EXAM #2: CYANOTIC HEART DISEASE Flashcards
What causes cyanosis?
Increased concentration of reduced Hb
What is acrocyanosis?
Cyanosis of the hands and feet in the newborn that is a NORMAL finding
What is isolated circumoral cyanosis?
Cyanosis of the lips seen often in cold, fair-skinned children
What is the utility of a hyperoxitest?
Test to determine the etiology of cyanosis
Outline the methology of the hyperoxitest.
1) Check baseline pO2
2) 100% O2 for 10 min
3) Recheck pO2
*Pulmonary= increase in pO2; Cardiac= no change
What is the utility of Prostaglandin E1?
Maintain the patency of the ductus arteriosus
What is the mnemonic to remember the causes of cyanotic heart disease?
5T’s:
1) Truncus Arteriosus
2) Transporition of Great Vessels
3) Tricuspid Atresia
4) Tetralogy of Fallot
5) Total anomalous pulmonary venous return
What defect is seen in TGA?
Transposition of the Great Arteries
- Aorta from RV
- Pulmonary from LV
What shunts can there be in TGA to maintain life?
1) VSD
2) PFO (patent foramen ovale)
3) PDA
*It is better to have 2x than 1x
What is this typical history for TGA?
Cyanosis from birth, especially in a male (full-term)
What is the typical presentation of TGA?
- Cyanotic, tachypneic infant in NO distress
- S2 is a single loud sound
Outline the medical management for TGA.
1) PGE1
2) Correct acidosis
3) Balloon atrial septostomy
What is the name for the Balloon atrial septostomy? Outline this procedure.
Rashkind Procedure
1) Catheter advanced across PFO
2) Balloon inflated and ripped back into RA
What is the surgical procedure to treat TGA?
Arterial switch operation
What is the major complication of the Arterial switch operation?
Getting the coronaries right
What are the four components of Tetralogy of Fallot (TOF)?
1) Large VSD
2) Overriding aorta
3) RV outflow obstruction (pulmonary stenosis)
4) RVH
*Based on a malalignment of the infandibular septum
What direction will blood shunt through the VSD in TOF?
Right-to-left b/c of resistance due to pulmonary stenosis/ overriding aorta
What causes the murmur in TOF?
RV outflow tract obstruction
*Note that a quieting murmur is v. concerning–RV is failing
What is the classic CXR in TOF?
Boot-shaped heart
What is a TET spell?
Hypoxic spell induced by spasm of the RV that presents as:
1) Sudden onset rapid breathing
2) Irritability
3) Prolonged crying
4) Increased cyanosis
5) Decreased heart murmur
How do you treat a TET spell?
1) Infant knee-chest
2) Oxygen
3) Morphine
4) Sodium bicarbonate
What are the two surgical procedures to treat TOF?
1) Blalock-Taussig shunt
2) Full surgical repair
What is the Blalock-Taussig shunt?
Connection between PA to Brachiocephalic a.
This is a surgical shunt similar to a PDA
What is Tricuspid Atresia?
Absent tricuspid valve with hypoplastic RV
What is the role of the ECG in Tricuspid Atresia?
Left axis and LVH in cyanotic child is diagnostic
What is are the medical steps to treat Tricuspid Atresia?
1) PGE1
2) Balloon atrial septostomy
What surgical procedures are indicated for Tricuspid Atresia?
1) BT
2) Fontan procedure
What is the Fontan procedure?
IVC/SVC directed straight into the PA
What is a Truncus Arteriosus?
Single arterial trunk leaves the heart giving rise to:
- Systemic circulation
- Pulmonary circulation
- Coronary circulation
*Large VSD is associated
What are the signs of Truncus arteriosus?
1) Wide pulse pressure
2) Single S2
How are Truncus arteriosus cases managed medically?
Anti-CHF meds temporarily
How is Truncus arteriosus surgically managed?
Complete repair early in life that involves:
1) Separation of the PA and connecting it to the RV via a conduit
2) Patching the VSD
What is Total Anomalous Pulmonary Venous Return (TAPVR)?
Pulmonary veins DO NOT return to the LA; instead, they return to the RA
*Need the ASD
What is the utility of the CXR in TAPVR?
V. helpful in evaluating obstruction
- Obstruction= normal heart and pulmonary edema
- Non= cardiomegaly
How do you surgically manage TAPVR?
Reconnect the pulmonary venous confluence back to the LA
