[Cyanotic heart disease. Part 2]

Tetralogy of Fallot is the most common cyanotic heart disease. Its operative mortality and long-term result are quite good in these days. At the late phase after the correction, pulmonary valve regurgitation associated with right side heart failure, aortic valve regurgitation, arrhythmia and sudden death become major adverse outcomes. Double-outlet right ventricle is a cyanotic heart disease with a wide spectrum of morphology and is divided according to the site of ventricular septal defect: subaortic, subpulmonary, doubly committed and remote type. Its operative methods are completely dependent on its morphology, and vary such as intracardiac tunnel repair, Rastelli type repair, arterial switch procedure and Fontan type repair. Left ventricular outflow tract obstruction is one of the most important problems after the correction. Recent operative strategies for the treatment of tricuspid atresia and single ventricle are quite similar and its final goal is the completion of right heart bypass operation using total cavo-pulmonary connection with staging strategy. Pleural effusion, ascites, protein loosing enteropathy and supraventricular arrhythmia are major adverse outcomes after Fontan type repair, while extracardiac total cavopulmonary connection is expected to reduce the incidence of supraventricular arrhythmia.