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Causes
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S/S
Cyanosis
Tachypnea
Feeding difficulties
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D/E
History collection
Physical examination- auscultation:
murmurs vary Chest X-ray: pulmonary vascular
marking and may be heart size vary
ECG
Echocardiogram
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Mgt
Stabilize with PGE1infusion
Inotropic support
Intubation and ventilation as needed
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Surgical mgt
The ductus arteriosus (the normal connection
between the aorta and the pulmonary valve) will
likely close if the prostaglandin E1 infusion is
stopped. Another pathway for blood to reach the
lungs to receive oxygen must be surgically
created.
A series of operations are performed in the first
two years of life that will re-route blood so that
enough oxygen is added to the bloodstream tomeet the child's needs.
There are two surgery type: first surgery
(neonates) and second surgery (6to9months) and
third surgery (18 months to 3 years)
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Blalock-Taussig shunt (BT shunt) (1st surgery)
The first operation creates a pathway for blood to
reach the lungs. A connection is made betweenthe first artery that branches off the aorta (called
the right subclavian artery) and the right
pulmonary artery. Some of the blood traveling
through the aorta towards the body will "shunt"
through this connection and flow into thepulmonary artery to receive oxygen. However, the
child will still have some degree of cyanosis since
oxygen-poor (blue) blood from the right atrium and
oxygen-rich (red) blood from the left side of theheart mix and flow through the aorta to the body.
Pulmonary artery band: if pulmonary blood flow is
excessive
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Glenn shunt (2nd surgery)
A second operation, often performed at about 4 to 12
months of age, replaces the Blalock-Taussig shunt with
another connection to the pulmonary artery. In thisoperation, the Blalock-Taussig shunt is removed, and the
superior vena cava (the large vein that brings oxygen-poor
blood from the head and arms back to the heart) is
connected to the right pulmonary artery. Blood from the
head and arms passively flows into the pulmonary artery andproceeds to the lungs to receive oxygen. However, oxygen-
poor (blue) blood returning to the heart from the lower body
through the inferior vena cava will still mix with oxygen-rich
(red) blood in the left heart and travel to the body, so the
child will remain mildly cyanotic. This operation helps create
some of the connections necessary for the final operation,the Fontan procedure.
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Fontan procedure (third surgery)
This operation is often performed at about 18 to
36 months of age, and allows all the oxygen-poor(blue) blood returning to the heart to flow into the
pulmonary artery, greatly improving the
oxygenation of the blood. The Glenn shunt,
connecting the superior vena cava to the right
atrium, is left in place. A second connection is
made directing blood from the inferior vena cava
to the right pulmonary artery. This connection can
be created in slightly different variations,
depending on the method your child's surgeonprefers, and what is best for your child.
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TGA
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TGA means what?
Occurs when the PA arises off the LV
and aorta arises off the RV.
Associated lesions include ASD,VSD, PDA, PS and CoA
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S/S
Cyanosis
Tachypnea
Metabolic acidosis CHF
Feeding difficulty
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D/E
Same as previous
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Mgt
Stabilize with PGE1infusion
Inotropic support
Intubation and ventilation as needed
Antibiotics
Digoxin and diuretics
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Surgical mgt
Arterial switch operation
Rastelli operation: performed for
TG
A, VS
D and PS
Atrial switch operation: RA-mitral
valve-LV-PA and LA-tricuspid-RV-Ao
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