Cyanotic Congenital Heart Disease

Question 0

Tetralogy of Fallot

Answer 0

VSD
overriding aorta
pulmonary stenosis
RVH
*murmur d/t PS, not VSD

Question 1

Hypoplastic Left Heart Repair

Answer 1

Norwood procedure
Glenn operation
Fontan procedure

Question 2

Norwood procedure

Answer 2

Pulmonary artery disconnected from lung circulation and attached to ascending aorta so blood from RV goes to aorta and body.
- requires reconstruction of small (underdeveloped) aortic arch
- requires shunt to restore pulmonary blood flow so blood from RV also goes to lung
»> Blalock-Taussig shunt: subclavian artery to pulmonary artery
»> Sano shunt: RV to pulmonary artery

So blood from RV goes to lung and systemic at the same time: returns via SVC/IVC and pulmonary veins to RV

• oxygenated and deoxygenated blood mixed in RV
• p ox usu 70-80%

Question 3

Hypoplastic Left Heart before surgery

Answer 3

Maintain PDA with PGE1
Blood still tends to flow to lung as pulm vascular resistance falls but SVR high&raquo_space; avoid excess oxygen as this further deceased PVR and further shunts blood from systemic circulation&raquo_space; allow pulse ox 65-80%

Question 4

Glenn operation

Answer 4

SVC connected to pulmonary artery
- remove former Blalock-Taussig or Sano shunt
So now blood from head returns to lung (bypasses RV) then goes to RV, while blood from body still goes to RV via IVC, then blood from RV only goes to aorta/body so systemic blood only half mixed with deoxygenated blood
- pulse ox still 70-80%
- done ~6mos

Question 5

Fontan procedure

Answer 5

Connect IVC to pulmonary artery
So now blood from RV goes to body, returns via IVC and SVC directly to lung, then goes to RV then aorta and body
- done ~2-3yo
- pulse ox ~90%

Question 6

Tet Spells

Answer 6

usually in am or w/dehydration
too little pulmonary blood flow
sx: crying, inconsolable, absent/decreased pulm stenosis murmur, hyperpnea, decreased pulse ox c/w baseline
* increase SVR to promote pulm blood flow
knee to chest or squatting position
phenylephrine
* increase preload (IVF)
* hyper-oxygenate (decrease PVR, increase SVR)

Question 7

Long-Term Complications of unrepaired cyanotic CHD

Answer 7

Paradoxic emboli&raquo_space; stroke
- polycythemia increases viscosity
- relative anemia (iron-deficiency + polycythemia)
Fe deficiency makes RBC’s less malleable and is even greater
risk than polycythemia