Pathology

Question 1

Tetralogy of Fallot (TOF)

Answer 1

It is a Right-to-Left Shunt

Problem: “PROV”

• Pulmonary artery stenosis
• Right Ventricular Hypertrophy
• Overriding Aorta
• Ventricular septal defect (VSD)

Clinical Presentation: 
Mild -> Profound Cyanosis as ductus arteriosus constricts 
Strong Peripheral Pulses 
CXR = Heart looks like a boot-shape 
Echocardiogram confirms diagnosis 

Treatment:
Supportive Care, O2
Administer Prostaglandin E1 (PGE1) to keep the ductus open.
Needs surgery to fix

RRT:
Anticipate lower sat goals
Maybe need to intubate for Hypoxima
Watch for TET spell - O2 saturation can suddenly drop, If this happens comfort the infant or flex the knees forward and upward

Question 2

Transposition of the Great Vessels (TGV)

Transposition of the Great Arteries (TGA)

Answer 2

It is a Right-to-Left Shunt

Problem:
The pulmonary artery and Aorta have switched places
There is no mixing of the blood, you have two separate circulatory systems.

Clinical Presentation:

• Profound Cyanosis to hypoxia during the first week of life.
• CXR = Heart will have an egg-shape
• Large pre-post ductal SpO2 difference
• Echocardiogram will confirm the diagnosis

Treatment:

• Supportive care, O2 (but high O2 level will stimulate ductus to close)
• PGE1 to keep the ductus open
• Surgical Correction

RRT
Might need intubation for hypoxia