Cyanotic Heart

Question 1

What is Acrocyanosis?

Answer 1

Blue hands and feet

Not Pathologic

Question 2

What Pathologies will show a V/Q Mismatch?

Answer 2

Pneumonia
Cystic Fibrosis
Hyaline Membrane Disease

Question 3

How can you tell if it is the heart or the lungs involved?

Answer 3

Lung problem will respond to high flow O2

Heart problem will not.

Question 4

What is Prostaglandin E1 used for?

Answer 4

Continuous infusion maintains patency of Ductus Arteriosus

Question 5

What is done to Stabilize the Infant with CHD?

Answer 5

Temp Reg
Labs
Fluids
O2
Ventilation
Avoid acidosis
Inotropic Support of Circulation

Question 6

What are the 5 Ts of Cyanotic Heart Defects?

Answer 6

Truncus Arteriosus
Transposition of Great arteries
Tricuspid Atresia
Tetrology of Fallot
Total Anomalous pulmonary venous return

Question 7

What is Transposition of Great arteries

Answer 7

LV connected to Pulm Arteries
RV connected to Aorta
Not conducive to life! Shunt!
Need to find a way to mix the two circulations for survival

Question 8

What are PE findings in D-Transposition?

Answer 8

Cyanotic Tachypnic infant
S2 is single and loud
Heart murmur
Hepatomegaly

Question 9

What is the Med Management of D Trans?

Answer 9

Prostaglandin
Correct Acidosis
O2 for severe hypoxia
Baloon atrial Septostomy
Anti Congestive Meds

Question 10

What is the Rashkind Procedure?

Answer 10

Baloon Atrial Septostomy

Catheter with baloon inserted in jerked back to widen the Atrial Septal defect

Question 11

What is the Atrial Switch Operation?

Answer 11

Connect the BVs to the Atria that are doing the correct job.
RV goes to Circulation,
LV goes to Lungs

Question 12

What is the ARTERIAL Switch Operation?

Answer 12

Move the Coronary arteries over to the Left side so it has the capability to support the systemic circulation.

Question 13

What is the Tetrology of Fallot?

Answer 13

1) Large VSD
2) Overriding Aorta
3) RV outflow obstruction
4) RV hypertrophy
Blood flows more readily through the VSD bc of Stenosis of Pulmonary outflow tract.

Question 14

What is the natural history of the Tetrology of Fallot?

Answer 14

Gradually worsening Cyanosis
Hypoxic Spells
Growth retardation if severe

Question 15

How are Hypoxic Spells managed in Tet of Fallot?

Answer 15

Hold infant in knee-chest position
O2
Morphine
Sodium Bicarb to treat acidosis

Question 16

What are the Medical and Surgical Tx of Tetrolog of Fallot?

Answer 16

Medical: Eduaction about Tet spells
Surgical: Palliation with Blalock Taussing shunt
Patch to increase size of RV outflow tract

Question 17

What is the Repair Technique for Tet of Fallot?

Answer 17

VSD closed and Pulmonic artery Opened with a patch

Question 18

What is Tricuspid Atresia?

Answer 18

TC valve is absent
RV is Hypoplastic
Pulm Atresia or Pulm Stenosis
Small VSD
Is a reason for far left axis shift

Question 19

What is the management of Tricuspid atresia?

Answer 19

Wont survive without surgery

Prostaglandin and Blaylock-Taussing Shunt

Question 20

Waht is the Fontan Circulation?

Answer 20

Only one Ventricle
Connect IVC to Solo atrium and Pulmonary circulaton
Connect the Ventricle to the Aorta

Question 21

What is the Truncus Arteriosus?

Answer 21

A single arterial trunk leaves the heart to give rise to the Pulmonary, Stystemic, and Coronary bloodflow
Lungs have very high flow and surgical intervention needed
as long as PVR

Question 22

What is the Surgical Repair of Truncus arteriosus?

Answer 22

Direct LV flow out of the truncal valve via patch closure of VSD
Dissconnect Branch Pulm arteries from truncus and atach to RV-PA conduit