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Alternative Names Return to topTET; TOF
Definition Return to top
Tetralogy of Fallot refers to a group of four types of heart defects present at birth (congenital).
Causes Return to top
Tetralogy of Fallot is classified as a cyanotic heart defect because the condition causes low oxygen levels in the blood, which leads to cyanosis (a bluish-purple coloration to the skin).
The classic form of Tetralogy includes 4 defects within the heart structures:
At birth, infants may not show the signs of the cyanosis, but later may develop sudden frightening episodes (called "Tet spells") of bluish skin from crying or feeding.
Tetralogy of Fallot is considered rare. Patients with tetraology of Fallot have a higher incidence of major non-heart congenital defects.
The cause of most congenital heart defects is unknown. Multiple factors seem to be involved. Prenatal factors associated with higher than normal risk for this condition include maternal rubella or other viral illnesses during pregnancy, poor prenatal nutrition, maternal alcoholism, mother over 40 years old, and diabetes.
There is a high incidence of chromosomal disorders in children with tetralogy of Fallot, such as Down syndrome and Di George's syndrome (a partial gene deletion that results in heart defects, low calcium levels, and immune deficiency.)
Symptoms Return to top
Exams and Tests Return to top
A physical examination with a stethoscope almost always reveals a heart murmur.
Tests may include:
Treatment Return to top
Surgery to repair heart defects is always done when the infant is very young. Sometimes more than one surgery is needed. The first surgery may be done to help increase blood flow to the lungs. Surgery to correct the problem may be done at a later time. (Generally, a definitive corrective surgery is performed in the first few months of life.) Corrective surgery is done to widen part of the narrowed pulmonary tract and close the ventricular septal defect.
Tips for parents of children with tetralogy of Fallot:
Outlook (Prognosis) Return to top
Most cases can be corrected with surgery. Babies who have surgery usually do well. Ninety percent survive to adulthood and live active, healthy, and productive lives. Without surgery, death usually occurs by the time the person reaches age 20.
Patients who have continued, severe leakiness of the pulmonary valve may need the valve replaced.
Regular follow up with a cardiologist to monitor for life-threatening arrhythmias (irregular heart rhythms) is recommended.
Possible Complications Return to top
When to Contact a Medical Professional Return to top
Call your health care provider if new unexplained symptoms develop or if the patient is having an episode of cyanosis (blue skin).
Prevention Return to top
There is no known prevention.
References Return to top
Zipes DP, Libby P, Bonow RO, Braunwald E, eds. Braunwald's Heart Disease: A Textbook of Cardiovascular Medicine, 8th ed. St. Louis, Mo; WB Saunders; 2007.Update Date: 12/10/2007 Updated by: Mark A Fogel, MD, FACC, FAAP, Associate Professor of Pediatrics and Radiology, Director of Cardiac MR, The Children's Hospital of Philadelphia. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.