Cyanotic Heart Disease

Question 1

acrocyanosis

Answer 1

blue hands and feet

Question 2

what is circumoral cyanosis

Answer 2

blue around the mouth

Question 3

hyperoxitest: what is it

Answer 3

• check baselinearterial Po2 in room air
• give patient 100% O2 for 10 min
• recheck arterial PO2
• ->if pulmonary shows rise in PO2 to at least 100mgHg
• -> if heart right to left shunt will demonstrate no significant increase in PO2

Question 4

Prostaglandin E1 -what for?

Answer 4

-only IV infusion to maintain patency of the ductus arteriosus

Question 5

The 5 T’s - Cyanotic heart defects:

Answer 5

• truncus arteriosus
• transposition of the great arteries
• tricuspid atresia
• tetralogy of fallot
• total anomalous pulmonary venous return

_ASK PEOPLE ABOUT THING TO REMEMBER IT!

Question 6

D-transposition is:

Answer 6

blue blood goes back out to body and red blood from lungs goes back to the lungs
(aorta arises from RV and pulomary trunk arises from LV)

Question 7

D-transposition - gender prevalence?

Answer 7

-MALES

Question 8

Fix for the D-transposition:

Answer 8

arterial switch + switch the coronaries

Question 9

Tetralogy of Fallot (TOF)- what four issues?

Answer 9

• large ventricular septal defect
• aortic valve overrides vetrcular septum
• r ventricular outflow obstruction=tract is smaller bc septum anterior and to the right
• R ventricle hypertrophy

Question 10

TOF- what makes the condition worse:

Answer 10

-the smaller the R ventricular outflow then the more R to left shunting = more blue

Question 11

Tetralogy of fallot - history?

Answer 11

-cyanotic (become more blue as ductus arteriosus closes up after birth) and murmur hear at birth

Question 12

Hypoxic tet spell - what condition? what happens?

Answer 12

• Tetralog of fallot
• peak incidence between 2 and 4 months
• sudden onset of rapid, deep breathing, irritability, prolonged crying, increased cyanosis, decreased heart murmur
• constriction of RV outflow tract (muscle spasm/smpathetic activation?)

Question 13

Blalock Taussig shunt procesure is for?

how does it fix?

Answer 13

• for TOF

- replace PDA with something?

Question 14

Tricuspid atresia

Answer 14

• tricuspid valve doesnt develop properly=RV doesnt dev properly
• small VSD often present
• majority have pulmonary atresia or stenosis

Question 15

Tricuspid atresia on ECG?

Answer 15

LVH often! bc the left ventricle is really the only ventricle around!

Question 16

Goal surgery for tricuspid or mitral valve atresia?

Answer 16

• fontan procedure (done after BT shunt procedure - allows for child/heart to grow a little) - systemic venous blood diverted directly to pulmonary arteries
• LV supports systemic circulation and the venous circulation goes straight into the lungs

Question 17

truncus arteriosus

what hapens>

Answer 17

one great vessel leaves the heart conncted inbetween both ventricles (sits BW VSD) and out to both lungs and systemic circulation

Question 18

least cyanotic disease?

Answer 18

truncus arteriosus

Question 19

Total anomalous pulmonary venous retrun (TAPVR)

-whats going on here?

Answer 19

-pulmonary veins come back to heart but not the LA –> goes North or south to go to the RA! – ALL RED AND BLUE BLOOD MIXES IN RA