Thanks to advances in both medical care and surgical techniques, over recent years, survival for patients with congenital heart disease (CHD) has increased significantly, with about 90% of patients now reaching adulthood. However, as CHD patients grow older, their relative risk of developing coronary artery disease (CAD) increases. Moreover, it has been demonstrated that the majority of adult congenital heart disease (ACHD) patients has at least one cardiovascular risk factor. On the other hand, common complications, such as atrial fibrillation (AF) may evolve into a major clinical concern and can be difficult to manage medically.This review aimed at examining the current pharmacological treatment strategies for primary and secondary prevention of CAD, medical and interventional treatment for supraventricular arrhythmias, as well as optimal medical strategies for ACHD patients with CAD and AF.