source of morbidity and mortality. In accordance with the increasing number of adult patients with CHD (ACHD) a , the need for noncardiac surgical and interventional procedures is increasing, both in the elective and acute settings. Therefore, there is a practical need for anesthesiologists to be familiar with the pathophysiologic considerations. In this review, Disclosure Statement: None.