Tetralogy of Fallot is a specific pattern of four heart malformations: a ventricular septal defect (VSD, or a “hole in the heart”), aorta misplaced over the VSD, narrowing of the pulmonary artery, and hypertrophy (thickening) of the right ventricle.
Tetralogy of Fallot occurs in 2 to 3 per 10,000 live births.
When tetralogy of Fallot is suspected, further evaluation includes a specialized evaluation of the fetal heart (“fetal echocardiogram”). As tetralogy of Fallot is frequently associated with other physical abnormalities and genetic syndromes, genetic counseling and testing are recommended. Prenatal consultations with pediatric cardiology, pediatric cardiothoracic surgery, and neonatology specialists are recommended to discuss the prognosis and management after birth. Prenatal care will be managed by a Maternal-Fetal Medicine specialist, an obstetrician with special training and expertise in high-risk pregnancies.
Newborns will be evaluated and cared for by a team involving pediatric cardiology and neonatology specialists. Outcomes vary widely based upon the underlying arrhythmia and are influenced by the presence of other abnormalities or genetic syndromes. In stable newborns, surgical repair may not be performed until several months of life.