Tetralogy of Fallot (TOF) Tetralogy of Fallot is the most common congenital heart defect causing cyanosis (bluish discoloration

of skin caused by oxygen-poor blood reaching the general circulation). An embryologic failure of the right ventricular outflow tract to form properly results in the 4 cardiac abnormalities characteristic of TOF: - ventricular septal defect VSD (an abnormal opening between the two lower chambers of the heart) - pulmonic stenosis PS (a narrowed area within the main pulmonary artery, at, above or below the pulmonary valve) - a malpositioned aorta in which the entrance to the aorta over-rides the VSD - an overly muscular right ventricle (ventricular hypertrophy) Signs and Symptoms: If the PS is minimal, blood will shunt from left-to-right across the VSD, and the patient may demonstrate signs and symptoms of too much blood flow to the lungs: -shortness of breath, heart enlargement, right heart failure. If the PS is moderate, the patient may have a balanced circulation in which right heart pressures are elevated just enough to match the left heart, and relatively little shunting will occur across the VSD. These patients may demonstrate very mild or no symptoms. If the PS is significant, blood will shunt from right to left across the VSD, causing the patient to develop cyanosis, a symptom of having a lower than normal oxygen content in their arterial blood. Many of these patients will also have Tet spells sudden increases in the right-to-left shunting that result in severe cyanosis. Diagnosing Children Severe heart disease generally becomes evident during the first few months after birth. Some babies are blue or have very low blood pressure shortly after birth. Other defects cause breathing difficulties, feeding problems, or poor weight gain. Minor defects are most often diagnosed on a routine medical check up. Minor defects rarely cause symptoms. While most heart murmurs in children are normal, some may be due to defects.

Surgical management: Blalock-Taussig Shunt Placement Placement of a Blalock-Taussig shunt (BT shunt) is a closed-heart procedure performed as a first stage to correct Tetralogy of Fallot. Placement of a shunt is usually a temporary measure designed to alleviate symptoms until such time that the patient is in better condition to undergo complete repair. The surgical approach to the repair of TOF is individualized to each patient based on age, anatomy, and symptoms. Based on this assessment, surgery can be undertaken in 1 or 2 stages: 1st stage: Blalock-Taussig shunt (artificial shunt created between aorta and pulmonary artery to increase blood flow to lungs) 1st or 2nd stage: Complete TOF repair (complete repair VSD patched to prevent right-to-left shunt, RV outflow tract widened to increase blood flow to lungs)

Before the Surgery 1. If at all possible, it is important that the patient be free of infection prior to going to surgery. This includes dental cavities, so a dental check-up and any dental work required should be completed within 6 weeks prior to surgery. A letter is required from the dentist clearing the patient for surgery. In addition, if the patient develops other signs and symptoms of an infection, such as a fever, runny nose, diarrhea, or vomiting, contact the surgeons office immediately. 2. If the patient is taking aspirin, contact the cardiologist to ask when to discontinue taking the aspirin. Aspirin is generally discontinued 14 days prior to surgery to minimize the risk of excessive bleeding. However, there may be a medical reason to continue the aspirin, so it is important to check with the cardiologist. 3. Patients undergoing cardiac surgery frequently need blood products. Patients and families interested in donating blood should call the Congenital Heart Surgery Clinic and ask to speak to a clinic nurse

Designated (or Directed) Donation: Blood donated will be used for the patient designated. For congenital heart surgery patients, blood donors must be the same blood type as the patient. For Designated Donation, it is very important before you donate that you call the surgeons office to let us help you coordinate the donation. The pertinent patient information we need when you call is: the patients weight blood type surgery date type of surgery The best time to donate blood is one week prior to surgery and no later than three days prior to surgery. There is a cost associated with directed donor blood that may not be covered by your insurance. It will be the familys responsibility to cover the cost of directed donor blood that is not covered by insurance. 4. If the patient is taking blood thinners such as Coumadin or Lovenox, please contact the Congenital Heart Surgery Clinic and ask to speak to the clinic nurse. These patients may need to be hospitalized a few days prior to surgery to discontinue these medications and convert to Heparin prior to surgery. This decision will be coordinated between the patient, surgeon and cardiologist. 5. A cardiologist performs a physical exam and multiple tests will be performed to ensure the patient is well for surgery. A chest x-ray (CXR), an electrocardiogram (EKG), and lab work will be performed as part of the preoperative process