Decreased pulmonary blood flow (cyanotic)

8/7/2019 Decreased pulmonary blood flow (cyanotic)

1/19


2/19


3/19


4/19

Causes


5/19

S/S

Cyanosis

Tachypnea

Feeding difficulties


6/19

D/E

History collection

Physical examination- auscultation:

murmurs vary Chest X-ray: pulmonary vascular

marking and may be heart size vary

ECG

Echocardiogram


7/19

Mgt

Stabilize with PGE1infusion

Inotropic support

Intubation and ventilation as needed


8/19

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)


9/19

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


10/19

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.


11/19

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.


12/19

TGA


13/19

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


14/19


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16/19

S/S

Cyanosis

Tachypnea

Metabolic acidosis CHF

Feeding difficulty


17/19

D/E

Same as previous


18/19

Mgt

Stabilize with PGE1infusion

Inotropic support

Intubation and ventilation as needed

Antibiotics

Digoxin and diuretics


19/19

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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Decreased pulmonary blood flow (cyanotic)