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Tetralogy of Fallot (TOF) is one of the most common congenital heart disorders (CHDs). This condition is classified as a cyanotic heart disorder, because tetralogy of Fallot results in an inadequate flow of blood to the lungs for oxygenation (right-to-left shunt) (see the following image). Patients with tetralogy of Fallot initially present with cyanosis shortly after birth, thereby attracting early medical attention.

Anatomic findings in tetralogy of Fallot.

Typical features
The 4 features typical of tetralogy of Fallot include right ventricular (RV) outflow tract obstruction (RVOTO) (infundibular stenosis), ventricular septal defect (VSD), aorta dextroposition, and right ventricular hypertrophy. Occasionally, a few children also have an atrial septal defect (ASD), which makes up the pentad of Fallot. The basic pathology of tetralogy is due to the underdevelopment of the right ventricular infundibulum, which results in an anterior-leftward malalignment of the infundibular septum. This malalignment determines the degree of RVOTO. The clinical features of tetralogy of Fallot are generally typical, and a preliminary clinical diagnosis can almost always be made. Because most infants with this disorder require surgery, it is fortunate that the availability of cardiopulmonary bypass (CPB), cardioplegia, and surgical techniques is now well established. Most surgical series report excellent clinical results with low morbidity and mortality rates. See also Tetralogy of Fallot With Pulmonary Stenosis, Tetralogy of Fallot With Pulmonary Atresia, and Tetralogy of Fallot With Absent Pulmonary Valve.

Historical information
Louis Arthur Fallot, after whom the name tetralogy of Fallot is derived, was not the first person to recognize the condition. Stensen first described it in 1672; however, it was Fallot who first accurately described the clinical and complete pathologic features of the defects. Although the disorder was clinically diagnosed much earlier, no treatment was available until the 1940s. Cardiologist Helen Taussig recognized that cyanosis progressed and inevitably led to death in infants with tetralogy of Fallot. She postulated that the cyanosis was due to inadequate pulmonary blood flow. Her collaboration with Alfred Blalock led to the first type

Lillehei performed the first successful open repair for tetralogy of Fallot using controlled crosscirculation. This image shows completed blocking with a Taussig shunt The pioneering Blalock-Taussig shunt surgical technique opened a new era in neonatal cardiac surgery. .of palliation for these infants. and the Waterston shunt (from the ascending aorta to the right pulmonary artery) followed. Less than half a year later. Development of the Potts shunt (from the descending aorta to the left pulmonary artery). with the advent of cardiopulmonary bypass by Gibbons. with another patient serving as oxygenator and blood reservoir. the Glenn shunt (from the superior vena cava to the right pulmonary artery). In 1944. another historic era of cardiac surgery was established. The following year. numerous advances in surgical technique and myocardial preservation have evolved in the treatment of tetralogy of Fallot. Scott performed the first open correction in 1954. Blalock operated on an infant with tetralogy of Fallot and created the first Blalock-Taussig shunt between the subclavian artery and the pulmonary artery (see the image below). Since then.