Tetralogy of Fallot Flashcards
Tetralogy of Fallot (TOF) - what is it?
Congenital heart condition where patients have 4 heart abnormalities
TOF - what are the 4 abnormalities?
- Stenosis - narrowing of right ventricular outflow tract, so not as much deoxygentaed blood can leave, lead to…
- Right Ventricular Hypertrophy - wall gets thick, heart looks boot-shaped on XRAY
-
Ventricular septal defect - shunting of blood between ventricles
- RV outflow obstruction causes pressure to be greater on this side, therefore deoxygenated blood shunts from right side into left side
- Aorta overrides V septal defect - picture attached shows where overriding aorta is located, and all 4 TOF features
TOF - cause?
Cause unclear
thought to be due to chromosome 22 deletions
DiGeorge Syndrome
TOF - clinical features?
Babies:
Cyanosis, bluish-purple skin - lips, fingertips
Clubbing in fingers and toes
Any decrease in 02 - causes feeding difficulties, failure to gain weight, failure to develop normally
TOP - what is a cyanotic spell, ‘tet spell’?
- They will appear normal
- Then if moving around, means increased 02 demand
- Heart pumps more deoxygenated blood
- Cyanosis
- Child squats down to reduce cyanosis
- Do this to slightly kink femoral arteries → increased vascular resistance in peripheral arteries → increased pressure in systemic circulation → increases pressure in left side greater than the right side, so temporarily reverses shunt from right to left, to left to right → reduces cyanosis
TOF - how do you treat ‘tet’ spell?
Keeping calm, giving 02 and IV fluids, medications
TOF - diganosis?
Echocardiography - can be done prenatally
TOF - management?
Cardiac repair surgery:
- First year
- Septal defect closed
- RV outflow tract enlarged
- If points 2 and 3 done, resolves other two issues as well
What murmur is heard?
- ejection systolic murmur due to pulmonary stenosis (the VSD doesn’t usually cause a murmur)