Tricuspid Atresia  It is characterized by absence of the tricuspid valve. and usually diminished pulmonary circulation  Openings are also present in the atrial and ventricular walls  PDA . a small right ventricle.


Causes .

S/S  Cyanosis  Tachypnea  Feeding difficulties .

auscultation: murmurs vary  Chest X-ray: pulmonary vascular marking and may be heart size vary  ECG  Echocardiogram .D/E  History collection  Physical examination.

Mgt  Stabilize with PGE1 infusion  Inotropic support  Intubation and ventilation as needed .

 There are two surgery type: first surgery (neonates) and second surgery (6to9months) and third surgery (18 months to 3 years) .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.  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 to meet the child's needs. Another pathway for blood to reach the lungs to receive oxygen must be surgically created.

Some of the blood traveling through the aorta towards the body will "shunt" through this connection and flow into the pulmonary 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 the heart mix and flow through the aorta to the body. Blalock-Taussig shunt (BT shunt) (1st surgery) The first operation creates a pathway for blood to reach the lungs. A connection is made between the first artery that branches off the aorta (called the right subclavian artery) and the right pulmonary artery.  Pulmonary artery band: if pulmonary blood flow is excessive .

the Fontan procedure. often performed at about 4 to 12 months of age. Blood from the head and arms passively flows into the pulmonary artery and proceeds to the lungs to receive oxygen. replaces the Blalock-Taussig shunt with another connection to the pulmonary artery. However. 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. This operation helps create some of the connections necessary for the final operation. the Blalock-Taussig shunt is removed. so the child will remain mildly cyanotic. Glenn shunt (2nd surgery) A second operation. In this operation. . oxygenpoor (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.

and what is best for your child. . 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. This connection can be created in slightly different variations. depending on the method your child's surgeon prefers. A second connection is made directing blood from the inferior vena cava to the right pulmonary artery. greatly improving the oxygenation of the blood. The Glenn shunt. is left in place. connecting the superior vena cava to the right atrium.


PS and CoA . PDA.TGA means what?  Occurs when the PA arises off the LV and aorta arises off the RV.  Associated lesions include ASD. VSD.


Causes  Same as previous .

S/S      Cyanosis Tachypnea Metabolic acidosis CHF Feeding difficulty .

D/E  Same as previous .

Mgt      Stabilize with PGE1 infusion Inotropic support Intubation and ventilation as needed Antibiotics Digoxin and diuretics .

Surgical mgt  Arterial switch operation  Rastelli operation: performed for TGA. VSD and PS  Atrial switch operation: RA-mitral valve-LV-PA and LA-tricuspid-RV-Ao .

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