Tetralogy of Fallot Flashcards

1
Q

Tetralogy of Fallot (TOF) - what is it?

A

Congenital heart condition where patients have 4 heart abnormalities

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2
Q

TOF - what are the 4 abnormalities?

A
  1. Stenosis - narrowing of right ventricular outflow tract, so not as much deoxygentaed blood can leave, lead to…
  2. Right Ventricular Hypertrophy - wall gets thick, heart looks boot-shaped on XRAY
  3. Ventricular septal defect - shunting of blood between ventricles
    1. RV outflow obstruction causes pressure to be greater on this side, therefore deoxygenated blood shunts from right side into left side
  4. Aorta overrides V septal defect - picture attached shows where overriding aorta is located, and all 4 TOF features
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3
Q

TOF - cause?

A

Cause unclear

thought to be due to chromosome 22 deletions

DiGeorge Syndrome

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4
Q

TOF - clinical features?

A

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

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5
Q

TOP - what is a cyanotic spell, ‘tet spell’?

A
  • 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
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6
Q

TOF - how do you treat ‘tet’ spell?

A

Keeping calm, giving 02 and IV fluids, medications

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7
Q

TOF - diganosis?

A

Echocardiography - can be done prenatally

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8
Q

TOF - management?

A

Cardiac repair surgery:

  1. First year
  2. Septal defect closed
  3. RV outflow tract enlarged
  4. If points 2 and 3 done, resolves other two issues as well
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9
Q

What murmur is heard?

A
  • ejection systolic murmur due to pulmonary stenosis (the VSD doesn’t usually cause a murmur)
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