Critical Care Management of the Adult with Tetralogy of Fallot

Tetralogy of Fallot (TOF) is the most common form of cyanotic congenital heart disease and usually is corrected in infancy with low mortality rate; after repair, pulmonary regurgitation is common, but in general surgery is not required until adulthood. Pulmonary valve replacement (PVR) is the most frequent operation in adult TOF patients, while complete correction in natural history is less frequent in developed countries but can take place in those patients coming from developing countries where a cardiac surgery program is not available. Operated TOF patients can suffer also from residual defects which derive from previous surgeries. Morbidity and mortality rates after reoperation are usually low, but different conditions can increase the postoperative risk. Adult TOF patients requiring any surgery are always a challenge for anesthesiologists and intensivists, and a very good knowledge of the pathophysiology and associated treatments is important to reduce the incidence of adverse events correlated mainly to right ventricle (RV) failure.