Tetralogy of Fallot (TOF) Aubree Tschudy

1. Congenital Heart Defects

1.1. more commonly in children with Down syndrome or DiGeorge syndrome http://www.heart.org/HEARTORG/Conditions/CongenitalHeartDefects/AboutCongenitalHeartDefects/Tetralogy-of-Fallot_UCM_307038_Article.jsp#.WA1TIFfwCPU

1.2. Children with tetralogy of Fallot are at increased risk for endocarditis.

1.2.1. In some patients, a shunt operation may be done first to provide more blood flow to the lungs

1.2.2. Complete repair tends to be done early in life, but in some cases it can be done in adulthood

2. Tetralogy of Fallot (teh-TRAL-uh-jee of duh-low) is a rare condition caused by a combination of four heart defects that are present at birth. http://www.mayoclinic.org/diseases-conditions/tetralogy-of-fallot/basics/definition/con-20043262

2.1. A large ventricular septal defect (VSD) Pulmonary (PULL-mun-ary) stenosis. Right ventricular hypertrophy (hi-PER-tro-fe) An overriding aorta. https://www.nhlbi.nih.gov/health/health-topics/topics/tof

2.1.1. A VSD is a hole in the septum between the heart's two lower chambers, the ventricles. The hole allows oxygen-rich blood from the left ventricle to mix with oxygen-poor blood from the right ventricle.

2.1.2. Pulmonary Stenosis This defect involves narrowing of the pulmonary valve and the passage from the right ventricle to the pulmonary artery. In pulmonary stenosis, the pulmonary valve cannot fully open. Thus, the heart has to work harder to pump blood through the valve. As a result, not enough blood reaches the lungs.

2.1.3. Right Ventricular Hypertrophy With this defect, the muscle of the right ventricle is thicker than usual. This occurs because the heart has to work harder than normal to move blood through the narrowed pulmonary valve.

2.1.4. In tetralogy of Fallot, the aorta is located between the left and right ventricles, directly over the VSD. As a result, oxygen-poor blood from the right ventricle flows directly into the aorta instead of into the pulmonary artery.

3. Get; TOF; Congenital heart defect - tetralogy; Cyanotic heart disease - tetralogy; Birth defect - tetralogy https://medlineplus.gov/ency/article/001567.htm

4. The Centers for Disease Control and Prevention (CDC) estimates that each year about 1,660 babies in the United States are born with tetralogy of Fallot.1 In other words, about 1 in every 2518 babies born in the United States each year are born with teratology of Fallot. http://www.cdc.gov/ncbddd/heartdefects/tetralogyoffallot.html

4.1. Tetralogy of Fallot may be diagnosed during pregnancy or soon after a baby is born.

5. Signs and symptoms http://emedicine.medscape.com/article/2035949-overview

5.1. Difficulty with feeding

5.2. Most infants are smaller than expected for age

5.3. Cyanosis of the lips and nail bed is usually pronounced at birth

5.4. After age 3-6 months, the fingers and toes show clubbing

5.5. A systolic thrill is usually present anteriorly along the left sternal border

5.6. A harsh systolic ejection murmur (SEM) is heard over the pulmonic area and left sternal border

5.7. During cyanotic episodes, murmurs may disappear

5.8. In individuals with aortopulmonary collaterals, continuous murmurs may be auscultated

5.9. Failure to thrive

5.10. Episodes of bluish pale skin during crying or feeding (ie, "Tet" spells)

5.11. Exertional dyspnea, usually worsening with age

6. In most cases, tetralogy of Fallot is sporadic and nonfamilial. The incidence in siblings of affected parents is 1-5%, and it occurs more commonly in males than in females. The disorder is associated with extra cardiac anomalies such as cleft lip and palate, hypospadias, and skeletal and craniofacial abnormalities.  http://emedicine.medscape.com/article/2035949-overview#a7